Index

A Acquired isolated proximal RTA, 1286 Abdominal distension, 605 Acromegaly, 1976 Abdominal mass, 605–606 ACTH, 1070–1071 Abdominal pressure, 1788 α-actinin-4, 815 Abnormal kidney number/location, 680 Actin-regulating protein Rho-GAP 24, 816 Abnormal LUT anatomy, 1794–1797 Action myoclonus-renal failure syndrome (AMRF), 822 Abnormal voiding, 1794 Active transport, 64 Aboriginal communities, 961 Activity and chronicity indexes, 1460–1461 Accelerated atherosclerosis, 1475 ACTN4, 815 ACE2, 1956 Acute catheter placement, 2158 ACEI. See Angiotensin conting enzyme inhibitors (ACEI) Acute cystitis, 1696 Acellular tissue matrices, 577 Acute glomerulonephritis, 707, 959–975 Acetate, 264, 2233 Acute kidney injury (AKI), 707, 720, 1130–1131, Acetazolamide, 1286 2101–2125, 2139–2161, 2173–2174, Acetohydroxamic acid, 1832 2617–2622 Achondroplasia, 285 parenchymal injury, 1696 Acid, 248–250 Acute Kidney Injury Network (AKIN), 2140 Acid-base balance, 223–224, 234–235, 1277–1278, Acute metabolic acidosis, 235 2241–2242 Acute nephritic syndrome, 960, 1003 in CKD, 272–273 Acute pyelonephritis, 1696 in neonates, infants and children, 268–271 Acute renal failure, 843 and nutrition, 267–268 Acute stress, 1968 Acid-base disorders, 414 Acute symptomatic stone events, 1853–1855 hypokalemia and, 406 Acute tubular necrosis (ATN), 2116, 2146, 2147, 2563 Acid-base handling, in distal nephron, 408 Acute tubulointerstitial nephritis, 1411–1420 Acid-base homeostasis, 250, 407–415 etiologic classification of, 1415 daily acid load, 408 prognosis, 1420 perturbations, 408 Acute tubulointerstitial nephritis with uveitis syndrome, respiratory, 409 1361, 1376 simple, 408–409 Acyclovir, 1668–1669 Acid load, 268 Adaptation, nephron, 2178 Acidosis, 2144, 2175, 2190, 2219, 2233 ADCK4, 821 Acquired forms, of Fanconi syndrome Addison's syndrome, 1291 acute tubulointerstitial nephritis with uveitis syndrome, Adefovir, 1378, 1670 1376 Adenine phosphoribosyl transferase (APRT) deficiency, ankylosing spondylitis, 1376–1377 1594 anorexia nervosa, 1377 Adenosine, 1960, 2106 autoimmune interstitial nephritis, 1377 Adhesions, 998 distal renal tubular acidosis, 1376 molecules, 987 legionella pneumonia, 1377 Administrative AKI diagnostic codes, 2144 membranous nephropathy, 1377 ADPKD. See Autosomal dominant polycystic kidney multiple myeloma, 1376 disease paroxysmal nocturnal hemoglobinuria, 1376 α-1 adrenergic blockers, 1809 Sjögren syndrome, 1376 Adrenomedullin, 195

# Springer-Verlag Berlin Heidelberg 2016 2697 E.D. Avner et al. (eds.), Pediatric Nephrology, DOI 10.1007/978-3-662-43596-0 2698 Index

Adrenomedullin peptide (ADM), 1960 Aminoaciduria, 1357–1358, 1369 Adult renal cells, 545–547 Aminoglycosides, 2144 Adult renal stem/progenitor cells, 545–555 Amlodipine, 2050 African Association of Nephrology (AFRAN), 2614 Ammonia, 252, 1278, 1410 Age, 963 production and transport, 254–255 Air embolism, 2444 Ammoniagenesis, 1528 AKI follow-up, 2161 Ammonium, 250, 1278 Alagille syndrome, 793 Amniotic fluid (AF), 99 Alarm therapy, 1804 Amniotic fluid and placental stem cells (AFPSC), 576 Albright's hereditary osteodystrophy (AHO), 308 Amniotic fluid index (AFI), 116–117 Albuminuria, 1529–1530 Amniotic fluid volume, 116, 128 Albuminuria category, 2213 Amphotericin, 1290 Albuterol, 223 Amphotericin B, 1671–1672 Aldosterone, 192, 263, 964, 1957, 2241 Amyloidosis, 2445 deficiency, 1291 ANA. See Antinuclear antibody (ANA) resistance, 1292 Anaphylatoxin, 900, 905, 928, 944 Alemtuzumab, 2558 ANCA. See Anti-neutrophil cytoplasmic antibody Alfacalcidol, 2234 (ANCA) Algorithm, 2003 Anemia in CKD, 2220–2221 Alkaline phosphatase, 2231–2232 in chronic kidney disease, 2222 Alkalosis, metabolic, 402–403 definition, 2221–2222 Alkylating agents, 857–860, 865, 1066 evaluation, 2223–2225 Allergy, 841 hemoglobin values in children, 2222 Allograft rejection pathophysiology, 2222–2223 acute rejection, 2478–2479 treatment, 2225–2228 chronic rejection, 2479–2480 Anemia, renal transplantation complication, 2590–2591 effector mechanisms, 2474–2478 Angiogenesis, 915 hyperacute rejection, 2478 Angiokeratoma corporis, 1596 Allorecognition, 2557 Angiomyolipomas, 686 direct pathway of, 2460–2462 Angioplasty, 2006 indirect pathway of, 2462, 2464 Angiotensin, 196 MHC, 2458–2459 Angiotensin converting enzyme (ACE) gene, 1556 semi-direct pathway of, 2464 Angiotensin converting enzyme