Index

A ABO immune disease, 1159–1160 Aarskog–Scott syndrome, 1876 ABO blood group heterospecificity, 1159 Abbie’s syndrome, 2266 ABO incompatibility, 1160 Abdomen carboxyhemoglobin, 1159 circumference, 151 clinical manifestations of, 1160 distension, 884 DAT-positive newborns, 1160 endocrine diseases, 1857, 1866 IVIG therapy, 1160 gastroenteritis, 1360 UGT1A1 , 1160 gastrointestinal malformations, 1296, 1307, 1310, Abortion 1311, 1318 ethical problems, 88 hemorrhage, 1505 genetic causes, 52 neonatal , 1740 hemolytic conditions, 1157 neonatal malignancies, 1656 prenatal hemorrhage, 1500–1504 pneumoperitoneum, 884 smoke, 711–712 renal diseases, 1964, 1970 spontaneous, 132 surgical emergencies, 1335 Abruptio placentae, 711, 1116 ventilation techniques, 986, 990 Abscess examination, 465 bacterial and fungal imaging studies , 1747 defects of the abdominal wall, 1298–1301 cerebral, 1730, 1731, 1741, 1752 duct, 1312 renal, 1745 duodenal obstructions, 1304 skin, 1751 duplication of the alimentary tract, 1316 liver, 1248 esophageal atresia, 1285, 1286 Absolute count (ANC), 870, 1550, 1551, 1562 Hirschsprung’s disease, 1347 Absorption, 515–521, 698 intestinal malrotation, 1319 fat, 515–517 intestinal obstructions, 1305–1311 glucose, 518 meconium plug syndrome, 1341 micronutrient, 519–521 necrotizing enterocolitis, 1383 , 517–518 persistence of the omphalo-mesenteric duct, 1312 Absorptive enterocytes, 506 pylorus, 1301 Acardia, 141–142 palpation, 459 ACA stroke, 2265 Abdominal Acceleration mass, 748 of fetal heart rate, 113, 121 paracentesis, 927 of fetal maturity, 839–840 ultrasound, 1227 Accessory tragus, 2423 wall, defects, 1296–1301 Accidental extubation, 862 cloacal exstrophy, 54 ACE inhibitors, 1122 exomphalos, 60 Acetyl-choline, 510 gastroschisis, 1298–1301 Acholic stools, 1225 omphaloceles, 1297–1298 , 67–68 Abnormal diffusion, 770 Acid–base Abnormal intrapleural tissue (AIPT), 930 measurement, 413–414 Abnormally placed pulmonary tissue (APPT), 930 status, 768

# Springer International Publishing AG, part of Springer Nature 2018 2459 G. Buonocore et al. (eds.), Neonatology, https://doi.org/10.1007/978-3-319-29489-6 2460 Index

Acidemia, 120 furosemide, 1946 fetal, 14, 120 human studies, 1950 intrapartum asphyxia markers, 411–412 hyperkalemia, 1947 propionic and methylmalonic, 1817 hyperphosphatemia, 1947 Acidosis, 1187 hypertension, 1947 and asphyxia (see Acidemia) hypocalcemia, 1947 and brain damage, 2174, 2179, 2207, 2302 hyponatremia, 1946–1947 and calcium disturbances, 653–662 hypoxemic-hypoxic insults, 1941–1942 and fetal distress, 106 imaging, 1945 and heart disease, 1053–1063, 1065–1076 intrinsic causes, 1940 management during pregnancy, 115, 124 low-dose dopamine, 1945–1946 metabolic, 206, 413, 663 mannitol, 1946 and NEC, 1373–1387 neonatal kidney, 1937–1938 renal tubular, 1929, 1930, 1949 nephrotoxic drugs, 1940–1941 respiratory, 411, 768 non-oliguric ARF, 1943 and respiratory distress syndrome, nutrition, 1948 980–983 oliguric ARF, 1943 and surfactant therapy, 997 outcomes, 1949 weaning from ventilation, 984 physical features, 1942–1943 Acinar development, 889 plasma creatinine, 1943–1944 , 2399 plasma cystatin C, 1944 Acquired coagulation disorders plasma urea, 1943 HIE and TH, 1449 postrenal causes, 1940 IVH, 1449 prerenal causes, 1939 liver dysfunction and liver failure, 1445 RRT, 1948–1949 VKDB, 1446 sepsis, 1942, 1948 Acquired immune deficiency syndrome septic shock, 1781 (AIDS), 1685 urinalysis, 1943 Acrocyanosis, 475, 1042, 2395–2396 urinary indices, 1944 Acrodermatitis, zinc deficiency, 671 respiratory distress syndrome (ARDS), 1003, 1781 Acrofacial Nager type, see Nager syndrome and subacute non-structural cardiac ACTH (adrenocorticotropic hormone), see Hormones abnormalities, 721 Activated partial thromboplastin time (aPTT), 1438 Adamkiewicz artery, 2269 disseminated intravascular ADAMTS-13, 1458 coagulation (DIC), 1478–1479 Adaptation Activated protein C (aPC), 35, 1779 cardio-respiratory, 23 Active and passive cooling, 299 fetus, 20, 116, 119, 122 Active failures, 309 gastrointestinal tract, 518, 543 Activin A, 2307, 2308 preterm infant, 22–23 Acute Adaptive immunity, 1576, 1583, 1590, 1794 bilirubin encephalopathy (ABE), 1155, 1200 Addressins, 1577 chorioamnionitis, 2235 Adenosine, deaminase deficiency, 1612 fatty liver of pregnancy, 1815 Adenosine triphosphate (ATP), 1163, 1497 heart failure, 1101–1102 Adenovirus, 869 lymphoblastic leukemia (ALL), 1656 pneumonia, 1741–1743 myeloid leukemia (see Leukemia) SCIDs, 1612–1614 non-lymphoblastic leukemia (ANLL), 1656–1657 thrombocytopenia, 1478 otitis media (AOM), 1748 Adrenal crisis, 1845 pain scale, 369 Adrenal gland renal failure (ARF), 1781, 1935–1952 congenital adrenal hyperplasia, 473, 1120 acidosis, 1947 disorder of gonadal development, 1837–1839 animal studies, 1950 3b-hydroxysteroid dehydrogenase, 1900–1902, 1906 blood count, 1943 11-hydroxylase, 1900–1902 convulsions, 1947 Adrenal hemorrhage, 1116, 1120 definition, 1938 Adrenal hypoplasia congenita, 1842 early diagnosis, 1949–1950 Adrenal insufficiency, 1837–1845 fetal kidney, 1937 clinical presentation, 1844 fluid challenge and rehydration, 1945 diagnostic evaluation, 1844–1845 fluid management, 1946 etiology, 1840–1844 Index 2461

molecular basis, 1839–1840 Alanine aminotransferase (ALT), 1227, 1691, 1821 treatment, 1845 Albinism, 2415 Adrenoleucodistrophy, 473 Albright’s hereditary (AHO), 1859 Adverse drug reactions, 704 Albumin, 387, 434, 518, 524, 559, 654, 753, 816 Aeromonas hydrophila, 1359 exchange, 1200 Aflibercept, 2373–2374 Alcohol Agammaglobulinemia, 1532, 1609–1610 fetal alcohol syndrome, 2138 Agammaglobulinemia either X linked (XLA) See also Maternal, drug abuse and bacterial infections, 1609 Alexander disease, 1827 characteristics, 1609 Alexis wound retractor (AWR) device, 1300 diagnosis, 1609–1610 Alimentary tract duplications, 1314–1317 genetic defects, 1609 A-lines, 914 symptoms, 1609 Alkaline phosphatase (ALP), 660, 1215, 1227, 1857 treatment, 1610 Alkalosis, 520, 610, 644 Agenesis of corpus callosum (ACC), see Corpus hypocalcemic, 654, 657 callosum agenesis metabolic, 658, 768 Age-specific nomogram, 1148 respiratory, 768, 948 AGREE evaluation tool, 319 Alloantibodies, 1476 Agyria, 2115, 2132, 2329 Allogeneic hematopoietic stem cell (HSC) Aicardi–Goutieres syndrome (AR), 2235, 2276 transplantation, 1845 , 2122 Alloimmune AIDS, 752, 1674, 1684–1689, 1844 neonatal , 1565 See also Human immunodeficiency virus neutropenia, 1542, 1565 Air thrombocytopenia, 1477 embolism, 1135 Alloimmunization, 1161 leak, 874, 996 Allopurinol, 904, 2156–2157 and MAS, 794 bronchopulmonary dysplasia, 904 syndromes, 795 hypoxic-ischemic encephalopathy, 2192 transport, 297 Alopecia, 2420, 2421 trapping, 899, 985 Alpha-1-antitrypsin deficiency (AATD), 1213–1214, and MAS, 794 1235–1236 Airway Alpha-fetoprotein (AFP), 1650, 1656, 1853, 2129–2130 compliance, 901 Alpha-1 proteinase inhibitor (α1P1), 904 disease 5-Alpha-reductase deficiency, 1907 airway obstruction, 778, 901 Alport’s syndrome, 1476 bronchoscopy, 752 Alterations in surfactant lipid, 898 endotracheal intubation, 386, 429–431, Aluminium, 614 794, 901 Alveolar extubation, 815, 862, 902 capillary dysplasia, 754–755, 830, 919 INSURE, 434 development, 890 laryngomalacia, 770 disorders, 928–929 cyst, 744 phase, 890 mechanical ventilation, 209 rhabdomyosarcoma, 1650 respiratory distress syndrome, 980, 997 surface, 802–803 subglottic stenosis, 386, 898 ventilation, 765–766 surfactant therapy, 995–1004 Alveolar-capillary membrane formation, 736 tracheomalacia and bronchomalacia, 737, 898, Alveoli, 875 901, 1284 Ambient humidity, 329–336 tracheostomy, 209 Ambiguity, 224 vocal cord paralysis, 1087 Amblyopia, 247 weaning from ventilation, 861, 984 of the cortico-spinal system, 272 granulomas, 901 Ambulance, 296 management in neonate, 385 American Academy of Pediatrics (AAP) guidelines, 1155, obstruction, 901 1158, 1163 reflexes, 1029 hospital discharge, late preterm infants, 181 resistance, 763–765 jaundice, 180 Alagille syndrome, 1213, 1226, 1238–1239, 1922 American College of Critical Care Medicine (ACCM), 1777 diffuse xantomata, 1214 Amiel-Tison neurologic assessment at term typical facies, 1213 (ATNAT), 2059 2462 Index

Amikacin, 1737 hemolytic disease, 1156–1161 hydrops, 1515–1521 disorders, 472 hypoproliferative anemias, 1506–1508 enteral feeding, 595–602 in hypoxia, 772 inborn errors of metabolism, 1808 immune-mediated hemolytic disease, 1498–1500 maple syrup urine disease, 2100 Kasabach–Merritt syndrome, 2419 non-ketotic hyperglycinemia, 1823, 2077, 2100 neonatal reference intervals, 1491–1497 parenteral nutrition, 605–616 oxidative stress, 449 transporters, 518 perinatal hemorrhage, 1503–1504 Amino acid-based formula, 581 of the newborn, 460 Aminoglycosides, 1736 postnatal hemorrhage, 1504–1505 Amiodarone, 1520 of prematurity, 1506–1508 Ammonia, 602 prenatal hemorrhage, 1500–1503 , 8, 10, 96, 1156–1157, 1517–1519 preterm neonate, 477 Amnioinfusion, 794, 927 SIDS, 1027 Amnioscopy, 112 syndromes associated with congenital anemia, 1507 Amniotic bands, 51, 2422 Anencephaly, 4, 2129 Amniotic fluid, 8, 54, 112 Anesthesia, 383–393 and chorioamnionitis, 96, 98–100 implications in newborn, 385 index, 112, 747 principal drugs, 387 Amoxicillin, 1745, 1748 Aneuploidy, 8, 57, 133 Amphotericin, 1753, 1781 Angelman syndrome (AS), 46 Ampicillin, 1734, 1736, 1737, 1743, 1754 Angiogenesis, 890 Amplitude integrated EEG (aEEG), 417, 2083, 2084, Angiomas, 2341–2342 2086–2088 Angiopoietin-2, 893–894 Amputation, 2423 Animal models, 906 Amylase, 518, 596 Aniridia, 463, 1121, 2346–2347 Anaerobic bacteria, 1731 Ankyloglossia, 1277 Anaesthesia Annular , 1303 induction and tracheal intubation, 385–386 Anomalies of transparency, 2344 intraoperative management, 384–387 Anophthalmia, 2342 vs. neonatal physiology, 385 Ano-rectal malformations, 1321 neurotoxicity risk, infants, 392 associated anomalies, 1323 outside operating room, 388–389 classification, 1321–1322 pre-anaesthesia assessment, 384 diagnosis, 1324 pre-term and ex-premature infants, 387 embryogenesis, 1321 regional, 387–388 incidence, 1321 Analgesia, 198, 389–393 postoperative treatment, 1327 effects of pain, 390 symptoms, 1323 ketamine, 392 therapy, 1324–1327 midazolam, 392 Antenatal diagnosis opioids, 391 fetal hydrops, 1516 pain in neonatal intensive care unit, 390 heart disease, 1066 paracetamol, 391 Antenatal glucocorticoid therapy (AGC), 825, 839, 840 Anastomosis, resection and primary, 1398 Antenatal screening, 1689 Anastomotic leaks, 1291 Antenatal steroids, 905, 998, 1001, 1017 Androgen insensitivity syndrome, 1907, 1908 Antepartum testing Anemia, 772, 1699 biophysical profile, 111–112 causes of hemorrhage in the fetus and newborn, 1501 cardiotocography (see Cardiotocography) coagulation disease, 1471–1484 Doppler examination, 115–121 congenital dyserythropoietic, 1507 Anterior anus, 21 congenital Anterior choroidal artery stroke, 2266 erythropoietic porphyria, 2408 Anthropometric variables, 158 infection, 1505–1506 Antibiotic(s) Diamond–Blackfan, 1507 early neonatal sepsis, 1539, 1540 erythrocyte membrane, abnormalities in, 1497–1498 early onset GBS infections, 1754–1756 erythrocyte transfusion, 1508–1509 neonatal septic shock, 1773–1782 erythropoietin, 670, 1490–1491, 2193 preterm delivery, 134–135, 207 Fanconi, 1507 prophylaxis, 1759, 1781 Index 2463

stewardship, 1761–1762 and head-elevated positioning, 1024 treatment, sepsis, 1735 oscillating waterbed/mechanosensory stimulation, 1027 anemia, 1500 oxygen administration, 1026 fetal and perinatal infection, 1703–1723 red blood cell transfusions, 1027 feto-neonatal , 1575–1595 synchronized nasal ventilation (S-NIPPV), 1024 gastrointestinal tract, 523 treatment, 1023–1027 hyperbilirubinemia, 1157–1158 preterm baby, 476 leukocytes anomalies, 1529–1530 resuscitation, 428, 430 neonatal tetanus, 1750 score to assess apnea severity, 102 neonatal viral infection, 1785–1790 treatment plan for apnea of prematurity, 1024 screen, 1157 , 2145, 2146 vaccination and neonatal immunity, 1798 chorioamnionitis, 101 Anticardiolipin antibodies (ACA), 1464 immunology, 1576 Anticonvulsant, 1629 inflammatory mechanisms, 81 congenital malformations, 51 kernicterus, 1171 hypoxic ischemic syndrome, 2173 meconium aspiration syndrome, 798 infants of diabetic mothers, 726 oxygen toxicity, 443 neonatal asphyxia, 417 Apparent diffusion coefficient (ADC), 2096, 2100 neonatal electroencephalography, 2085 Appendicitis, 1318, 1334, 1336 pain, 392 Approach to low risk newborn, 395–406 Anticonvulsive drugs, 2172 Appropriate for gestational age (AGA), 149, 175 Antidiuretic hormone (ADH), 1118, 1845–1847 Aquaporin, 1924 Anti-D titer, 1157 Aqueduct stenosis, 2140 Anti-epileptic drugs, 684–686 Arachidonic acid (AA), 527 Antiepileptic medications, 2291 , 56 Antigen-presenting cells, 100, 1584, 1616 Arachnoid cyst, 2139 Antihypertensive, 1120 Arching, 1200 Anti-inflammatory therapies, 1634 Arginase, 1823 Anti-Kell isoimmunization, 1161 Arginine, 527, 941, 1387 Antimicrobial prophylaxis Argininosuccinic acid, 1823 antibiotic prophylaxis, 1759 Aromatase deficiency, 1904 antifungal prophylaxis, 1760–1761 Aromatic amino acid decarboxylase (AADC) Antioxidants, 452–453 deficiency, 1825 Anti-oxidative agents, 1628 Arrhythmia, 1103, 1202, 1520, 1750 Antiphospholipid syndrome (APLS), 1460 atrial tachyarrhythmia, 1103–1106 Antiretroviral drugs, 1685 clinical algorithm, 1104 Antiretroviral therapy, 1688 ventricular tachyarrhythmia, 1106–1107 Antiviral treatment, 1695 Arterial Antley–Bixler syndrome, 1876 blood gas measurements, 847 Antrum, 513 blood pressure, 1113 Aortic calcification, 663, 1120, 1518 arch interruption, 57, 1070 catheters, 1202 coarctation, 57, 1043, 1054–1055, 1076, 1120 ischemic stroke, 2226 stenosis, 1054, 1076 spasm, 2238 valve stenosis, 1076 thrombosis, 1463 , 65, 1261–1263 Arterial switch operation (ASO), 1070 Apgar score, 20, 198, 400, 410–413, 1029, 2166–2167, Arterial tree abnormalities, 924–925 2174 Arthritis, septic, 1745–1747 Apical junction complexes (AJCs), 524 , 748 Aplasia cutis, 56, 463, 2421 Artifacts, 914–915 Apnea, 178, 778–782 Ascending amniotic infection, 1733 IVH in preterm infants, 2077 Ascending cholangitis, 1235 neonatal anesthesia, 387, 388 Ascites, 1221, 1235, 1516, 1517 of prematurity, 784, 1021–1032 Ascorbic acid, 521 caffeine, 1024–1026 Aspartate aminotransferase (AST), 1227 CO2 concentration, 1027 Asphyxia, 198, 409–419, 1129, 1187, 1479, 2098, 2175 continuous positive airway pressure, 1024 acid base measurements in umbilical cord blood, doxapram, 1026 413–414 2464 Index

Asphyxia (cont.) Atrioventricular septal defect (AVSD), 1061–1063 activin A, 230 Atropine, 547 amplitude integrated EEG, 417 Attachment, affect, 23, 196, 212 anemia, 1489–1509 Attention deficit hyperactivity disorder aspiration syndrome, 791–798 (ADHD), 714 blood lactate at birth, 414–415 Audit brain damage, 2067–2068 clinical, 253–254, 286 cerebral ultrasound, 243 National Neonatal Audit Programme, 253 chorioamnionitis, 100 Auditory congenital diaphragmatic hernia, 950–956 brainstem cyanosis, 1043 evoked potential (BAEP), 1191 , 1833–1885 response (ABR), 247, 1173 ethical problems, 198 canal atresia, 66 fetal scalp blood sampling, 412 dys-synchrony (AD), 117 fetus, 11, 12, 121 neurotoxicity (AN), 117 hyperbilirubinemia, 1169–1181 recognition/music, 375–376 hypertension, 1119–1120 speech and language, 2037–2038 hypocalcemia, 654 Audits and patient safety walk rounds, 311 hypothermia, as medical treatment, 436, 2098–2099, Autoantibodies, 1476 2193–2194 Autoimme polyendocrinopathy-candidiasis ectodermal hypoxic-ischemic encephalopathy, 198, 411, dystrophy syndrome 412, 415, 416, 2098–2099, (APECED), 1616 2143–2160 Autoimmune diseases, 1692 meconium aspiration syndrome, 791–798 Autoimmune neutropenia (AIN), 1535–1536 MOF (multiorgan failure), 416 Autoimmune thrombocytopenic purpura, 1477 necrotizing enterocolitis, 1373–1387 Autoregulatory escape, 508 neonatal EEG, 2085–2088 Autosomal recessive neuroimaging, 2091–2110 agammaglobulinemia, 1609–1610 oxygen toxicity, 45 (see also Agammaglobulinemia either perinatal outcome, 160 X linked (XLA)) polycitemia, 1125–1137 polycystic kidney, 1119, 1964 pulmonary air leakage, 873–884 Axillary temperature, 181 renal dysfunction and failure, 1919–1952 Axonal sprouting, 2280 resuscitation of newborn, 423–436 Aztreonam, 1737 Aspiration pneumonia, 899 Azurophilic granules, 1580 Aspirin non-steroidal anti-inflammatory drugs, 1940 qualitative disorders of platelets, 1481 B Asplenia, 1061, 1215, 1245 Babies with diarrhea, 1366 Assist/control (A/C), 856–857 Babinski reflex, 241, 2059, 2076 Assisted reproductive technologies Baby-friendly hospital initiative (BFHI), 562–564 (ART), 31, 131 Bacillus cereus, 2215 Assisted ventilation, 209, 429, 2169 Back-transports, 294 Associations, 51–53 Bacteria Asthma, 107, 679 anaerobic, 1731 Astigmatism, 2363 Gram-negative, 869, 1730–1731 Astrocytoma, 1643 Gram-positive, 1729–1730 Asymmetric tonic neck reflex, 460 probiotic, 527–528 Asymptomatic Bacterial colonization, 1377 bacteriuria, 30 Bacterial infections, 1566–1567, 1727–1764 hyperthyrotropinemia, 1871 blood cultures, 173 pneumothorax, 881 clinical features, 1735 Ataxic cerebral palsy (CP), 238 conjunctivitis, 174 Atelectasis, 771, 787, 795, 898, 980 diagnosis, 1731–1733 Athetoid cerebral palsy (CP), 238 etiology, 1729–1731 Atopic , 2408–2410, 2412 laboratory tests, 1732 Atrial meningitis, 1739–1741 flutter/fibrillation, 1044, 1105–1106 molecular diagnostics, 1732–1733 septal defect, 1049, 1057, 1061, 1072 noma neonatorum, 1750 Index 2465

omphalitis, 1748–1749 Bile, 1225 osteomyelitis and septic arthritis, 1745–1747 duct otitis, 1748 paucity, 1226, 1236 pneumonia, 1741–1743 perforation, 1226 routes of transmission, 1733–1734 salts, 516 sepsis, 1733–1739 secretion, 1225 staphylococcal complications, 1749 Bile acid, 1231, 1233, 1234 syphilis, 1703–1710 synthesis, inborn error, 1220 tetanus, 1750 Bile salt-stimulated lipase (BSSL), 516 therapy, 1735–1738 Biliary atresia (BA), 1146, 1210–1211, 1228, 1244–1247 tuberculosis, 1707–1710 bile duct dilatation, 1247–1248 urinary tract, 1743–1745 causes, 1244 Bacterial vaginosis (BV), 31, 99 clinical forms of, 1234 Bactericidal/permeability-increasing protein clinical signs, 1245 (BPI), 1580 description, 1244 Bacteriuria asymptomatic, 30 diagnosis, 1245–1246 Bacteroides fragilis, 1731 embryonic form of, 1234 Ballard score, 46 etiology and pathogenesis, 1234, 1245 Banana sign, 2130 histologic findings, 1230, 1234 Barbitures, 387 Kasai portoenterostomy, 1246–1247 Bardet–Biedl syndrome, 1880 malabsorption, 1234 Barlow’s test, 466l in premature infants, 1238 Baro-and volu-trauma, 889 prognosis, 1247 Barotrauma, 837 solitary liver cyst, 1249–1250 , 156, 1557, 1564 surgery, 1227, 1246–1247 Bartter syndrome, 1846 therapy, 1246–1247 Basal vein of Rosenthal, 2273 Biliary diversion procedures, 1237 Basophils, 1427 Biliary drainage, 1233 Bax protein, 2152 Biliary tract malformations Bayley scales of infant development, 245, 2039 biliary atresia (see Biliary atresia (BA)) compartement defects congenital bile duct dilatation, 1247–1248 agammaglobulinemia either X linked (XLA), solitary liver cyst, 1249–1250 1609–1610 Bilirubin hyper IgM syndrome (HIGM), 1610–1612 conjugation genetics, 1147–1148 B-cells, 1587–1589, 1609 Crigler–Najjar syndrome, 1147, 1155, 1177, BCG vaccination, 1612 1179, 1203 Bcl-2 family of , 2151 and encephalopathy, 1146, 1163, 2077 Beckwith–Wiedemann syndrome (BWS), 60–61, 1297, clinical symptoms, 1174 1852, 1876 diagnosis, 1175 Behavior, 2037, 2038 epidemiology, 1172–1173 and psychological diagnosis and disabilities, Gilbert syndrome, 1147, 1162 2046–2047 metabolism, 1144–1145 and psychology, 2039 neurotoxicity, 1188 Behavioral assessment scale, 2060 toxicity, 1174 Bell staging criteria, 1374 unconjugated, 1146–1147 Benchmarking, 252, 254, 286, 311 See also Hyperbilirubinemia; Jaundice Benign recurrent intrahepatic cholestasis (BRIC), Bilirubin albumin ratio trial (BARTrial), 1191 1233, 1238 Bilirubin-induced brain damage, 1189 Benzodiazepines, 387, 392, 684 Bilirubin-induced neurologic dysfunction (BIND), 1155, Bernard–Soulier syndrome, 1480 1170, 1172–1174, 1181 Beta trace protein, 1925 clinical symptoms, 1171–1172 Bevacizumab, 2371–2373 diagnosis, 1173–1175 β-glucuronidase, 1145 risk factors, 1171 17β-HSD3 deficiency, 1908 treatment, 1175–1177 Bhutani nomogram, 1153, 1155–1156, 1162 Bilirubin-related brain damage, 1155 Bianchi’s minimal Binding affinity of albumin, bilirubin, 1153 invasive management, 1299 Binding capacity, 1153 Bianchi technique, 1325, 1326 Binding of bilirubin to albumin, 1164 Bifidobacteria, 527, 528 Biologic agents, 62 2466 Index

Biomarkers, 892–898, 2176 Blue-green spectrum, 1187 Biophysical profile (BPP), 111–112 Bochdalek’s diaphragmatic hernia, 1318 amnioscopy, 112 Body amniotic fluid index, 112 fat, 4 Biotin, 521 length, 158–159, 663 preterm nutrition, 592 temperature (see also Thermoregulation) vitamins, 521, 673 axillary, 181 Biotinidase deficiency, 473, 1809, 1811, 1817–1818, 1829 thermal environment, 323–343 Biotrauma, 836–837 weight, 62, 157, 158, 185, 197, 589 Birth and surfactant, 802 defects, 1697 Body-mass index (BMI), 29 rate (preterm), 10–11, 134–135, 256, 474 Bolus feeding, 598 trauma, 19, 21, 1116 Bombesin-like peptide, 897 weight Bonding, 211, 214 and esophageal atresia, 1286 of infant and mother, 191, 196, 401 and neutropenia, 1564 Bone and PDA, 1080 dysostoses, 64–67, 1276 Birthmarks, 2416–2418 marrow, 1428–1429 pigmented b., 2416–2417 aspiration, 1651 vascular b., 2418 biopsy, 1483 Bladder exstrophy, 1961, 1971–1972 transplantation, 1541, 1614, 1615 Bleeding accretion, 648, 663–667 disorders (see Coagulation, disorders) morphogenetic protein (BMP) , 936 time, 1482 , 67–70 Blepharophimosis, 2341 osteomyelitis, 1745–1747 Blisters sucking, 2397 osteopenia of prematurity, 664–667 See also Pustules, blisters and erosion osteoporosis, 69 Bloch–Sulzberger syndrome, see rickets (see Rickets) Blood turnover, 666 analysis, 766, 2174–2175 Bosentan, 950 respiratory distress syndrome, 477–478 Bowel and enterostomy formation, 1397 aspiration syndrome, 866 , 64, 1259 bank, 1201 , 56, 67 culture, 1217, 1731 Brady-arrhythmias, 1107–1108 bacterial and fungal infection, 1727–1764 Bradycardia, 112, 122 flow, 1126, 1127, 1133, 1134 neonatal hypercalcemia, 659 group incompatibility (see ABO immune disease) resuscitation of the newborn, 426–428 lactate concentration, 414 and SIDS, 1021–1032 loss (see Hemorrhage) Brain pressure, 476 abscess, 2215 causes of hypertension, 1120 blood flow, 368 causes of neonatal shock, 1116 congenital malformations, 2125–2141 coarctation, 464 injury, 2166, 2173, 2176 neonatal blood pressure, 111, 1113 cerebral hemorrhage, 2201–2218 pre-eclampsia, 33 cerebral outcome, 238–240, 2315–2321 selected drugs in hypotension and cerebral plasticity, 265–274 hypertension, 1122 EEG abnormalities, 2084–2085 treatment of hypertension, 1121 fetal injuries, 11–12 treatment of hypotension, 1116 hypoxic-ischemic encephalopathy, 2143–2160 variation in the neonate, 1115 hypoxic-ischemic syndrome, 2165–2179 products (see Transfusion) and inflammation, 75, 2019–2026 sampling neonatal brain damage, 2295–2309 delivery, 1416–1417 neuroimaging studies, 2091–2110 fetal scalp, 123–124, 412 neuroprotective strategies, 2185–2197 transfusion (see Transfusion) , 259–260 volume, 433–434 plasticity, 265–274 Blood–brain barrier (BBB), 1627 tumors, 1654–1655 Blue diaper syndrome, 1861 symptoms, 1654 Blue fluorescent lamps, 1196 treatment, 1657–1658 Index 2467

See also Central nervous system Bronchopulmonary dysplasia (BPD), 23, 76, 78, Brain-derived neurotrophic factor (BDNF), 2307 83, 85, 88, 161–162, 258, 259, Brain-sparing effect, 119 887–906, 998, 999, 1001, 1003, Brainstem auditory evoked potential (BAEP), 1191 1004, 1017, 1019 Branchial cleft cysts, 2423 alterations in ventilation/perfusion ratio, 771–772 Branching morphogenesis, 1920 biomarkers in, 892–898 Branchio-oto-renal syndrome, 1922 clinical aspects, 898–899 Brazelton scale, 22, 2060 clinical diagnosis, 898 Breastfed infants, 545, 547 radiological findings, 898–899 Breastfeeding, 373–374, 523, 631–632, consensus definition, 889 681–683, 1686–1688, 1691, cystic BPD, 899 1693 differential diagnosis, 899 analgesic role, 373–374 dysregulated, 889 controversies early phase of, 898 contraceptive methods, 570 established BPD, 898 cytomegalovirus (CMV), 568–569 etiology and pathogenesis, 889–892 group B streptococcus (GBS), 569 baro-and volutrauma, 891 hepatitis B virus (HBV), 568 genetics, 889 hepatitis C virus (HCV), 568 hyperoxia, 890–891 medications to mother, 569–570 lung immaturity, 890 new pregnancy, 569 postnatal infections, 891 persistent organic pollutants (POPs) prenatal infections, 890 exposure, 569 role of cytokines, 891–892 radiological contrast agents, 570 evolving phase of, 898 HCV infection, 1691 high risk infants, 258 HIV infection, 1686 and intrauterine growth restriction, 161–162 hyperbilirubinemia, 1147 neurodevelopmental outcomes, 901–902 IDM, 721–723 and patent ductus arteriosus, 1082 initiation of, 402 pathology, 889 See also Milk physiologic definition, 889 Breast milk, 1385 prematurity, 890 supply prevention, 905–906 and baby weight gain, 566–567 prognosis, 899–902 low urine output, 567–568 pulmonary outcomes, 899–901 See also Milk steroid treatment, 1017 Breath-holding apneas, 778 surgical ligation of PDA, 891 Breathing therapy, 902–906 apnea, 779 vitamin A supplementation, 673–674 chemical regulation, 782–784 Bronchopulmonary sequestration (BPS), 930 control of, 775–787 clinical aspects, 743 patterns at rest, 776–778 differential diagnosis, 743 periodic breathing and apnea, 778–782 etiology and pathogenesis, 743 pulmonary reflexes, 784–785 Bronze baby syndrome, 1195, 1198 head’s paradoxical reflex, 429 Bruising and abrasions, 462 Hering–Breuer reflex, 983 Brush border membrane, 518 respiratory muscle, 786 Bruton’s agammaglobulinemia, 1609–1610 resuscitation of newborn, 427–428 See also Agammaglobulinemia either Breech delivery and GMH-IVH, 2206 X linked (XLA) British Guideline Development Group β2-subunit, 1533–1534 (GDG), 1368 Budesonide, 904 Bronchial branching process, 735 Bullae Bronchial tree abnormalities, 928 erythropoietic protoporphyria, 2408 Bronchial ultrastructural anomalies, 919–921 herpes gestationis, 2407 Bronchial walls disorders, 928 neonatal pemphigus vulgaris, 2407 Bronchiolitis, 177, 985, 1789, 1790 suckling blisters, 2397 Bronchoalveolar lavage, 892 Bullosa, see Epidermolysis bullosa Bronchogenic cyst (BC), 930 Bullous ichthyosiform erythroderma, 2411 Bronchomalacia, 737, 898, 901 Bullous impetigo, 2401, 2402 See also Tracheomalacia Buphthalmos, 2346 2468 Index