inhibitors (ACEI), Alpha-adrenergic blockers, 2048 708, 722, 866, 1001, 1954, 2053–2055, Alpha-1 antitrypsin deficiency, 792–793 2219, 2239 Alpha intercalated cell, 1279 Angiotensin II, 263, 993, 1550 Alpha-methyldopa, 2048 subtype 1 receptor, 995 Alport syndrome, 607, 779–786, 817 Angiotensin receptor blockers (ARB), 866–867, 1001, Alstrom syndrome, 794–795 2055–2056, 2219 Alternate pathway, 962 Angiotensin type 1 receptor, 1755 Alternative complement pathway, 989 Anillin, 816 Alternative designs, 483 Anion exchanger (AE1), mutations in, 1288 Alternative hypothesis, 474 Anion gap, 260, 1359 Alternative pathway (AP), 930–933 Δ Anion gap, 413 Aluminum, 2234 Anionic charge, 851 Alveolar capillary dysplasia with misalignment of Ankylosing spondylitis (AS), 1376–1377 pulmonary veins (ACDMPV) syndrome, 1765 ANLN, 816 Ambulatory blood pressure monitoring (ABPM), 1532, Anorexia nervosa, 1361, 1377 1909, 2000, 2026–2027, 2236 ANOVA, 489 Ameliorate nephrocalcinosis, 1835 Antenatal Bartter syndrome, 227, 1203–1205 American Academy of Pediatrics, oral rehydration clinical manifestations, 1205–1207 recommendations, 381–382 differential diagnosis, 1207 American diet, 348 with sensorineural deafness, 1209–1211 American Society of Pediatric Nephrology, 2666 Antenatal renal hypoplasia, 116 Amiloride, 1221 Anterior lenticonus, 781 Amino acid transport Antibiotics, 967–968 molecular structure of, 1160 dosing, pediatric renal patients, 2281–2288 regulation of, 1158–1160 of staphylococcal infection, 971 Amino acid transporter branches I and II, 1162 treatment, 974 Index 2699

Antibiotic therapy, 1832 APRT deficiency Antibody, 884 description, 1844 Antibody-mediated allograft rejection (AMR), enzyme activity, 1846–1847 952–953 prevalence, 1844 Antibody-mediated immunity, 884, 1409 renal histological examination, 1847 Anti-C5 antibody, 908 treatment, 1847 Anticardiolipin antibody, 992 APSGN superimposed in IgA nephropathy, 967 Anticholinergics, 1801, 1804, 1808 AP-1 transcription factor, 810 Anti-coagulation, 2159–2160 AQP2, normal physiology of, 1310 Anticonvulsants, pediatric renal patients, 2286–2287 Aquaporins, 183 Anti-DNA, 963 Arachidonic acid (AA), 919, 2105–2106 Anti-DNAse B, 967 ARB, 2239 Antiemetic treatment, 1854 Arbeitsgemeinschaft fur pediatrische Nephrologie, Anti-factor H autoantibodies, 949 855, 859 Antifungal agents, pediatric renal patients, 2285 Archivos Latinoamericanos de Nefrologia Pediatrica, Anti-GBM antibody nephritis, 786, 893 2632 Antigen-antibody reactivity, 960 Argentina, 2633 Antigen presenting cells (APCs), 2557 Arginine vasopressin (AVP), 190–191, 196, 199, Antigen-presenting cells (APCs), 2459–2471 200, 1309–1312 Anti-glycan antibodies, 993 ARHGAP24, 816 Anti-hypertensive medications, 968, 2041–2045, ARHGDIA, 823 2286–2287 Aromatic amino acid transporters, 1167 Anti-IgG, 963 ARPKD. See Autosomal recessive polycystic kidney Anti-IgG deposits, 965 disease Anti-inflammatory diet, kidney disease, 350–351 Array comparative genomic hybridization (aCGH), 431 Antimicrobial prophylaxis Arrhythmia, 2236 agents, 1730–1731 Arterial or arteriolar sclerosis, 998 controveries, 1732–1733 Arterial stiffness, 1926 limitations, 1731–1732 Arterio-venous (a-v) fistula, 2446–2447 recent randomized trials, 1732 Artificial sphincter, 1803 Anti-MPO antibody, 901 ASCT subfamily, 1160–1161 Antimuscarinic therapies, 1809 Ask-Upmark kidney, 2013 Antinatriuretic effect, 193 ASPN Anti-neutrophil cytoplasmic antibody (ANCA), clinical care and workforce updates, 2667–2669 707, 719–720, 963, 973, 992 education, 2670–2671 glomerulonephritis, 900 mission, 2666 positivity, 707 organizational structure, 2666 Antinuclear antibody (ANA), 715 research, 2669–2670 Antioxidants, 922 Assent, 496 Anti-phospholipid (APL) antibodies, 733, 1454–1456 Association, 157 Anti-PLA2R antibody, 952 Association for Assessment and Accreditation of Antisera, 714 Laboratory Animal Care International, 448 Antistreptococcal antibodies, 967 Asymptomatic hyponatremia, 387–388 Antistreptolysin O, 967 Asymptomatic proteinuria, 1064–1065 Antithrombin III, 843 Atgamfi, 2554–2555 Anti-Thy1 nephritis, 887, 910, 914 Atherosclerosis, 2236, 2238 Antituberculosis agents, pediatric renal patients, 2285 ATP depletion, 1661 Antiviral agents, pediatric renal patients, 2285 Atrial natriuretic peptide (ANP), 193, 964, 1958 Antiviral drugs, 1668 Atrial receptors, 189 Antiviral therapy, 741 Atubular glomeruli, 1332 ANZDATA Registry Australian and New Zealand Atypical hemolytic uremic syndrome (aHUS), Paediatric Nephrology Association (ANZPNA) 908, 948–949, 1503–1504, 1509 Registry, 2673–2678 Auscultatory measurements, 1908 Apical ectodermal ridge (AER), 280 Australia, 2673 APOL1, 825 Australian and New Zealand Dialysis and Transplant Apolipoprotein L1 (APOL1), 2217 Registry (ANZDATA), 2676 Apoptosis, 994, 1131, 1659 Autoantibody, 441–442, 893 Apparent mineralocorticoid excess (AME), 265, 404, Autoimmune, 1222 1970, 2067 Autoimmune interstitial nephritis, 1361, 1377 2700 Index

Autoimmune pluriglandular hypoparathyroidism, Barttin, 1210 302–303 Base excess, 258–259 Autoimmune reactivity, 963 Baseline SCr, 2142 Automated PD (APD), 2382 Basiliximab, 2556 Autonomic dysreflexia, 1977 B cell, 893 Autosomal dominant (type Ia), 1288 B-cell apoptosis defect, 1466 Autosomal dominant Fanconi syndrome, 1361, 1375 Bcl-2, 994 Autosomal dominant hypocalcemia (ADH), 1222, Beckwith-Wiedemann syndrome (BWS), 1879–1880 1225–1226 Behçet disease, 1444–1445 Autosomal dominant polycystic kidney disease, 1835 Belatacept Evaluation of Nephroprotection and Efficacy as Autosomal dominant polycystic kidney disease a First-line Immunosuppression Trial (ADPKD), 685 (BENEFIT), 2557 clinical features, 1118–1121 Belimumab, 1474 diagnosis, 1121–1122 Belize, 2633 epidemiology and genetics, 1117–1118 Beta-adrenergic blockers, 2048–2049 pathology, 1118 Beta-blockers, 2239–2240 prognosis of, 1123–1124 Beta intercalated cells, 1278 treatment and complications, 1122–1123 Beta-2-microglobulin, 2436 Autosomal dominant renal-coloboma syndrome Bevacizuma, 1675–1676 (RCS), 122 Bicarbonate, 248–249, 256–258, 264, 2242 Autosomal recessive distal RTA, 1289 Bicarbonate reclamation, 1275 Autosomal recessive Fanconi syndrome, NaPi-IIa gene Bilateral disease, 1892–1893 mutation, 1361, 1375 Bilateral hydronephrosis, 109 Autosomal recessive polycystic kidney disease Bilateral renal agenesis (BRA), 120 (ARPKD), 685 Bilateral renal hypoplasia/dysplasia (BRHD), 120 biliary ductal plate malformation, 1106 Bioavailability, 2269 clinical features, 1109–1111 Biofeedback, 1802 congenital hepatic fibrosis, 1109 Biofilms, 1831 diagnosis, 1111–1112 Bioimpedance, 188 epidemiology and genetics, 1106–1107 Bioinformatics, 434–437 hepatobiliary lesions, 1109 Biomarkers, 1004–1005, 2150 hepatorenal fibrocystic disorder, 1106 renal injury, 1730 pathology, 1107–1109 Biomaterials, 576–577 prognosis of, 1115–1116 acellular tissue matrices, 577 renal collecting ducts, cystic dilations of, 1106 naturally derived, 577 treatment and complications, 1112–1115 synthetic polymer, 577–578 Autosomal recessive with (type Ib) hearing loss, 1288 Biopsy needle, 709 Avascular necrosis (AN), 2589 Birmingham Reflux Study, 1718 Axenfeld-Rieger syndrome, 1765 Birth weight, 1918–1919 Azathioprine, 1010–1011, 1470 Bisoprolol, 2049 Azathioprine (AZA), 2560–2561 Bisphosphonates, 857 Azoospermia, 860 BK virus (BKV), 741, 2578–2579 Bladder, 579–580 abnormalities, 683–684 B augmentation, 1802 B7.1 (CD80), 853 capacity, 1786 Bacterial infections diary, 1790 in nephrotic children, 844, 845 dysfunction, after kidney transplantation, 1811 renal transplantation, 2574–2575 exstrophy, 1794–1795 Bacterial virulence factors, 1697 function, 1782 Bahrain, 2651 inflammation, 1788 Bangladesh, 2643 malfunction, 1780 Barakat syndrome, 307 neck suspension, 1803 Bardet-Biedl syndrome (BBS), 794 pain, 1789 Barker hypothesis, 2277 Bladder bowel dysfunction (BBD), 1789 Baroreceptors, 189 renal injury risk factors, 1722 Bartonella henselae infection, 2579 Blinding/masking, 480 Bartter and Gitelman syndrome, 405 Blocking, 482 Bartter-like syndrome, 1222, 1226 Blood-borne viruses, 2451–2452 Index 2701