Burst-suppression (BS), 2085 fetus, 596 Byler disease, 1237 glucagon-like peptide, 538–540 See also Progressive familial intrahepatic cholestasis glycoprotein deficiency, 519 metabolism disorders congenital carbohydrate malabsorption, 662 C congenital disorders of glycosylation, 1813 Café-au-lait spots, 2414 galactosemia (see Galactosemia) Caffeine, 863 glycogen storage disease, 1518, 1557, 1564, 1813 apnea of prematurity’s interventions, 1024 hereditary fructose intolerance, 1217, 1220 citrate, 1025 pyruvate dehydrogenase deficiency, 1820 treatments during ventilation, 863, 1002, 1003 parenteral intakes for ELBW and VLBW infants, 606 Calcaneovalgus, 2389 parenteral nutrition, 608–609 Calcitonin, 652–653, 661, 723, 1862 recognition domain, 804 Calcium, 520, 639–667 Carbon dioxide calcitonin, 652–653 blood level control, 848–849 disturbances, 653–662 breathing chemical regulation, 782–784 neonatal hypercalcemia, 659 dissociation curve, 76, 768 neonatal hyperparathyroidism, 659–661 fetal arterial blood flow, 119 neonatal hypocalcemia, 653–659 fetal oxygenation, 106 nephrocalcinosis in preterm infants, 661–662 production, 336 fortifier, 600–601 transcutaneous monitoring, 476 gluconate Carbon monoxide, 711 hypocalcemia treatment, 658, 722–724, 1859 Carboxyhemoglobin, 1159 parenteral nutrition, 614 Cardiac hormonal regulation catheterization, 1047–1048 FGF23 and phosphatonin defects, 1683 (see also Cardiovascular system; Heart) peptides, 653 prevalence, 141 parathyroid hormone, 649–650 tamponade, 883 vitamin D, 650–652 dysfunction, 2171 intestinal absorption, 642–644 tamponade parenteral nutrition, 592–593, 609–610 causes, 1095 physiology, 641–646 definition, 1094 placental transport, 641–642 emergency treatment, 1095–1096 preterm human milk, 599 Cardiomyopathy, 1044 renal excretion, 644–645 Barth syndrome, 1564 requirement, 645–646 inborn errors of metabolism, 1808 serum calcium, 641 infants of diabetic mothers, 725 urinary, 1857 Cardiopulmonary bypass, 1008, 1009 Calcium-sensing receptor, 649 Cardiorespiratory monitoring, 475–476 defect, 662 Cardiotocography, 10–11, 112–115, 2299 Callosal agenesis, 2100 contraction stress test, 114–115 , 68, 1905 description, 112–113 Campylobacter spp., 1359 non stress test, 113–114 Canalicular phase, 736, 890 Cardiovascular system, 1037–1063 Cancer, see Tumor(s) cardiac catheterization, 1047–1048 Candida spp., 1750, 2401 causes of heart failure in newborns, 1044 albicans, 2401 cyanosis, 1042–1043 vaginitis,99 duct-dependent pulmonary blood flow, 1048–1053 Capillary critical pulmonary valve stenosis, 1048–1049, hemangioma, 460 1073–1074 malformation, 2419 Ebstein’s anomaly, 1052–1053, 1072 Captopril, 798, 1121, 1530 pulmonary atresia with intact ventricular septum, Caput succedaneum, 462 1049–1051, 1075 Carbamazepine, 1233, 1479, 1530 tetralogy of Fallot, 1051–1052, 1072–1073 Carbamyl phosphate synthetase deficiency, 1820 duct-dependent systemic blood flow, 1053–1058 Carbapenems, 1736 aortic stenosis, 107, 1054 Carbimazole, 1530 coarctation of the aorta, 1054–1055, 1076 Carbohydrate, 518 hypoplastic left heart syndrome, 1057–1058 digestion and absorption, 518–519 interrupted aortic arch, 1055–1057, 1076 Index 2469

heart failure in the newborn, 1044 Central nervous system (CNS), 4, 2127, 2128, 2130, 2132 fetal and neonatal circulation, 1038–1040 disorders of CNS cell number, 2137 indications for neonatal interventional disorders of cortical connectivity, 2137–2139 catheterization, 1048 and ventriculomegaly, 2139–2140 indications for palliative procedure, 1067 intracranial arachnoid cysts, 2139 left-to-right shunt lesions malformations, 2113–2123 atrioventricular septal defect, 1062–1063 cerebellar hypoplasia, 2131 ventricular septal defect, 1061–1062 congenital, 2125–2141 lesions with complete intracardiac mixing developmental disorders of cerebellum, complex single ventricle, 1060–1061 2130–2132 total anomalous pulmonary venous return, holoprosencephaly, 2127–2128 1059–1060, 1072 and subcortical band eterotopia, truncus arteriosus, 1060, 1070 2115–2119 Norwood procedure, 1069 midline or vermis malformations, 2130–2131 parallel circulation/transposition of the great arteries, neuronal tube defects, 2128–2130 1058–1059, 1070–1072 polymicrogyria, 2120–2122 polycythemia and hyperviscosity, 1125–1137 pontocerebellar hypoplasia, 2131–2132 preoperative management, 1066–1070 prenatal diagnosis, 2127 regulation of pulmonary vascular resistance and and meconium aspiration syndrome, 423, 436 pulmonary blood flow, 1040 neuroimaging, 268, 2091–2110, 2299–2301 Care neuronal migration disorders, 2133 ELBW infants lissencephaly, 2132–2134 nutritional support, 209 polymicrogyria, 2120–2122, 2134–2135 ventilatory support, 209 schizencephaly, 2135 See also perinatal care and rubella associated defects, 1683–1684 Carnitine, parenteral nutrition, 611 See also Brain Cartilage hair hypoplasia (CHH), 808, 1564, 1615 Central venous pressure, 986, 1516 Casein Centrum semi-ovale stroke, 2269 intestinal absorption, 643 Cephalhematoma, 19, 462 parenteral preterm nutrition, 612 Cephalic pustulosis, 2399 Caspase-3, 2154 Cephalosporins, 1736 Cataract Cerebellar congenital, 463, 2345 hematoma, 2230 congenital muscular dystrophies-associated, 2328–2331 hemorrhage, 2010, 2106 inborn errors of metabolism, 1810 hypoplasia, 2131 rubella-associated, 63, 1684 vermis agenesis, 2130–2131 varicella-associated, 2399 Cerebral Catch-up growth, VLBW infants, 591 artery pulsatility index, 118 Cathepsin K, 894 atrophy, 352 Catheterization blood flow, 11–12, 368, 385, 436 cardiac, 1047–1048 and hypoxic-ischemic encephalopathy, 2145 interventional, 1048 and hypoxic-ischemic syndrome, 2023 umbilical vessels, 397 in preterm, 1115 Caudal epidural anaesthesia, 387 circulation, 118, 1115 CD5 expressing B-cells, 1589 cortex, 367 CD40L deficiency, 1611 edema, 119, 352, 460 Cefotaxime, 1737, 1740 allopurinol, 2192 Ceftazidime, 1737, 1740 ischemia, 11–12 Celiac trunk, 507 plasticity, 265–274 Cell-cycle regulation, 2115 language, 268–270 Cell-free fetal DNA, 1157 sensori-motor system, 270–272 Cell-mediated immunity, 1584–1585 visual system, 272 Cellular death pathways, 891 sinus and venous thrombosis, 2097–2098 Cellular immunity, 1586 ultrasound, 243, 2091–2110 Cellular metabolism, 149 venous thrombosis, 2097–2098 Cellulitis and omphalitis, 1748–1749 , 246, 2006 Central apnea, see Apnea Cerebral palsy, 241, 351, 410 Central cord syndrome, 2269 ataxic, 238 Central core disease (CCD), 2325 athetoid, 238 2470 Index

Cerebral palsy (cont.) Cholesterol, 520 early markers of poor outcome, 238–239, 241–243 Alagille syndrome, 1213 gross motor function classification system for, 239 Cholestyramine, 1232 IUGR, 164 Choline, 803, 817 neonatal care, 351 Chondrodysplasia, 1615 outcome, 2315–2321 Choreoathetosis, 1191 preterm birth adverse outcomes, 2318–2320 Chorioamnionitis (CA), 75–78, 207, 827, 890, 906, spastic, 238 1630, 2022 Cerebral sinovenous thrombosis (CSVT), 1464, 2093, acute and chronic diseases, 100–102 2095, 2097–2098, 2215, 2226 antenatal factors, 98 Cerebroplacental ratio (CPR), 119 fetal consequences, 101 Cerebrospinal fluid (CSF), 1491 incidence, 98 Ceruloplasmin, 446 infants born late-preterm to term, 102 Cesarean section, 1691 infants born very preterm, 102 and GMH-IVH, 2206 infections associated with, 99 history of the pregnancy and delivery, 19 meconium, 100 neonatal resuscitation practices, 349–351 morbidity prevention, 102 and transient tachypnea of the newborn, 177, 867–868 neonatal management, 1756 CHARGE syndrome, 54, 385, 1914 neurologic consequences, 101–102 Chediak–Higashi syndrome, 1528, 1534, placenta and fetal membranes study, 96 1536–1538, 2415 pulmonary consequences, 101 Chemical agent, 62–63 villitis of unknown etiology, 100 , 894, 1526, 2021 without microbes, 100 Chemoreceptors, 119 Chorioangioma, 1210, 1503 control of breathing, 779, 783 Chorionic villus sampling (CVS), 10, 1156, 1904 response to doxapram, 1026 Choroid plexus, hemorrhage, 2103, 2201–2218 Chemotactic agents, 524 Chromatin, 42–44, 47 Chemotaxis, 1526–1527, 1578–1579 Chromosomal abnormalities, 55–57, 1256 Chemotherapy, in neonates, 1645–1646 Chromosome Chemotrypsin, 517 abnormalities, 55–57 (see also individual syndromes) Chest Beckwith–Wiedemann syndrome, 60– compression, 415, 424 61, 1876 examination, 464–465 Cornelia de Lange syndrome, 58 tube drainage, 882 cri-du-chat syndrome, 56 X-ray DiGeorge syndrome, 1615 bronchopulmonary dysplasia, 898 , 61–62 coarctation of the aorta, 1054–1055 , 59 cyst bronchogenic, 745 , 59–60 lung agenesis, 746 Prader–Willi/Angelman, 46, 60 pneumomediastinum, 879 , 47 pneumopericardium, 883–884 Rubinstein–Taybi syndrome, 58–59 pulmonary hemorrhage, 867 Silver Russel syndrome, 61 Chiari malformation, 2140 Smith–Lemli–Opitz syndrome (SLOS), 62 Chloramphenicol, 405 trisomy 8, 56–57 Chloride trisomy 13 (Patau syndrome), 56 absorption, 520, 643 trisomy 18 (Edwards syndrome), 56 congenital chloride diarrhea, 1846 trisomy 21 (Down syndrome), 55–56 meconium ileus, 1310–1311 Turners’ syndrome, 57 mineral source, 614 Wolf–Hirschhorn syndrome, 56 parenteral nutrition, 610 rearrangements, 1616 Chloride-bicarbonate anion exchanger, 519 Chronic Chloroquine, 1530, 2009 bilirubin encephalopathy, 1190, 1200 3-Chlorotyrosine, 894 fetal distress, 110 , 463, 770 fetal hypoxia, 793 Cholecystokinin (CCK), 548–549 granulomatous disease, 1534–1535 Choledochal cyst, 1226, 1249 idiopathic neutropenia of prematurity, 1567 Cholera, 1358 kidney disease, 164 Cholestasis, 1210–1214, 1244, 1245, 1809, 1826 lung disease, 1018, 1019 See also Neonatal cholestasis pain scale, 369 Index 2471

protein-energy malnutrition, 626 perfringens, 1731 pulmonary insufficiency, 750 tetani, 1748 (see also Bronchopulmonary dysplasia) Cloverleaf , 65 renal failure, 1950–1952 Clover syndrome, 1322 anemia, 1952 Coagulase-negative Staphylococci, 1539–1540, 1729 arterial hypertension, 1952 pneumonia, 457 causes of, 1950–1951 Coagulation, 1433, 1459 failure to thrive and anemia, 1951 anticoagulant proteins, 1434 fluid, electrolyte and acid–base balance, cascade, 1462 1951–1952 developmental hemostasis, 1433 hypertension and congestive heart failure, 1951 disorders, 161, 1431–1450 malformative uropathies, 1951 platelet disorders, 1471–1484 nephrotic syndrome, 1951 platelet disorders: platelet function in neonates, nutrition, 1951 1481–1483 RRT, 1952 platelet disorders: qualitative disorders, Chylomicrons, 517 1480–1481 Chylothorax, 752–754 platelet disorders: quantitative disorders, Ciliary abnormalities, 919–921 1474–1480 Cimetidine, 1313 EPCR and TM in, 1435 dilating effects, 1081 fibrinogen concentrate, 1450 Ciprofloxacin, 1709 fibrinolytic proteins, 1434–1436 Cirrhosis, 1239, 1692, 1694 neonatal bleeding disorders (see Neonatal bleeding biliary cirrhosis, 1210–1212 disorders) peroxisomal disorders, 1813 neonatal platelets, 1436 Citrobacter, 1730 pathways of, 1433, 1434 Clamydia PCC, 1450 cause of neonatal pneumonia, 869, 1741–1743 plasma, 1450 trachomatis, conjunctivitis, 404 platelet function tests, 1438–1439 Clara cell secretory protein (CCSP), 894 proteins, 1457 Classical binary physiology, 1895 PT and aPTT, 1438 Classic galactosemia, 1220 recombinant factor VIIa, 1450 Clearance, 699 reference ranges in neonates, 1439 Clear cell sarcoma of kidney, 1654 thrombelastography/thromboelastometry, 1438 Cleft lip/cleft palate (CLCP), 63, 401, 1267–1270 Coagulopathy, 2210, 2217, 2218 , 68–69 Coarctation of the aorta (CoAo), 1054–1055, 1076 DiGeorge syndrome, 1057 Cocaine, 7, 31, 51 endocrine disorders, 1833–1885 See also Maternal, drug abuse Goldenhar syndrome, 61–62 Cochrane analysis, 1777 Opitz syndrome, 1914 Cochrane review, 1017 Patau syndrome, 56 , 2415 Pierre–Robin syndrome, 54 Codeine, 391, 683 schisis association, 1285 Coffin–Siris syndrome, 2131 SLOS syndrome, 62 Cognition and very preterm birth, 230–232 and smoke, 711–712 Cognitive function Walker–Warburg syndrome, 2329 and BPD, 902 Clinical congenital brain lesions, 266 audit, 253–254, 286, 293 epidemiology of adverse outcome, 2316 governance, 252, 286–287 neuroprotective treatments, 2155–2160 scoring system, 400, 1175, 2070 outcome of preterm babies, 245–246 Clinical chorioamnionitis, 97 Cognitive neuroscience, 220 Clinical pathways (CPs), 319–320 Cognitive outcome, preterm birth, 2319–2321 Clip and drop technique, 1399 CO-hemoglobin, 1144 Clitoroplasty, 1912 COL4A1, 2206 Cloacal malformations, 54 COLAA2, 2206 prune belly syndrome, 55 Colic atresia, 1308–1310 Clonazepam, 2172 Colipase, 516 Clonidine, 2153 Colipase-dependent pancreatic lipase, 516 Clostridium spp., 1554 Collagen, 1472 difficile, 1358 Collodion baby, 2410 2472 Index

Coloboma, 463, 2340, 2347 heart disease (CHD), 464, 899 association with genetic syndromes, 56, 61, 66 clinical presentation, 1041–1043 of iris, 2347 preoperative management, 1066 Colon, 515 hypothyroidism Colonic atresia, 1307 central, 1865–1866 Colonization of gastrointestinal tract, 522 clinical signs, 1867 Colostrum, 1593 epidemiology, 1865 Combined antiretroviral (cARV) therapy, 1686 etiology, 1865 Combined immunodeficiencies (CID), 1614, 1615 follow-up, 1869–1870 Comfort, 223 peripheric hypothyroidism, 1867 Common variable immunodeficiency (CVID), 1610 scintigraphy, 1868 Communicating pneumothorax, 880 screening programs, 1867–1868 Communication, 312 therapy, 1869 Compassion, 223 thyroid dysgenesis, 1866 Complaints, 312, 349 thyroid dyshormonogenesis, 1866–1867 Complement factors, 1579 thyroid ultrasonography, 1868 Computed tomography transient, 1867 chest, 929 immunodeficiencies head, 2091–2110 agammaglobulinemia either X linked (XLA), See also Imaging 1609–1610 Computerized provider order entry (CPOE), 313 autoimmune polyendocrinopathy-candidiasis Confidential enquiries, 253, 254, 287 ectodermal dystrophy syndrome Configurational isomers, 1194 (APECED), 1616 Conflicts in neonatal care, 193 cartilage hair hypoplasia (CHH), 1615 Congenital combined immunodeficiencies (CID), 1614 adnexal disorders, 2420–2421 DiGeorge syndrome (DGS), 1615 adrenal hyperplasia, 1840 (see also Adrenal) hyper IgM syndrome (HIGM), 1610–1612 adrenal hypoplasia (see Adrenal) immunodysregulation, polyendocrinopathy, alveolar capillary dysplasia (ACD), 925–926 , X-linked (IPEX), 1616 amegakaryocytic thrombocytopenia (CAMT), 1476 Nijmegen breakage syndrome (NBS), anomalies of the kidney and urinary tract 1615–1616 (CAKUT), 1960 SCID neonatal screening test, 1617 aplasia cutis, 2421 severe combined immunodeficiencies (SCIDs), candidiasis, 1751 1612–1614 cataracts, 63, 463, 2345, 2346 Wiskott–Aldrich syndrome (WAS), 1614 chylothorax, 927 infection, 1505–1506 cystic adenomatoid malformation, 929–930 intrauterine pneumonia, 869 characterization, 742 ion transport defects, 519 clinical aspects, 742 large hyperlucent lobe, 740, 928–929 differential diagnosis, 742 leukemia, 1656 therapy and treatment, 742 lobar emphysema (CLE), 744–745 diaphragmatic hernia, 740, 810, 817–818, 950–956, lung cysts, 744 964, 1010 malformations, 49–70 acute pulmonary hypertension, 951–954, classification, 50–51 965–967 disruptions, 62–64 characterization, 950 disruptions: biologic agents, 62 chronic pulmonary hypertension, 955, 967–968 disruptions: chemical agents, 62–63 outcomes, 955–956 genetic counselling, 52 prognostic factors, 964 prenatal diagnosis, 52 pulmonary hypertension, 964–965 mandibular diseases, 1270–1271 respiratory distress, 919 megacolon, 1346 subacute (late) pulmonary hypertension, melanocytic nevi, 1658 954, 967 mesoblastic nephroma (CMN), 1653 disorders of glycosylation, 1808, 1809, 1811, 1813, muscular dystrophies (CMD), 2328 1816, 1827 myasthenic syndromes (CMSs), 2332–2333 dyserythropoietic anemia, 1507 myopathies (CMs), 2324–2328 fiber type disproportion (CFTD), 2327 , 2330 fibrosarcoma (CF), 1651 naso-lacrimal-duct obstruction, 2343 heart defects, 712, 1038 neutropenia, 1550 Index 2473

with extra hematopoietic manifestations, RDS, 980 1555–1557 renal function, 977 with innate and adaptive deficiency, 1554–1555 respiratory effects of, 976 pneumonia, 1741 side effects, 985–986 pulmonary lymphangiectasia (CPL), 926–927 tracheobronchomalacia, 985 pulmonary malformations, 737 Continuous tracheal gas insufflation (CTGI), 904 rubella infection, 1683–1684 Continuous venovenous hemofiltration, 1949 rubella syndrome (CRS), 1683, 1684 Contraction stress test (CST), 114–115 (see also Rubella virus) Contralateral disturbances, 2263 surfactant protein abnormalities, 921–924 Contrast studies, 749 syphilis, 1706 Control of breathing thoracic malformations, 740–742 (see also Solid and breathing patterns at rest, 776–778 cystic lung diseases) chemical regulation, 782–784 tubulopathies, 1929 periodic breathing and apnea, 778–782 tumors (see Tumor(s)) respiratory muscles, 786 varicella syndrome, 1677 upper airways and pulmonary reflexes, 784–785 Congestive heart failure (CHF), 1043 Convection, 323–343 computed tomography and magnetic resonance, 1048 Conventional mandatory ventilation (CMV), 851–853 initial evaluation, 1044–1047 Conventional ventilation vs. HFOV in MAS, 796 in newborn, 1048–1063 Convergent , 2341 Conjuctivitis, 99 Coombs test, 1414 Conjugated hyperbilirubinemia, see Neonatal cholestasis Copper, 670 Conjugate meningococcal B vaccine, 1799 Cord blood monocytes, 1527 Conjugate meningococcal C vaccine, 1799 Cord-clamping, 1128, 1129, 1131, 1135 Conjugate pneumococcal vaccine, 1799 Cord occlusion, 143–145 Conjunctivitis, 404, 1747 Cordocentesis, 1669, 2217 Pseudomonas, 1731 Corneal dystrophy, 2344 Conners Rating Scale, 2039 Corneal opacity, 2344 Conradi–Hunermann syndrome, 1810, 2409 Cornelia de Lange syndrome, 58, 2396 Consent, 19, 285, 353–354 Coronavirus, 869 Conservative forces, 763 Cor pulmonale, 898 Constipation, 659, 1347 Corpus callosum agenesis, 62, 1836, 2116, 2127, 2134, Continuous 2136, 2138 intravascular blood gas monitoring, 849 Corrected end-tidal carbon monoxide (ETCOc), 1155, respiratory function monitoring 1156, 1159 pressure and flow wave, 849–850 Cortical development, malformations, 2132 tidal volume wave, 850 , 797, 1016, 1017, 1117, 1852, Continuous positive airway pressure (CPAP), 429, 2214, 2217 971–990 IVH prevention, 2213 atelectasis, 985 lung disease, 1017 Benveniste system, 977 shock therapy, 1115 bronchiolitis, 985 vaccines, 1799–1800 cardio-circulatory system, 976 Cortisol clinical applications, 980 hypertension, 1121 congenital cardiopathies, hyperafflux, 985 hypotension, 1115 continuous and variable flow system, 977 PDA, 1082 continuous flow systems, 977 sepsis, 1781 endotracheal tube, 979 Cotinine, 711–713 mask, 979 Counselling, 52, 192–194 facial chamber, 979 antenatal, 185, 192, 197, 282 head-box, 979 genetic, 52, 62, 192, 1285, 2120 infant flow system, 977–979 parents, 223 intracranial pressure, 977 (see also Nasal continuous postnatal, 185, 192 positive airways pressure (N-CPAP)) Couplet care, 361 nasal masks, 980 Cow’s milk, 599 nasal prongs, 979–980 Coxsackie viruses, 1478, 1518, 1786 nasopharyngeal tube, 979 Cranial compression encephalopathy, 2232 neonatal apnea, 983–984 Cranial ultrasound, 2091–2110 post-extubation phase, 984–985 Craniofacial dysostoses, 64 2474 Index

Craniostenosis syndromes pulmonary valve stenosis, 1073–1074 apert syndrome, 1261–1263 respiratory and cardiac causes, 1100 , 1261 severe Ebstein’s anomaly, 1052–1053, 1072 Smith–Lemli–Opitz syndrome, 1263 total anomalous pulmonary venous connection, treatment, 1265–1266 1059–1060 Craniosynostoses, 463 transposition of great arteries, 1058–1059 syndromal, 64 tricuspid atresia, 1067 syndromic, 65–66, 68 truncus arteriosus, 1060 C-reactive protein (CRP), 474, 1634, 1732, Cyclic neutropenia (CyN), 1530–1532, 1788, 1795 1554, 1565 Creatine kinase, 352, 2176, 2324 Cyclooxygenase inhibitors, 1083, 1940 Creatinine, 1978, 1980 Cyst acute renal failure, 1943 arachnoid, 2139 lactate/creatinine ratio, 416–417 branchial cleft cysts, 2423 CRH (corticotropin-releasing hormone), see Hormones bronchogenic, 744, 919, 929, 930 Cri-du-chat syndrome, 56, 1518 congenital solitary liver cyst, 1249–1250 Crigler–Najjar syndrome, 1147, 1155, 1177, 1179, cystic periventricular leukomalacia, 2203 1199, 1203 dermoid, 2421–2422 Critical gums, 463 aortic stenosis, 1054 congenital solitary liver cyst, incident, 307 1249–1250 incident reporting, 309–310 lung, 737 Cromolyn, 904 porencephalic, 2217 Crossed renal ectopia, 1963 Cystatin C, 1925, 1944, 1982 Cross-talk process, 735 Cystic Crouzon syndrome, 65, 1261 adenomatoid malformation of lung, 742–743 , 65 duplications, 1315 Crying, 21, 22, 369, 460 dysplastic kidney, 1964 CRYO-ROP study, 2353, 2360, 2361 fibrosis, 1310 Cryptorchidism, 1913, 1914 causes of hypernatremia, 1846 Cryptosporidium parvum, 1358, 1611 meconium ileus, 1310–1311 C-terminal fragment of type I procollagen, 897 screening, 473 Cuddliness, 2062 transmembrane regulator protein, 833 Cushing syndrome, 1904 hygroma, 2420 Cutaneous and chylothorax, 753–754 herpes, 1674 and hydrops fetalis, 1518 infection, 2401 and Turners’ syndrome, 57 injuries, 307 kidneys Cutis and causes of hypertension, 1119–1120 aplasia (see Aplasia cutis) periventricular leukomalacia (PVL), 240 marmorata, 2396 Cystourethrography, 1970 CXCL12, 2021 Cytochrome c protein, 2151 Cyanosis, 1042–1043 Cytokinemia, 1634 and bronchopulmonary dysplasia (BPD), 898 Cytokines, 522, 892, 1622, 1633–1634, 2021 clinical features-general observation and Cytomegalovirus measurements, 460 clinical manifestations, 1667–1668 coarctation of the aorta, 1054–1055 diagnosis, 1668–1669 congenital heart disease, 1065–1076 epidemiology, 1665–1666 definition, 1042, 1099 fetal infections, 1663–1678 differential cyanosis, 1042 infection, 1665 duct dependent pulmonary circulation lesions, clinical manifestations, 1099–1101 1667–1668 Fallot’s tetralogy, 1051–1052 diagnosis, 1668–1669 hypoplastic left heart syndrome, 1057–1058 epidemiology, 1665–1666 hypoxia test, 1099 newborn evaluation, 1669 interrupted aortic arch, 1055–1057 pathogenesis, 1666–1667 and persistent pulmonary hypertension of the postnatal therapy, 1670 newborn (PPHN), 948–949 prenatal therapy, 1669 pulmonary atresia, 1053 prevention, 1670–1671 Index 2475

pathogenesis, 1666–1667 impetigo, 1749, 2402 prevention, 1670–1671 incontinentia pigmenti, 2406–2407 treatment, 1669–1670 infantile acropustulosis, 2398 Cytotoxic T-cells, 1665 Langerhans cell histiocytosis, 2408 mastocytosis, 2405–2406 miliaria, 2399 D neonatal cephalic pustulosis and neonatal Dandy–Walker malformation, 2131 acne, 2399 D antigen, 1156 neonatal pemphigus, 2407 Data collection, 299 pityriasis rubra pilaris, 2413 D-Dimers, 1465 , 2413 Deafness, 247 red scaly skin, 2410–2413 congenital rubella syndrome, 1684 scabies, 2402 and late congenital syphilis, 1705 seborrhoeic dermatitis, 2412 Death, perinatal, see Mortality staphylococcal scalded-skin syndrome, 1749, 2401 Decelerations of fetal heart rate, 121 syphilis, 1703–1710 Decision-making, 184, 194–195, 219–227 toxic erythema, 2398 Decision value, 223 transient sterile pustular disorders, 2398–2399 Defensins, 524 varicella, 1676–1678 Deformations, 51 vascular birthmarks, 2418 Dehydrated patients assessment, 1368 hemangiomas, 2418–2419 Dehydration, 1367–1369 zinc-deficiency dermatosis, 2408 fluid therapy types, 1367–1369 sebaceous hyperplasia, 2397 Delayed visual maturation, 246 subcutaneous fat necrosis, 2397–2398 Delivery indications, late preterm births, 174 sucking blisters, 401, 2397 Delivery room Dermoid cysts, 2421–2422 preparation, 397–398 Development, 536 neonatal resuscitation supplies, 397 care, 357–362 thermal stability, 398–400 NIDCAP, 360 Dendritic cells, 1583, 1594–1595, 1689 of gut function, 537, 538, 540 Denis–Drash syndrome, 1876 Developmental, 2033, 2034, 2036, 2038 Dentritic cells, 526 coordination disorder (DCD), 239 Denys–Drash syndrome, 1950 dysplasia of the , 466, 2389 Deoxycortocosterone (DOC), 1900 hemostasis, 1433 Dermatology origins of health and disease, 46 adnexal disorders, 2420–2421 plasticity, 42, 43, 46–47 aplasia cutis, 2421 Dexamethasone, 814, 1016–1019, 1799, 1904 birthmarks and naevi Dextrocardia, 747, 1045 epidermal naevi, 2417–2418 Dextrose, 397, 723 congenital tumors mellitus, 176 dermoid cysts, 2421–2422 Diabetic Kasabach–Merritt syndrome, 2419 fetopathy, 718–727 Langerhans cell histiocytosis, 1658, 2408 cardiac abnormalities, 725 leukemia, 2422 disorders of iron metabolism, 721 neuroblastoma, 1646–1647 growth disorders, 721–723 vascular tumors, 2418–2419 hyperbilirubinemia, 725 milia, 2397 hypocalcemia, 723–724 neonatal lupus erythematosus, 2413 hypoglycemia, 723 pustules, blisters and erosions hypomagnesemia, 723–724 atopic dermatitis, 2412 iron deficiency, 724–725 bullous ichthyosis, 2411 metabolic syndrome, 726 Candida, 1753, 2401 neonatal metabolic complications, 722 drugs, 2412 neurodevelopment, 726–727 eosinophilic pustulosis, 2398 neuropathology, 725–726 epidermolysis bullosa, 2404–2405 polycythemia, 724 Herpes simplex, 2399–2400 pulmonary status, 725 hyper-IgE syndrome, 2408 mother, 718–727, 1130, 1136 ichthyoses, 2411 pregnancy, 478 immunodeficiencies, 2410 Diamond–Blackfan syndrome, 1506, 1507, 1518 2476 Index

Diaphragm, 786 ethics and sex assignment, 1909–1911 Diaphragmatic hypospadias, 1914 eventration, 748 I-DSD Registry project, 1898 hernia, 817–818, 950–956, 1003 karyotype abnormalities, 1898–1899 Diarrhea, 1355–1363, 1365–1371, 1787 medical management in newborn, 1911–1912 babies with, 1366 sex chromosome, 1898 Diastrophic dysplasia, 68–69 surgical management, 1912–1913 pseudodiastrophic dysplasia, 69 46,XX, 1899–1904 Diazepam, 31, 1750 46,XY, 1904–1909 Diazoxide, 1852 Disproportionate Intrauterine Growth Intervention Trial At Diet, see Nutrition Term (DIGITAT), 18 Differential cyanosis, 938 Disruptions, 51, 62–64 Differentiation, 892 Disseminated intravascular coagulation (DIC), 1116, Difficult decisions, 220 1478–1479, 1779 Diffusion, 766 Dissipation forces, 763 Diffusion tension brain imaging (DTI), 361 Distichiasis, 2341 Diffusion weighted imaging (DWI), 2096, 2099, Distribution, 698 2100, 2259 Diuretics, 1860 DiGeorge syndrome (DGS), 57, 657, 1615, bronchopulmonary dysplasia, 903 1877, 2117 furosemide, 1946 Digestion, 515 hypertension, 1121 Digital anomalies, 67 mannitol, 1946 Dihydrotestosterone deficiency, 1907 Diverse animal models, 938 Dilated cardiomyopathy, 1564 Diverticular theory, 1315 Barth syndrome, 1564 Dizygotic twinning, 130–131 Dilute surfactant, 796 DNA Dimethylacetals (DMA), 895 chromosomal abnormalities, 55–57 Dipalmitoyl phosphatidylcholine (DPPC), 804, 831 damage, 450 Diphtheria, tetanus, and pertussis (DTP) epigenetic mechanisms, 42–45 vaccine, 1798 fragmentation, 2154 Diphtheria vaccine, 1689, 1798 investigations for inborn errors of metabolism, Diplegia, 241 1826–1828 Direct acting antiviral agents (DAAs), 1691, 1692 methylation, 43 Direct antiglobulin test (DAT), 1154, 1156 Dobutamine, 1117, 2171 Direct fluorescent T. pallidum test Docosahexaenoic acid (DHA), 527 (DFA-TP), 1705 Dohle bodies, 1566 Disability, 180, 288 , 1210 early markers of poor outcome, 237–248 Dopamine, 662, 1115, 1117, 1945–1946 and extremely preterm infants, 238–240 Doppler and high risk infants, 256–261 assessment, 1157 outcome of VLBW, 2031–2048 examination and preterm birth, 186–198, 476 utero-placental vessels, 115–116 Disaturated phosphatidylcholine (DSPC), 811–813, 815, examination–fetal vessels, 118–120 817, 818 examination–feto-placental vessels, 116–118 Discharge, 181, 208 indices, 115 body composition, 622 velocimetry instruction for endocrine disease middle cerebral arteries, 153 follow up, 287–289 umbilical arteries, 153 neonatal screening, 208 uterine arteries, 152–154 parental education, 208 Dorsal motor nucleus of vagus, 511 post discharge nutrition, 619–633 Double outlet right ventricle, 1056 protein and energy needs, 622 Downsyndrome,9,55–56, 198–199, 1303, 1518, 1537, 1538 special care needs, 209 associated abnormalities planning, 214 atrioventricular septal defect, 1062–1063 Dislocated lens, 1810 chylothorax, 753–754 Disorders of prematurity, 84, 88 congenital pulmonary lymphangiectasia, 926–927 Disorders of sex development hematological abnormalities, 1530, 1536 classification, 1897 Hirschsprung’s disease, 1347 cryptorchidism, 1913 skin manifestation, 2410 Index 2477

Doxapram, 1026 Dwarfism apnea, 1024 camptomelic, 68 Doxepin, 1530 cartilage-hair hypoplasia, 1564 Drug-induced osteodysplastic primordial, 69 neutropenia, 1530 rhizomelic short limb, 68 thrombocytopenia, 1479–1480 , 1914 Drugs thanatophoric dwarfism, 68, 748 absorption, 698 Dying babies, see Mortality abuse (see Maternal, drug abuse) Dyserythropoietic anemia, 1507 anesthetic, 387–389 (DKC), 1532–1533 developmental pharmacokinetics, 698–699 Dyskinetic cerebral palsy (CP), see Athetoid cerebral developmental pharmacology and therapeutics, palsy (CP) 698–706 Dysmorphic pulmonary vasculature, 891 elimination, 699 Dysmorphism formulations, 702 in endocrine diseases of newborn, 1857 labeling, 678 in malformations of cortical development, 2116 metabolizing enzymes, 699 in muscle-eye-brain (MEB) disease, 2329–2330 neonatal hypertension selected drugs, 1122 in Potter sequence, 54–55 nephrotoxic drugs, 1940–1941 in Smith–Lemli–Opitz syndrome, 1922 non-steroidal anti-inflammatory drugs, 1940, in Walker–Warburg syndrome, 2329 2189–2190 Dysmotility, 1292 during pregnancy and breastfeeding, 31, 677–689 Dysostoses, 64–67 resuscitation of the newborn, 433–434 craniofacial, 64–66 steroid and drug metabolism, 1781 limb, 67 stress relieving drug, 197–198 thoraco-vertebral, 66–67 therapeutic drug monitoring, 700–702 Dysphagia, in branchial cleft cysts (BCC), 2423 therapy, 695 Dysplasia, 19, 51 treatments during ventilation, 863 alveolar capillary, 754–755 Dual emission x-ray absorptiometry (DEXA), 630 bronchopulmonary (see Bronchopulmonary dysplasia) , 1877 dental, 1172 Duct-dependent diastrophic, 68–69 CHD, 297 hip, 282, 466 pulmonary blood flow, 1048–1053 neuronal intestinal, 515, 1348 pulmonary circulation lesions, 1099–1101 osteochondrodysplasias, 67–70 systemic blood flow, 1053–1058 pseudodiastrophic, 69 systemic circulation lesions, 1097–1099 renal (see Renal dysplasia) Ductus septo-optic, 54 arteriosus, 5, 951 Dysregulated angiogenesis, 891 closure, 1040 fetal and neonatal circulation, 1038–1040 patent ductus arteriosus, 206, 477, E 1079–1087 Early developmental care (EDC), 360 patent ductus arteriosus: epidemiology, Early diagnosis, 2342, 2348 1080–1081 Early enteral nutrition, 593 patent ductus arteriosus: pharmacological Early infantile epileptic encephalopathy (EIEE), 2288 treatment, 1083–1086 Early myoclonic epileptic encephalopathy (EMEE), 2288 patent ductus arteriosus: physiology and Early onset disease (EOD), 1774, 1775, 1781 pathogenesis, 1081–1082 Early-onset neonatal pneumonia, 1741 patent ductus arteriosus: surgical treatment, 1086–1087 abnormalities, 459 and RDS, 839 in Apert syndrome, 65 venosus, 120 in Cornelia de Lange syndrome, 58 Duodenal aspirate analysis, 1230 in cri-du-chat syndrome, 56 Duodenal atresia, 1303–1304, 1333, 1334 in DiGeorge syndrome (DGS), 1615 Duodenal obstructions in Down syndrome, 55–56 classification, 1303–1304 in Edwards syndrome, 56 extrinsic, 1302 in maternal hyperphenylalaninemia, 63 intrinsic, 1302 in Nager syndrome, 66 Duration of therapy, sepsis, 1737 in Noonan syndrome, 59–60 2478 Index

Ears (cont.) neuroprotective strategies, 2185–2197 in osteodysplastic primordial dwarfism, 69 timing of neonatal brain damage, 2295–2309 in , 65–66 End-diastolic flow (EDF), 117 in Turners’ syndrome, 57 Endocrine cells, 506 in Wolf–Hirschhorn syndrome, 56 Endocrine disorders, 1833–1885 hearing evaluation, 247 adrenal insufficiency, 1837–1845 Ebstein’s anomaly (EA), 1043, 1052–1053, 1072 hypercalcemia, 1837–1845, 1859–1862 Echocardiography, 749, 772, 938, 1055, 1071 hyperglycemia, 1853–1855 Echovirus, 1217, 1787 hyperinsulinism, 1848–1853 Eclampsia, see Pre-eclampsia hypernatremia, 1845–1848 Ectopic ureters, 1971 hypocalcemia, 1857–1859 , 67 hypoglycemia, 1848–1853 Edwards syndrome, 56 hyponatremia, 1845–1848 EEC syndrome, 67, 1878 hypopituitarism, 1835–1837 Ehlers–Danlos syndrome, 927 of sexual development, 1893–1914 ELA2 (ELANE) gene , 1551–1552 of thyroid function, 1862–1870 Elastin, 737 Endogenous thrombin potential (ETP), 1459 Elastogenesis, 890 Endoscopic retrograde cholangiopancreatography Elective cesarean section, 1686, 1687 (ERCP), 1230 Elective deliveries and gestational length, 174 Endothelial-derived relaxing factor (EDRF), 936 Electrocardiogram, fetal, 124–125 Endothelial progenitor cells (EPCs), 2357 Electroencephalography, 2081–2089, 2301–2302 Endothelial protein C receptor (EPCR), 1435 amplitude integrated EEG, 417 Endothelin, 508, 895, 1941 EEG-monitoring, 2086–2089 Endothelin-1 (ET-1), 895–896, 1942 effects of medications, 2085 Endothelin-receptor antagonist, 900 normal EEG maturation, 2082–2084 Endothelium, 1458 seizures, 2085–2086 Endotracheal Electrolytes, 206, 519, 591, 607, 1789 intubation, 424, 429–431, 901 imbalance tubes, 397 in epidermolysis bullosa, 2404–2405 Endotracheal intubation, 429 in meconium aspiration syndrome, 795 Endotracheal tube, 861, 979 Electronic fetal monitoring (EFM), 121–123, End-tidal carbon monoxide (CO), 897 412, 794 Energy, 106, 403, 515, 537, 541 cardiotocography, 112–115 density, 626 electronic external or internal monitoring, parenteral nutrition, 606–608, 610–611, 615 122–123 protein and energy needs in preterm infants, 622 fetal oxygenation, 123–125 VLBW infant, 588, 591, 592 fetal electrocardiography, 124–125 eNose, 1384 pulse oximetry, 123 Enteral heart rate, 114, 122–123 feedings, 1386, 2171 Ellis–van Creveld syndrome, 1062, 2131 nervous system, 510–511 Embolism, pulmonary gas, 884 nutrition, 601 Embryo crown-rump length (CRL), 110 bolus continuous feeding, 597–598 Embryofetopathies, 62 early enteral feeding of the VLBW infant, 595–602 Embryonal rhabdomyosarcoma (ERMS), 1650 early enteral nutrition, 593 Embryonic phase, 735, 890 expressed mother’s milk, 599 Encephalitis, 1673 human milk banks and donor milk, 599–600 Encephalocele human milk fortifiers, 601 in Potter sequence, 54–55 intragastric feeding, 598 in Walker–Warburg syndrome, 2329 late enteral nutrition, 593 Encephalopathy minimal enteral feeding, 597 bilirubin encephalopathy, 1146–1147, oral feeding, 598 1169–1181, 2077 preterm formula, 601 biochemical basis of hypoxic-ischemic preterm human milk, 599 encephalopathy, 2143–2160 transpyloric feeding, 598 clinical aspects and treatment of hypoxicischemic rehydration syndrome, 2165–2179 mechanism of, 1366 neonatal encephalopathy types of, 1366–1367 postasphyxial, 415–416 Enteric nervous system (ENS), 509 Index 2479

Enterobacteriaceae, 522, 1730, 1736 Epipupillary membrane persistence, 2345 Enterobacter spp., 1748 Epispadias, 1961, 1971 E. sakazakii, 1730 Epstein–Barr virus, 1478, 2415 Enterococci, 1730 Epstein pearls, 463 Enterocolitis, 1346, 1348, 1351 Equipment for neonatal Hirschsprung associated E. (HAEC), 1347, 1348 anesthesia, 390 necrotizing E. (NEC), 162, 260, 1373–1387, resuscitation, 397 1395–1400 transport services, 296 surgical treatment, 1395–1400 Erb’s palsy, 466, 726 Enterohepatic circulation, 517 Erosions, see Pustules, blisters and erosion Enterokinase, 517 Error, 307, 309, 312 Enteroviruses Erythema infectiosum (EI), 1697 characteristics, 1786–1787 Erythema toxicum neonatorum, 460 and chorioamnionitis, 99 Erythroblastosis, 478, 1518, 1529 clinical aspects, 1787–1788 Erythrocytes, 107, 118, 139, 205, 415, 417, 419, 440, 441, clinical manifestations, 1788 446, 449, 579 ,661, 670, 767, 1027, 1130, 1152, differential diagnosis, 1788 1201, 1217, 1220, 1406–1418, 1424, 1426, epidemiology and pathogenesis, 1787 1472, 1475, 1481, 1483, 1490–1494, and liver, 1220 1497–1502, 1505–1507, 1509, 1519, 1527, and neonatal infections, 1785–1790 1533, 1576, 1615, 1632, 1682, 1699, 1813, clinical aspects, 1787–1788 1825, 1823, 1854, 1979, 2176, 2277, differential diagnosis, 1788 2296–229, 2308, 2436, 2437, 2441, 2456 epidemiology and pathogenesis, 1787 blood sampling, 1417–1418 prognosis, 1788–1789 erythrocyte count, 1410 therapy and treatments, 1789 erythrocyte indices, 1410–1414 prognosis, 1788–1789 hematocrit, 1408–1410 therapy and treatments, 1789 hemoglobin concentration, 1407–1408 Enzyme immunoassay (EIA), 1715 morphology, 1416 Enzymes neonatal reference intervals for, 1491–1497 and cofactors, 1473 reticulocyte count, 1414–1416 digestion and absorption, 515–521 site of sampling, 1416–1417 non-immune hemolytic disease, transfusion, 1378, 1508–1509 1161–1164 Erythromycin, 404, 904, 1748 oxidative stress, 444–447 Erythropoiesis, 721, 725, 1130 See also Individual enzymes Erythropoietic protoporphyria, 1816, 2408 Eosinophilic gastroenteritis (EGE), 1302 hepatoerythropoietic porphyria, 2409–2410 Eosinophils, 1420–1423 Erythropoietin (Epo), 161, 670, 721, 1130, 1416, Epibulbar dermoids, 2344–2345 1490–1491, 1628 Epicanthus, 2341 Escherichia coli, 1358–1359 EPICure study, 17, 18, 399, 2316, 2319 E-selectin, 896 Epidermal growth factor, 512 Esophageal anastomosis, 1290 Epidermolysis bullosa, 2404–2405, 2420 Esophageal atresia, 1281–1293 Epidural analgesia, 387 associated anomalies, 1284–1285 Epidural anesthesia, 112, 387, 400 classification, 1283–1284 Epigenetic mechanisms, 42–45 clinical description, 1285 definition, 42 common types, 1283 developmental plasticity and disease risk, diagnosis, 1285 46–47 emergency ligation, 1287 DNA methylation, 43 genetic counseling, 1285 genomic imprinting, 45 non-treatment selection, 1287 histone modifications, 42–43 operative approach, 1287–1288 non-coding RNAs, 44–45 pathophysiology, 1284 reversibility, 47 postoperative management, 1290 Epilepsy, 2120, 2279 preoperative management, 1285–1286 medications during pregnancy, 679 risk categorization and prognosis, 1286 neonatal seizures, 2291 thoracoscopic approach, 1288–1290 neuroimaging studies, 2098 Esophageal complications and outcomes, 1290–1292 pyridoxine-dependent epilepsy, 2227 Esophageal replacement, 1292 See also Seizures ESPGHAN 2014 guidelines, 1369, 1371 2480 Index

Essential fatty acid (EFA), 609, 611, 616, 1216, 1233 neonatal intensive care units, 205–207 deficiency, 1233 nutritional support, 209 See also Fat re-hospitalization, 209 Estimated fetal weight safe transportation, 209 preterm fetus, 10–11 timing of home discharge, 208 fetal growth restriction, 133–134 ventilatory support, 209 Ethical issues and problems, 185 Extremely preterm (EP) infant, 185 attitudes toward extremely preterm and critically sick attitudes towards, 190–191 neonate, 190–191 critically sick neonates, 190 best interests of the infant, 188–190 decision-making in EP Infants, 194–195 laws and guidelines, 187–188 informing and counselling parents, 192–194 limits of viability, 187–188 laws and guidelines, 187–188 Euthanasia, 225 malformations, 198 in Netherlands, 198 neonatal morbidities and long-term outcome, 186 Evaporative heat loss, 329–330 outcome prediction, 187 Eventration, diaphragmatic, 748 pain and stress relieving drugs, 197 Evidence based medicine (EBM), 2430–2432 quality of life, 188–190 Evidence-based practice, 318 resuscitation after birth, 196 Evoked potentials (EPs), 2178 rights of newborn and parents, 191 Exchange transfusion, 1738 severe asphyxia, 198 Excipient, 703 survival, 185–186 Excitotoxicity, 81, 1626, 2013, 2020, 2146, 2148, withdrawing of intensive care, 196–197 2189–2191 withholding proactive antenatal care, 196 Exercise capacity, 901 withholding vs. withdrawing of intensive care, 195–196 Exhaled nitric oxide (NO), 897 Extrinsic risk factors, sepsis, 1733 Exit procedure, 741 Extubation Exomphalos, 60–61, 748 accidental extubation, 862 Exothermic mattresses, 340–341 and NCPAP Exposed seropositive mother, 1685 and surfactant, 434, 815 External genitalia, 1896 weaning the infant from ventilation, 861, 984 Extracorporeal membrane oxygenation (ECMO), 178, Eye(s), 463 659, 795, 797, 942, 1002, 1003, 1007–1012, damage, 1198 1481, 1776, 1779, 1782, 2217–2218 delayed visual maturation, 246 changes in requirements with time, 1012 dislocated lens, 1810 circuit, 1009 examination at birth, 21, 459, 463 cost-effectiveness, 1012 ocular malformations, 2339–2348 eligibility for, 1009–1010 albinism, 2415 management during, 1009 aniridia, 2346–2347 morbidity, 1011 congenital anomalies of the iris and pupil, mortality, 1010–1011 2346–2348 outcome, 1011 congenital cataract, 463, 2345 survival rates and use of, 1008 corneal malformations, 2343–2345 techniques, 1008–1012 crystalline lens anomalies, 2345–2346 veno-arterial ECMO, 1009 eyelid malformations, 2340–2342 veno-venous ECMO, 1009 malformative glaucoma, 2346 Extracranial hemorrhage (ECH), 1442 malformative glaucoma: , 2343 Extramedullary hematopoiesis, 1236 malformative glaucoma: microcornea, 2343–2344 Extrapleural approach, 1287 malformative glaucoma: naso-lacrimal-duct Extrapulmonary shunt, 770–771 malformation, 2343 Extreme hyperbilirubinemia, 1156, 1158, 1161 malformative glaucoma: ocular bulb Extreme jaundice, 1162 malformations, 2329, 2342–2343 Extremely low birth weight (ELBW) infant, 22, 177, 349 malformative glaucoma: Peter’s anomaly, 2344 admission to the NICU, 205–207 malformative glaucoma: retinopathy of approach to the care in delivery room, 204 prematurity, 162, 2349–2378 basic approach, 204–207 malformative glaucoma: visual evaluation, 246–247 cardiovascular problems, 206 and neonatal states classification scale, 22 first hours of life, 205 Eyelid malformations information and psychosocial intervention, 203–210 anomalies of rima (Palpebral Fissure), 2341–2342 long-term morbidities, 207 congenital anomalies, iris and pupil, 2346–2348 Index 2481

corneal malformations, 2343–2345 Ferritin, 722, 724, 1217 crystalline lens anomalies, 2345–2346 Fetal developmental anomalies, 2340–2341 breathing movements, 100, 111, 739 naso-lacrimal-duct malformation, 2343 development, 4, 11, 17, 738 ocular bulb malformations, 2342–2343 distress, 105–125 position anomalies, 2342 antepartum testing, 111–121 biophysical profile, 111–112 cardiotocography (see Electronic fetal monitoring) F causes of reduced fetal oxygenation, 107 Face mask, 979 clinical diagnosis, 109–111 neonatal resuscitation supplies and equipment, 397 Doppler examination, 115–121 ventilation, 429 identification, 111 Facial clefts, 1266 during labor surveillance, 121–125 and LPS growth cleft lip cleft palate, 1267–1270 restriction, 133–134 congenital mandibular diseases, 1270–1271 heart rate monitoring, 412 Facilitated tucking, 375 hydrops, 1515–1521 Factor, F antenatal evaluation, 1519 F. II, 1444 causes, 1518 F. VII, 1444 treatment, 1519 F. VIII, 1466 lung liquid, 177, 833–834 F. XI, 1444 medicine, 279–289 Factor, F. V Leiden, 35, 1466 surgery Failure mode and effect analysis tools (FMEA), 311 hydrops, 1515–1521 Fallot’s tetralogy, 55, 57, 748, 1043, 1048, 1051–1052, intrathoracic lesions, 741 1072–1073 neural tube defects, 2128–2130 Familial hemophagocytic lymphohistiocytosis Fetal breathing, 739 (FHLH), 1218 Fetal circulation, 1038 Familial hypocalciuric hypercalcemia, 1861 Fetal distress Familial isolated hypoparathyroidism, 1858 antepartum testing (see Antepartum testing) Fanconi anemia, 1476, 1507, 1878 definition, 106 Fanconi–Bickel syndrome, 1878 diagnosis, 106, 109–111 Fasciitis, 1749 labor surveillance, 121–125 Fat oxygenation, 106–109 body composition of infants, 622 Fetal electrocardiography, 124–125 breast milk Fetal fibronectin (fFN), 32 catch-up growth, insulin resistance and visceral Fetal gasping, 792 obesity in preterm infants, 625–627 Fetal growth digestion and absorption, 515–517 abnormal, 149–151 fat soluble vitamins, 520–521 biometric measurement, 151 fluids and electrolytes, 610 charts, 150–151 parenteral intakes for ELBW and VLBW infants, 606 defects, 149 thermal environment, 177, 338 normal, 149–151 FATP4, 517 pattern of, 154 Fat soluble vitamins, 520 restriction Fatty acid oxidation disorders, 1808, 1810, 1816, consequences, 134 1821–1822, 1829 definition, 133–134 Fatty acid protein transporter, 517 diagnosis, 134 Favism, 1162 epidemiology, 134 Feeding, 206 etiology, 134 continuous feeding, 598 Fetal hydrops, 1156, 1697 difficulties, 178 Fetal hypoxia, 2174 late preterm infants, 178 Fetal infection, 1683, 1696 enteral feeding of the VLBW infant, 595–602 Fetal inflammatory response syndrome See also Breastfeeding; Milk (FIRS), 78–79, 98, 522 FEF25-75%, 901 Fetal iron metabolism disorders, 721 Feminizing genitoplasty (FG), 1912 Fetal lung maturity, 1017 Fentanyl, 387, 390 Fetal oxygenation, 721 Fenton–Haber Weiss reaction, 441 Fetal programming hypothesis, 2297 2482 Index

Fetal renal pelvis dilatation, 1973–1974 Fluid Fetal saccular period, 875 deficits, 386 Fetal scalp blood sampling (FBS), 123, 124, 412 loss, 386 Fetal thyroid hormone production, 1864 management, 1370 Fetal to maternal hemorrhage (FMH), 1500, 1502 therapy Fetal weight, 153 in acute renal failure, 1945 gain, 589–590 in BPD, 902 Fetomaternal hemorrhage, 1502 in chronic renal failure, 1951–1952 Feto-neonatal in dehydration, 1365–1371 adaptive, 1576–1589 in ELBW infant, 205 innate, 1576–1582 intraoperative, 386 Fetoplacental circulation, 115 in neonatal hydrops, 1520 Fetoplacental hemorrhage, 1503 recommendations for parenteral, 607–608, 610 Fetoscopic endoluminal tracheal occlusion (FETO) Fluoxetine, 940 therapy, 956 Focal bacterial infections, see Bacterial infections Fetus, 1921 Focal brain lesions, 2253 amniotic fluid volume, 112 Fogarty catheter, 1287 oligohydramnios, 749, 1942 Folate, 521 polyhydramnios, 512, 919 Folic acid aneuploidy, screening tests for, 8–10 minimum content in feeding for preterm, 592 causes of hypoxia, 11 and neural tube defect, 2128–2130 CNS, 4 Follicle-stimulating hormone (FSH), see Hormones cocaine effects on, 7, 31 Follow-up diagnosis of well-being, 8–11 after discharge, 287–289 fetal circulation, 5 early markers of poor outcome, 237–248 fetal growth regulation, 6–8 cerebral palsy, 238–239, 241–243 fetal metabolism, 5 hearing evaluation, 247 fetal response to injury, 12–19 mental retardation, 239–240 growth, 6–8 neuroimaging predictors, 243–246 injuries, 11–12 traditional neurological examination, 240–241 causes of hypoxia, 11–12 visual evaluation, 246–247 invasive tests, 10–11 outcomes of high risk infants multiple pregnancy, 129–145 neuroimaging predictors, 243–246 postnatal development examination, 462 hemodynamic aspects, 13–14 Food supplements, 527 preterm fetus, 10–11 Forced expiratory flow, 901 timing of delivery and management, Foregut, 505 17–19 Formula fed nutrition, 536 response to injuries, 12–19 Formula-feeding ultrasound, 8 development, 630–631 virilization, 1893–1914 growth, 629–630 FEV1, 901 Formula milk, see Milk Fever, 1200 Formulations, 698 Fiber-optic pad, 1196 Fortified breast milk, see Milk Fibrinogen, 1473 Foxe1, 1864 Fibrinolysis, 1458 Framing bias, 223 Fibroblast growth factor-2 (FGF-2), 896 Franceschetti syndrome, 66, 1276 Fibroblastic tumors, 1653 See also Treacher–Collins syndrome Fibronectin, 894 , 1922 Fibrosarcoma, 1651 Frasier syndrome, 1877 First-line antituberculosis drugs, 1709 Fredet–Ramstedt pyloromyotomy, 1302 Fistulae, 925 Free bilirubin, 1148 Floppy infant, 2078 Free radicals, 441, 2148 Flow cytometry, 1482 in cerebral cortex of newborn piglets, 2148 Flow in superior vena cava, 2205 generation under hypoxic conditions, 2148 Flow velocity, fetus, 1157 inhaled nitric oxide and neuroprotection, Flucloxacillin, 1736 2149–2150 Fluconazole, 1753 nitric oxide, 2149 prophylaxis, 1759–1761, 1781 Frontal , 1257–1259 Index 2483

Fructose intolerance, 1825 E.coli, 1358 , 752 epidemiology, 1356 Fukuyama congenital muscular dystrophy (FCMD), 2329 etiology, 1357–1360 See also Muscle disease intractable diarrhea, 1363 Full term parasites, 1359–1360 and cutis marmorata, 2396 preventive measures and control, 1362–1363 definition, 11, 158 rotavirus, 1357–1358 general observation and measurements, 460 Salmonella spp, 1359 and heat exchange, 328 Shigella spp, 1359 and hyperbilirubinemia, 478 therapeutic approach, 1362 and hyperglicemia, 1853–1855 transmission, 1360 infants, 545, 546, 548 Gastroesophageal reflux, 178, 512, 898, 1292 and ponderal index, 157 Gastrografin enema, 1311 serum calcium concentration, 655 Gastrointestinal serum immunoglobulin levels, 1589 function and hormones, 535–549 and subcutaneous fat necrosis, 2397–2398 anatomic and functional maturation, 507 Functional residual capacity cholecistokinin, 548–549 (FRC), 760, 868, 974, 976, 983 digestion and absorbtion, 515–521 Functioning, 247 ghrelin, 543–546 Fungal infections, 1750–1754 glucagone-like peptide 1, 537–538 clinical manifestations, 1752 glucagone-like peptide 2, 538–540 clinical pictures, 1751 glucose-dependent insulinotropic peptide, 541–542 diagnosis, 1752 host defense, 521–528 duration of antifungal treatment, 1753 motilin, 547–548 frequency, 1751 neural control of motor function, 509–515 mortality and morbidity, 1752 obestatin, 546–547 risk factors, 1751 organogenesis and function, 505–507 sources, 1751 oxyntomoduline, 540–541 treatment, 1753 peptide YY, 542–543 Fungal sepsis, 1478 physiology, 503–528 Funisitis, 98, 1632 vascularization, 507–508 Furosemide, 1946 malformations hypertension, 1122 abdominal wall defects, 1296–1301 increased risk of PDA, 1082 duodenal obstruction, 1302–1305 in renal failure, 1946 duplications, 1314–1317 intestinal malrotation, 1317–1321 intestinal obstruction, 1305–1311 G persistence of the omphalo-mesenteric duct, Galactose malabsorption, 1811 1311–1314 Galactosemia, 1220, 1809–1811, 1820, 1825 pylorus anomalies, 1301–1302 congenital cataract, 2345 motility, 513 screening, 473 rare surgical emergencies, 1332 Galactosialidosis Gastrointestinal-associated lymphoid tissue eye finding, 1810 (GALT), 1376 skin-hair finding, 1809 Gastroschisis, 1298–1301 Gamma-aminobutyric acid (GABA), 11, 784 Gaucher disease, 1210, 1813, 1816 Gamma glutamyl transpeptidase (GGT), 1227, 1236, 1237 Gender effect, quality in neonatal care, 258 Ganciclovir, 1670 Gene Gas monitoring, 476 expression, 42–47 Gastric therapy acid, 517 chronic granulomatous disease, 1534–1535 distension, 986 epidermolysis bullosa, 2404–2405 emptying, 512–513 severe combined immunodeficiencies, 1612–1614 lipases, 515–516 unconjugated hyperbilirubinemia, 1179 Gastroenteritis, 1355–1363 Generalized body hypothermia, 2159–2160 Aeromonas hydrophila, 1359 General movements (GMs), 241, 2066–2067 Campylobacter species, 1359 Genetic(s), 906 clinical aspects, 1360–1361 congenital malformations and syndromes, 49–70 diagnosis, 1361–1362 counselling, 52 (see also Counselling) 2484 Index

Genetic(s) (cont.) Gill arch syndrome, 1272–1276 developmental plasticity, 46–47 Gingivostomatitis, 1673 DNA methylation, 43–44 Glanzmann’s thrombasthenia, 1480 epigenetic mechanism, 42–45 Glaucoma congenital, 463, 2346 genomic imprinting, 45 Glial fibrillary acidic protein (GFAP), 418–419, 2306 histone modifications, 42–43 Glioma, 1654 regulatory RNAs, 44–45 Glomerular filtration rate (GFR), 647, 1937, 1980–1981 Genetic cholestasis, 1211–1214 Glomerulotubular balance, 648 management, 1214–1215 Glossoptosis, 1271 Genetic predisposition, 889 Glucagon, 403 Genetic susceptibility, 88 Glucagon-like-peptide 1 (GLP-1), 537–538 Genitalia examination, 465 Glucagon-like-peptide 2 (GLP-2), 538–540 See also Sexual development disorders Glucocorticoids, 1628 Genodermatosis, 2406, 2420 in hypercalcemia, 661 -wide association analyses (GWAS), 827 Glucose Genomic imprinting, 45, 52, 58 carbohydrate digestion and absorption, 518–519 Genomic testing, 354 disorders of glucose metabolism, 720–721 Genotypes, 1689, 1692 homeostasis, 543, 608–609, 652 Gentamicin, 1736 impaired glucose homeostasis (see Hyperglycemia; Germ cell tumor, 1643, 1910 Hypoglycemia) Germinal matrix haemorrhage-intraventricular intake for VLBW infant, 615 hemorrhage (GMH-IVH), 478, monitoring of glucose, 403–404, 478 2010–2011, 2104 in parenteral nutrition, 592–593, 607, 611 development of, 2206 risk of maternal glucose intolerance, 719–720 diagnosis of, 2207–2210 Glucose-dependent insulinotropic polypeptide (GIP), etiology of, 2204 541–542 ibuprofen, 2215 Glucose metabolism disorders, 720 incidence of, 2203, 2204, 2206 Glucose-6-phosphate dehydrogenase (G6PD) deficiency, indomethacin, 2214 1161–1163 mechanical disruption, 2204 function of, 1161 neuropathology and pathogenesis, 2203–2204 genetics of, 1161 outcomes, 2212–2213 G6PD-associated kernicterus, 1163 pathogenesis of, 2204 and hemolysis, 1161–1162 phenobarbital, 2214 testing for, 1162–1163 prevention of, 2213 UGT1A1*28, 1162 risk of, 2204–2206, 2214 Glucose-6-phosphate-transporter (G6PT) protein, severity of, 2215 1556 vitamin K, 2214 Glucose transporter type 1 (GLUT1) deficiency Germinal tumors (GT), 1648–1650 syndrome, 1823 Gestational Glutamate, 510, 2190, 2194 age, 110, 149, 185 enteral nervous system, 510–511 accurate assessment of, 149 oxygen toxicity, 446, 2189 assessment of, 110–111, 158, 467–468 receptor, 2005 and birthweight guidelines, 351 Glutamine, 527, 1814 chronological criterion of, 350 Glutaric aciduria neurological criteria, 468 type I, 473 age at birth type II, 473 vs. IQ, 232 GLUT family of facilitative glucose transporters, 518 special educational needs, 231 Glycine, 1814 diseases Glycoconjugates, 522 transepidermal water loss, 329 Glycogen storage disease, 1226, 1813 Gestational alloimmune liver disease (GALD), 1218 hydrops causes, 1518 Gestational risk factors, 1459 hypercalcemia, 1860 GFI1 , 1554 with lactic acidosis, 1820 Ghrelin, 543–546 with neutropenia, 1557, 1564 Giant lacunar infarction, 2266 Glycogen storage disease type Ib, 1557 Giant , 1298 Glycol Giant platelets, 1481 polyethylene, 1215 Gilbert’s syndrome, 1147, 1162, 1181 propylene, 2395 Index 2485