Blood gas results, 256–260 Calcium-sensing receptor (CaSR), 292, 1221, 1838 Blood pressure (BP), 1554, 2031, 2219, 2226 gain-of-function, 1222 control, 1558 loss-of-function, 1222 in GS, 1219–1220 Caloric, 2244 variability, 1909 Cameron index, 842 Blood pressure load (BPL), 1911 Campath-1H, 2556 Bloom's syndrome, 1880 Canadian Association of Pediatric Nephrology B lymphocytes, 741, 1452–1453 (CAPN), 2666 BMD. See Bone mineral density (BMD) clinical care and workforce updates, 2667–2669 BMP. See Bone morphogenetic protein (BMP) education, 2670–2671 Body mass index (BMI), 638 mission, 2666 Body water, 363–365 research, 2669–2670 Bolivia, 2633 Cancer risk, 1476 Bone, 1393, 2243 Cancer seeding, 712 age, 646, 2247 Cancer stem cells, in kidney, 555 biopsy, 2232 CaOx crystallization inhibitors, 1395 deformity, 2233 Capillary lumen filling up, 717 loss and fracture risk, 2588 Capillary thrombosis, 949 marrow, 988, 1366 Captopril, 867 marrow depression, 860 Carbapenems, 1665 metabolism in CKD, 2176 Carbonate, 2233 and mineral factors, measurement frequency, Carbonic acid, 268 2231–2233 Carbonic anhydrase (CA), 252 Bone mineral density (BMD), 2229 Carbonic anhydrase II, 1290 Bone morphogenetic protein (BMP), 280 Carboplatin, 1672–1674 Botulinum-A toxin (BTX-A), 1801–1802, 1809 Carboxypeptidase N, 939 Bowman's space filling up, 719 Cardiac index (CI), 1952 Bradykinin release syndrome (BRS), 2160 Cardiac output (CO), 1952 Brazil, 2634 Cardiac surgery, 2143 Breakthrough UTI (BT-UTI), 1737 Cardiovascular, 2227, 2235–2241 Breast feeding, 1921 Cardiovascular complications, renal transplantation, 2580 Bronchopulmonary dysplasia (BPD), 1977 Cardiovascular disease, 2010, 2012, 2358–2360 BSND, 1210 Cardiovascular disease with CKD Buffering system, 249–250 evaluation and treatment, 2238–2241 pathophysiology, 2236 prevalence, 2236–2238 C risk factors, 2235 C1, 967 Carnitine, 2223, 2452 C3, 967, 988 Carotid, 2238 C4, 967, 989 arch receptors, 190 Cadmium, 1378, 1681 artery intima-media thickness, 1924 Caffeine, 2033–2034 Carotid intimal medial thickness (cIMT), 2238 CAKUT, 817 CARPEDIEM, 2437 Calcimimetics, 2235, 2367 Carvedilol, 2049 Calcimimetic therapy, 2367 Case-control studies, 478–480 Calcineurin inhibitors, 1067, 1511, 1974 Case series, 477 cyclosporine A, 1676, 2558–2559 Casual blood pressure, 1910–1911 NAPRTCS analysis, 2560 β-catenin, 280, 1876 tacrolimus, 2559–2560 Catheters, 2249–2250 Calciphylaxis, 2360–2361 Catheter size, 2158 Calcitriol, 2229, 2232, 2234–2235 Cationic aminoaciduria, 1169, 1172–1179 Calcium, 1921, 2038, 2228 Cationic antigens, 962 handling, developmental changes in, 345 Cat-scratch disease, 2579 and phosphate metabolism, 648 Causal inference, 475 and phosphorus diets, 2361–2362 C5b-9, 949 recommended intake of, 346 C4b2a, 933, 935 Calcium channel blockers (CCB), 2050–2052, 2154, 2240 C4b2a3b, 935 Calcium homeostatsis, 272 C3bBb, 931 Calcium phosphate plaques, 1836 C3bBbC3b, 933 2702 Index

C4-binding protein, 936 Chinese herbs, 1286, 1377 CC chemokines, 912 Chinese herbs nephropathy, 1683 C1 complex, 933 Chi-square, 489 C3 convertase, 930 Chlorambucil, 857 CD40, 899 Chloroquine (CQ), 1473 CD59 (Protectin), 936 Cholecalciferol, 2234 CD80, 898 Chromium, 1378 CD151, 824–825 Chronbach's alpha, 489 CD2AP, 725, 810, 814, 816, 819 Chronic calcineurin inhibitor toxicity, 740 CD2AP mutation case reports, 725 Chronic granulomatous disease (CGD), 1466 CD2-associated protein, 814 Chronic infections, 729 CDC42, 823 Chronic kidney disease (CKD), 272–273, 342, 720, CD4+ helper T cell, 897 993, 1533–1534, 1724, 1913, 1972, CD89-IgA complexes, 1004 2061–2063, 2145, 2692, 2693 C3 dominant glomerulonephritis, 707 additional contributing factors, 2175–2176 C/EBP homologous protein (CHOP), 1678 addressing metabolic considerations in, 2192 Celiprolol, 2049 adipokines, 2310–2312 Cell-mediated immunity, 883–884, 962, 1408–1409 adrenal hormone axis, 2306–2307 Cell number, 4 for age percentile Cell proliferation, 909 estimated dry weight and body mass index, 654 Cellular casts, 610 head circumference, 655 Cellular reprogramming, 526, 528, 545, 558 height and length, 653 Cellular variant, 849 velocityof height and length, 653–654 Central nervous system, 1366 carbohydratemetabolism, 2307–2310 Cephaloridine, 1665 causes, 2177 Cephalosporins, 1665 developmental stage, 2312–2315 CERA. See Continuous erythropoiesis receptor activator dietary guidelines, 354–356 (CERA) epidemiology in European countries, 2215 Cerebral receptors, 190 etiology of growth impairment, 647–649 Cerebrotendinous xanthomatosis, 1835 gonadotropic hormone axis, 2297–2300 Cerebrovascular accidents, 1348 growth and development evaluation Cetuximab, 1233 dietary intake, 652–653 CFHR5 nephropathy, 950 normalized protein catabolic rate (nPCR), 655 C3 glomerulonephritis, 1036 growth and nutritional assessment, 655 C3 glomerulopathy, 950–951, 966 growth failure animal models, 1040–1041 anemia, 2319 autoimmune forms, 1041–1042 disturbances of