Glycoprotein IIb/IIIa (GPIIb–IIIa) complex, intrapartum asphyxia, 411–412 1472, 1473 methodological criteria, 317–318 Glycosylation disorders, 2238 pain management, 390–391 GM1 gangliosidosis, 1816 parenteral intakes for ELBW and VLBW infants, 607 GMH-IVH, see Germinal matrix haemorrhage- postnatal care, 458 intraventricular hemorrhage (GMH-IVH) protocols and checklist, 312 Goat milk formula, 581 resuscitation of the newly born, 425–426 Goblet cells, 506 Gut Goldberg–Shprintzen syndrome, 1347, 2117 and bilirubin metabolism, 1144–1145 Goldenhar syndrome, 61, 62, 1276, 2344 development, 505–506 epibulbar dermoids, 2344–2345 digestion and absorption, 515–521 Gonadal development disorders, 1904–1905 hormones and gastrointestinal function, 535–549 Klinefelter syndrome, 1898 in host defense, 521–528 ovarian development, 1899 hypoxia, 597 testicular development, 1900, 1904 maturation, 538, 546, 548 XY females, 1904 micronutrients and vitamins, 673 Gonadoblastoma, 1649 motility, 509 Gonads, 1895 peptides, 537–549 Gonococcal ophthalmia, 404 and pneumoperitoneum, 883–884 Gonorrhoea, 404 vascularization, 507–508 G6PC3 mutations, 1557 Gut-associated lymphoid tissues (GALT), 525 G6PD deficiency, 1187 Gynaecology GRADE approach, 317, 319 hymen abnormalities, 465 Graft-versus-host disease, 1202, 1612, 2410 neonatal breast development, 464 Gram-negative bacteria, 1730–1731 Gram-positive bacteria, 1729–1730 Granulocyte colony-stimulating factor (G-CSF), H 1540–1542, 1628, 1636, 1738 Haemolysins, 2215 Granulocyte-macrophage colony-stimulating Haemophilus influenzae factor, 1524, 1542–1543, neonatal sepsis, 1736 1585, 1738 otitis, 1748 Granulocyte transfusion, 1540 vaccine (see Vaccines) Granuloma gluteale infantum, 2422 Haemophilus type b (Hib) vaccine, 1798 Griffiths scales, 245 Haldane effect, 76, 768 Gross motor function classification system for cerebral Hammersmith Neurological Examination palsy (GMFCS), 239 (HINE), 240 Group A Streptococcus, 1748 Hands Group B Streptococcus (GBS), 869, 1730, 1774 in achondroplasia, 67–68 antepartum GBS prophylaxis, 1782 in Apert syndrome, 65 EOD, 1781 in arachnodactyly, 56, 59 flow chart, 1736 in cartilage-hair hypoplasia, 1564 implementation of, 1774 in ectrodactyly, 67 incidence of, 1774 in Edwards syndrome, 56 Growth, 892 in epidermolysis bullosa, 2404–2405 Growth hormone (GH), 1852 in lobster claw, 67 See also Hormones in , 67 Growth hormone releasing hormone (GHRH), in Pfeiffer syndrome, 65–66 see Hormones in physical examination, 458–459 Growth Restriction Intervention Trial (GRIT), in , 67 17, 121 in Prader–Willi syndrome, 60 Guidelines (GL), 315–320 as route of transmission, 1733–1734 care, 187–188 in rubor and acrocyanosis, 2395–2396 clinical governance, 286–287 in Turners’ syndrome, 57 definition, 316 in zinc deficiency, 671 discharge late preterm, 181 Hard tissue abnormality, 1257 formulation of, 318–319 Harlequin ichthyosis, 2411 ILCOR (International Liaison Committee on Hashimoto’s thyroiditis, 1874 Resuscitation), 196, 397, 425, 429 HAX1gene mutation, 1556 intraoperative fluid therapy, 386 HBGB1, 1626 2486 Index

H2 blockers, 1378 Hemangioma, 2418–2419 Head circumference, 157, 158, 462–463 capillary, 460 Hearing, 239, 463 liver, 1656 defects, 1683, 1684 Hematocrit, 1128, 1129, 1133, 1136, 1200, 1408–1410 deficit, 1669 polycythemia, 724, 1125–1137 evaluation, 247 Hematology, 1405–1429 impairment, 239 anemia (see Anemia) loss, 247 neonatal thrombocytopenia, 1475–1480 See also Deafness platelets Heart disorders, 1471–1484 auscultation, 464–465 Hematoma congenital heart disease, 1037–1063, cord, 1503 1065–1076 See also Hemorrhage electrocardiography, 124–125 Hematopoietic stem cell transplantation (HSCT), 1533, failure, 1043 1535, 1612, 1615–1617 failure, in Newborn, 1043 Hematuria, 1943 fetal heart rate, 121–123 coagulation disorder, 1475 rate, 428 hypertension, 1119 Heart block, 1030, 1044 Heme oxygenase, 1146 first-degree, 1107 Heme oxygenase-1 (HO-1), 1154, 1164 second-degree, 1107 Heme-oxygenase inhibitors, 1147 third-degree, 1102, 1107, 1108 Hemifacial microsomias, 1276 Heat balance, 325 Hemihypertrophy, 60 Heated beds, 328 Hemiplegia, 242 Heat exchange Hemisyndrome, 2253 heated beds, 328 Hemivertebrae, 61, 67 incubators, 328 Hemochromatosis, 1217 infant body surface and environment Hemodialysis, 1949 through conduction, 327 Hemodilution, 1130, 1135 through convection, 326, 327 Hemodynamic responses, 368 through evaporation, 326 Hemofiltration, 1781 through radiation, 326 Hemoglobin (Hb), 402–403, 484, 486, 487, water loss from skin, 326 1407–1408 infant respiratory tract and environment, 327–328 catabolism, 1144 phototherapy effect, 337 concentration, 1407–1408 respiratory water loss, 336, 337 corpuscular, 1412 infant skin and environment, 329 fetal, 5, 106, 767 ambient humidities, 331 reference range, 1408, 1409 care on heated bed, 334–335 Hemoglobin distribution width (HDW), 1414 first day after birth, 329–331 Hemoglobin-oxygen (Hb-O2), 486 first hours after birth, 329 Hemolysis, 1196 during first weeks after birth, 331–333 Hemolytic phototherapy, 334 anemia radiant heaters, 333–334 ABO antibodies, 1159 skin barrier maturation rate, 334 hereditary elliptocytosis, 1164 skin-to-skin care, 335–336 hereditary pyropoikilocytosis, 1164 thin clothing effect, 336 hereditary spherocytosis, 1163–1164, 1413 transepidermal water loss, 329 pyruvate kinase deficiency, 1163 radiant heaters, 328 red cell enzymopathies glucose-6-phosphate respiratory water dehydrogenase deficiency, 1161–1163 before and after intubation, 337 diseases, 1498–1500 evaporative heat loss with gestational age, ABO immune disease, 1159–1160 337–338 antibodies, RhD, 1160–1161 during mechanical ventilation, 338 G6PD deficiency, 1161–1163 during phototherapy, 338 hereditary ovalocytosis, 1164 skin-to-skin contact, 342–343 hereditary pyropoikilocytosis, 1164 water loss from airway, 327 hereditary spherocytosis, 1163–1164 HELLP syndrome, 1815 hereditary stomatocytosis, 1164 Hemangioendothelioma, 1643 infection, 1164 Index 2487

pyruvate kinase deficiency, 1163 glycogene storage disease type Ib, 1557 Rh isoimmune hemolytic disease, 1156–1159 G6PC3 mutations, 1557 risk, 1154–1156 Hermansky–Pudlak syndrome 2, 1556 hyperbilirubinemia (see Hyperbilirubinemia) myelocathexis, WHIM syndrome, 1555 Hemophagocytic lymphohistiocytosis (HLH), 1218–1219 severe congenital neutropenia, 1551–1554 Hemorrhage Shwachman–Diamond syndrome, 1556 cause of, 1501 RBC membrane defects, 1163 cerebellar, 2106–2108 spherocytosis, 1163–1164 cerebral, 2201–2218 tyrosinemia, 1220 extracranial (see Hematoma) Hereditary fructose intolerance intracranial, 11, 2202–2203 (HFI), 1220 intrathoracic petechial, 1029 Hereditary tyrosinaemia type 1 (HT1), 1220 parenchymal (venous infarction), 2105 Hering–Breuer reflex, 983 perinatal, 1503–1504 Hermansky–Pudlak syndrome, 1556 periventricular (see Periventricular Hernia leukomalacia) diaphragmatic, 817–818, 950–956 postnatal, 1504–1505 inguinal, 465 prenatal, 1501 Herpes simplex virus infection pulmonary, 838, 866–867 clinical manifestations, 1672 See also Adrenal hemorrhage diagnosis, 1674 Hemorrhagic stroke, 2227 epidemiology, 1671 Hemostasis, 1473 in immunodepressed children, 1674 neonatal hemostasis, 1438 pathogenesis, 1671–1672 primary, 1432 prevention, 1675–1676 secondary, 1432 prognosis, 1675 tertiary, 1432 treatment, 1674–1675 Hemostatic defect, 1460 type 1 and 2 infection, neonatal, 1219 Hepadnavirus family, 1692 Heterotopia Heparinization protocol, 2277 Genetic Basis and Diagnosis, 2119–2120 Hepatic haemangioma (HH), 1656 Genetic Counselling, 2120 Hepatitis laboratory investigations, 2120 HBV, 1692–1695, 1798–1799 phenotype, 2120 HCV, 1689–1692 Heterozygotes, 1162 idiopathic neonatal, 1236 Heubner’s artery stroke, 2267 Hepatitis B immunoglobulin (HBIG), 1695 HIES, see Hyper IgE syndrome Hepatitis B vaccine, 1695, 1798 High frequency oscillation, 1012 Hepatitis B virus (HBV) ventilation, 2084 clinical aspects, 1693–1694 High frequency oscillatory ventilation, etiology and pathogenesis, 1692 205–206, 859–861, 901 prevention, 1694–1695 Highly active antiretroviral therapy (HAART), 1685, therapy and treatments, 1695, 1698 1686, 1688 transmission, 1692–1693 High-pitched cry, 1200 Hepatitis C virus (HCV) High resolution computed tomography (HRCT), 900 clinical aspects, 1691–1692 High voltage slow activity (HVS), 2084 diagnosis, 1692 HIGM, see Hyper IgM syndrome etiology and pathogenesis, 1689 Hindbrain arterial ischemic stroke, 2269 therapy and treatment, 1692 Hindgut, 505 transmission, 1689–1691 Hippocratic Oath, 184 Hepatobiliary scintigraphy, 1215, 1227–1229 , 466 Hepatoblastoma (HB), 1656 Hirschsprung’s disease, 509, 515, 1307, 1310, 1345–1351 Hepatocarcinoma (HCC), 1694 clinical aspects, 1347–1348 Hepatocyte growth factor (HGF), 896 diagnosis, 1348 Hepatomegaly, 1225 etiology and pathogenesis, 1346–1347 Hereditary disorders, 1480 prognosis, 1351 neutropenia, 1549–1557 treatment, 1348–1350 associated with glucose-6-phosphatase Histone modifications, 42–43 complex disorders, 1556–1557 Holder pasteurization, 574 associated with poikilodermia, 1557 Holoprosencephalic sequence, 53–54 cyclic neutropenia, 1554 Holoprosencephaly, 54, 2127–2128 2488 Index

Home care, ELBW, 207–215 secretion reflex, 562 Home discharge, 208 sharing, 570 Homocystinuria, 35 skin-to-skin contact (STS), 564–565 Horizontal/nosocomial routes of transmission, sudden unexpected postnatal collapse (SUPC), sepsis, 1733 prevention, 564–565 Hormones Human milk bank (HMB), 574 calcitonin, 652–653 Human milk oligosaccharides (HMOs), 580 chorionic gonadotropin (hCG), 6, 35 Human morality, 221 follicle-stimulating h. (FSH), 131 Human parvovirus B19 and gastrointestinal function, 535–549, 598 clinical aspects, 1696–1697 growth h. (GH), 60 diagnosis, 1697–1698 parathyroid h. (PTH), 639–667 disorders, 1697–1699 parathyroid hormone-related protein (PTHrP), 650 etiology and pathogenesis, 1695–1696 placental production, 35 prognosis, 1698 stress, 390 therapy and treatments, 1698–1699 Horner’s syndrome, 466 transmission, 1696 Horseshoe kidney, 1960 Humidification, 849, 989 Hospital readmission, 172, 177, 181, 208 Hyaline membrane disease, 824 Host defenses, 522 Hydralazine, 1122 Human cerebral cortex, 2114 Hydrocephalus, 1654, 2105 Human chorionic gonadotropin (hCG) Hydrocortisone, 904, 1635, 1912 levels, 35 Hydrolyzed formula, infant, 581 See also Hormones Hydronephrosis, 1960, 1967–1969, 1974 Human errors, 309 antenatal, 1976 Human immunodeficiency virus (HIV), 1684–1689 non obstructed, 1978 clinical manifestations, 1685 postnatal radiology, 1974–1975 diagnosis, 1685–1686 Hydrops, 1699 etiology and pathogenesis, 1684–1685 Hydrops fetalis (HF), 1156, 1157, 1515–1521 MTCT prevention, 1686–1687 aetiology, 1517 prognosis, 1688 antenatal evaluation, 1519 therapy and treatments, 1688–1689 diagnosis, 1517 transmission, 1685 epidemiology, 1516 transmission through maternal milk, 1688 pathogenesis, 1516 See also AIDS prognosis, 1521 Human milk, 361, 452, 516 treatment, 1517–1521 baby-friendly hospital initiative (BFHI), 562–564 Hydrothorax fetal, 748 bioactive substances, 560 Hydroxyl radical, 443 breastfeeding controversies (see Breastfeeding, Hyperammonemia, 1823 controversies) disorders presenting with, 1823 calibration phase, 562 disorders presenting without, 1823–1825 clinical approach, neonatal weight loss, 565 Hyperbilirubinemia, 179–180, 478, 725, 1143–1149 delayed lactation, 566 associated with breastfeeding, 1147 ejection reflex, 562 cholestasis, 1210–1214 feedback inhibitor of lactation (FIL), 562 metabolic disorders with, 1826 feeding recommendations, 561 in IDM, 725 formula, 557–582 insufficient nutrition, 1146 health risks, formula fed, 561 treatment, 1185–1203 infant formula (see Infant formula) unconjugated, 1146–1147, 1151–1164 International code of marketing, breast-milk See also Kernicterus substitutes, 564 Hypercalcemia, 659, 1859–1862 lactogenesis, 561–562 hyperparathyroidism, 659–661 LATCH charting system, 566 idiopathic infantile hypercalcemia, 1861 low breast milk intake causes, 568 subcutaneous fat necrosis, 1861, 2397–2398 low breast milk supply, 566–568 , 1861 neonatal intensive care unit (NICU), human milk Hypercaloric formula, 582 nutrition (see Neonatal intensive care unit Hypercapnia, 2356 (NICU), human milk nutrition) Hyperglycemia, 478, 1853–1855 neonatal weight loss, 565–566 causes, 1853–1854 nurtritional value, 558–561 clinical pesentation and complications, 1854 Index 2489

definition, 1853 in hepatoerythropoietic porphyria, 2409–2410 diagnosis, 1854 in neurofibromatosis, 2414 maternal, 53, 719–720 Hypertrophic pyloric stenosis (HPS), 1301–1302 neonatal diabetes mellitus, 72, 719, 1855–1857 Hyperventilation, 784 screening, 1854 pulmonary hypertension, 94 Hyper IgE syndrome, 2408 Hyperviscosity, 724, 1125–1137 Hyper IgM syndrome, 1610–1612 Hypoadrenalism, see Adrenal insufficiency Hyperinflation, 795 Hypoalbuminemia, 1192, 1516 Hyperinsulinism, 1821, 1848–1853 Hypocalcemia, 649, 659, 723–724, 1857–1859 Hyperkaliemia in IDM, 723 causes, 1846 Hypogalactia, 566, 568 definition, 1845 , 1610, 1614, 1779–1780 management, 1848 Hypoglycemia, 161, 478, 723, 1820–1821, 1848–1853 Hyperlipidemia, 1238 in IDM, 723 Hypernatremia, 1845–1848 Hypomagnesemia, 656, 657, 723–724 Hyperoxemia, 489–492 in IDM, 723 Hyperoxia, 80–81, 360, 447–449, 490–492, Hyponatremia, 1845–1848, 1946 890–891 causes, 1846 test, 1043, 1045 definition, 1845 Hyperparathyroidism, 659–662 investigation, 1847–1848 congenital parathyroid disorders, 1858–1859 management, 1848 maternal, 649 renin-angiotensin-aldosterone system, 1845–1846 Hyperphenylalaninemia, 51, 63 Hypoparathyroidism, 660, 1858, 1859 Hyperphosphatemia, 653 Hypoperfusion, in hypoxia, 772 acute renal failure, 1947 Hypophosphatasia, 1861 Hypophosphatemia, 1861 in Apert syndrome, 65 Hypophosphatemic rickets autosomal dominant in cri-du-chat syndrome, 56 (ADHR), 1883 in facial cleft, 1266 Hypophosphatemic rickets autosomal recessive in Jansen’s metaphyseal chondrodysplasia, 1861 (ARHR), 1884 in Noonan syndrome, 59–60 Hypophosphatemic rickets X-linked dominant (XLH), 1883 in Opitz syndrome, 1914 Hypopituitarism, 1835–1837 in Pfeiffer syndrome, 65–66 biochemical/hormonal measurements, 1836–1837 in Wolf–Hirschhorn syndrome, 56 clinical presentation, 1836 Hypertension, 1111–1122 etiology, 1835 causes, 1119–1120 genetic analysis, 1837 clinical features, 1119 imaging, 1837 diagnostic approach, 1120–1121 management, 1837 intracranial, 65, 1257 Hypoplastic kidney, 1958–1959, 1961, 1964 maternal, 34, 1528–1529 Hypoplastic left heart syndrome, 938, 1057–1058, 1066, outcomes, 1121 1067, 1069 pre-eclampsia, 33 Hypoproliferative anemias, 1506–1508 pulmonary, 488 Hypospadias, 1913–1914 treatment, 1121 Hypotension, 1111–1122 Hyperthermia, 400, 475, 1788 causes, 1115 Hyperthyroidism clinical features, 1115 fetus, 1872–1873 definition, 1113 neonate, 1871–1874 incidence, 1115 clinical signs, 1872 outcomes, 1118 diagnosis, 1873 treatment, 1116–1118 etiology, 1872 Hypothalamus, 1836 Graves’ disease, 1872–1874 Hypothermia, 161, 181, 400, 404, 426, 475, 478, mutations of TSH receptor, 1874–1875 1627–1628, 1788, 2157, 2193 treatment, 1873–1874 as medical treatment, 436, 2193–2194 sporadic congenital non-autoimmune, 1874 therapy, 436 Hypertonia, 1200 Hypothyroidism, 671 Hypertrichosis, 2397 neonatal screening, 473 in Cornelia de Lange syndrome, 58 See also Congenital hypothyroidism in erythropoietic protoporphyria, 2408 Hypotonia, 467, 2324 2490 Index

Hypoventilation, 769–770 transient congenital hypothyroidism, 1867 Hypovolemia, 433, 1116 twinning, 131 Hypoxanthine, 2176 event, 309–311 Hypoxemia, 11–13, 115, 119, 489, 721, 934, 938 hypercalcemia, 1860 Hypoxemic respiratory failure (HRF), 951 twinning, 131 Hypoxia, 11–12, 410–414, 417, 443, 487, 2296–2298 Ibuprofen abnormal diffusion, 770 prophylaxis, 1084 acid–base balance, 108–109 risks of treatment, 1085–1086 alterations in ventilation/perfusion ratio, 771–772 treatment, 1084–1085 anemia and hypoperfusion, 772 ICA stroke, 2265 and apnea, 781, 1021–1032 I-cell disease, see Lysosomal storage disease asphyxia syndrome, 160, 410–411, 1479 Ichthyosis, 1810, 2411–2412 cause of, 11–12, 769–772 Idiopathic characterization, 107 infantile hypercalcemia, 1861 extrapulmonary shunt, 770–771 neonatal hepatitis, 1236 fetal oxygenation, 107–109, 721 I-DSD Registry project, 1898 fetal response, 12–19 Iinducible protein 10 (IP-10), 892 focal cerebral infarction, 2013–2014 IL-6, 1626 hemodynamic consequences, 109 IL-10, 1626 hypoventilation, 769–770 IL-18, 1624 hypoxic-ischemic encephalopathy, 2143–2160 Ileal tubular duplication, 1317 markers of tissue injury, 415–419 IL-1 receptor antagonist (IL-1RA), 896 neuroimaging, 2176–2179 IMAGe syndrome, 1878 parasagittal injury, 241 Imaging patent ductus arteriosus, 1080 neuroimaging (see Neuroimaging) perinatal hypoxia, 351–353 positron emission tomography (PET), 268 respiratory distress syndrome, 997 Immature kidney, 1926 treatment of hypoxic-ischemic syndrome, Immature platelet fraction (IPF), 1427 2169–2173 Immature reticulocyte fraction (IRF), 1416 Hypoxic-ischemic encephalopathy (HIE), 240, Immune 1449–1450, 2098 modulation, 522 asphyxial marker, 415 molecules, 2021 blood lactate concentrations, 414–415 paralysis, 1780 encephalopathy, 415–416 prophylaxis, 1762 multiorgan failure (MOF), 416 system, 522 urinary lactate/creatinine ratio, 416–417 adaptive immune system, 1583–1595 Hypoxic-ischemic (HI) injury, 2023 B-, 1587–1589 Hypoxic-ischemic syndrome innate immunity, 1576–1582 clinical picture, 2167–2169 monocytes and macrophages, 1581–1582 diagnosis, 2173–2179 mucosal immune system, 1591–1595 epidemiology, 2166 natural killer cells, 1590–1591 pathology and outcome, 2166–2167 , 1562–1563, 1576–1581 treatment, 2169–2173 (see also Hypoxic-ischemic T-lymphocytes, 1583–1587 encephalopathy) thrombocytopenias, 1476–1478 tolerance, 522 Immunodeficiency I complex disease, 1615–1617 Iatrogenesis autoimmune polyendocrinopathy-candidiasis incidence of, 306 ectodermal dystrophy syndrome risk management (see Risk management) (APECED), 1616 Iatrogenic cartilage hair hypoplasia (CHH), 1615 disorders DiGeorge syndrome, 57, 1615 blood loss, 1501 immunodysregulation, polyendocrinopathy, delivery, 18 enteropathy, X-linked (IPEX), 1616 epidemiology, 306–309 Nijmegen breakage syndrome (NBS), 1615–1616 hypercalcemia, 659 SCID neonatal screening test, 1617 hyperglycemia, 1853 defects of B-cell, 1612 hyponatremia, 1846 defects of T-cell, 1612–1614 prematurity, 33 HIV (see Human immunodeficiency virus) Index 2491

hyper IgM syndrome, 1610–1612 with prenatal onset, 1808–1813 late-onset neonatal skin and hair findings, 1809 neutropenia associated with poikilodermia, small molecule diseases, 1809, 1812 Clericuzio type, 1557 transient hyperammonemia, 1820 Shwachman–Diamond syndrome, 1556 treatment, 1828–1829 neutropenia, 804, 806–807, 935, 951, 1549– urea-cycle disorders, 1217 1557, 1562 Incontinentia pigmenti, 2235, 2276, 2403, 2406–2407, autoimmune neonatal, 1535–1536 2414, 2422 Chediak–Higashi syndrome, 1528, 1534, 1536 Incubators, 328 chronic granulomatous disease, 1534–1535 Indomethacin, 1084–1086 isoimmune neonatal, 1529–1530 Induced bradicardia, 794 short-limbed dwarfism, 1564, 1861 Inducible protein 9 (IP-9), 892 Wiskott–Aldrich syndrome, 1476, 1614 Ineffective erythropoiesis, 1153 Immunodysregulation, polyendocrinopathy, enteropathy, Infant AVR prophylaxis, 1686, 1687 X-linked (IPEX), 1616 Infant formula Immunoglobulin G (IgG), 1635 compositional requirements, 580 in colostrum, 559 contamination, 579 deficiencies, 524, 526 energy and nutrients composition, special formulas, hyper IgE syndrome, 2408 578–579 IgM, 1159 medical reasons for substitute, 578 hyper IgM syndrome, 1610–1612 nutritional characteristics, 579–580 intravenous, 1202, 1478, 1540–1541 special formulas, 580–582 secretory, 1593–1594 Infantile serum levels, 1589 hypertrophic pyloric stenosis, 509 Immunoglobulin serum levels, 1608–1609 polycystic disease (see Polycystic kidney disease) Immunohistochemical techniques, 352 Refsum disease, 1813 Immunological development, 1794–1795 Infants of diabetic mothers (IDM), 53 Immunoprophylaxis, 1694, 1695 glucose intolerance (see Maternal glucose intolerance) Immunosorbent agglutination assay (ISAGA), 1716 metabolic syndrome, 726 Impaired alveolarization, 891 neurodevelopment, 726–727 Impaired elimination, 1153, 1154 pathologic conditions, 719 Imperforate anus, 1972 Pedersen hypothesis and diabetic fetopathy, 718, 719 Impetigo bullosa, 1730, 1749, 2402 Infected seropositive mother, 1685 Inborn errors, bile acid synthesis, 1212 Infections, 889, 1164 Inborn errors of metabolism, 1805–1829 bacterial (see Bacterial infections) with cardiac presentation, 1808 bone and joints, 1745–1746 clinical and biochemical findings, 1807, 1811 fetal, 1663–1678 clinical presentations, 1807–1808 fungal, 1750–1754 disorders of glycosylation, 1813 intrauterine, 31 fructose 1,6-bisphosphatase deficiency, 1820 otitis media, 1748 glycogen storage disease, 1557, 1564, 1808, 1813 postnatal infections, 891 disorders of subcellular organelles prenatal infections, 889–892 fatty acid oxidation defects, 1821, 1829 septic shock, 1775 lysosomal storage diseases, 1811–1813 toxoplasmosis, 1711–1723 etiology, 1807 urinary tract (UTI), 1743–1745 eye findings, 1810 viral (see Viral infection) hematological findings, 1809 See also Neonatal, infections investigations, 1826–1828 Infectious pneumonia, 899 large molecule diseases, 1812–1813 Inferior mesenteric artery, 507 with liver involvement, 1809, 1825 Infiltrates, 898 maternal metabolic disease, 1813–1815 Inflammation, 75, 77, 79, 81, 83, 85, 87, 88, 891, 2020, medications, 1829 2188, 2190, 2194 with non-immune hydrops presentation, 1816 CNS vulnerability, 2026 organic acid disorders, 473 preterm delivery, 2022 isovaleric acidemia, 473, 1818 systemic, 2020 methylmalonic acidemia, 473, 1808, 1817 term brain injury, 2023 propionic acidemia, 473, 1808, 1818 Inflammatory pathogenesis, 1807 anti-inflammatory agents, 2188–2189 with postnatal onset, 1826 biomarkers, 2305 2492 Index

Inflammatory (cont.) Intermittent positive pressure ventilation, 856–857, 898, inflammation in brain injury, 2023–2026 905, 1024 mechanism, 73–89 Internal cerebral vein, 2271 mediators, 1621 International Liaison Committee on Resuscitation pro-and anti-inflammatory cytokines, 83 (ILCOR), 424, 425, 428, 430, 431, 434, 435 response, 524 International Neuroblastoma Staging System (INSS), 1647 Influenza, 869, 903, 1586, 1799 Interphase, 832 vaccination, 1796 Interrupted aortic arch (IAA), 1055, 1076 vaccine, 1799 Interstitial cells of Cajal (ICC), 509 Informed consent, 353–354, 1137 Interstitial lung disease, 899 Inguinal hernia, 465, 1972 Intertertiary transfer, 294 Inhaled nitric oxide (NO) therapy, 900, 905 Intestinal PPHN, 942 gangrene, 1400 in premature newborn, 945–948 macrophages, 523–524, 1594–1595 Inheritance, 52–70 malrotation, 1317–1321 Inherited coagulation disorders mucus, 524 disorders of fibrinogen, 1443–1444 obstruction, 1333 FII, FV, FVII, FX, FXI, FXII and combined occlusion, 1333–1335 FV/FVIII deficiencies, 1444 perforation, 1333–1334 FXIII deficiency, 1444–1445 transplantation, 1385 hemophilias, 1441–1443 Intestinal T cells, 1592 hereditary combined VK deficiency factor Intracardiac thrombosis, 1461 disease, 1445 Intracerebellar haemorrhage, 2209–2210 PAI-1 deficiency, 1445 Intracerebral calcifications Von Willebrand disease, 1443 in Aicardi–Goutieres syndrome, 2122 Inherited metabolic diseases, 1805–1829 in GMH-IVH, 2206 Inherited metabolic disorders, 1220 in intracranial hemorrhage, 11, 2201–2218 Inhibitor of heme oxygenase, 1203 in subarachnoid hemorrhage, 2216 Innate immune responses, 2022 in subdural hemorrhage, 2216 Innate immune system, 522 in subgaleal hemorrhage, 2216–2217 Innate immunity, 1622–1626 Intracranial Inositol, 803, 904 hypertension, 65 prophylaxis of bronchopulmonary dysplasia, 904 pressure, 977 iNO therapy, 298 Intracranial hemorrhage (ICH), 478, 1442 Insulin, see Hyperinsulinism Intractable diarrhea, 1363 Insulin-like Intragastric feeding, 598 growth factor, 36 Intralobular sequestration, 743 binding protein, 36 Intraoperative fluid therapy, 386 growth factor-1 (IGF-1), 2356–2357 Intrapartum antibiotic prophylaxis, 1754–1756 Insure, 434 Intrapartum asphyxial markers INSURE technique, 999 electronic fetal monitoring (EFM), 412 (ID), 239 fetal scalp blood sampling (FBS), 412 Intelligence quotient, 246, 2037 meconium in utero, 412 Intensive care, 195–196, 390–391 Intrapartum fetal monitoring, 121 admission, 205–207 Intrapulmonary cystic malformations, 740–742 See also Perinatal intensive care Intrathoracic defect Interburst intervals (IBI), 2082, 2085 cystic adenomatoid malformation, 742–743 Interferon, 525 diaphragmatic hernia, 950–956 Interferonγ (IFNγ), 892, 1535, 1587, hydrothorax, 748 1708–1709 Intrauterine Interferon regulatory factor (IRF), 525 asphyxia, 793 Interleukins (IL), 892–893 candidiasis, 99 IL-4, 13, 893 erythrocyte transfusions, 1699 IL-8, 893 fetal death, 1697 IL-10, 893 infection, 62 (see Infections) IL-16, 893 pneumonia, 1741 IL-1β and 6, 893 transfusion, 1157, 1158 Intermittent Mandatory Ventilation (IMV), Intrauterine growth restriction (IUGR), 14, 113, see Conventional Mandatory Ventilation (CMV) 133–134, 149 Index 2493

diagnosis of, 151–154 , 1257 early onset, 155–157 treatment, 1260 intermediate onset, 157 , 1260 late onset, 157 Isolated hypoaldosteronism, 1843 modes of delivery, 155–157 Isomerism, Mechanical Abnormalities in Neonates, neonatal aspects, 147–165 930–931 Intravenous immunoglobulin (IVIG), 1158, 1160, 1202, Isoniazid, 1530, 1709 1484, 1530, 1610, 1612, 1614, 1698, 1737 8-Iso-prostaglandin F2alpha, 897 neonates infection treatment, 1541 Isovaleric acidemia, see Inborn errors of metabolism pretermbirth infection prevention, 1540–1541 very low birth weight infants infection prevention, 1541 J Intravenous vasodilator drug therapy, 949 Jagged 1 gene (JAG1), 1213 Intraventricular hemorrhage (IVH), 1016, 1018, 1019, Jansen’s metaphyseal chondrodysplasia, 1861 1449, 2077 Jarcho–Levin syndrome, 1960 See also Germinal matrix haemorrhage-intraventricular Jaundice, 179–180, 460, 477, 1146–1147, 1224, 1227, hemorrhage (GMH-IVH) 1234, 1237 Intrinsic factor, 521 breast milk jaundice, 1147 Intrinsic risk factors, sepsis, 1734 diagnosis, 1173 Intubation and tracheal suctioning, 794 neonatal cholestasis, 1210 INtubation-SURfactant-Extubation (INSURE) physiological, 1148–1149 approach, 434, 982 treatment, 1175–1177 In vitro fertilization, 8 128–145 See also Hyperbilirubinemia Iodine, 671 Jejunal and ileal atresia, 1306–1308 human milk content, 670 Jejunoileal atresia, 1333 recommended intake, 670 Jeune syndrome, 1958 Iodine-123 scintigraphy, 1868 Jitteriness, 657, 723, 724, 1132 IPEX syndrome, 519 Job’s syndrome, 2408 IPPV, see Intermittent positive pressure ventilation Joints, septic arthritis, 1745–1747 Iris coloboma, 2347 Joubert syndrome, 1965, 2130 Iron, 520, 670–671 Jug-handle posturing, 2333 deficiency, in neonates, 724 human milk content, 670 oxigen toxicity, 439–453 K recommended intake, 670 , 1836, 1879 Irradiance, 1195 Kangaroo care, 22, 193, 197, 213, 360, 374, 399, 548, 989 Irritability, 22, 2062 Kangaroo mother care (KMC), 360, 574–575 in atrioventricular septal defect, 1062–1063 Kaposiform hemangioendothelioma, 2419 in bilirubin neurotoxicity, 1171 Kasabach–Merritt syndrome, 1479, 2419 in blue diaper syndrome, 1861 Kasai hepatoportoenterostomy, 1235 in congenital syphilis, 2402–2404 Kearns–Sayre syndrome, 1859, 1879 in hypertension, 1119–1120 Kenny–Caffey syndrome, 1859, 1879 in hypocalcemia, 723–724, 1857 Keratinocyte growth factor (KGF), 896 in hypomagnesemia, 723–724 Keratoconjunctivitis, 1673 in infection, 1708 Kernicterus, 1146, 1154–1156, 1159, 1160, 1162, 1164, in neonatal acute liver failure, 1217 1169–1181, 1187–1191, 1200 in neonatal polycythemia, 724 description, 1170 Ischemia, 410, 1378 epidemiology, 1172–1173 Ischemic damage, 1221 risk factors, 1171 Ischemic neonatal strokes, 2226 Ketamine, 387, 392 Ischemic perinatal stroke (IPS), 1464, 2252 dose, 390 Ischemic stroke, 2097 Ketoacidosis, 1820 Isofurans, 449 Ketone bodies, 1821 Isoimmune hemolytic disease, 1187 Kidney Isoimmune neonatal neutropenia, 1529–1530 acute renal failure, 1937 Isoimmunization, 1156–1161, 1499 agenesis, 1961–1962 Isolated craniostenosis chronic kidney disease, 164 brachicephalia, 1259 classification of renal anomalies, 1960–1961 frontal plagiocephaly, 1257–1259 development, 1920–1921 2494 Index

Kidney (cont.) Langerhans cell histiocytosis (LCH), 1658, 2408 fetal kidney, 1018, 1027 Language, 246, 266, 268–270, 288, 2037–2038 hydronephrotic, 1974 Lanugo, 2395, 2397 hypoxemic-hypoxic insults, 1941–1942 Laparoscopic assisted ano-recto-plasty (LAARP), 1325 laboratory management, 1978–1987 Large bowel defects, see Gastrointestinal, malformations multicystic dysplastic kidney, 1964 Large for gestational age (LGA), 149 neonatal kidney, 1919–1932 , 1879 Potter sequence, 54–55, 748, 1284–1285 Laryngeal tumors, 1643, 1653–1654 chemoreflexes (LCR), 784 Kidney injury molecule 1 (KIM-1), 1985 mask, 389, 1000 Kimura’s anastomosis, 1305 Laryngomalacia, 770 Kimura technique, 1304 Laryngospasm, 784 KL-6, 897 Laryngotracheobronchitis, 1788 Klebsiella, 1376, 1730, 1742 LATCH charting system, 566 oxytoca, 1743 Late enteral nutrition, 593 pneumoniae, 1743 Latent failures, 309 Kleihauer Betke stain, 1500, 1503 Late onset disease (LOD), 1775, 1781 Klippel–Feil malformation, 66 Late-onset neonatal pneumonia, 1741 Klippel–Feil syndrome, 66–67 Late preterm infants (LPI), 22, 171–181 Klippel–Trénaunay syndrome, 462 American Academy of Pediatrics (AAP) Klumpke’ guidelines, 180 palsy, 466 apnea, 178 syndrome, 726 birth hospitalization morbidity, 176 Kostmann’s syndrome, 1528, 1536, 1555, 1564 feeding difficulties, 178 Krabbe disease, 1827 hospital discharge guidelines, 181 Kyphoscoliosis, 69, 2325 hyperbilirubinemia, 179 , 67 incidence and prevention, 173 long term outcomes, 180 morbidity, 176 L mortality, 175 Labor preterm infants, 173 chorioamnionitis, 95–103 readmission, 177 delivery room preparation, 95–103 respiratory distress syndrome (RDS), 178 history of delivery, 19 vs term infant morbidity, 176 neonatal resuscitation, 349–350, 397 vs. term infants, complications, 175 pre-eclampsia, 33 thermal instability, 177 preterm delivery, 28–33, 173–175 transient tachypnea of the newborn (TTNB), 177 problem of preterm labor, 474–475 Laurence–Moon syndrome, 1880 risk factors of preterm delivery, 28–33 LBW, see Low birth weight surveillance, 121–125 LCHAD (long chain 3-hydroxy-acyl-coenzyme A fetal distress identification, 121 dehydrogenase), 1815 fetal heart rate, 121–123 L-cysteine, 446 Laboratory evaluations, 1227 Leadership and safety culture, 311–312 Laboratory tests, 1732 Leaky lung syndrome (LLS), 898 Lactase, 518 Learning disabilities (LD), 239 Lactate, 412, 414–415, 601 Lecithin/sphingomyelin (L/S) ratio, 813, 840 dehydrogenase, 352, 1348 Left-to-right shunt lesions, 1061–1063 ringer’s lactate, 434 atrial septal defect, 1061 urinary lactate, 416–417 atrioventricular septal defect, 1062–1063 Lactation, 679 ventricular septal defect, 1043, 1061–1062 support, 1197 Left ventricular outflow obstruction See also Milk (LVOTO), 1053 Lactic acidosis, 1820 Legal issues LactMed, 679 crux of the matter, 350–351 Lactoferrin, 522, 894, 1387, 1580 informed consent, 353–354 Lactogenesis, 561–562 medical negligence claims, 349 Lactose, 518 parental rights, 191–192 LAD, see Leukocyte adhesion deficiency standards of care, 176, 254 Ladd’s bands, 1303, 1318 Legg–Calvè–Perthes disease, 712 Index 2495

Leigh disease, 1810, 1827 Lingual lipase, 515 Leiner’s disease, 2410 Link stroke to (birth) trauma, 2231 Lemon sign, 2130 Lipid hydroperoxide (LOOH), 894 , 47, 548 Lipids, 515 Less invasive surfactant administration (LISA), 987 Lipofibromatosis, 1653 Less invasive surfactant application, 2206 Lipoid adrenal hyperplasia, 1906 Lethal congenital malformations, 220 Lipopolysaccharide (LPS), 1376 Leukemia, 1538, 1656–1657, 2422 Lipoprotein lipase (LPL), 516 congenital, 1656, 2422 Liquid ventilation, 904 Down syndrome and myeloproliferative, 1656 Lissencephaly (LIS), 2115–2119, 2132–2134 and neutropenia, 1554 cerebellar hypoplasia, 2115 Leukemoid reaction, 1530, 1538 genetic basis and diagnosis, 2115–2118 Leukocyte adhesion deficiency (LAD), 1533–1534 genetic counselling, 2119, 2120 type I, 1533–1534 laboratory investigation, 2119 type II, 1533–1534 microcephaly, 2137 type III, 1533–1534 phenotype, 2118 Leukocytes, 524, 1418–1424 type II, 2329 abnormalities, 1527–1543 See also Central nervous system, malformations frequency, 1528 Listeria monocytogenes, 19, 99, 869 in newborn, 1536 neonatal pneumonia, 869 antibiotics, 1539–1540 Lithium, 1835 autoimmune neutropenia, 1530, 1535–1536 Live birth, 187, 257 clinical aspects, 1536–1537 Liver differential diagnosis, 1537–1538 biopsy, 1230, 1234 drug induced neutropenia, 1530 disease, 164, 1207–1221 dyskeratosis congenita, 1532–1533 acute fatty liver of pregnancy, 1815 eosinophils, 1420–1423 and alfa1-antitrypsin deficiency, 1235–1236 function, 1525–1526 cholestasis granulocyte colony-stimulating factor, 1541–1542 cholestasis: of the newborn, 1207–1221 granulocyte-macrophage colony stimulating factor, cholestasis: in premature infant, 1238 1542–1543 cirrhosis, 1210–1211 granulocyte transfusions, 1540 inborn errors of metabolism, 1809 growth factors, 1567–1568 dysfunction, 1825–1826 intravenous immunoglobulin (IVIG), 1540–1541 neonatal acute liver failure, 1216 isoimmune neutropenia, 1529–1530 transplantation, 1211, 1214, 1216, 1218, kinetics, 1524–1525 1219, 1221 leukemoid reaction, 1530 tumors, 1643, 1655–1656 leukocyte count, 1418–1420 See also Hepatitis moderately common abnormalities, 1529–1530 Lobar emphysema, 740, 744–745, 928 physiology, 1524–1527 Lobar hematoma, 2228 prematurity functional differences, 1526–1527 Long-chain acyl-coenzyme A dehydrogenase deficiency prognosis, 1538–1539 (LCAD), 1883 rare abnormalities, 1530–1536 Long-chain 3-hydroxy-acyl-coenzyme A dehydrogenase site of sampling, 1418 (LCHAD) deficiency, 1815 Leukokoria, 2347–2348 Long-chain polyunsaturated fatty acids Leukomalacia, see Periventricular (LCPUFA), 527 leukomalacia parenteral nutrition, 592–593 Leukotriene E 4, 897 Long philtrum, 58 Levetiracetam, 2291 Long-term morbidity, quality in neonatal care, Leydig cell, 1895 256–261 hypoplasia, 1908 Long-term neurodevelopmental outcome, Lidocaine, 2290, 2291 1158–1159 dose and route, 1428 Louw–Barnard theory, 1306 seizures, 2395 Low–bilirubin kernicterus, 1190 Life course approach, 47 Low birth weight, 22, 204–207, 712 Light-emitting diode (LED) l, 1196 assisted ventilation, 209 Limb, 466 cardiovascular problems, 206 dysostoses, 67 discharge, 208 movement, 22, 111, 2066 early-onset sepsis, 207 2496 Index

Low birth weight (cont.) in gastrointestinal tract, 526 late-onset sepsis, 207 in inborn errors of metabolism, 1808 long term outcome, 260–261 in lamina propria and intra-epithelial compartments, nutrition, 558, 596, 606 1592–1593 Lower esophageal sphincter (LES), 512 lymphocytes interaction, 1590 Lower limbs abnormalities, neonatal physical T-lymphocytes, 1583–1587 examination, 466 Lymphoid depletion syndrome, 1780–1781 Lowe syndrome, 1810, 1827 Lymphoma, 1476, 2406 Low grade hemolysis, 1162 Lymphopenia, 1609, 1612, 1614, 1615, L-selectin, 894, 897 1780–1781 Lumirubins, 1194, 1197 Lysosomal storage disease, 1811–1813 Lysosomal storage disorders, 1812–1813 abnormalities of perfusion, 838–839 Lysosomal trafficking regulator (LYST), 1534 alveolar capillary dysplasia, 754–755 Lysozyme, 522, 894 chylothorax, 753–754 congenital cyst adenomatoid, 742–743 M lobar emphysema, 744–745, 928 lung cyst, 744 in brain tumors, 1654 pulmonary lymphangiectasia, 751–753, in errors of metabolism, 1811 926–927 in lupus erythematosus, 2413 development, 734–737, 890, 906 in osteochondrodysplasias, 68, 69 edema, 838 Macroglossia, 1278 elastic properties, 761–763 in Beckwith–Wiedemann syndrome, 60–61 fetal lung liquid, 447 in inborn errors of metabolism, 1811 inflammation, 795 in Pompe disease, 2331 injury, 793 Macrolide antibiotics, 904 intrapulmonary cysts, 740–742 Macronutrients, 537 lavage, 796 Macrophage(s), 524, 891, 893, 896, mechanics, 760–761 1581–1582 neonatal pneumonia, 869 in gastrointestinal tract, 526 physiology, 759–772 Macrophage migration inhibitory factor (MIF), 894 postnatal development, 737–738 Macrosomia, 63, 718–727 pulmonary Magnesium, 2191–2192 agenesis, 745–747 recommended intake, in preterm infants, 61, circulation, 767 606, 614 hemorrhage, 866–867 sulfate, 2155–2156 hypoplasia, 396, 486 Magnetic resonance, 86, 206 interstitial emphysema, 875–877 antenatal diagnosis, 740, 2165 malformations, 740–755 neuroimaging, 1167, 2091–2110, 2300 sliding, 914 spectroscopy, 2177 structure, 874–875 Magnetic resonance angiography (MRA), surfactant 2259 metabolism, 814 Magnetic resonance cholangiography (MRCP), 1230 transient tachypnea, 867–868 Magnetic resonance imaging (MRI), 900, 2093, ultrasound, 915–916 2095, 2177 volume, 764 Maintenance fluid, 386 Lupus erythematosus neonatal, 1226, 2411 Majewski syndrome, 1880 Lutein, 453 Major malformations, 50 Luteinizing hormone (LH), see Hormones Malaria, 1202, 1226 Lyell syndrome, 1719 Malformations Lyme disease, 752 biliary tract, 1243–1250 Lymphangioma, 1642, 2420 brain, 2002–2003 Lymphatic malformations, 2420 cardiac, 1037–1063 Lymphatic tree abnormalities, 926 diagnosis and treatment, 1065–1076 Lymphocytes congenital, 35, 49–70, 175, 177, 476 B-lymphocytes, 1587–1589 associations, 52–53 in bone marrow aspirate, 1428 classification, 50–51 in chylothorax, 753–754 disruption, 62–64 Index 2497

dysostoses, 64–67 during pregnancy on neonate, 721 osteochondrodysplasias, 67–70 breastfeeding, 721–723 sequences, 53–55 cardiopulmonary adaptation, 725 syndromes, 55–62 growth disorders, 720–723 craniostenosis, 1257–1266 hyperbilirubinemia, 725 in infants of diabetic mother, 719 hypocalcemia and hypomagnesemia, 723 in intrauterine growth restriction, 160 hypoglycemia, 723 letal, 198–199 iron deficiency, 724 ocular, 2339–2348 neuropathology, 725 orofacial, 1255–1279 polycythemia, 724 of posterior segment, 2348 pulmonary status, 725 pulmonary, 733–755 risk, assessment and management, 722 sequence, 54 Maternal hyperparathyroidism, 1858 skin, 2422–2423 Maternal hypertension, 1528 in smoke exposure, 711–712 Maternal hypoxia, 7 urinary tract, 1743–1745, 1955–1987 Maternal immunity, 1665 vascular, 461, 2419–2420 Maternal inflammatory diseases, 7 Malformations of cortical development (MCD), Maternal nutrient intake, 6 2132–2138 Maternal nutrition, 46 features of, 2115 Maternal risk factors, sepsis, 1733 megalencephaly, 2115 Maternal thrombophilia, 1459–1460 postzygotic mutatitions, 2115 Maternal uterine artery blood flow, 6–7 Malformative glaucoma, 2346 Maternal vaccination, 1796–1798 Malignancies, 1641–1658 Maternal viral load, 1685 See also Tumor(s) Matrix metalloproteinases (MMPs), 894 Malondialdehyde, 894 MMP-2, 896 Malpractice claims, against neonatologists, 349 MMP-8, 895 Malrotation, 505 MMP-9, 896 Managed clinical network, 255 Maturation Management, 1158–1159 central nervous system, 21 , 1274 gut, 538, 546, 548 Manganese, 670 May–Hegglin anomaly, 1481 Maple syrup urine disease, 1814, 1818, 1828, 2100 McCune–Albright syndrome, 1874 Marfan syndrome, 59, 2139, 2345 MCD, see Malformations of cortical development (MCD) Marie Unna hypotrichosis, 2421 M cells, 525 Marijuana, 7 McKusick–Kaufman syndrome, 1904 Mast cells, 526, 897, 2405–2406 Meacham syndrome, 1877 Mastocytosis, 2405–2406 Mean arterial pressure (MAP), 1776, 1781 Maternal Mean corpuscular hemoglobin (MCH), 1412, 1413 antiphospholipid antibodies, 2238 Mean corpuscular hemoglobin concentration (MCHC), cigarette smoking, 7 1412–1414 disease, 3, 19 Mean corpuscular volume (MCV), 1410, 1412, 1413 drug abuse, 7, 21, 31, 63 Mean platelet volume (MPV), 1483 alcohol, 63 Measles, 1688, 1689, 1799 benzodiazepines, 684 Mechanical ventilation, 79–80, 843–863, 2316 opioids, 683–684 arterial blood gas measurements, 847 mortality, 33 caffeine treatment, 863 acute fatty liver of pregnancy, 1815 chest X-ray, 847 HELLP syndrome, 1815 complications management Maternal-fetal pharmacotherapy, 680–681 accidental extubation, 862 Maternal glucose intolerance endotracheal tube, 861 during fetal period pneumothorax, 862 acute and subacute non-structural cardiac pulmonary interstitial emphysema, 862 abnormalities, 721 drug treatments, 863 fetal iron metabolism disorders, 721 indications for, 846–847 glucose metabolism disorders, 720 apnea, 846 growth disorders, 720–723 oxygenation, 847 reduced fetal oxygenation, 721 respiratory failure, 846–847 during periconceptional period, 719–720 infection investigation, 847 2498 Index

Mechanical ventilation (cont.) Medium-chain acyl-coenzyme A dehydrogenase managing complications, 861–862 deficiency, 1883 muscle relaxing drugs treatment, 863 Medium chain triglycerides (MCT), 1233 principles of, 847–851 Medullary thyroid carcinoma, 1346 blood gas measurment, 849 Medulloblastoma, 1654, 1655 blood level control, 848–849 Megalencephaly, 68, 2123 flow wave, 849 Megalocornea, 2343 heating and humidification, 849 Megaureter, 1938, 1960 oxygenation improvment, 847–848 Melanin pigmentation, 2414–2422 pressure wave, 849 Melanocytic neuroectodermal tumor of infancy (MNTI), tidal volume wave, 850 1657–1658 problems, 846 Mendelian inheritance, 52 sedation treatment, 863 Meningioma, 1643 synchronised intermittent positive pressure ventilation, Meningitis, 1739–1741 856–857 antibiotic therapy, 1740 techniques of, 851–861 clinical diagnosis, 1739 assist control, 856–857 clinical signs, 1739 conventional mandatory ventilation, 851–853 diagnosis, 1740 high frequency ventilation, 859–861 microrganisms, 1739 neurally adjusted ventilator assist, 859 mortality and morbidity, 1741 pressure support ventilation, 859 prognosis, 1741 synchronised intermittent ventilation, 856 treatment, 1740 triggered ventilation, 854–856 viral, 1788 volume guarantee ventilation, 857–859 Meningocele, 2128, 2422 weaning from mechanical ventilation, 984 Mental developmental indices (MDI), 2321 weaning infant, 861 Mental retardation (MR), 239–240 Meckel–Gruber syndrome, 55, 1922, 1958, 1960, 2130 Meropenem, 1736, 1737 Meckel’s diverticulum, 1311–1314, 1347 Merosin negative CMD, see Muscle disease Meconium, 100, 434, 514, 1377 Mesenchymal hamartoma, 1656 composition, 792 Mesenchymal stem cells (MSCs), 956 obstruction, 1340, 1341 Mesenterium commune, 1317 Meconium aspiration syndrome (MAS), 434, 791–798, Mesoblastic nephroma, 1643, 1653 810, 816–817, 985, 1010 Mesonephros, 1920 clinics and therapy, 794–798 Messenger RNA, 45, 649 definition, 792 Metabolic pathogenesis, 792–793 abnormalities, 1256 prevention strategies, 794 acidosis, 413, 1816–1818 (see also Acidosis) severity, 796 bone disease Meconium ileus, 1310–1311, 1341, 1343 bone mineral accretion, 663–667 Meconium plug syndrome bone turnover, 646 clinical presentation, 1341 calcium and phosphorus homeostasis, 639–667 contrast enema technique, 1343 , 650–652 definition, 1340 disorders, 2099 differential diagnosis, 1341 inborn errors of metabolism, 1805–1829 etiology/pathogenesis, 1340 risk, 164 Hirschsprung’s disease and cystic fibrosis, 1340 screening, 473 hypermagnesiemia/eclamptic mothers, 1340 syndrome, 14, 164, 327, 726 hypoglicemia/maternal gestational diabetes, 1340 Metabolomics, 1986–1987 management, 1342 Metaiodobenzylguanidine (mIBG) scan, 1647 maternal drugs, 1341 Methemoglobinemia, 944 prognosis, 1344 Methimazole, 1530, 1873 sepsis, 1341 Methyldopa, 1530 surgical procedures, 1343 Methylmalonic acidemia, 1817 Meconium stained amniotic fluid, 792 Methylxanthines, 902 Mediastinal teratomas, 1650 MHC class II antigen deficiency, 1614 Medication during pregnancy and breast feeding, 677–689 Microbial associated molecule pattern (MAMPs), 525 Medication error (ME), 307, 312–313 Microbial tests, 1731–1732 Medium-chain acyl-CoA dehydrogenase (MCAD), 1811, Microbiome, 521 1819, 1821 Microbiota, 521, 1377 Index 2499

Microcephaly, 63, 181, 2128, 2135, 2137 Minimal enteral feeding (MEF), 572, 597 in cri-du-chat syndrome, 56 Minimal neurological dysfunction, 239–240 in disorders of CNS cell number, 2137 Minimum alveolar concentration (MAC), 386 in fatty acid oxidation disorders, 1821–1822 Minor malformations, 50 in Nager syndrome, 66 Minor neurological dysfunction (MND), 239 in Nijmegen breakage syndrome, 1615–1616 Minute ventilation, alteration in sleep, 778 in osteodysplastic primordial dwarfism, 69 miR-7, 898 in phenylketonuria, 1813 miR-152, 898 in Rubinstein–Taybi syndrome, 58–59 miR-206, 898 in Wolf–Hirschhorn syndrome, 56 miR-219, 897–898 Microcornea, 2343–2344 miR-30a-3p, 898 Microflora, 522 miR-133b, 898 Microglia, 1624, 1627, 2194 Miscarriage Microglossia and aglossia, 1279 and fetal loss Micrognathia definition, 132–133 in camptomelic dwarfism, 68 diagnosis, 133 in cri-du-chat syndrome, 56 epidemiology, 133 in DiGeorge syndrome, 1615 etiology, 133 and drugs, 63 See also Abortion in muscle-eye-brain disease, 2329–2330 Mismatch, 46 in , 69 Missense mutation, 1147 in peroxisomal disorders, 1813 Mitochondria, 2195 in , 1271–1272 Mitochondrial disease, see Inborn errors of metabolism in Treacher Collins syndrome, 66 Mitochondrial oxygen radical formation, 443 Micronutrients, 519–521, 669–674 Mitochondrial respiratory chain disorders, 1219 Microorganisms, 522 Mitochondrial superoxide theory, 443 Micropenis Mitochondrial trifunctional protein deficiency, 1880 in hypopituitarism, 1836 Mitogen-activated protein kinase (MAPK), 525 in Smith–Lemli–Opitz syndrome, 62 Mixed gonadal dysgenesis, 1899 Microphthalmia, 2341–2343 See also Sexual development disorders in Patau syndrome, 56 Mode of delivery, 1691, 1693 in Walker–Warburg syndrome, 2329 Moderate hypoxic ischemic encephalopathy, 298 MicroRNA (miRNA), 2307 Molecular diagnostics, 1732 Microsatellite marker, 60 Molybdenum, 670 Microvilli, 505 Mongolian spots, 460, 2416 Microvillus inclusion disease, 519 Monoamniotic (MA) twinning, 142–143 Midazolam, 392 Monochorionic placenta, 132 neonatal anesthesia, 387, 390, 392 Monocyte, 1525–1527, 1581–1582 Middle cerebral artery (MCA), 1157 counts in the bone marrow aspirate, 1427 pulsatility index, 118 deactivation, 1780 stroke, 2262–2263 and macrophages, 1581–1582 Midgut, 505 Monocyte chemoattractant proteins (MCP) 1, 1α,1β, 2 and Migrating action potential complexes (MAPCs), 509 3, 894 Migrating motor complex (MMC), 509 Monogenic disorders, 57–62 Mild growth failure (MGF), 625 Mononuclear phagocytes, 1525–1526 Milia, 460, 2397 Monosomy of chromosome X, see Turners’ syndrome Miliaria, 2399 Monozygotic twinning, 131 Milk Moral decision-making, 221 bank, 600 Moral dimension, 221–222 formulas, 601 decision making, 220–221 fortifier, 600–601 parental decision making, 223–224 human, 213, 375, 452, 643 physician role, 224–226 micronutrients content, 670–671 Morbidity, 258–260 vitamins content, 672–673 brain pathology, 259–260 See also Breastfeeding in high risk infant, 256–261 Miller–Dieker syndrome, 2117 inlate preterm infants, 176 Milrinone, 950 lung disease, 258–259 Minerals and electrolytes, 591 Moro reflex, 2056 Minimal androgen insensitivity syndrome, 1907 Morphine, 319 2500 Index

Mortality, 1018 Multiple-hit hypothesis, 2023 chronic lung disease, 258 Multiple pituitary hormone deficiencies (MPHD), 1844 herpes simplex virus, 2399–2400 Multiple pregnancies, see Multiple births infant, 135 Mumps vaccine, see Vaccines in late preterm infants, 175 Muscle disease maternal, 19, 33, 287 congenital muscular dystrophies (CMD), and morbidity clinical review, 311 2328–2331 sepsis, 1735 Fukuyama CMD, 2329 septic shock, 1775 merosin negative CMD, 2328–2329 surfactant treatment, 998 muscle-eye-brain disease, 2329–2330 transpyloric feeding, 598–599 myotonic dystrophy, 2330 Mosaic 8 chromosome trisomy, 56–57 Walker–Warburg syndrome, 2329 Motherhood, 210, 211 congenital myopathies, 2324–2328 Motherisk program, 679 central core disease, 2325 Mother to child transmission (MTCT) congenital fiber type disproportion, 2327 prevention, 1686–1688 multi-minicore disease, 2327–2328 risk factors, 1691 myotubular myopathy, 2326–2327 Motilin, 547–548 nemaline myopathies, 2325–2326 Motility, 241 metabolic myopathies, 2331–2332 dysmotility, 1292 mytochondrial, 2331–2332 fetal, 1872 Pompe disease, 2331 gastrointestinal, 509–515, 547, 598 myasthenia gravis, 2332 in meconium plug syndrome, 1340 spinal muscular atrophy, 2333 in necrotizing enterocolitis, 1375–1376 Muscle-eye-brain disease (MEB), 2329–2330 in perforation, 1333–1334 Muscle relaxing drugs, 863 Motor development of VLBW infants, 2040 Muscle tone examination, 467, 2056–2057 Motor neuron disorders, spinal muscular atrophy Muscular dystrophy, see Muscle disease pontocerebellar hypoplasia type 1, 2333 Musculoskeletal conditions, 2389 respiratory distress syndrome type 1, 2333 Musculoskeletal infection in newborn, 2390 Mouth, 463 Mutations small, 55, 60, 1057 DCX of, 2115 MRI findings, neurological examination, 240–241 FLNA gene, 2119 Mucin, 524 missense, 2118 Mucocutaneous candidiasis, 1752 somatic mosaic, 2123 Mucolipidoses, 182 Myasthenia gravis, see Muscle disease , 1808 Myasthenic syndromes, 2332–2333 Mucosal immune system, 1576–1595 Mycobacterium tuberculosis, 1707, 1742 Mucous cells, 506 Mycoplasma spp., 473 , 65 Mydriasis, 359 Müllerian duct aplasia, unilateral renal agenesis, and Myelin basic protein (MBP), 2307 cervicothoracic somite anomalies Myelin/myelination, 2002 (MURCS), 1880 disorders (see also White matter injury) Multicystic dysplastic kidney, 1964 Alexander disease, 1827 and hypertension, 1119–1120 Krabbe disease, 1827 Multi-minicore disease (MmD), 2327–2328 peroxisomal disorders, 1813, 1827 Multiorgan failure (MOF), 416 Myelocathexis, 1555 Multiple births, 129–145 Myelomeningocele, 2130 complications, 132–134 Myenteric (Auerbach) plexus, 510 cord occlusion, 143–145 Myocardial disease, 1044 preterm delivery, 134–135 Myocarditis, 1787 twin reversed arterial perfusion syndrome, 136–138 Myofibromatosis, 1653 twin-to-twin transfusion syndrome, 141–142 Myogenic response, 508 epidemiology, 130–131 Myoglobinuria, see Renal, failure fetal growth, 133–134 Myoinositol, 898 mortality and morbidity, 257 Myopathies, see Muscle disease placentation (chorionicity, zygosity), 131–132 Myopia, 2363 single intrauterine death, 140–141 Myotonic dystrophy, see Muscle disease types of twins, 130 Myotubular myopathy (MM), 2326–2327 Multiple carboxylase deficiency, 1817–1818 Mytochondrial myopathies, 2331–2332 Index 2501

N pathogenesis, 1375–1378 N-acetyl cysteine (NAC), 904 peritoneal drainage (PPD)/laparotomy, 1397 NADPH, 1161 and pneumoperitoneum, 1396 NADPH-oxidase, 2025 prevention, 1385–1387 Nager syndrome, 66 primary peritoneal drainage (PPD)/laparotomy, 1396 Nails disorders, 2420–2421 radiographic findings, 1381 nail patella syndrome, 1922, 2420 resection and primary anastomosis, 1398 Nalmefene, 1232 surgical intervention outcome, 1400 Naloxone, 390, 1232 surgical options for advanced, 1397 Naranjo algorithm, 704 surgical treatment, 1395–1400 Nasal continuous positive airways pressure (N-CPAP), Needle aspiration, 882 898, 904–905, 981 Neisseria gonorrhoeae, 404, 1748 airways suction, 990 Nemaline myopathies (NM), 2325–2326 attachment (headset and nasal prongs), 988–989 NEMO gene defects, 1611 circuit, positioning of, 989 Neonatal, 213–215, 348 CURPAP study, 982 behavior, 2031–2048, 2059–2066 efficacy of, 981, 984 care, 279–289 feeding, 990 in the delivery room, 397–398 humidification and heating, 989 cerebral palsy, 351 incidence of, 987 development, 21–22 (see also Neurodevelopment) infant, positioning, 989 examination, 457–468 monitoring, 986–987 abdomen, 465 nasal-cutaneous lesions, 986 assessment of gestational age, 467–468 nebulization of pharmaceuticals, 985 chest, 464–465 phrenic nerve, paralysis of, 985 ears, 463 pressure level, 986 eyes, 463 pulmonary hyperdistension, 985 face, 462–463 in TTN, 985 general observation, 460 weaning, 988 genitalia, 465 Nasal-cutaneous lesions, 986 head and skull, 462–463 Nasal intermittent positive pressure ventilation (NIPPV), hips, 466 898, 905 informed consent, 353–354 Nasolacrimal duct obstruction, 2343 limbs, 466 Nasopharynx congenital defect, see Cleft lip/cleft mouth, 463 palate neck, 464 Na taurocholate cotransporting polypeptide neurological, 466–467 (NTCP), 1225 nose, 463 National Health Service (NHS), 280 resuscitation practices, 349–351 Natural killer cells, 1590–1591 skin, 460–462 Natural twinning, 131 spine, 465–466 Near infrared spectroscopy (NIRS), 1083, 2176 timing of perinatal hypoxia, 351–353 Neck examination, 464 hereditary neutropenia (see Hereditary, neutropenia) Necrosis, 2256 infections Necrotizing enterocolitis (NEC), 162, 260, 522, 597, 1071, antimicrobial prophylaxis, 1759–1761 1202, 1373–1387, 1479, 1567 bacterial (see Bacterial infections) biomarkers, 1379, 1380 breast milk, 1762 bowel and enterostomy formation, 1397 early onset, 207 bowel specific biomarkers, 1380–1381 fungal, 1750–1754 classical diagnosis, 1378 laboratory investigation, 473–474 complications, 1385 lactoferrin, 1762–1763 and endothelial dysfunction, 508 late onset, 207 indications for surgery in infants with, 1396 prevention, 1754–1764 infants with, 1396 prevention: hand hygiene, 175 and IUGR, 162 probiotics, 1763 laboratory findings, 1379 skin care, 1763–1764 management, 1384–1385 and thrombocytopenia, 1478 near infrared spectroscopy, 1384 viral (see Viral infection) nutritional supplements, 1387 intensive care (see Intensive care) panintestinal disease, 1398 malignancies (see Tumor(s)) 2502 Index