water and electrolyte metabolism, clinical outcomes, 1047–1048 2319 clinical presentations, 1042–1044 growth hormone, 2325–2332 complement system, 1037–1040 hormonal factors, 2320 diagnosis, 1045 malnutrition and inflammation, 2318–2319 differential diagnosis, 1045–1046 metabolic acidosis, 2319 genetic forms, 1042–1043 renal osteodystrophy, 2320 histopathology, 1037 socioeconomic factors, 2320–2321 treatment, 1046–1047 treatment, 2321–2325 α3 chain of collagen type IV, 819, 896 growth in children α4 chains of collagen IV, 819 acidosis control, 658 Charcot-Marie-Tooth (CMT) disease, 794, 815, 823–824 enhanced dialytic clearance, 660 Chemoattractant, 913, 939, 995 exposure to corticosteroids, 660 Chemokine(s), 911–914 metabolic bone disease, 658–659 Chemokine-chemokine receptor, 2476 morbidity and mortality, 655 Chemotaxis, 995 implications, treatment, 2191–2194 Chemotherapy, 1511–1512, 1880, 1894 in infants and children, 414–415 Childhood-onset type 1 diabetes (T1D), 1546 model, 2178 Childhood polyarteritis (PAN), 1433–1436 normal phases of growth, 647 Childhood vasculitides, 1432 nutrition, 656–658 Chile, 2633 nutritional management, 342 China, 2640 patterns progression, 2176–2177 Chinese herbal medicine (Sairei-to), 1011 progression, regulatory elements, 2181 Index 2703

protein energy wasting, 649–652 Classic Bartter syndrome, 1211–1215 biochemical disturbances, 650–651 classic renal glycosuria, 1188 energy intake, 651–652 Classification of molecular mechanism, 1313 low body weight, weight loss, 651 Claudin-16, 1228 muscle mass reduction, 651 Claudin-19, 1228 pubertal development, 647 ClC-Ka, 1211 recombinant human growth hormone therapy, 659–660 ClC-Kb, 1211 risk factors, 2173–2176 CLCNKA and CLCNKB, 1211 cardiovascular factors, 2175 CLDN16, 1228 scarring in, 2186 Clean intermittent catheterization (CIC), 1802 short stature, 656 Clearance, of IgA1, 990 sodium and water losses, 658 Clevidipine, 2056–2057 stages of, 2172 Clinical characteristics, 972–973 thyroid hormone axis, 2304–2306 Clinical pharmacology Chronic kidney disease in children (CKiD), 2236 clearance, 2270–2271 anemia, 2220, 2227 (see also Anemia in CKD) drug absorption, 2269 causes, 2214–2216 drug disposition, developmental changes, 2275 classification, 2212–2214 gastric acid, 2275 cohort, causes in, 2217 gastrointestinal tract, 2275 glomerular filtration rate evaluation, 2208–2212 gut motility, 2275 incidence, 2214–2216 hepatic clearance, 2272 interventions, 2216–2220 influence of food, 2275 natural history, 2216–2220 plasma protein binding, 2274 prevalence, 2213–2216 renal clearance, 2272–2274 risk factors, 2216–2220 saturable elimination, 2274 study baseline patient characteristics, 2216 three-compartment model, 2269 Chronic kidney disease mineral and bone disorder volume of distribution, 2269–2270 (CKD-MBD) Clinical Practice Guidelines, 2688 definition, 2227, 2350 Clinical symptoms, 1457 diseases after kidney transplantation, 2368–2370 Clinicopathologic correlations, 1013–1014 evaluation, 2231–2233 Cloaca and urogenital sinus, 1796–1797 growth hormone therapy, 2368 Clonidine, 2047 management, 2233–2235 Clotting cascade, 918 pathogenesis, 2350–2354 Clusterin, 936 pathophysiology, 2228–2231 Cluster sampling, 485 Chronic liver disease, 1005–1006 CMV, 721 Chronic metabolic acidosis, 235, 648 C3NeF, 907–908 Chronic pyelonephritis, 721 CNNM2, 1234 Chronic respiratory acidosis, 413 Coagulation cascade, 916–918 Chronic tubulointerstitial nephritis, 1420–1421 Coagulation modifying agents, 1009 etiologic classification of, 1422 Coarctation, 2010 treatment and prognosis, 1421, 1423 Coarctation of aorta (CoA), 1974 Chrousos syndrome, 1970 Cobalamin metabolism, 1509–1510 Churg Strauss Syndrome (EPA/CSS), 1442 Cochrane review, 2246 Cidofovir, 1378, 1669 Cockayne syndrome, 794 Cigarette smoking, 2033 Coenzyme Q10 (COQ10, ubiquinone), 820 Cilastatin, 1665 Cofilin, 2115 C1 inhibitor, 936 Cognitive and psychosocial development, 2247–2249 Circadian pattern, 2000 Cognitive changes, 1926 Circadian rhythm, 1953–1954 Cohort design, 480 Circulating antibodies to extracellular domain, 1222 COL4A3, 816–817 Circulating immune complex, 894, 896 COL4A4, 816–817 Circulating volume, alterations, 366 Colicky abdominal pain, 1851 Circumcision, 1705 Collagen(s), 742 Cisplatin, 1378, 1672–1674, 2148 Collagen IV, 990 Citrate, 264, 385–386, 2159, 2234 Collagen type III glomerulopathy, 791 Citrate lock, 2159 Collapsing glomerulopathy, 849 Classical complement pathway, 996 Collapsing variant, 849 Classical pathway (CP), 933–935, 962 Collecting system development, 17 2704 Index