Neonatal (cont.) retinoblastoma (RB), 1657 pain, 365–377 soft tissue sarcoma (STS), 1650–1653 non pharmacological management, 371–372 teratomas, 1648–1650 scales, 369 therapeutic principles, 1645–1646 resuscitation, 397, 423–436 Neonatal care ethical problems, 187–199, 223 hyperoxemia and hyperoxia hazards, 490–492 juridical regulation, 349 O2 saturation by pulse oximetry septic shock, 1773–1782 monitoring, 484–489 transport, 291–301 monitoring: alarms in, 488 Neonatal abstinence syndrome (NAS), 686–688 monitoring: hyperoxemia, 489 Neonatal acute liver failure (NALF) monitoring: hypoxemia, 489 causes, 1217 monitoring: hypoxia, 487 definition, 1216 monitoring: time and sensitivity, 489 etiology, 1216 oxygen assessment in blood, 483–484 management, 1221 supplemental oxygen, 483 Neonatal alloimmune thrombocytopenia, 1477, 2217 target ranges Neonatal anaesthesia, 387 in delivery room, 495–497 neurotoxicity risk, 392 for preterm infants, 492–495 Neonatal arterial ischemic stroke (NAIS), 2226 tissue oxygenation, 489–490 Neonatal asphyxia Neonatal care quality definition, 411 assessment, 252 incidence, 410–411 benchmarking, 254 Neonatal assessment at birth clinical audit, 253 blood pressure monitoring, 476 confidential enquiries, 254 cardiorespiratory monitoring, 475 definition, 252 temperature determination, 475 domains, 252 transcutaneous blood gas monitoring, 476 education and knowledge translation, 255 Neonatal autoimmune neutropenia, 1530 improvement, 255 Neonatal Behavioral Assessment Scale (NBAS), 713, managed clinical networks, 255 2060, 2062 morbidity, 258 Neonatal bleeding disorders, 1439 brain pathology, 259 acquired coagulation disorders (see Acquired longer term outcome, 260 coagulation disorders) lung disease, 258–259 bleeding assessment tool, 1440 necrotising enterocolitis, 260 inherited coagulation disorders (see Inherited visual disorder, 260–261 coagulation disorders) mortality, 257–258 laboratory investigations, 1440–1441 gender effect, 258 Neonatal blood pressure, 1114 geographical variation, 258 Neonatal brain damage limits of viability, 257 biomarkers, 2303, 2308 multiple birth rates, 257 cardiotocography and fetal Doppler studies, 2299 preterm birth rates, 256 clinical assessment, 2302, 2303 National and International group, 255 electroencephalography, 2301, 2302 outcome, 253 fetal programming hypothesis, 2297 Neonatal care units hematology, 2302 intensive care, 283 neuroimaging, 2299, 2301 levels, 283, 284 placental pathology, 2297, 2299 special care, 283 Neonatal brain injury, 76, 81, 83, 85, 87 Neonatal cerebral sinovenous thrombosis (NCSVT), 2227 Neonatal cancer Neonatal cervical spinal cord injury, 2388 brain tumors, 1654–1655 Neonatal cholestasis causes, 1643 abdominal ultrasound, 1227 clinical features and diagnosis, 1643–1645 Alagille syndrome, 1238–1239 congential tumors, 1657–1658 α1-AT deficiency, 1235–1236 definition, 1642 biliary atresia, 1233–1234 germinal tumors, 1648–1650 BRIC, 1238 kidney tumors, 1653–1654 classification of, 1225 leukemia, 1656–1657 clinical presentation, 1225–1226 liver tumors, 1655–1656 definition, 1224 neuroblastoma, 1646–1648 description, 1209 Index 2503

duodenal aspirate analysis, 1230 sleep protection, 359–360 ERCP, 1230 stress, 359 hepatobiliary scintigraphy, 1227–1229 Neonatal intestinal obstruction, 1347 idiopathic neonatal hepatitis, 1236 Neonatal intestinal perforation, 1333 incidence of, 1224 Neonatal intracranial haemorrhage, 2202–2203 laboratory evaluations, 1227 Neonatal jaundice liver biopsy, 1230, 1234 definition, 1146 management of, 1230–1235 physiological, 1148–1149 MRCP, 1230 Neonatal lung diseases, surfactant metabolism, pathophysiology, 1225 see Surfactant metabolism, in neonatal PFIC, 1236–1238 lung diseases premature infants, 1238 Neonatal metabolic complications, in IDM, 722 principal causes, 1210–1214 Neonatal mortality, 1018 spectrum of etiology, 1210 Neonatal MRI, 241 treatment, 1215–1216 Neonatal myasthenia gravis (NMG), 2332–2333 Neonatal depression, 410 Neonatal neuroblastoma (NBL), 1643, 1646–1648 Neonatal diabetes mellitus (NDM), 1855–1857 clinical findings, 1647 Neonatal electroencephalography, 2081–2082 staging, 1647 abnormal, 2084 therapy and prognosis, 1647 effects of medications, 2085–2086 Neonatal neutropenia monitoring, 2086–2088 definition, 1562 normal maturation, 2082–2084 severe chronic neutopenia, 1562 (see also Severe seizures, 2085 chronic neutropenia (SCN)) Neonatal emergencies, causes, 1333 Neonatal neutrophils, 1526 Neonatal Emergency Transport Service (NETS), 293 Neonatal palliative care, 220 Neonatal encephalopathy (NE), 415–416, 2174, 2175 Neonatal patient, 220 Neonatal hemochromatosis, 1216–1218 Neonatal physiology, 696–698 Neonatal herpes, 1673 Neonatal pneumonia Neonatal hyperbilirubinemia, 517 etiology of, 869 Neonatal immunoprophylaxis, 1693 incidence of, 868 Neonatal individualized developmental care and morbidity, 868 assessment program (NIDCAP), 360, 2061 Neonatal renal vein thrombosis, 1461 Neonatal infant stressor scale scores, 359 Neonatal screening test, SCID, 1617 Neonatal intensive care unit (NICU), 175, 205, 208, 210, Neonatal seizures 306, 307, 309, 311, 312, 2084, 2086, 2089 diagnosing seizures, 2289–2290 admission to, 205–207 incidence, 2288 description, 358 prognosis, 2292 human milk nutrition treatment, 2290–2292 breastfeeding epidemiology, 570–571 Neonatal sepsis, 1633–1634 enteral feeding, advancement, 572 Neonatal severe hyperparathyroidism, 1861 expression and storage, 574 Neonatal shock fortification, 573–574 PPHN, 1776 infant subpopulation basis, 571–572 preterm newborns, 1776–1779 kangaroo mother care (KMC), 574–575 term newborns, 1775–1776 milk bank, 574 Neonatal stroke minimal enteral feeding (MEF), 572 ACA, 2265 oropharyngeal colostrum, 572 anterior choroidal artery stroke, 2266 preterm infant benefits, 572 clinical presentation, 2253–2255 preterm infant, oromotor competence, 576 definition, 2226 preterm infant, tube to breast transition, EEG findings, 2274 575–577 epidemiology and definition, 2252–2253 preterm milk fat content, 572–573 genetic aspects, 2274 preterm milk protein content, 573 hindbrain arterial ischemic stroke, 2269 preterm milk vs.breast milk, 573 ICA, 2265 semi-demand feeding, 577–578 MCA, 2262–2263 light, 359 MRI, 2258–2261 noise, 358 PCA, 2265 oxidative stress, 360 perforator stroke, 2266–2269 pain, 359, 390 prognosis, 2278–2280 2504 Index

Neonatal stroke (cont.) Nephron number reduction, 1932 spinal cord arterial ischemic stroke, 2269 Nephrotic syndrome, 1705, 1951 treatment guidelines, 2276–2278 Netherton’s syndrome, 2409, 2411 ultrasound, 2257–2258 NETS type, 301 venous thrombosis, 2271–2276 Neu–Laxova syndrome, 1810 Neonatal tetanus, 1750 Neural crest cells, 509 Neonatal toxic shock syndrome-like exanthematous Neurally adjusted ventilator assist (NAVA), 859 disease, 1749 Neural tube defects, 2128–2130 Neonatal transport index (NTI), 293 Neuroanatomical substrate, 221 Neonatal undernutrition, 1926 Neuroblastoma, 1646–1647 Neonatal varicella, 1677 See also Neonatal neuroblastoma (NBL) Neonatal withdrawal syndromes, 686–689 Neuroblastoma amplified sequence (NBAS) gene, 1221 Neonate of diabetic mothers, 718–727 Neurocutaneous melanosis, 2416 calcium and phosphorus alterations, 656 Neurodevelopment Neonates in BPD infants, 901 asphyxia, 160 extremely preterm infants, 186 auxological evaluation of, 157–160 IDM, 726–727 developmental abnormalities infants of diabetic mothers, 726–727 abnormal intrapleural tissue, 930 VLBW infants, 2031–2048 abnormally placed pulmonary tissue, 930 Neurodevelopmental outcomes, 901–902 bronchial tree abnormalities, 928 Neuroendocrine responses, 368 bronchial walls, disorders of, 928 Neurofibromatosis, 2414 bronchogenic cyst (BC), 930 Neurofurans, 450 bronchopulmonary sequestration, 930 Neuroimaging, 2091–2110, 2218, 2299–2301 congenital cystic adenomatoid malformation, arterial and venous neonatal stroke, 2095–2098 929–930 asphyxia, 2098–2100 congenital large hyperlucent lobe, 928–929 cerebellar hemorrhages, 2106–2107 pulmonary hypoplasia, 927–928 cerebral ultrasound, 243 solid and cystic lung diseases, 929–930 cognitive evaluation, 245 infections, 160 cranial transfontanellar ultrasound, 2093 mechanical abnormalities, 930–931 early evaluation of hypoxic-ischemic injury, 417–418 mortality, 160 GMH-IVH, 2103–2105 neurological outcome, 163–164 hydrocephalus, 2105 orofacial malformations hypoglycemia, 2099–2101 classification, 1256–1257 magnetic resonance imaging, 2093–2095 craniostenosis syndromes, 1260–1266 metabolic disorders, 2099–2103 facial clefts and LPS, 1266–1271 MRI, 243–245 Gill arch syndrome, 1272–1276 parenchymal hemorrhage/venous hemifacial microsomia, 1276 infarction, 2105 isolated craniostenosis, 1257–1260 predictors of outcome, 243–246 Pierre Robin sequence, 1271–1272 preterm newborn brain, 2101–2103 tongue malformation, 1276–1279 pseudocysts, 2105–2106 parenchymal conditions ultrasound, 2094–2096 arterial tree abnormalities, 924–925 white matter, premature babies, 2108–2110 congenital alveolar capillary dysplasia, 925–926 Neurological disorders, 1698 congenital chylothorax, 927 Neurological examination (NE), 240–241, 466–467, congenital pulmonary lymphangiectasia, 926–927 2056–2057 congenital surfactant protein abnormalities, ATNAT, 2059 921–924 bilirubin encephalopathy, 2077 fistulae, 925 Hammersmith neurological examination, 2062–2065 lymphatic tree abnormalities, 926 hyypoxic ischemic events and full-term infant, primary ciliary dyskinesia, 919–921 2068–2076 venous tree abnormalities, 925 IVH, 2077 smoking mothers (see Smoking) jitteriness, 2077 Neostigmine, 2332 major aims of, 2056 Nephroblastoma, see Wilms tumor metabolic disease, 2077 Nephrocalcinosis in preterm infants, 661–662 NBAS scoring form, 2060, 2062 See also Hypercalcemia neuromuscolar disorders/spinal cord disease, Nephrogenesis, 699, 1920 2077–2078 Index 2505

Prechtl screening, 2060–2061 Nevus PVL, 2076 anemicus, 2414 schemes of examinations, 2056 of Ito, 2416 screening examination, 2056 of Ota, 2416 sick newborn infant, 2067–2068 Newborn, 1924 spontaneous movement patterns, 2066–2067 ACMG expert panel recommendations, 473 stiff baby syndrome, 2077 apnea, 785 See also Electroencephalography assessment at birth (see Neonatal assessment at birth) Neurologic disability, 2033 expiration, 786 Neurologic/neurosensory, 2036, 2040–2043 infants Neuromuscular disorders (NDs) with CDH, 817 CMD, 2328–2331 heat balance (see Heat exchange) CMs, 2325 pneumonia in, 815 metabolic myopathies, 2331–2332 with RDS, 811 myasthenia, 2332 thermal delivery room management, 339–343 spinal muscular atrophy, 2333 infections, 473–474 Neuronal intestinal dysplasia (NID), 515 inhalation of carbon dioxide, 782 Neuronal migration disorders, 2003–2004, 2133 metabolic screening, 472–473 lissencephaly, 2132–2133 positioning and airway clearing, 400 polymicrogyria, 2134–2135 postnatal assessment, 401 schizencephaly, 2135 resuscitation, 398 Neuronal nuclear Ca++ influx, 2150–2151 stimulation, 400 Neuron specific enolase (NSE), 2306, 2307 Niacin, 521, 672 Neuropathology, in IDM, 725 recommended intake for preterm infant, 592 Neuropeptide Y, 510 NICE 2009 guidelines, 1371 Neuroprostanes, 450 Nicotine, 711, 713, 714 Neuroprotection, 2194, 2195 replacement therapy, 714 Neuroprotective strategies, 2185–2197 NICU network neurobehavioral scale Neutropenia, 1609, 1611, 1780 (NNNS), 2076 associated with Rh hemolytic disease, 1529 Niemann–Pick disease, 1813 autoimmune, 1530, 1535–1536 Nijmegen breakage syndrome (NBS), 1615–1616 definition, 1525, 1562 Nipples supernumerary, 2423 drug induced, 1530 Nitric oxide (NO), 507, 905, 1012 with glucose-6-phosphatase complex in preventing BPD, 905 disorders, 1556 NKX2-1, 1864 and infection, 1780 N-methyl-D-aspartate (NMDA) with poikilodermia, Clericuzio type, 1557 and cerebral damage, 2146 prognosis, 1538–1539 and enteral nervous system, 510 therapy, 1539–1543 receptors, 2146–2148 See also Hereditary, neutropenia Noblett technique, 1311 Neutrophil gelatinase-associated lipocalin (NGAL), 895, Noise, 358–359 1984–1985 Noisy open wards, 358 Neutrophils, 891, 1424–1427, 1562–1563, 1576–1581 Noma neonatorum, 1750 antibodies, 1529 Nomograms, 1148 in bone marrow, 1427 Non-autoimmune congenital myasthenic syndromes, 2332 chemotaxis, 1578 Non-coding RNAs (ncRNAs), 44–45 and chorioamnionitis, 96 Noncommunicating pneumothorax, 880 concentration, 1524–1525 Noncytotoxic innate immune cells, 1591 degranulation, 1580 Non-enzyme antioxidant factors, 445 development, 1576 Nonimmune hydrops, 1807, 1812, 1816 distribution, body, 1524 Non-immune hydrops fetalis (NIHF), 1516 gelatinase-associated lipocalin, 1984–1985 Non-nutritive sucking (NNS), 374–375, 512 granules, 1527 Non protein bound iron (NPBI), 446 intracellular killing, 1579–1580 Non punitive, anonymous, and timely reporting system, 310 life span, 1524 Non-steroidal anti-inflammatory drugs (NSAIDs), 1940 phagocytosis, 1579 neuroprotective effects, 2189–2190 transendothelial migration, 1577 See also Patent ductus arteriosus See also Neutropenia Non stress test (NST), 113–114 Neutrophil storage pool (NSP), 1524 Non-syndromal craniosynostoses, 64–65 2506 Index

Non-treponemal tests, 1705 iris and pupil, 2346–2348 Noonan syndrome, 59–60, 899, 1476, 1518, 1880 ocular bulb, 2342–2343 Norepinephrine, 510 Ocular toxoplasmosis (OT), 1713, Norovirus, 1358 1717, 1719 Norwood procedure, 1069–1072 Oculocutaneous albinism, 2415 Nose, 463 and Hermansky–Pudlak syndrome, 1556 Nosocomial infection, 307 Oligodactyly, 67 prevention, 1756 Oligodendrocytes, 2007 CVC-related infections, 1758–1759 Oligohydramnios, 54 hand hygiene, 1758 renal dysfunction, 1940 Notochord split syndrome, 1315 syndrome, 750–751 Nuchal translucency (NT), 9 Oligosaccharides, 522 Nuclear factor-kappa B (NF-κB), 525, 895, 1626 Omega-3 fatty acids, 2377 Nucleated red blood cells (NRBC), 417, 1492, Omenn’s syndrome, 2410 1494, 2302 Omics sciences, 2432 Nucleotides, 522, 527 Omphalitis, 1748–1749 Nucleus ambiguous, 511 Omphalocele Nucleus tractus solitarius, 511 cardiac and chromosomal Numbers needed to treat, 1197 anomalies, 1297 Nutrient requirements, 589 description, 1297 for VLBW infants, 592 diagnosis, 1297 See also Parenteral nutrition , 1297 Nutrition prognosis, 1298 catch-up growth, 591 surgical treatment, 1297–1298 energy requirements, 590–591 therapy, 1297 fetal weight gain, 589–590 Omphalo-enteric duct, 1311 minerals and electrolytes, 591 Ondansetron, 1232 nutrient delivery, 592 Onset severity definition, 1717, 1718 early enteral nutrition, 593 Open lung approach, 949 late enteral nutrition, 593 Open pneumothorax, 880 parenteral nutrition, 592–593 Ophthalmia, 404, 1747–1748 parenteral, 605–616 Ophthalmia neonatorum, 1747 post-discharge, 209, 619–633 Opioids, 391, 2157 protein intake, 590 Opisthotonos, 1200 protein requirements Opitz syndrome, 1914 empirical approach, 590 Opportunistic infection (OI), 1685, 1688 factorial approach, 589–590 Optic nerve hypoplasia, 1836 recommended intake, 592 and hypopituitarism, 1836 for VLBW infant, 557–582 See also Septo-optic dysplasia See also Breastfeeding Oral Nutritional support, 206, 209 feeding, 598 Nutritive sucking, 512 sweet solutions, 372–373 Oral rehydrating solution (ORS), 1366 Oral rehydration therapy (ORT), 1362, 1366 O Organic acidemias Obesity, 713 methylmalonic, 1818 Obestatin, 546–547 propionic, 1818 Obstructive apnea, 778 Organic anion transporting proteins Obstructive uropathy (OATP), 1225 PUJO, 1967–1969 Organization of perinatal care, 279–289 PUVs, 1965–1967 care of high risk newborns, 281–289 VUJO, 1969 clinical governance, 286–287 Octreotide, 1852 delivery room, 282 Ocular complication, 1719 follow-up after discharge, 287–289 Ocular defects, 1683 medical staffing, 286 Ocular malformations, 2339–2348 neonatal unit, 282–289 corneal, 2343–2345 nurse staffing, 284–286 crystalline, 2345–2346 Ornithine transcarbamylase deficiency, 1823 eyelid, 2340–2342 Orofacial malformations, newborns Index 2507

classification, 1256–1257 assessment in blood, 483–484 craniostenosis syndromes, 1260–1266 saturation by pulse oximetry facial clefts and LPS, 1266–1271 alarms in, 488 Gill arch syndrome, 1272–1276 in delivery room, 495–497 hemifacial microsomia, 1276 hyperoxemia, 489 isolated craniostenosis, 1257–1260 hypoxemia, 489 Pierre Robin sequence, 1271–1272 hypoxia, 487 tongue malformation, 1276–1279 monitoring, 484–489 Orthopedic diagnoses and syndromes, 2390 for preterm infants, 492–495 Orthopedic surgery, 2387–2390 time and sensitivity, 489 Ortho-tyrosine., 450 supplemental, 483 Ortolani’s test, 466 tissue oxygenation, 489–490 O2 saturation by pulse oximetry (SpO2) Oxygenation monitoring fetal alarms in, 488 causes, 107 hemoglobin, 484 definition, 106 hyperoxemia, 489 hypoxia (see Hypoxia) hypoxemia, 489 physiology, 106–107 hypoxia, 487 and ventilation, 2170–2171 time and sensitivity, 489 Oxygenation index (OI), 947 target ranges Oxygen saturation, 906 in delivery room, 495–497 Oxygen toxicity, 439–453 for preterm infants, 492–495 antioxidant, 452–453 Oscillatory potentials (OPs), 2366 carbonyl groups, 450 Osmolality, of amino acid solution for preterm clinical aspects, 449–453 infant, 611, 613 glutathione, 445 Osteochondrodysplasias, 67–70 hydroxyl radical, 441 achondroplasia, 67–68 iron binding proteins, 446 camptomelic dwarfism, 68 lipid peroxidation, 449–450 diastrophic dysplasia, 68–69 NADPH oxidase, 442 osteodysplastic primordial dwarfism, 69–70 non protein binding iron (NPBI), 446 osteogenesis imperfecta, 6 oxidative stress, 444–447 pseudodiastrophic dysplasia, 69 prevention, 449–453 thanatophoric dwarfism, 68 protective effect of human milk, 452 Osteodysplastic primordial dwarfism, 69–70 reactive oxygen species, 441–444 Osteogenesis imperfecta, 69 Oxyntomodulin (OXM), 540–541 Osteomyelitis, 1745–1747 clinical signs, 1746 definition, 1745 P prognosis, 1747 Pachygyria, 1813, 2115, 2117 risk factors, 1746 Packed red blood cells (PRBC), 1201 treatment, 1747 PaCO2 and high-frequency oscillatory Osteopenia of prematurity, 664–667 ventilation, 860 Osteopetrosis, 1508 Pain in neonates and anemia, 1507 anatomy, 366 Othahara syndrome, 2288 healthcare professional pain/anxiety, 370 Otitis media, 1748 parents pain/anxiety, 370 Outcomes, 1129, 1132, 1133, 1135, 2034, peripheral receptor development, 366–367 2035, 2037 procedural pain, 369–370 functional, 2046–2047 auditory recognition/music, 375 VLBW, 2039–2040 breastfeeding, 373–374 Ovine ductal ligation model, 939 faciliated tucking and swaddling, 375 Ovotesticular DSD, 1899 non-nutritive sucking (NNS), 374 Oxidant stress, 939 olfactory/aromatherapy recognition, 376 Oxidative injury, 1193 oral sweet solutions, 372–373 Oxidative stress, 360, 445 rocking, 375 biomarkers, 2304, 2305 sensorial saturation, 376 Oxidized glutathione, 1161 skin-to-skin care, 374 Oxygen, 802 stress responses, 368 2508 Index

Pain in neonates (cont.) pharmacological interactions, 1931–1932 subplate zone in cerebral cortex, 367–368 urinary tract anomalies, 1930 therapeutic tripod, 371 Patient-triggered ventilation, 854–856, 905 Palate, see Cleft lip/cleft palate and BPD, 905 Pallister–Hall syndrome, 1881 Pearson syndrome, 1507, 1508 Pallor, 426, 460 Pedersen hypothesis, 718, 719 Palpebral ptosis, 2341, 2342 Pediatric claims, 348 Pancreatectomy, 1852 Pediatric surgery, 1645 Pancreatic bile salt stimulated lipase, 516 Pegaptanib, 2373 Pancuronium, collateral effects, 1120 Pelvic kidney, 1962 Paneth cells, 506, 524, 1376 Pelviureteric junction obstruction (PUJO), 1967–1969 Panintestinal disease, 1398–1399 clinical presentation, 1967 Pantothenic acid, 672–673 differential diagnosis, 1969 recommended intake for preterm infant, 592 fetal intervention, 1968 PaO2 alveolar ventilation, 765 management, 1967–1968 Paracetamol, 391–392 pathogenesis, 1967 Parainfluenza, see Pneumonia postnatal management and prognosis, 1968 Parallel circulation/trasposition, 1058–1059 Peña’s technique, 1325 Paraneoplastic syndrome, 1861 Pendred syndrome, 1881 Parasagittal injury, 241 Penicillin Parasympathetic preganglionic fibers, 511 in congenital syphilis, 1707 Parathyroid disorders, 1858–1859 in early neonatal sepsis, 1539 Parathyroid hormone-related protein (PTHrP), 895 Pentoxifylline, 797 Parechovirus, 1786 Pepsin, 517, 895 Parenchymal molecular defects, 921–924 Pepsinogen, 517 Parental Peptidases, 518 decision making, 223–224 Peptide growth factors, 895–896 education, 208, 210 Peptide YY (PYY), 542–543 Parenteral nutrition (PN), 592–593, 605–616, 1238 Peptostreptococcus spp., 1731 amino acid solutions, 613 Percheron’s artery, 2268 lipid emulsions, 613 Pericardiocentesis, 1095–1096 recommended intakes, 606 Perinatal asphyxia, 298 in stable growing period, 610–612 hypoxic-ischemic encephalopathy (HIE), 411 in transitional period, 607–610 intrapartum asphyxial markers (see Intrapartum in VLBW infant, 592–593 asphyxial markers) Parents and family support, 212–215 Perinatal brain damage, 2187 Parent’s pain/anxiety, 370 Perinatal care Parietal cell, 517 antenatal counseling and support, 282 Partial Brown–Séquard syndrome, 2269 clinical audit, 286 Partial exchange transfusion, 1158 data collection, 287 Parvoviridae, 1695 delivery room care and resuscitation, 282 Parvovirus B19, 1695–1699 designation of neonatal services, 282–283 Patau syndrome, 56 family-centered neonatal care, 283 Patent ductus arteriosus (PDA), 477, 839, 891, 1079–1087 follow-up groups, 287–289 epidemiology, 1080–1081 guidelines, 286–287 management, 1083–1086 medical staffing, 286 persistent ductus arteriosus, 1002 models of care, 281 physiology and pathogenesis, 1081–1082 neonatal care categories, 284 Pathogen-associated molecular patterns (PAMPs), 525 neonatal transport system, 280 Pathologic unconjugated hyperbilirubinemia nurse staffing, 284–286 definition, 1153 performance of neonatal service, 287 etiology and pathogenesis, 1152–1154 perinatal outcome reporting, 288 hemolytic conditions, 1154–1156 standards, family-centered care, 283–284 immune hemolytic disease, 1156–1161 Perinatal decisions, 223 nonimmune hemolytic disease, 1161–1164 Perinatal hypoxia, 351–353 Pathophysiology, fetal and neonatal kidneys Periodic breathing, 778–782 congenital tubulopathies, 1929 Peripheral blood monocyte, 1525–1527 immature kidney, 1926–1929 Peripheral receptor development, 366–367 nephron number reduction, 1919–1932 Peripheral smear, 1482 Index 2509

Peritoneal dialysis, 1948–1949 hormonal regulation, 649–653 Peritoneal drainage (PPD)/laparotomy, 1397 magnetic resonance spectroscopy, 2177 Periventricular leukomalacia (PVL), 2007, 2076–2077, recommendations for parenteral intakes, 606 2108 requirements, 591 neuroimaging, 2108–2110 Phosphorylated insulin-like growth factor binding nodular heterotopia, 2119 protein-1 (phIGFBP1), 32–33 and oxidative stress, 360 Phosphorylation, 525 and quality of NICU, 259 Photodegradation, 1193 Periventricular nodular heterotopia (PNH), 2119, 2120 Photoisomerization, 1193 Periventricular venous infarction (PVI), 2228 Photoisomers, 1196 Permanent neonatal diabetes mellitus (PNDM), 1855, Phototherapy, 179, 1158, 1160, 1164, 1187–1190 1856, 1881 aggressive phototherapy, 1192 Peroxisomal disorders, 1813, 2100 cycled phototherapy, 1193 Persistence of pulmonary hypertension (PPHN), 829, 830 home phototherapy, 1200 Persistent intensive phototherapy, 1187, 1199 cloaca, 1321 Physical environment, water and heat exchange, 323–343 fetal circulation, 935 between infant’s body and environment, 326–328 Müllerian duct syndrome, 1909 between respiratory tract and environment, 336–338 pulmonary hypertension, 797, 933–956 Physical examination of newborns, 457–468 Persistent pulmonary hypertension of the newborn abdomen, 465 (PPHN), 298, 934 assessment of gestational age, 467, 468 clinical physiology and evaluation, 937–938 chest and auscultation of heart and lungs, 464, 465 clinical syndrome, 935 ears, 463 diseases associated with, 935 eyes, 463 nitric oxide (NO) therapy, 942–945 general observation and measurements, 460 treatment, 940–942 genitalia, 465 ventilator management, 948–949 head and face, 462, 463 Persistent/recurrent hypoglycemia, 1849–1850, 1852 hips, 466 Pertussis, vaccine, see Vaccines limbs, 466 Peter’s anomaly, 2344 mouth, 463 Peyer’s patches, 526, 1591–1592 neck, 463, 464 Pfeiffer syndrome, 65–66 nose, 463 Phagocytosis, 1579 skin, 460, 462 Pharmacodynamics, 696 spine, 465, 466 Pharmacokinetics, 696 Physical forces, 738 Pharmacological interactions, fetal and neonatal Physicians kidneys, 1931 clinical governance, 286–287 Pharmacology, 698–706 medical staffing, 286 Pharmacotherapeutic approaches, 906 nurse staffing, 284–286 Pharmacovigilance, 695, 704 Physiologic anemia, 1496 Phenobarbital, 1228, 1232, 2172, 2290 Physiologic neonatal jaundice, 1152 clearance of bilirubin, 1179 Phytomenadione, see Vitamin K Phenotypic variants, 50 , 2415 Phenylalanine, increased concentration, 1813 Pierre Robin sequence, 54, 1271–1272 Phenylephrine, 1120 Pilot Kernicterus Registry, 1161 Phenylketonuria, 1809, 1811, 1813–1815 Pinard fetal stethoscope, 122 maternal, 1813 Pinocytosis, 518 screening, 472 Pituitary hormone deficiency, see Hypopituitarism Phenytoin, 2290 PiZZ phenotype, 1236 Phimosis, 1743 Placenta, 1696, 1714 pH measurements, umblical cord blood, 413–414 circulation, 115–121 Phosphate, disorders of homeostasis, 662–663 pathology and brain damage, 2297–2299 Phosphatidylcholine (PC), 803, 811–814, 816–818, 831 Placental disfunction, 1667 Phosphatidylglycerol, 803, 831 Placental growth factor, 897 Phosphatidylinositol-glycan biosynthesis class Placentation, 131–132 F (PlGF), 36 in multiple pregnancies, 131–132 Phospholipids, 803 Placing and stepping reflexes, 467 Phosphorus, 639–667 Plagiocephaly, 64, 1260 content in milk, 600 Plan-Do-Act-Study method, 312 2510 Index