Collectins, 935 diagnosis, 765–766 Colloid solutions and volume resuscitation, 384–386 Finnish type, 756 Colombia, 2633 genetic findings prevalence, 754 Colony-forming units (CFU), 1699 management, 766 Columbia classification, 849 management of infants with, 767 Combination therapy, 1010–1011 podocin gene mutations (NPHS2), 760–761 Combined proximal and distal RTA, 1283 Congenital nephrotic syndrome of the Finnish type Common variants, 426–428 (CNF), 812 Complement, 890, 905–908 Congenital obstructive uropathy Complement activation, 962–963 animal models, 1751, 1753 pathways, 928–930 classification, 1756–1757 Complement component C2, 933–934 description, 1750 Complement component C3, 930 epidemiology, 1750 Complement component C4, 933–934 Excretory Urography, 1763 Complement control protein modules (CCPs), 1505 genetics, 1753–1756 Complement-derived chemotactic factors, 953 magnetic resonance imaging, 1760–1761 Complement factor H gene cluster (CFHR1), 987 MMIHS, 1769 Complement factor H related proteins (CFHR), 1506 postnatal diagnosis, 1758–1759 Complement levels, 967, 973 prenatal diagnosis, 1757–1758 Complement-mediated glomerular injury, 937 pressure perfusion studies, 1763 Complement profile, 939 prune belly syndrome, 1769 Complement receptor 1 (CD35), 936 radionuclide renal scintigraphy, 1761–1763 Complement receptor 2, 938 ultrasonography, 1759–1760 Complement regulatory proteins, 907 ureteropelvic junction obstruction (see Ureteropelvic Complement system, 928, 1037, 1039–1040 junction obstruction (UPJO)) Complete remission, 1472 ureterovesical junction obstruction, 1767–1769 Complex traits, 427–428 voiding cysturethrogram, 1760 Complotype, 928 Congenital renal hypoplasia/dysplasia. See Renal Computed tomographic angiography (CTA), 2006–2007 hypoplasia/dysplasia (RHD) Concentric LVH, 2236 Congenital vs. acquired reflux nephropathy, 1721 Confidence intervals, 490–492 Connective tissue growth factor (CTGF), 915–916, 1551 Confidentiality and privacy, 496 Constipation, 1786–1787 Conflicts of interest, 495 Consumption, 928 Confounder, 481 Continuing Professional Development (CPD), 2688 Congenital abnormalities of the kidney and urinary tract Continuous ambulatory PD (CAPD), 2382 (CAKUT), 347 Continuous erythropoiesis receptor activator (CERA), Congenital adrenal hyperplasia (CAH), 397, 1291, 1970, 2226 2017, 2067–2068 Continuous incontinence, 1788 Congenital and acquired syphilis, 975 Continuous renal replacement therapies (CRRT), Congenital anomalies of the kidney and urinary tract 391, 2154, 2281, 2288 (CAKUT), 115, 119–123, 128, 806, 1716, 2215 Continuous venovenous hemodiafiltration (CVVHDF), Congenital Fanconi Syndrome, 1284 393, 2157 Congenital forms of distal RTA, 1288 Continuous venovenous hemodialysis (CVVHD), 2157 Congenital hepatic fibrosis, 1109 Continuous venovenous hemofiltration (CVVH), 2157 Congenital kidney and urological disorders Convection, 2435 abnormal kidney number/location, 680 Convective mass transfer, 2386 bladder abnormalities, 683–684 Convenience sampling, 485 crossed ectopia, 682 Cooperative Clinical Trials in Pediatric Transplantation duplex collecting system, 682–683 (CCTPT) program, 2556 ectopic ureters, 683 Co-peptin, 632 horseshoe kidney, 680 Copy number variations (CNVs), 426, 430, 439–440 megaureter, 680 COQ2, 821 ureteroceles, 680 COQ6, 821 ureteropelvic junction obstruction, 680 CoQ10 biosynthesis defects, 1591 urethral abnormalities, 684 Corneal crystals, 1336 vesicoureteral reflux, 684 Coronary artery, 2230 Congenital nephrotic syndrome (CNS), 725, 753–770 Correlation coefficient, 489 classification, 754 Cortical BMD, 2229 congenital syphilis, 764 Cortical collecting duct (CCD), 230 Index 2705

Cortical collecting tubule sodium transport developmental Cystic fibrosis, 2144 features, 82 Cystic kidney diseases (CKDs), 1103 Corticosteroid, 840, 1009–1010, 1468, 1734 Cystine, 1330 monotherapy, 1065–1066 binding protein assay, 1338 therapy, 1419 concentrations, 1338 treatment, 971 crystals, 1174 Costa Rica, 2633 crystals of, 1330 Costimulatory molecules, 898, 2458 depletion, 1330 Costimulatory signals, 2464 stones, 1175 Counseling, 2033 transport, 1330 Countercurrent mechanism, 200–201 Cystine-depleting regimens, 1342 COX-2 inhibitor, 1679 Cystinosin loss, 1332 Cox proportional hazards methods, 491 Cystinosis, 1284–1285, 1331–1332, 1339, 1341, 1361, C1q, 989 1367, 2626 C1q glomerulopathy, 850 diagnostic age, 1338 CRB2, 813 Cystinuria, 1172, 1339, 1848 Creatinine, 614–618, 2209 CKD, 1848 normal value, 2278 hypotonia-cystinuria syndrome, 1849 Crescentic glomerulonephritis, 917 prevalence, 1848 Crescents, 965, 998 thiol drugs, 1849 Critically ill children, 2143 treatment, 1849 Crossed ectopia, 130, 682 Cystometric techniques, 1791, 1793–1794 Cross-sectional study, 477 Cystoplasty, 1801 Cryoglobulinemia, 715 Cytokine, 852, 884, 888, 897, 987, 1410, 1551, 2223 Cryoglobulins, 729 Cytomegalovirus (CMV), 754, 2575–2576 CTNNB1, 1876 Cytotoxic T lymphocyte-associated antigen 4 (CTLA4), CTNS, 1331 2466, 2557 mutation, 1332 mutation analysis, 1339 transgene, 1346 D Ctns-/-mouse, 1333 Daclizumab, 2556–2557 Cubam, 1355 Daily Recommended Intake (DRI), 2245 Cubilin, 1658 Darbepoetin-alfa, 2226 definition, 1361 Daytime incontinence, 1788 recycling of, 1362 Debilitating conditions in poststreptococcal nephritis, 960 structure of, 1357 Decay-accelerating factor (CD55), 936 Cuff sizes, 1999 De-differentiation, 849 Cumulative prevalence, 840 Defective urinary acidification, 1527 Curled Tenckhoff catheters, 2390 Defects in carbonic anhydrase, 1283 Cushing syndrome, 1976, 2016, 2070 Deferasirox, 1378 CXC chemokines, 912, 913 Definition, AKI, 2140 CXXC5, 1876 Deflazacort, 857 CyA nephrotoxicity, 1676 Defluxffi, 1734 Cyberbullying, 2031 Deformation, 157 Cyclers, 2391 Degradome, 441 Cyclin dependent kinase inhibitors, 840 Dehydration, 1358, 1360, 1367, 1379 Cyclin M2, 1234 clinical assessment, 376 Cyclooxygenase, 919–920 Delayed graft function (DGF), 2563 Cyclophilins, 2558 Delayed type hypersensitivity, 899 Cyclophosphamide, 857, 865, 1011, 1468 Dense deposit disease (DDD), 707–708, 729–731, Cyclosporine, 860, 865, 868 907, 1037 Cyclosporine A (CSA), 1015, 2558–2559 Dense deposits, 950 CystagonR, 1343–1344 Dent disease, 1284, 1361, 1840, 1842 CYSTARANTM, 1345 Dent 2 disease, 1363 Cystatin C, 618, 2146, 2150, 2152, 2209, 2274 Dent's Disease type 1, 1285 Cysteamine, 1330, 1342–1345 Dent's Disease type 2, 1285 eyedrops, 1345 Denys-Drash syndrome (DDS), 818, 1872, 1879–1880 therapy, 1343 Dependent variables, 474 Cysteine, 1330 Deposition, 889 2706 Index

Deposits, IgA, 986 Dominant IgA deposits, 969–971 Desmopressin, 1534, 1804 Dominican Republic, 2632 Detrusor, 1783 Donor specific antibodies (DSA), 952 overactivity, 1801 Dopamine, 1958, 2153–2154 Detrusorectomy, 1802 Double–stranded DNA (dsDNA), 905 Developmental outcomes, 2247 Doxercalciferol, 2366 Diabetes mellitus, 735, 1976–1977 D-penicillamine, 1175–1176 post-transplant, 2583–2584 Driving forces, 2384–2387 Diabetic nephropathy (DN), 2064–2065 Drug-induced definition, 1546 lupus, 1465 stages, 1546–1547 stones, 1832–1833 Diacylglycerol kinase epsilon gene (DGKE), 1507 Drug removal Diacylglycerol-protein kinase C (DAG-PKC) pathway, and dosing during CRRT, 2281–2288 1553 during intermittent hemodialysis, 2280–2281 Diagnosis and clinical features, 1174–1175 by peritoneal dialysis, 2288–2289 Dialysis, 1973, 2624–2625 Drugs Diaphanous autoregulatory/inhibitory domains abuse, 2017 (DAD/DID), 815–816 adefovir, 1378 Diastolic, 2237 Chinese herbs, 1377 Dibasic amino acids, 1158 cidofovir, 1378 Dicarboxylic aminoaciduria, 1181 cisplatin, 1378 Diet, 267, 1556, 2037 deferasirox, 1378 nutrition and metabolism, 2175 dosing, 2161 restriction, 1559 exposure, 1510–1512 Dietary history, 2003 ifosfamide, 1377 Dietary oxalate intake, 1395 imatinib mesylate, 1378 Differential diagnosis, between IgA-dominant PIGN and salicylate binding, 1377 IgA nephropathy, 970 Valproic acid, 1377 Differentiation process, 4 Drug therapy, pediatric renal patients, 2268 Diffuse mesangial proliferation, 840 clinical pharmacology, 2268–2277 Diffuse mesangial sclerosis (DMS), 725, 788, 812, 818 dosing, 2268 Diffuse proliferative glomerulonephritis, 737 dosing in neonatal period, 2277 Diffusion, 2435 human development and pharmacokinetic Diffusive transport, 2384–2385 alteration, 2276 Dihydropyridine (DHP), 2240 renal handling of drugs, 2277–2289 1,25-dihydroxy vitamin D3 (calcitriol), 2228 Drug toxicity, 708, 738, 741 Dilation of renal pelvis, 99 Drug transport