Plasma, 1127, 1131, 1201 Polycystic kidney disease (PKD), 1964–1965, 1974 acylcarnitine patterns, 1819 Polycystic ovary syndrome (PCOS), 30, 34 coagulation reactions, 1472 Polycythemia, 161, 1127, 1130, 1131, 1133 Plasmapheresis, 1233 blood flow effects, 1133 Plasminogen activator inhibitor-1 (PAI-1), 893, 895 coagulation, 1134 Plasticity, 2193, 2280 cord blood, 1128 Platelet activating factor (PAF), 1942 definition, 1128 Platelet-associated immunoglobulin G, 1482 frequency of symptoms, 1132 Platelet function analyzer 100 (PFA-100), 1482 and hyperviscosity, 724, 1125–1137 Platelets, 1424–1427, 1458, 1465 causes, 1129–1130 acquired disorders, 1481 clinical features, 1132 aggregometry, 1482 prevention and treatment, 1135–1136 clinical aspects, 1474 in IDM, 724 diameter, 1472 in neonates, 1129, 1136 disorders, 1471–1484 prevention, 1135 qualitative, 1480–1481 renal functions, 1134 quantitative, 1474–1480 risk factors, 1129 treatment, 1483–1484 screening, 1136 Wiskott–Aldrich syndrome, 161 Polydactyly, 67 function in neonates, 1473–1474, 1481–1483 Polyhydramnios, 136, 512, 919, 1334, 1517, 1669, hereditary qualitative disorders, 1480 1836, 1929, 1931 qualitative disorders, 1480–1481 Polymerase chain reaction (PCR), 1683, 1692, 1697, quantitative disorders, 1474–1480 1705, 1708, 1732 reference interval for platelet count, Polymicrogyria, 2023, 2120–2122, 2134–2135 1425–1426 Polymorphonuclear, 524 reticulated platelets, platelet distribution width Polysplenia and biliary atresia, 1245 and IPF, 1427 Polyunsaturated fatty acid (PUFA), 895 sampling site, 1424 Pompe disease (PD), 2331 thrombocytopenia, 1475 Ponderal index, 157 thrombocytosis, 1474–1475 Pontocerebellar hypoplasias, 2132 transfusions, 1483 POR deficiency (PORD), 1901 Plus disease, 2360 Porencephaly, 2140 Pneumomediastinum, 877–879 Porphyria, 2408–2410 clinical aspect and differential diagnosis, 878 Portal hypertension, 1235 definition, 877 after Kasai procedure, 1235 etiology and pathogenesis, 878 Portal vein thrombosis (PVT), 1461, 1463 risk factors, 878 Port wine stain, 461–462, 2419 treatment, 879 Position anomalies, 2345 Pneumonia, 815, 868–870, 1741–1743 Positive end expiratory pressure (PEEP), 429 aspiration, 899 Positive rolandic sharp waves (PRSW), 2085 Pneumopericardium, 882–883 Positron emission tomography (PET), 268 Pneumoperitoneum, 883–884 Postconceptional age (PCA), 2082, 2084 Pneumothorax, 862, 879–882 Post-discharge formula (PDF), 582 asymptomatic, 881 Post-discharge nutrition, preterm infants, classification, 880 619–633 clinical aspects, 880–881 breast-feeding, 631–632 definition, 879 catch-up growth, insulin resistance and visceral differential diagnosis, 881 obesity, 625–627 etiology and pathogenesis, 880 formula-feeding, 629–631 needle aspiration, 882 Posterior communicating artery (PCoA), 2268 prevention, 882 Posterior embryotoxon, 1238 risk factors, 879–880 Posterior limb of internal capsule (PLIC), 2099, 2102 selective intubation, 882 Posterior pituitary deficiency, 1837 thoracotomy, 882 Posterior sagittal ano-recto-plasty, 1325 PNH, see Periventricular nodular heterotopia (PNH) Posterior segment, malformations, 2348 Poland anomaly, 54 Posterior urethral valves (PUVs), 1951, 1974, 1975 Poliomyelitis, 1789 antenatal diagnosis, 1966 Polio vaccine, 1798 antenatal management, 1966 Poliovirus, 1786, 1788, 1789 postnatal management, 1966 Index 2511

prognosis, 1967 infants, 22–23, 76, 77, 79, 81, 83, 85, 474–476, 996, surgical treatment, 1966–1967 999, 1001, 1002 Posthemorrhagic ventricular dilatation (PHVD), 2207, asymptomatic hyperthyrotropinemia, 1871 2210–2218 formula, 601 See also Hydrocephalus human milk, 599 Post hoc analysis of preterm infants, 103 hypothyroidism with delayed TSH elevation, 1870 Posthypoxic brain injury, 2145–2146 hypothyroxinemia, 1870 Postnatal growth impairment, 162–163 morbidity and mortality, 171–181 Post-natal malnutrition and growth failure, 620–625 outcome, 186 Postnatal steroids, 1018–1019 transient congenital hypothyroidism, 1870–1871 Postprandial hyperemia, 508 and term newborn, 22–23 Potassium CNS development, clinical aspects of, 21–22 intake with parenteral nutrition, 610 pregnancy and delivery, history of, 19–21 phosphate, 614 Preterm baby Potential conflicts, infants, 223 hyperbilirubinemia, 478 Potter’s disease, 1286 hyperglycemia, 478 Potter sequence, 54–55 hypoglycemia, 478 Prader–Willi syndrome (PWS), 46, 60, 1881 intracranial hemorrhage, 478 Preauricular tags, 463 patent ductus arteriosus, 477 Prebiotics, 528 respiratory distress syndrome, 477–478 Prechtl screening neurological examination, 2061 risks for, 476 Prednisone, 1862 Preterm birth (PTB) Pre-eclampsia (PE), 33–36, 1459 adverse outcomes age, 34 cerebral palsy (CP), 2318–2320 biochemical markers, 35–36 cognitive outcomes, 2319–2321 biophysical markers, 35 morbidity determination, 2317–2319 chronic hypertension and renal disease, 34 mortality, 2316–2318 congenital and chromosomal anomalies, 35 problems in, 2317 definition, 33 sensory outcomes, 2321 family history of, 34 survival, 2316 multiple pregnancy, 35 age, 29 nulliparity, 34 ART, 31 obesity, insulin resistance, PCOS and diabetes, 34 bacterial vaginosis and intrauterine infection, 31 oocyte donation, 35 biochemical markers, 32–33 stress, 35 biophysical markers, 33 thrombophilic disorders, 34–35 cervical surgery, 30 Pregnancy-associated plasma protein A (PAPP-A), 36 definition, 28, 134 Pregnancy, endocrine disruptors in, 1909 diagnosis, 135 Pregnancy-induced hypertension (PIH), 1474, 1566 drugs and substances of abuse, 31 Prelabor rupture of fetal membranes (PROM), 97 epidemiology, 134–135 Premature infants, cholestasis, 1238 etiology, 135 Premature Infants in Need of Transfusion etnicity, 29 (PINT) study, 1508 genetic factors, 29 Premature rupture of membranes, 75 incidence and prevention, 173 and pulmonary hypoplasia, 747–750 local/systemic infections, 30 Prematurity, 208, 210, 890, 1375 multiple pregnancy, 31 Prenatal ARV regimens, 1686–1687 nutritional status, 29 Prenatal diagnosis, 52 obstetric history, 30 of congenital malformations, 52, 2127 prevention, 135 of fetal distress, 105–125 reproductive disorders, 30 screening tests, 8 risk factors, 28–33, 135, 1017, 1858 Prenatal hemorrhage, 1500–1503 short cervix and cervical insufficiency, 32 Prenatal sonography, 1651 smoking, 31 Pressure support ventilation, 859 socio-economic status, 29–30 Pressure ventilation, 794 stress, 30–31 Preterm systemic diseases, 30 delivery, 28–33, 2022 vaginal bleeding, 31–32 biochemical and genetic markers, 32–33 See also Very preterm birth risk factors, 28–29 Preterm formulas, 582 2512 Index

Preterm labor, problem of, 474–475 requirement by Preterm neonates, 536, 538, 540, 542, 543, 545, 547, 548 empirical approach, 590 Preterm neutrophils, 1579 factorial approach, 589–590 Prevention of early-onset group B streptococcal infection Protein S100, 2305, 2306 intrapartum antibiotic prophylaxis, 1754–1756 Proteomic neonatal management, 1756 nephrology, 1978–1979, 1984–1985 Prevention of premature birth, 905 oxidative stress, 450 Primary ciliary dyskinesia (PCD), 919–921 Proteus mirabilis, 1743 Primary immunodeficiency Prothrombin complex concentrate (PCC), 1450 agammaglobulinemia, 1609–1610 Prothrombin time (PT), 1437–1438 autoimmune polyendocrinopathy candidiasis Protocols, 320 ectodermal dystrophy syndrome, 1616 Proton magnetic resonance spectroscopy, 2099, and bacterial infections, 1608 2177, 2178 cartilage-hair hypoplasia, 1615 Protracted diarrhea of infancy (PDI), 519 classification, 1608 Proximal jejunostomy, 1398 diagnosis, 1608–1609 Prune belly sequence, 55 DiGeorge syndrome, 1615 Pruritus, 1231–1233 hyper IgM syndrome, 1610–1612 Pseudodiastrophic dysplasia, 69 immunodysregulation, polyendocrinopathy, Pseudoglandular phase, 735, 890 enteropathy, X-linked, 1616 Pseudohermaphroditism, see Sexual development MHC class II antigen deficiency, 1614 disorders Nijmegen breakage syndrome, 1615–1616 type 1, 1843 severe combined immunodeficiencies, 1612–1614 , 1859, 1884 Wiskott–Aldrich syndrome, 1614 Pseudomonas, 869, 1730–1731 Primary investigations in term and preterm infant, Pseudoparalysis, 63 471–479 Pseudopolyps, 901 clinical assessment findings for hypo-and Pseudopseudohypo parathyroidism, 1885 hyperthermia, 475 Psoriasis, 2413 metabolic screening, 472–473 Psychosexual development, 1896 monitoring of blood glucose, 478 Psychosocial interventions, 210–215 screening panel, 473 PTH (parathyroid hormone), see Hormones Primary peritoneal drainage (PPD)/laparotomy, 1396 PTHrP (parathyroid hormone-related protein), Primary transfer, 294 see Hormones Primitive neuroectodermal tumors (PNET), 1652, 1655 Ptosis, see Muscle disease Pro-and anti-inflammatory cytokines, 890 Puberty, 164 Probiotics, 1386, 1635 Pull-to-sit manoeuvre, 467 bacteria, 527 Pulmonary Procalcitonin, 1732 agenesis, 745–747 Procedural pain alveolar proteinosis, 753 non-pharmacological interventions, management with, atresia, 1047–1051 371–372 edema, 838 prevention, 369 and hemodynamic transition, 1040 recognition, 369–370 hemorrhage, 866–867 Progressive familial intrahepatic cholestasis (PFIC), 1211, hypertension, 933–956 1233, 1236–1238 nitric oxide, 941 Pronephros, 1920 oxidative stress, 946 Prophylactic treatment, 2291–2292 vasoactive mediators, 940 Propionic acidemia, 1817 hypoplasia, 927–930 Propranolol, 1873 lymphangiectasia, 751–753 and hypertension, 1122 physiology, 759–772 Prostacyclin, 950 airway resistance, 763–765 Prostacyclin analog, 900 alveolar ventilation, 765–766 Prostaglandin extrapulmonary shunt, 770–771 E1, 1046–1047 ventilation/perfusion ratio, 767, 771–772 E2 and tubular function, 1925 stenosis, 1048–1049 Protein, 517 trypsin-2, 895 digestion and absorption, 517–518 Pulmonary air leaks intake, 590 characterization, 874 oxidation, 450 incidence, 874 Index 2513

pneumomediastinum, 877–879 airway resistance, 763–765 pneumopericardium, 882–883 alveolar ventilation, 765–766 pneumoperitoneum, 883 chest in premature infants, 760 pneumothorax (see Pneumothorax) circulation, 767 pulmonary gas embolism, 884 diffusion, 766 pulmonary interstitial emphysema, 875–877 hypoxia causes subcutaneous emphysema, 884 abnormal diffusion, 770 Pulmonary artery banding (PAB), 1068 alterations in ventilation/perfusion ratio, Pulmonary atresia with intact ventricular septum 771–772 (PA-IVS), 1049–1051, 1075 anemia and hypoperfusion, 772 Pulmonary biomarkers, 892 extrapulmonary shunt, 770–771 Pulmonary ectopia, see Abnormally placed pulmonary hypoventilation, 769–770 tissue (APPT) lung Pulmonary edema, 889, 891 elastic properties, 761–763 Pulmonary embolism, 1463 mechanics, 760–761 Pulmonary gas embolism, 884 volume, 764 Pulmonary hemorrhage (PH) O2 and CO2 transfer, 767–769 diffuse, patchy infiltrates and right-sided ventilation/perfusion ratio, 767 atelectasis, 867 Pulmonary surfactant management of, 866 composition, 803–805 risk for, 866 isolation of, 803 surfactant therapy, 867 structure, 805 Pulmonary hypertension, 793, 898–900, 939, 964, 1010 Pulmonary underdevelopment acute, 965 abnormal intrapleural tissue (AIPT), 930 chronic, 967 abnormally placed pulmonary tissue (APPT), 930 subacute (late), 967 bronchial tree abnormalities, 928 Pulmonary hypoplasia (PH), 739, 927–928 bronchial walls, disorders of, 928 clinical aspects, 747–748 bronchogenic cyst (BC), 930 description, 747 bronchopulmonary sequestration (BPS), 930 diagnosis, 748–749 congenital cystic adenomatoid malformation (CCAM), differential diagnosis, 749 929–930 etiology and pathogenesis, 747 congenital large hyperlucent lobe, 928–929 prognosis, 750 pulmonary agenesis, 927 treatment, 749 pulmonary aplasia, 927 Pulmonary interstitial emphysema (PIE), 862, 875–877 pulmonary hypoplasia, 927–928 clinical presentation, 876 solid and cystic lung diseases, 929–930 complications, 877 Pulmonary valve stenosis (PS), 1073–1074 definition, 875 Pulmonary vascular media, 793 etiology and pathogenesis, 875–876 Pulmonary vascular resistance (PVR), 942, 1040 incidence, 875 Pulsatility index, 6, 13 and mechanical ventilation, 862 pulsatility index for veins (PIV), 120 risk factors, 875 Pulse oximetry, 123 treatment, 877 Pulses, 464 Pulmonary lymphangiectasia (PL), 751–753, 899 Pustular melanosis, 2398 Pulmonary malformations Pustule alveolar capillary dysplasia, 754–755 and infective diseases, 2399–2404 bronchopulmonary sequestration, 743–744 neonatal disorders with, 2398–2399 chylothorax, 753–754 Pustules, blisters and erosion, 2398–2399 congenital cystic adenomatoid malformation, 742–743 acrodermatitis enteropathica, 2407–2408 congenital lobar emphysema (CLE), 744–745 bullous impetigo, 2402 congenital lung cysts, 744 Candida spp., 2401 intrapulmonary cystic malformations, 740–742 congenital syphilis, 2402–2404 oligohydramnios syndrome, 750–751 epidermolysis bullosa, 2404–2405 pulmonary agenesis, 745–747 fungal infection, 2401–2402 pulmonary hypoplasia, 747–750 herpes gestationis, 2407 pulmonary lymphangiectasia, 751–753 herpes simplex, 2399–2400 Pulmonary mechanics, 712 hyper-immunoglobuline syndrome, 2408 Pulmonary outcomes, 899–901 incontinentia pigmenti, 2406–2407 Pulmonary physiology in neonates Langerhans cell histiocytosis, 2408 2514 Index

Pustules (cont.) arterial tree abnormalities, 924–925 mastocytosis, 2405–2406 congenital alveolar capillary dysplasia, 925–926 miliaria, 2399 congenital chylothorax, 927 neonatal cephalic pustulosis and neonatal acne, 2399 congenital pulmonary lymphangiectasia, 926–927 neonatal pemphigus vulgaris, 2407 congenital surfactant protein abnormalities, porphyrias, 2408–2410 921–924 scabies, 2402 fistulae, 925 staphylococcal scalded-skin syndrome, 2401 lymphatic tree abnormalities, 926 toxic erythema of the newborn, 2398 primary ciliary dyskinesia, 919–921 transient neonatal pustular melanosis, 2398 venous tree abnormalities, 925 varicella, 2399 Rare pulmonary causes of respiratory distress in neonates, Pyloric stenosis, 1301–1302 917–931 Pyloric web, 1301 congenital Pylorus, 513 lobar emphysema, 928 Pyridoxine, 521, 672, 2288 surfactant protein abnormalities, 919 metabolism error, 1823–1824 cystic lung disease, 919 Pyrimethamine, 1719 primary ciliary dyskinesia, 919–921 Pyruvate, 1820 Ratio of bilirubin to albumin (B/A), 1191 kinase deficiency, 1163, 1518 Rat models, 1375 Reactive airway disease, 900 Reactive hyperemia, 508 Q Reactive oxygen, 441–444, 889–891, 1579, 2173 Qualitative disorders, 1480 Reactive oxygen species (ROS), 441, 889, 1622–1626 hereditary disorders, 1480 excess of, 445 Quality evaluation, 299 hyperoxia, 447–449 Quality improvement (QI), 255, 319 NOX enzyme, 442–444 Quality indicators, 311 redox reactions, 445 Quasispecies, 1689 reperfusion, 444 sources of, 442 tissue damage prevention, 451–452 R Readmission, late preterm infants, 177 Radiant heaters, 328 Rebound in the TSB, 1199 Radiant infant warmer (RIW), 339–340 Recombinant activated factor VII, 1484 Radiant warmer, 398, 400 Recombinant CC10, 905 Radiation, 51 Recombinant erythropoietin (rEPO), 1474 therapy, 1646 Recombinant granulocyte colony-stimulating factor Radiograph, 915 (rG-CSF), 1562 Radiometer, 1196 administration to neonates, 1568 Ragged red fibers, 2332 randomized clinical trials testing treatment, 1569 Ramstedt’s pyloromyotomy, 1302 (see also Severe chronic neutropenia (SCN)) Randomized trials US FDA approved, 1570 postnatal steroids, 1018–1019 Recombinant superoxide dismutase (SOD), 905 prenatal corticosteroids, 1017 Recommended dietary intake (RDI), 621 Ranibizumab, 2373 Rectourethral fistula, 1324 Ranitidine Rectal temperature, 336 and neutropenia, 1530 Rectovaginal fistula, 1324 and PDA, 1082 Red blood cell (RBC) Rapid eye movement (REM) sleep, 22, 359, 783, 2084 antibodies, 1201 Rare lung diseases membrane, 1164 developmental abnormalities nucleated, 417, 2302 abnormal intrapleural tissue, 930 transfusion, 1378 abnormally placed pulmonary tissue, 930 Red cell enzyme deficiencies, 1154 bronchial tree abnormalities, 928 Red cells, 1151–1164 bronchial walls, disorders of, 928 and apnea, 1027 congenital large hyperlucent lobe, 928–929 and hematocrit, 1408 pulmonary hypoplasia, 927–928 and hydrops fetalis, 1518 solid and cystic lung diseases, 929–930 and hyperbilirubinemia, 725 mechanical abnormalities, 930–931 lifespan, 1507 parenchymal conditions and oxygen toxicity, 439–453 Index 2515

and polycythemia, 161 anomalies of the penis, 1972–1973 and pulmonary physiology, 767 classification of renal anomalies, 1960–1961 transfusion, 1027, 1130, 1509 CYR61, SSAT, Zf9, TSP-1, IL-18, Red scaly baby, 2410–2412 1985–1986 atopic dermatitis, 2409 cystatin C, 1982 ichthyoses, 2411 environmental factors, 1959 immunodeficiencies, 2410 fetal intervention, 1977–1978 psoriasis, 2413 fetal radiology, 1973–1974 seborrhoeic dermatitis, 2412 glomerular filtration rate, 1980 Reduced glutathione, 1161 kidney fusion, 1963 Reference ranges, 1406–1407 kidney ischemia, hypoxia and drug-induced blood hemoglobin concentration, 1492 toxicity, 1984 blood neutrophil concentration, 1420 KIM-1, 1985 coagulation assays, 1459 low molecular mass proteins, 1981–1982 hematocrit, 1410 metabolomics, 1986 hemoglobin, 1408, 1409 NGAL, 1984 MCV, MCH, 1412, 1413 non obstructed hydronephrosis, 1978 neutrophil, 1420, 1422 nonsyndromic, 1958–1959 platelet, 1425, 1426 obstructive uropathy, 1965–1969 Reference values, 2430 pediatric and neonatal nephrology, biomarkers in, Referral ophthalmologist, 2340 1978–1979 Reflexes postnatal radiology, 1974–1976 crossed extension reflex, 467 postnatal therapies, 1978 head’s paradoxical reflex, 429, 785 prevalence, 1960 Hering–Breuer, 785, 976, 983 prognosis, 1976–1977 irritant, 785 renal agenesis, 1961 Moro reflex, 2056 renal dysplasia, 1963–1965 palmar and plantar, 2509 syndromic, 1957–1958 pulmonary reflexes, 784–785 ureteral duplications and ectopia, 1970–1971 pupillary reflex, 359 urinary tract obstruction, 1978 rooting and sucking, 2059 VUR, 1969–1970 tonic neck reflex, 2059 Renal blood flow, fetus, 1921 upper airways, 784–785 Renal dysplasia, 1963, 1965 Refsum’s disease, 1210 aplastic/dysplastic kidney, 1963–1964 Regional anaesthesia, 387–388 cystic dysplastic kidney, 1964 Rehydration after diarrhea, 1365–1371 hypoplastic kidney, 1964 assessment of dehydrated patient, 1368 multicystic dysplastic kidney, 1964 composition of rehydrating solutions, 1367 polycystic kidney disease, 1964–1965 fluid management, 1370 Renal elimination capacity, 699 Renal Renal physiology, 1921–1932 excretion, 647–648 fetus, 1921–1924 failure newborn, 1924–1926 acute, 1781, 1935–1952 Renal replacement therapy (RRT), chronic, 1950–1952 1948–1949, 1952 glomerular function, 1921–1923 Renal vein thrombosis (RVT), 724, 1463, 1505 pathophysiology, 1921–1932 Repair, 892 tubular Reperfusion injury, 360, 444, 2173 acidosis, 1860 Reproductive function, 164 function, 1925–1926 Resistance tumors, 1653–1654 (see also Wilms tumor) antibiotic, 1781 Renal agenesis, 198, 751, 1961–1962, 1974 fetal and neonatal circulation, 1038–1040 bilateral renal agenesis, management of, 1961 forces, 763 diagnosis, 1961 index, 115 prognosis, 1962 to insulin, 626 unilateral renal agenesis, management of, 1962 lung physiology, 763–765 Renal and urinary tract malformations premature infants, 974 abnormalities of position, 1962–1963 renal vascular, 1921, 1924, 1939, 1941, 1945 albumin in urine and kidney disease, 1983 to TSH, 1865, 1867 anomalies of the bladder, 1971–1972 uterine artery, 116 2516 Index

Resistance to thyroid hormones, 1874–1875 abnormalities in lung perfusion, 838–839 Respiration monitoring, 475 biotrauma, 836 Respiratory complications, 826–827 acidosis, 426, 997 diagnostics, 829–831 acute disease differential diagnosis, 829–831 amniotic fluid aspiration, 899 genetic factors, 826–827 aspiration pneumonia, 99 high-permeability lung edema, 838 aspiration syndrome (see Meconium aspiration late-preterm and early term infants, 829 syndrome (MAS)) lung liquid clearance, delay in, 837–838 pleural effusions, 748 lung ultrasound, 829 pneumonia (see Pneumonia) patent ductus arteriosus, 839 pulmonary air leaks, 873–884, 997 pathogenesis, 836 pulmonary air leaks: pneumomediastinum, biotrauma, 836–837 877–879 hemorrhage, 838 pulmonary air leaks: pneumopericardium, 882–883 lung edema, 838 pulmonary air leaks: pneumoperitoneum, 883–884 patent ductus arteriosus, 839 pulmonary air leaks: pneumothorax, 879–884 perfusion abnormalities, 838–839 pulmonary gas embolism, 884 predisposing factors, 831–835 pulmonary hemorrhage, 838, 866–867 lung development, 835 pulmonary interstitial emphysema, 875–877 secretion of fetal liquid, 833–834 rare causes, 919 surfactant system, 831–833 transient tachypnea of the newborn, 177, 985 prevention of, 839 alkalosis, 662, 768, 1859 primary and secondary surfactant deficiency, 835–836 centre, 781 pulmonary haemorrhage, 838 collapse, duct-dependent systemic circulation, 1061 respiratory course and lung function, 827–828 depression, 385 risk factors, 825–827 distress, 795 genetic, 826–827 arteriovenous malformation, 2420 secretion of fetal lung liquid and induction of liquid double-inlet/double outlet ventricle, 1062 absorption, 833–834 left-to-right shunt lesions, 1061–1063 severity of, 829 total anomalous pulmonary venous connection, structural lung development, 834–835 1059–1060 surfactant system, 831–833 truncus arteriosus, 1060 symptoms and clinical function, 827–829, failure 980–983 complications, 861–862 course, 827–828 complications: conventional mandatory or preterm infants, 828–829 intermittent, 851–854 therapy, 997 complications: high frequency, 859–861 volutrauma and barotrauma, 837 complications: pressure support, 859 Respiratory syncytial virus (RSV), 900, 1789–1790 complications: SIMV, 856 clinical aspects, 1789 complications: SIPPV, 856–857 diagnosis, 1790 complications: triggered ventilation, 854–856 epidemiology and pathogenesis, 1789 complications: volume guarantee, 857–859 prognosis, 1790 complications: weaning, 861 therapy and treatments, 1790 drug treatments during ventilation, 863 Respiratory system, 973–976 mechanical ventilation, 843–863 congenital abnormalities, 740–755 muscles, 786 function rate, 337, 464, 477, 765 breathing, 775–787 reflexes, 784–785 chemical regulation, 782–784 head’s paradoxical reflex, 429, 785 patterns at rest, 776–778 Hering-Breuer reflexes, 785 periodic breathing and apnea, 778–782 irritant reflex, 785 respiratory muscles, 786 upper airways reflexes, 784–785 upper airways and pulmonary reflexes, support, 209, 903 (see also Mechanical ventilation; 784–785 Ventilation) malformations, 737, 740–755 Respiratory distress syndrome (RDS), 161–162, 178, 211, alveolar capillary dysplasia, 754–755 258–259, 477–478, 490, 810–815, 817, bronchopulmonary sequestration, 823–841, 980–983, 997, 999, 1001, 1003, 743–744, 919 1016, 1017, 1019, 1781 chylothorax, 753–754 Index 2517

ciliary primary dyskinesia, 919–921 choroidal involution, 2364 congenital emphysema, 744–745 classification, 2357–2361 congenital lung cysts, 744 rush disease, 2360 congenital lymphangiectasia, 926–927 severe, 2360–2361 cystic adenomatoid, 742–743, 929–930 stages, 2358–2359 cystic m., 740–742 zones, 2357–2358 oligohydramnios syndrome, 750–751 clinical presentation, 2360–2361 pulmonary agenesis, 745–747 complications, 2362–2367 pulmonary hypoplasia, 747–750, 927–930 evolution, 2361–2362 monitoring onset, 2361 carbon dioxide, 106, 782–783 cryotherapy, 2369 oxygen, 827 definition, 2354 oxygen: transcutaneous monitoring, 746 diagnostic examination, 2360 postnatal development, 464, 737 epidemiology, 2352–2353 Respiratory water functional changes, 2364–2367 evaporative heat exchange before and after genetic predisposition, 2377–2378 intubation, 337 historical background, 2351–2352 evaporative heat exchange during ophthalmologic follow-up, 2369 phototherapy, 338 and oxygen-independent factors, 2356–2357 evaporative heat loss in relation to gestational age, pain prevention, 2367 337–338 pathogenesis, 2354–2357 heat exchange during mechanical ventilation, 338 hypercapnia, 2356 Respiratory water loss (RWL), 327, 336, 337 IGF-1, 2356–2357 Resuscitation photocoagulation, 2370–2371 airways, 427 pre-retinal neovascularization, inhibition of, 2378 assessment, 400 prevention, 2375–2377 circulation, 433 progression of, 2354 documentation, 436 refractive errors, 2363 drugs, 433–434 regression, 2361 endotracheal intubation, 429–431 retinal microvasculature, preservation of, 2378 guidelines for, 425–426 risk assessment, 2369 heart rate, 428 risk factors for, 2362 indication for, 425 scleral buckling and vitrectomy, 2374–2375 meconium aspiration, 434 screening guidelines, 2367–2368 newborns, 398 severe ROP, 2360 oxygen supplementation, 431–433 stages in, 2358–2359 PEEP/CPAP, 429 strabismus, 2363 physiological and biochemical changes, 426 telemedicine approach, 2368–2369 post resuscitation care, 435–436 timing and rate of, 2361–2362 preparation and selection of risk cases, 424–425 treatment preterm infants, 434–435 anti-VEGF, 2371–2374 response to birth, 426–427 cryotheraphy, 2369 supplies and equipment, 397 photocoagulation, 2370–2371 ventilation, 428–429 scleral buckling, 2374–2375 withholding and withdrawing resuscitation, 435 visual field, loss of, 2363 See also Neonatal, resuscitation zones in, 2357–2358 Reticular dysgenesis, 1528, 1532, 1536, 1612 Retrocolis, 1200 Reticulated platelets, 1427, 1483 Retrolental fibroplasia (RLF), 2359 Reticulocytes, 1414–1416 Retrovirus, 1684 corrected count, 1416 Rett syndrome, 46 immature fraction, 1416 Reversibility, 47 Reticuloendothelial problems, 1667 Rhabdoid tumor (RT), 1643, 1650, 1652, 1654, 1860 Retinoblastoma (RB), 1643, 1657 Rhabdomyoma, 1643 Retinol, see Vitamin A Rhabdomyosarcoma (RMS), 1643, 1650–1651 Retinopathy of prematurity (ROP), 21, 162, 260, 2321, diagnostic procedures, 1651 2349–2378 treatment, 1657–1658 age at onset, 2361 types, 1650 anatomical abnormalities, 2362 RhD alloimmunization, 1161 anti-VEGF therapy, 2371–2374 RhD gene, 1157 2518 Index

Rh erythroblastosis, 1529 S Rh hemolytic disease, 1566 Saccular phase, 737, 890 Rhinovirus, 1786 Sacrococcygeal Rh isoimmunization, 1156–1159 pits, 466 Rhizomelic chondrodysplasia punctata, 1811 teratoma, 1505, 1518, 1650, 2130 Rhizopus, 2401 Safe transportation, 209 fractures, 664 Saldino–Noonan syndrome, see Noonan syndrome Riboflavin, 521, 671 Salmonella spp, 1359 Ribonucleic acid (RNA), 1691 Salmonella spp Rickets, 642, 653, 657, 664, 667, 1858 transmission, 1359 Rifampin, 1231, 1232, 1530, 1709 Saltatory syndrome, 2077 Right heart obstructive lesions, 1072–1073 Sanjad–Sakati syndrome, 1881 Right-to-left shunt lesions, 938, 1072–1073 Sarcoma, 1643, 1652 Right ventricular systolic pressure (RVSP), 900 Sarnat evaluation, 2070 Ring chromosome, 2129 Sarnat score, 2176 Risk benefit ratio, 221 Saturation monitoring, 123, 402, 475 Risk management, 305–313 Scabies, 2402 audits and patient safety walk rounds, 311 Scaphocephaly, 64, 1257, 1259 communication, 312 Scarring, 2396, 2399 epidemiology of iatrogenesis, 306–309 Scattered B cells, 1592 FMEA, 311 Schizencephaly, 2127, 2132 guidelines, protocols and checklist, 312 Schuster’s technique, 1298 iatrogenic event occurrence, 309–311 Scimitar syndrome, 743, 748 incident reporting systems, 309–310 Sclerocornea, 2344 investigation and causation analysis of incident, Screening 310–311 cystic fibrosis, 473 leadership and safety culture, 311–312 Down syndrome, 9 morbidity and mortality committee, 311 following normal birth, 282 Plan-Do-Act-Study method, 312 G6PD deficiciency, 1161–1163 prevention barriers, 309 hearing, 208, 247 prevention of MEs, 312–313 metabolic, 472–473 recovering barriers, 309 neonatal liver failure, 1217 reducing barriers, 309 pervasive developmental disorders, 2039 training, induction and competence, 312 phenylketonuria, 472, 473 Robertsonian translocation, 55 Sebaceous hyperplasia, 2397 Robinow syndrome, 1881, 1914 Seborrhoeic dermatitis, 2412 , 56 Secretin, 516 Root causes analysis, 310 Secretory diarrhea, 519 Rooting reflex, 467, 2059 Secretory IgA, 522 Rotating thromboelastogram (ROTEM), 1483 Secretory immunoglobulins, 1593–1594 Rotation-fixation process, 1317 Sedation, 389, 863 Rotavirus, 1245, 1357–1358, 1786, 1799 drugs and doses, infants, 390 Rothmund–Thompson syndrome, 2420 Seizures, 2085–2089, 2171 Royal College of Obstetricians and causes, 2288 Ginecologists, 286 clinical classification Royal College of Paediatrics and Child clonic, 2289 Health, 286 myoclonic, 2289 Rubella vaccine, 1684 subtle, 2289 Rubella virus, 51, 63, 1120, 1226, 1683–1684, 1786 tonic, 2289–2290 clinical aspects, 1683 EEG classification, 2088, 2289, 2290 congenital, 1684 etiology, 2288, 2289 diagnosis, 1683 hypoxic-ischemic encephalopathy, 2170, 2288 etiology and pathogenesis, 683, 1683 infection, 2288, 2289 prognosis, 1683–1684 intracranial hemorrhage, 2288, 2289 therapy and treatments, 1684 management and treatment, 2086, 2171–2173 transmission, 1683 metabolic, 2288 Rubinstein–Taybi syndrome, 58–59 hyperammoniemia, 1823 Rupture of membranes, prolonged, 748 hypoglycemia, 2288 Rush disease, 2360 pyridoxine-dependent epilepsy, 2291 Index 2519

other causes Severe congenital neutropenia (SCN), 1528, 1530–1532, early myoclonic encephalopathy, 2288 1550–1554 Otohara syndrome, 2288 Severe hemolytic episodes, 1161 prognosis, 2292 Severe hydrops fetalis, 1156 status epilepticus, 2086 Severe hypoxic ischemic encephalopathy, 298 Selective head cooling, 2158 Severe infantile hypophosphatasia, 1861 Selective intrauterine growth restriction of one twin Sex chromosome abnormalities, 57 (sIUGR), 139–140 Klinefelter syndrome, 1898 Selective intubation, 882 Turners’ syndrome, 57 Selective serotonin reuptake inhibitors (SSRI), 688–689, Sex differentiation, 1895 940 Sexual development disorders, 1893–1914 Selenium, 670, 671 cryptorchidism and hypospadias, 1913–1914 Sensori-motor system, 272 ethics and sex assignment, 1909–1911 Sepsis, 175, 478, 1116, 1187, 1733–1739, 1775 karyotype abnormalities, 1898–1899 anaerobic bacteria, 1731 medical management, 1911–1912 antibiotic resistance, 1738 nomenclature and classification, 1897–1898 bacterial, 1733–1739 sex determination and differentiation, clinical features, 1735 1894–1897 definition, 1733 surgical management, 1912–1913 early-onset sepsis, 79, 207, 261, 868, 1542, 1729, 46 XX disorders, 1898–1904 1756, 1761 46 XY disorders, 1904–1909 fungal, 1540 Shigella spp., 1359 incidence of, 1774 Shingles, 1676 late onset, 1540 Shock/circulatory failure, 1114, 1116 management, 1736 cardiac tamponade, 1094–1096 neonatal, 1775 definition, 1094 pathogenesis and transmission, 1733–1734 duct dependant systemic circulation lesions, 1097 prognosis, 1735 ductus arteriosus, 1043 and septic shock, 1775 septic, 1773–1782 therapy, 1735–1738 tension pneumothorax, 1096–1097 Septic types, 1094 arthritis, 1745–1747 Short shock, 1773–1782 bowel syndrome, 1308 Septo-optic dysplasia, 54, 1226, 1882 limbs, 67, 68 Sequences, 51, 53 Short chain fatty acids (SCFAs), 519, 522 Seroreverted seropositive mother, 1685 Short-term morbidity, quality in neonatal care, 256–261 SERPINA 1 gene, 1235 dystocia, 464, 720, 2078 Serratia marcescens, 2215 Shwachman–Bodian–Diamond syndrome, 1564 Serratia marcescens, 869 Shwachman–Diamond syndrome (SDS), 1528, 1532, Sertoli cells, 1895 1536, 1556, 1564 Serum C-IV, 897 Sibling, 1285, 1347, 2128, 2133 Severe chronic neutropenia (SCN), 1564 Sickle cell anemia, 473 autoimmune, 1565 Sildenafil, 900, 950 Barth syndrome, 1564 Silver–Russell syndrome, 46, 61, 1882 cartilage-hair hypoplasia, 1564 Simple pneumothorax, 880 cyclic neutropenia, 1565 Simplified alveoli, 891 glycogen storage disease type 1b, 1565 Simpson–Golabi–Behmel syndrome, 1922 Kostmann syndrome, 1564 Single intrauterine death, 140–141 medications, 1566 Sinovenous thrombosis, 2237, 2253, 2273 neutropenia not categorized as, 1564, 1566–1567 Sinus, bradycardia, 2194 screening for, 1570 Sjögren–Larsson syndrome, 1809 Shwachman–Diamond syndrome, 1564 Sjögren’s syndrome, 1107 Severe combined immunodeficiencies (SCIDs), Skeletal dysplasia, 460 1612–1614 Skin MHC class II antigen deficiency, 1614 abscess, 1751 Netherton’s syndrome, 2409, 2411 colour, 426, 772 Omenn’s syndrome, 2410 dimpling, 2078 reticular dysgenesis, 1528, 1532, 1536, 1612 infections, 1551 Wiskott–Aldrich syndrome, 1614 neonatal skin disorders, 2391–2423 2520 Index

Skin (cont.) Sodium-dependent D-glucose co-transport carrier system rash, 1752 (SGLT1), 518 temperature, 475, 772 Soft tissue development abnormality, 1256 Skin care, 1763–1764 Soft tissue sarcomas (STS), 1643, 1650–1653 Skin-to-skin (STS), 564–565 congenital fibrosarcoma, 1651 Skin-to-skin (STS) care, 374 fibroblastic tumors, 1653 See also Kangaroo mother care (KMC) rhabdoid tumor (RT), 1652 Skin-to-skin contact (SSC), 342–343 rhabdomyosarcoma, 1650–1651 parental care, 283 Solid and cystic lung diseases, 929–930 Skull Soluble intercellular adhesion molecule-1 (sICAM-1), cloverleaf, 65 895 neonatal examination, 462 Sotalol, 1520 Sleeping position, 208, 1029 , 1882 Sleep state, 780–781, 786 Soy formulas, infant, 581 Sliding sign, 914 Spastic cerebral palsy (CP), 238 Small Special care, 174 for gestational age, 158, 160, 1130 Special care needs, 209 left colon syndrome, 1348 Spectral irradiance, 1195 Small gestational age (SGA), 14, 149, 621, 624, 627 Spectral power, 1195 asymmetrical, 158 Spectroradiometer, 1187, 1196 infants, 175 Spectrum of light, 1195 symmetrical, 158 Spherocytosis, 1147, 1413 Small intestine, 513 Spina bifida, 4, 747, 1972, 2128, 2130 Small left colon syndrome, 1341 Spinal Smith–Lemli–Opitz syndrome, 62, 1263, 1810, abnormalities, 1904 1812, 1882 anaesthesia, 388 genital anomalies, 1906 cord arterial ischemic stroke, 2269 long philtrum, 58 muscular atrophy, 435, 748, 2333 microcephaly, 1906 Spinal muscular atrophy (SMA), Smoke-free workplaces, 711 2333–2334 Smoking Spine, 54, 67, 1654, 2128, 2422 behavioural aspects, 713 Spironolactone, 903 cleft lip and palate, incidence of, 712 Splanchnic nerves, 511 congenital heart defects, 712 Spleen, 159, 465, 1472 epidemiology, 710 Splenomegaly, 1225 habits, 710 Split notochord theory, 1315 Legg–Calvè–Perthes disease, 712 SPOCK2, 897 length and head circumference, 712 Spontaneous intestinal perforation (SIP), 1374 low birth weight, 712 Spontaneous motor activity, 241, 460, 2056 obesity, 713 Spontaneous preterm births (sPTB), 28, 29 pregnancy and fetus, 711 SRY gene, 1904 prenatal smoke exposure/ETS, 714 Stabilization, 295 pulmonary mechanics, 712 Stable isotopes, 812–815, 817, 818 and SIDS, 714 Staff systolic pressure, 713 education, 191, 313 tobacco smoke, pharmacology of, 711 medical, 187 , 713 Standard of care, 254, 601, 741 Smoking mothers, 709–714 STAN method, 2174 behavioral aspects, 713–714 Staphylococcal scalded-skin syndrome, 1749, 2401 congenital malformations, 712 Staphylococcus SIDS, 714 aureus, 869, 1527, 1582, 1729 Social interaction, 358, 2038 epidermidis, 1582, 1781 Sodium, 334, 434, 519, 610 Status epilepticus, 2069, 2086 balance, 606, 607 Steady state, 700 bicarbonate, 434, 1059 Stem cells, 524, 905 concentration disorders (see Hypernatremia; therapy, 2196 Hyponatremia) transplantation, 1611 excretion, 655 Steroidogenetic acute regulatory protein valproate, 1530 (StAR protein), 1901 Index 2521

Steroids, 797, 903, 1016, 1117–1118, 1235 Superior sagittal sinus, 2271 and drug metabolism, 1781 Superoxide dismutases (SOD), 441, 671, 905, 940 treatment of fetus and newborn, 1015–1019 Supportive care, 2171 randomized trials, 1018–1019 in neonates, 1646 Stickler syndrome, 54 Supraventricular tachycardia (SVT), 1053, Sticky eye, 463 1103–1105, 1518 Stiff baby syndrome, 2077 Surface area, 1187, 1197 Stillbirth, 711, 793, 1117 Surfactant(s), 259, 434, 477, 762 Stomas, 1397 administration, 206, 916 Stools, 519, 1238 and congenital diaphragmatic hernia, 817–818 Streptococcus deficit and oxygen toxicity, 448 faecalis, 1748 delivery, 905 Group A, 1748 endogenous production, 4 Group B, 869, 1730, 1774 function, 796 Guidelines GBS kinetics, 812–813, 815–818 flow chart, 1736 and meconium aspiration syndrome, 793, 796–797, neonatal sepsis, 1774 816–817 pneumoniae, 1590, 1609, 1748 molecular structure, 801–806 Stridor, 386, 827, 898, 1052, 1292 and neonatal pneumonia, 815 Stroke, 266, 350, 2095–2098 and prenatal corticosteroids, 814 neonatal, 2226 proteins–B,-C, 899 Structural heart disease, 938, 1044, 1066, 1118 pulmonary (see Pulmonary surfactant) Structural isomers, 1194 pulmonary hypoplasia, 747–750 Sturge–Weber syndrome, 938, 1044, replacement therapy, 995–1004 1066, 1118 size of the first dose, 998–999 Subarachnoid haemorrhage, 2216 surfactant preparation in clinical use, 999 Subarachnoid hematoma, 2230 timing of the first dose, 998 Subclavian artery, 64 synthetic, 806 Subcortical band heterotopia (SBH), 2115–2119 Surfactant metabolism, in neonatal lung diseases Subcutaneous emphysema, 884 CDH, 817 Subcutaneous fat necrosis (SFN), 1861 MAS, 815–817 Subcutaneous immunoglobulins (SCIG), 1610 neonatal pneumonia, 815 Subdural prenatal corticosteroids on surfactant synthesis, 814 haemorrhage, 2216 surfactant function and composition, 810–811 hemorrhage, 2216 surfactant kinetics, 812–813 Subgaleal surfactant pool size, 813 haemorrhage, 2216–2217 surfactant status, 814–815 hematomas, 1504 surfactant synthesis, secretion and clearance, Subglottic stenosis, 386, 898 811–812 Submucosal (Meissner) plexus, 510 surfactant therapy on surfactant synthesis, 813–814 Subplate zone in cerebral cortex, 367–368 Surfactant replacement therapy Substance P, 367, 508, 510 concomitant interventions, 1001–1002 Substrate, 221 efficacy, 997 Subventricular zone (SVZ), 2203 indications, 1002–1003 Sucking, 512 method of administration, 999–1000 blisters, 401 need for redosing, 1000 Sudden infant death syndrome (SIDS), 714, RDS, 997 1027–1032, 1788 size of first dose, 998–999 epidemiology, 1028–1029 timing of first dose, 998 genetic studies, 1030 type of, 1000–1001 pathology, 1029–1030 Surfactant synthesis pathophysiology, 1030 prenatal corticosteroids on, 814 prevention, 1030–1032 secretion and clearance, 811 risk factors, 1029 surfactant therapy on, 814 Sudden unexpected infant death (SUID), 1028 Surgical therapy, 1246–1247 Sudden unexpected postnatal collapse (SUPC), 564–565 Surrogate’s decision, 222 Sulphonamide, 1719 Susceptibility weighted imaging (SWI), 2104, 2261 Sunlight, 1200 sVEGFR1, 896 Superior mesenteric artery, 507 Swachman–Diamond syndrome, 1536 2522 Index

Swaddling, 375 Teratomas (TRT), 1643, 1648–1650, 1654 Swallowing, 21, 512 classification, 1649 difficulties, 178 sacrococcygeal, 1505, 1518, 2130 reflex, 512 Tertiary phase, 2191 Sweating, 328, 723 Tertiary units, 281 Swelling, 2255 Testes, 465 Swiss cheese–model, 309 Testicular Sympathetic postganglionic fibers, 511 regression, 1913 Symphalangism, 67 torsion, 465 Synactive theory, 360 Testosterone Synchronised Intermittent Ventilation (SIMV), 856 5-α-reductase, 1005 , 67, 1262, 1810, 2423 3-β-hydroxysteroid dehydrogenase, 1906 Syndromal craniosynostoses, 65–66 disorders of biosynthesis, 1905–1907 Syndromes, 51, 55–62 lipoid adrenal hyperplasia, 1906 Synthetic surfactants, 797, 806, 905 Tetanus, 1750, 1798 Syphilis, 19, 752, 1210 Tetralogy of Fallot (TOF), 1051–1052, 1072–1073 clinical manifestations, 1705 Tetraplegia, 241 congenital, 1703–1710 Tezosentan, 797 diagnosis, 1705–1706 Thalamic haemorrhage, 2215 epidemiology, 1704 Thalassemia etiology and pathogenesis, 1704 Thalassemia β, 473 follow-up, 1706 THAM, 434 treatment, 1706 Thanatophoric dwarfism, 68, 748 Systemic infection, 30, 1538, 1751, 2410 , 68 Systemic inflammatory response syndrome (SIRS), Theophylline, 1941 1629–1630 Theory of the difficult enteric recanalization, 1315 Systemic pathology, 2340–2348 Therapeutic Systemic-to-pulmonary artery shunt, 1067 index, 701 System malfunctions, 985 orphans, 696 Systolic pressure, 713 Therapeutic drug monitoring (TDM), 698, 700–702 Systolic time intervals, 938 Therapeutic hypothermia (TH), 1449, 1529 Thermal delivery room management cap/hat, 341 T combination of interventions, 342 Tachycardia, 112 delivery room temperature, 339 supraventricular, 1053, 1518 exothermic mattress, 340–341 ventricular, 1030, 1102, 1106–1107, 1845 humidified and heated gas, 341–342 Tachypnea, transient, 172, 177, 867–868, 985 interventions, 339, 340 Tachyzoite, 1713, 1714 physiological mechanisms, 339 T and B lymphocytes and macrophages, 523 radiant infant warmer, 339–340 Tandler theory, 1306 wrap, 341 Tau protein, 2307 Thermal environment, 323–343, 478 Taurine, 527, 613 Thermal homeostasis, in newborn infant, see Heat T-cell(s), 1583–1595, 1612–1614 exchange compartement defects Thermal instability, late preterm infants, 177 combined immunodeficiencies (CID), 1614 Thermoregulation, 161, 181, 607 severe combined immunodeficiencies (SCIDs), The timing of perinatal hypoxia, 351 1612–1614 Thiamine, 521, 671 T cell receptor excision circle (TRECS), 1617 Thiobarbituric acid (TBA) test, 449 Tdap vaccination, 1796 Third space fluid loss, 386 Technetium, 1868 Thompson score, 2169 Teicoplanin, 1736, 1781 Thoracocentesis, 927 Teicoplanin prophylaxis, 1781 Thoracoscopic approach, 1288–1290 Telomer, 1532 Thoracotomy, 882 Temperature measurement, 475 Thoraco-vertebral dysostoses, 66–67 Temporal biochemical changes, 2154 Tendon reflex, 467, 2077, 2333 chylothorax, 753–754 Tension pneumothorax, 880, 1096–1097 pneumothorax, 879–882 Teratologic dislocation, 2389 Thrombin activation and generation, 1458–1459 Index 2523

Thrombocytopenia, 1202, 1474–1480, 1669, 1779 Tonic seizure, 2070 causes of, 1475 Topiramate, 2291 classification of, 1475 TORCH syndrome, 1718 clinical signs of, 1475 Torsades de Pointes, 1106 due to decrease platelet production, 1475–1476 Torticollis, 466 from increased platelet destruction, 1476–1480 Total infections, 1478 anomalous pulmonary venous, 1059–1060, 1072 in mother and fetus, drug induced, 1479 body water, 698 in neonate, 1475 colonic aganglionosis, 1346 pattern of, 1475 parenteral nutrition, 607 Thrombocytopenia-associated multiple organ failure, amino acid, 610 1775, 1779 lipids, 611 Thrombocytopenic purpura, 1477, 1530, 1779 Total brain necrosis, 2166 Thrombocytosis, 1474–1475 Total serum bilirubin (TSB) level, 179 Thromboelastography (TEG), 1483 Touwen infant neurological examination Thrombomodulin (TM), 1435 (TINE), 239 Thrombophilia testing, 1466 Toxic erythema, 2398, 2403 Thrombophilic disorders, 34–35 Toxoplasma gondi infection (TgI), 1712 Thrombopoietic factors, 1484 clinical aspects, 1717 Thrombosis, 1202, 1473, 1479 differential diagnosis, 1718 Thromobcytopenia with absent radii syndrome epidemiology, 1714 (TAR), 1476 etiology and pathogenesis, 1713 Thumbs, 58, 66, 467, 2421 laboratory diagnosis, 1715 Thymus, 1587, 1615 maternal diagnosis, 1715 Thyroid newborn diagnosis, 1715–1716 stimulating hormone (see Hormones) prognosis, 1720 ultrasonography, 1868 therapy and treatments, 1718 Thyroid-releasing hormone (TRH), see Hormones Toxoplasmosis, 1711–1723 Thyrotoxicosis, 1130 clinical aspects, 1717–1718 Thyroxine, 905 pathogenesis, 1713–1714 Tibia, 1428 therapy, 1718–1720 Tidal volume, 428, 760 Trace elements, 520 Tight junctions, 524 alternant, 777 Tin-mesoporphyrin (SnMP), 1198, 1203 parenteral nutrition, 612 Tissue inhibitor of metalloproteinases (TIMP), 895 preterm human milk, 599 TIMP-1, 896 Trachea, 734 TIMP-2, 896 esophageal atresia, 1282 Tissue macrophages, 1525 Tracheal aspirates (TA), 892 Tissue oxygenation, 489–490 Tracheal occlusion To accelerate lung growth (TOTAL) T-lymphocytes trial, 956 circulating T-cells, 1584–1585 Tracheal secretions, 892 development, 1583–1584 Tracheobronchomalacia, 985 funcion, 1585–1586 Tracheomalacia, 898, 901, 1284, 1292 T-cell receptor (TCR) repertoire, 1584 Tractography, 2260 See also T-cell(s) Transanal anorectoplasty (TAP), 1324 Tobacco, 713 Transcobalamine II, 521 pharmacology of, 711 Transcranic magnetic stimulation (TMS), 270 prenatal smoke exposure/ETS, 714 , 42 Togavirus, 1683 Transcutaneous bilirubin, 1148, 1160 Tolazoline, 1941 Transendothelial migration, 1577 Toll-like receptors (TLRs), 525, 1633, 2022 Transepidermal water loss (TEWL), 326, 329 Tongue fissure, 1279 Transfer, 185, 283, 294 Tongue malformations Transforming growth factor β, 895 ankyloglossia, 1277 Transfusion, 1408, 1508–1509 macroglossia, 1278 fresh frozen plasma, 1130, 1738, 1779 microglossia and aglossia, 1279 granulocytes, 1540 tongue fissure, 1279 platelet transfusion, 1483–1484, 1614 Tongue tie, 463 red cell transfusion, 1027, 1117 See also Ankyloglossia twin-to-twin (see Twin-to-twin transfusion) 2524 Index

Transient Trophic factors, 2193 hypogammaglobulinemia, 1610 Trophic feeding, 592, 593 hypoglycemia, 1849 True hermaphroditism, 1899 tachypnea, 135, 172, 177, 753, 985 TRUFFLE study, 121 Transient myeloproliferative disorders (TMD), 1657 Truncus arteriosus, 1056, 1060, 1062, 1070 Transient neonatal diabetes mellitus (TNDM), 1855, Trypsin, 517 1856, 1881 Trypsinogen, 517 Transient tachypnea of the newborn (TTN), 177, 985 Tube feeding, 214, 548 incidence of, 867 Tuberculin skin reaction, 1708 insufficient fluid absorption, 868 Tuberculosis, 869, 1226, 1707–1710, 1742 pathophysiology of, 867 clinical patterns, 1708 in premature infants, 868 diagnosis, 1708 radiographic findings of, 868 etiology and pathogenesis, 1707 risks for, 867 prevention, 1709 surfactant deficiency, 868 therapy and treatment, 1709 Transillumination, pneumothorax, 880 Tuberous sclerosis, 2414 Transitional care, 283 Tubular duplications, 1315 Transitional hyperbilirubinemia, 1153 Tubulinopathies disorders, 2119 Transitory (benign) neonatal cholestasis, 1211 Tumor(s), 1641–1658 Translation, 192, 255 acute leukemia, 1656 Transparency anomalies, 2345 brain, 1643, 1654–1655 Transport neonates, 291–301 congenital, 2421–2422 costs, 299, 301 dermoid cysts, 2421–2422 equipment, 296 germ cell, 1643 patient stabilization, 295 gluteal granuloma, 2422 personnel, 295–296 histiocytosis, 1226, 1643, 1658 Transport systems, 1225 incidence, 1642–1643 Transposition of the great arteries (TGA), 1058–1059, Kasabach–Merritt, 1479, 2419 1070–1072 Langerhans cell histiocytosis, 1658, 2408 Transpyloric feeding, 598–599 leukemia, 1656–1657 Trans-umbilical-laparoscopically-assisted-approach, 1313 liver, 1655–1656 Transumbilical pyloromyotomy, 1302 neuroblastoma, 1226, 1643, 1646–1647 Transverse sinus, 2273 primitive neuroectodermal, 1652 Traumatic birth injuries, 2389 renal, 1643, 1653–1654 Treacher–Collins syndrome, 66 retinoblastoma, 1657 See also Franceschetti syndrome rhabdoid, 1652 Treatment sarcoma, 1652 futility, 223 teratoma, 1648–1650 parental decision making, 223–224 transient myeloproliferative disorders, 1656–1657 steps in decision making, 223–224 vascular, 2418–2419 Treponemal tests, 1706 Wilms, 1653–1654 Treponema pallidum, 1704 Tumor necrosis factor-α (TNF-α), 895 Triangular cord sign, 1227 Tumor related hypercalcemia, 1861 Tricuspid atresia, 1047, 1053, 1067, 1072 Turbulent flow, 763 Tricuspid regurgitant jet velocity (TRJV), 900 Turners’ syndrome, 57, 1898, 1899 Triggered ventilation, 854–856 congenital heart disease, 1055 Triggering receptor expressed on myeloid cells-1 foot lymphedema, 57 (TREM-1), 1635 webbed neck, 464 Triglyceride, 515, 611, 1233 Twin anemia-polycytemia sequence (TAPS), 138–139 Trigonocephaly, 63, 64, 1260 Twin births, 130 Trimethoprim, 1235, 1530 acardiac, 141, 142 Triple birth, 623 chorionicity, 131–132, 134 TRIPS score, 299 dizygotic twins, 130–131, 826 Trisomy, 55–57, 435, 1130, 1518 fetal complications, 132–134 T. 8, 56 iatrogenic twinning, 131 T. 13 (see Patau syndrome) monoamniotic twins, 142–143 T. 15, 1518 monozygotic twins, 131 T. 18 (see Edwards syndrome) preterm delivery, 134–135 T. 21 (see Down syndrome) selective feticide, 141 Index 2525

twin reversed arterial perfusion, 141–142 hernia, 465, 506 zygosity, 131–132 vein, 1039 Twin gestation research, 1378 catheterization, 1136 Twin pregnancy, fetal complications Umbilical venous (UVC), 1202 fetal growth restriction, 133–134 Unacylated ghrelin (UAG), 543–546 miscarriage and fetal loss, 132–133 Unbound bilirubin, 1148 Twin reversed arterial perfusion (TRAP) Unbound/free bilirubin, 1191 syndrome, 141–142 Unconjugated hyperbilirubinemia, treatment for, Twin-to-twin transfusion syndrome (TTTS), 64, 136–138, 1177–1181 1130, 1566 Undescended testes, 465, 1972 serial amnioreduction, 137 Unlicensed/off label, 695 Twin-twin disruption sequence, 64 Unsaturated fatty acids, 516 Type 2 diabetes, 713 Upper Type I and II pneumocytes, 890 airways Type I interferon (IFN) signalling, 2276 reflexes, 784–785 Tyrosinemia, 473, 1808, 1809, 1811, gastrointestinal bleeding, 1018 1815, 1825 limbs Tyrosinemia type I, 1825 abnormalities, 1653 neonatal examination, 466 U Upper esophageal sphincter (UES), 512 Ubiquitination, 525 Urea, 1811 Ubiquitin carboxyl-terminal esterase L1, 2305 cycle disorders, 1217 Ubiquitin carboxyl-terminal hydrolase L1 (UCHL1), Urea cycle, 1822 418–419 disorders, 1814, 1817, 1820, 1821, 1823, 1827 UDP-Glucuronosyltransferase (UGT), 1145 Ureaplasma spp., 103, 904 Ultradian rhythms, 359 urealyticum,98–99 Ultrasonography, 109 Uremia and/or renal failure, 1481 Ultrasound Ureteric bud (UB), 1920 abdominal, 1227, 1234 Ureterocele, 1970–1971 antenatal diagnosis Uridine 5'-phospho-glucuronosyltransfererase 1A1 congenital heart disease, 1066 (UGT1A1), 1153, 1160, 1162, 1164 hydronephrosis, 1974 Urinalysis, 1943 cardiac defects, 1067 Urinary lactate/creatine ratio, 416–417 cerebral, 243 Urinary tract cranial, 206, 259 anomalies, 1930, 1955–1987 Dandy–Walker malformation, 2131 anomalies of the bladder, 1971–1972 Down syndrome, 55 anomalies of the penis, 1972–1973 fetal growth assessment, 134 classification, 1960–1961 hip, 282 differential diagnosis, 1973–1976 hydrops fetalis, 1516 etiology, 1957–1959 microcephaly, 2137 laboratory management, 1978–1987 neural tube defects, 2128–2130 lactate/creatinine, 416–417 PVL, 243 obstructive uropathy, 1965–1969 transvaginal, 135, 2130 renal agenesis, 1961–1692 umbilical artery, 116, 117 treatment options, 1977–1978 Ultraviolet B (UVB) radiation, 1232–1233 ureteral duplications and ectopia, 1970–1971 Ultraviolet (UV) light, 1194 vesicoureteral reflux (VUR), 1969–1970, 1976–1977 Ulysses syndrome, 2431 Urinary tract infections, 1743–1745 Umbilical clinical signs, 1744 artery diagnosis, 1744 catheterization pathogens, 1743 catheterization: single, 53, 465 treatment, 1744 pulsatility index, 117 Urine cord osmolality, 1944 blood output of newborns, 608 blood: acidemia, 124, 413–414 specific gravity, 608 blood: sampling, 412 Urioste syndrome, 1882 clamping, 1129 Ursodeoxycholic acid (UDCA), 1215, 1231–1232 prolapse, 12 Urticaria, 460, 2411 2526 Index

U.S. Preventive Services Task Force, 902 assist control ventilation, 856–857 Uterine blood volume and blood pressure, 434 artery Doppler wayform, 34, 35 complications of intubation, 861–862 artery resistance index, 116 endotracheal tubes, 397 rupture, 2297 extubation, 862 Utero-placental vessels, 115–116 HFOV, 205–206, 859–861 intubation for, 429–431 IPPV, 1024 V ventilator settings, 849 Vaccines patient-triggered, 854–856 BCG, 1612 pressure support ventilation, 859 conjugate meningococcal group C, 1798, 1799 tracheostomy, 209 diphtheria-tetanus-pertussis, 1689, 1798 volume guarantee ventilation, 857–859 Haemophilus influenzae type b (Hib), weaning from, 209, 861 1689, 1798 Ventilation/perfusion ratio, 767, 771–772 hepatitis B (HBV), 1692–1695, 1798–1799 Ventilator associated pneumonia (VAP), 869, 1742 influenza, 1799 Ventilator-induced injury, 891 measles-mumps-rubella, 1689 Ventilator-induced lung injury, 948 and varicella, 1799 Ventilatory support, 209 polio, 1612, 1798 Ventricular rotavirus, 1357–1358, 1799 septal defect, 1061–1062 VAC device, 1298 tachycardia, 1030, 1102, 1106–1107, 1845 VACTERL association, 1283 Ventricular fibrillation, 1107 VACTERLS association, 52–53 Ventriculomegaly (VM), 2127, 2139–2140, 2213 Vaginoplasty, 1912 Vermont Oxford Network (VON), 254, 255, 287 Vagus nerve, 511 Vertebral anomalies, 748 Vancomycin, 1781 Very long-chain acyl-coenzyme A dehydrogenase Vanishing testis syndrome (VTS), 1913 deficiency (VLCAD), 1883 Varicella infection Very low birth weight (VLBW), 1774, 1776, 1777, clinical manifestations, 1677 1780, 1781, 2033, 2038, 2046, 2316 complications, 1677 dystonia, 241 diagnosis, 1677–1678 outcomes, 2039–2040 epidemiology, 1676 Very low birth weight infants (VLBWI), 212, 358, pathogenesis, 1676 620–623, 626, 632, 1418, 1420, 1508 treatment, 1678 Very preterm birth Varicella zoster, 63, 1676–1678 adult outcomes, 234 Vascular disruption, 63–64 behavioural outcomes, 232 Vascular endothelial cells, 1625 brain injury after, 230 Vascular endothelial growth factor (VEGF), 896, 1636, cognition and, 230–232 2352, 2354 and developmental changes after, 230 Vascular malformations, 2418 lung function, 230 congenital alveolar capillary dysplasia (ACD), motor function, 233 925–926 somatic outcomes, 233–234 congenital chylothorax, 927 Vesicoureteral reflux (VUR), 1744, 1969–1970, congenital pulmonary lymphangiectasia (CPL), 1975–1977 926–927 postnatal management, 1970 fistulae, 925 prognosis, 1970 lymphatic tree abnormalities, 926 Vesicoureteric junction obstruction (VUJO) venous tree abnormalities, 925 diagnosis, 1969 Vascular tumors, 1479, 2418–2419 management, 1969 Vasculopathy, 2297 Vibrio cholerae, 1359 Vasoactive intestinal peptide (VIP), 510 Video-assisted approach, 1304 Vasopressin, 1118 Video-assisted thoracoscopy (VATS), 741 VATER association, 1284, 1882 Villi, 505 VATER syndrome, 1284 Villitis of unknown etiology (VUE), 100 Vehicles for transport, 296–297 Viral infection(s), 1217, 1219–1220, 1785–1790 Venous malformations, 2420 Coxsackie viruses, 1226, 1478, 1786 Venous tree abnormalities, 925 cytomegalovirus, 1663–1678 Ventilation, 843–863 enteroviruses, 1786–1789 Index 2527

HBV, 1692–1695 Volume-targeted ventilation, 905 HCV, 1689–1692 Volume therapy, 433–434 herpes viruses Volutrauma, 429, 837, 846, 948 herpes simplex virus, 1671–1676 Volvulus, 1320 HIV, 19, 600, 1684–1689 neonatorum, 1318, 1319 human parvovirus B19, 1695–1699 Vomiting, 1334 influenza and parainfluenza, 1742 Von Gierke disease, 1565 measles, 1689, 1799 Von Willebrand disease (VWD), 1443 mumps, 1689, 1799 Von Willebrand factor, 1458 neonatal hepatitis, 1236 in Neonatal Intensive Care Unit, 1786 respiratory syncytial virus (RSV), 1789–1790 W rubella, 63, 1683–1684 , 2415 varicella, zoster, 63, 1676–1678 WAGR syndrome, 1885 Viral tissue tropism, 1665 Walker–Warburg syndrome (WWS), 2129–2131, 2329 Viremia detection, 1686 Wallerian degeneration, 2257 Virilization, 1907 Warburg syndrome, 2117 Virilization Warm response, 336 and adrenal insufficiency, 1841–1842 WAS mutation, 1555 Vision in preterm infant, 246 Water, 519 Visual insensible loss, 336 disability, 239 Watershed dysfunction, 2279 injury, 2266 evaluation, 246 lesions, 2167 impairment, 246 Water-soluble vitamins, 521 system, 272–273 Werdnig–Hoffmann syndrome, 435, 2333 Vitamin, 520, 671–674 WHIM syndrome, 1555 parenteral nutrition solutions, 612 White lung, 914 Vitamin A, 327, 520, 672–674 White matter injury breast milk, 599 and asphyxia, 2098–2099 fortified breast milk, 600 and perinatal vulnerability, 1999–2014 preterm formula, 601 and virus infections, 1786 Vitamin B, 671–673 WHO, ORS (oral rehydrating solution), 1367 breast milk, 520 Williams syndrome, 1861, 1885 deficiency, 520 cholestasis, 1210 recommended intake, 672 Wilms tumor (WT), 1653–1655, 1964 Vitamin B1, 521 Wilson–Mikity syndrome, 830, 899 Vitamin B2, 521 Window of vulnerability, 77, 82, 86 Vitamin B6, 521 Wingspread classification, ano-rectal Vitamin B12, 521 malformations, 1322 Vitamin C, 521, 2376–2377 Wiskott–Aldrich syndrome (WAS), 1476, 1609, 1614 Vitamin D, 520, 640, 650–652 Withholding/withdrawing life sustaining treatment, 195 deficiency, 651, 652, 654 decision making, 194 Vitamin D-dependent rickets, type I (VDDRI), 1883 guidance, 192 Vitamin D-dependent rickets type II (VDDRII), 1883 withdrawal process, 197 Vitamin E, 452–453, 674, 905, 2375–2376 Withhold/terminate treatment, 220 Vitamin K, 405, 520, 521, 674 Wolffian ducts, 1895 deficiency, 405 Wolff–Parkinson–white syndrome, 1053, 1103 recommended intake, 405 Wolf–Hirschhorn syndrome, 56 Vitamin K deficiency bleeding (VKDB), 1445–1449 Wolfram syndrome, 1836 Vitamin K epoxide reductase complex 1 (VKORC1), 2206 Wolman disease, 1811 VKORC1, see Vitamin K epoxide reductase Work of breathing (WOB), 973, 977 complex 1 (VKORC1) World Health Organization (WHO), 1648 VLBW, see Very low birth weight (VLBW) Wound infection, 1087 V’max25, 901 Vocal cord paralysis, 1087 Voiding cystourethrogram, 1745 X Volume guarantee ventilation, 857–859 Xanthine oxido-reductase system, 442 Volume of packed red blood cells (VPRC), 1408 Xeroderma pigmentosum, 2415 2528 Index

X-inactivation, 1162 Z X-linked , 1210, 1226, 1813, , 1843 1922, 2100 agammaglobulinemia, 1609–1610 Zidovudine (ZDV) prophylaxis, 1686–1688 chronic granulomatous disease, 1534–1535 Zinc, 671 hydrocephalus, 2139–2140 enteral rehydration, 1366 XX males, 1900 human milk formula, 670 XY females, 1904 recommended intake, 606 XY gonadal dysgenesis, 1904–1905 Zoster infection congenital varicella syndrome, 1677 maternal infection control, 1678 Y YKL-40, 896 Yolk sac, 505