systems, 1656 Dimercapto-succinic acid (DMSA), 2013, 2014 Dry ice, 713 Dipstick testing, 1698–1699 Dual energy X-ray adsorptiometry (DXA), 2233 Dipyridamole, 1010 Dubre, 1330 Directed differentiation of human ESCs/iPSCs into Duplex collecting system, 682–683 embryonic renal stem/progenitor cells, 540–545 Duplication anomalies, 104 Directed differentiation of mouse ESCs/iPSCs into Dysautonomias, 2069 ESCs/iPSCs, 535–540 Dysfunctional voiding, 1806–1808 Directed differentiation of pluripotent stem cells, 534–535 Dyslipidemia, 1554, 1558–1559, 2220, 2237–2238, Direct renin inhibitors, 2052 2584–2586 Disruption, 157 Dysmorphic red blood cells, 706 Disseminated intravascular coagulation, 722 Dysplasia, 157 Distal convoluted tubule developmental transport, 79–80 Dysplastic tissue, 121 Distal nephron, 1278 Dysregulation, 928 Distal renal tubular acidosis, 1286–1290, 1361 Dysuria, 1788 Distal RTA, 1842 Distal RTA without hearing loss (type Ic), 1289 Distal tubule acidification developmental aspects, 82 E Diuretics, 263–264, 400, 1395, 2046, 2149, 2154 EAAT subfamily, 1160 DMS. See Diffuse mesangial sclerosis (DMS) Eagle-Barratt Syndrome. See Prune belly DNase 1L3 mutation, 1466 syndrome (PBS) DNA sequencing, 428–429 Ear, 1366 Dobutamine, 2154 EAST/SeSAME syndrome, 1230 Index 2707

Eccentric LVH, 2236 End stage renal disease (ESRD), 1535 Echocardiography, 2239 dialysis, 1537 Ectopic kidney, 129–130 genetic modifiers, 1536–1537 Ectopic ureters, 683 kidney transplantation outcomes, 1537–1538 Ecuador, 2633 rates of progression, 1536 Eculizumab, 949, 1508 End-stage renal disease (ESRD), 342, 722, 1335 Edema, 599, 840, 841, 964 End stage renal failure, 1475–1476 Effective circulating volume, 365–366 Energy, 2244 Effector T cells, 899 Enteral cation exchange resins, 400 eGFR, 2145 Enteric coated mycophenolic acid (EC-MPA), 2562 Eicosanoids, 904, 919–921 Enuresis, 1534, 1788, 1803 Electrochemical driving force, 231–232 Environmental factors, 840 Electrolyte abnormalities, 2592–2594 Enzyme AGT, 1390 Electrolyte balance, 2241–2242 Enzyme linked immunosorbent assay Electrolyte disturbances, 2148 (ELISA), 2560 Electrolyte management, 2153 Eosinophilic Polyangiitis (EPA), 1442 Electromyography (EMG), of pelvic floor, 1793 Eosinophils, 1414 Electron dense deposits, 974, 988 Eosinophiluria, 1413 Electron dense deposits (humps), 966 Epidemic outbreaks, 961 Electron dense subepithelial deposits (humps), 962 Epidemiology, 2140, 2143–2145 Elevated urinary PGE2 excretion, 1203 Epidemiology of Diabetes Interventions and El Salvador, 2633 Complications (EDIC), 1554 Embolic disease, 973 Epidermal growth factor, 916 Embryological considerations, 1782 Epidermal growth factor (EGF) gene, 1232 Embryonic renal stem Epidermal growth factor receptor progenitor cells, 530–533 (EGFR), 1129 Embryonic renal stem/progenitor cells, human Epidermolysis bullosa, 824 ESCs/iPSCs into, 540, 543–545 Epigenetics, 1918 Embryonic renal stem/progenitor cells, mouse Episodic hypertension, 2000, 2003 ESCs/iPSCs into, 535–536, 539–540 Epispadias, 1794 Embryonic stem cells, 573 Epithelial cell sodium channels (ENaC), 404 EMP2, 825 Epithelial foot processes, 999 Encapsulating peritoneal sclerosis (EPS), 2416 Epithelial growth factor, 1233 Encephalopathy, 2247 Epithelial membrane protein 2, 825 Endemic calculi, 1824 Epithelial-mesenchymal transformation, 1411 Endocapillary hypercellularity, 998 Epithelial sodium channel (ENaC), 198 Endocapillary proliferation, 965 Epithelial sodium channel (ENaC) blockers, 2047 Endochondral ossification, 280 Epitope shifting, 2463 Endocrine, 1366 Eplerenone, 1221, 2057 Endocrine abnormalities, 2013–2017 Epsilon aminocaproic acid (EACA), 1534 Endocrine disorders in chronic kidney disease Epstein and Fechtner syndromes, 791 insulin-like growth factor axis, 2300–2304 Epstein-Barr virus, 741, 742 pathophysiological mechanisms, 2295–2297 Epstein-Barr virus infections and PTLD, Endocytic pathways 2576–2577 dysruption of, 1356 Ergocalciferol (D2), 2234 impaired actin metabolism, 1364 ER stress, 1664 megalin/cubilin-mediated, 1355 Erythromycin, 968 receptor-ligand complexes, 1357 ESA, 2225, 2227 receptor-mediated, 1362 ESC Endogenous antigens, 894 progenitor cells, 530–533 Endoplasmic reticulum (ER), 1656 ESCAPE trial, 2215 Endoscopic treatment, 1734 ESPN working groups, 2683–2684 Endothelial cells, 886, 890–891 ESRD, 737 Endothelial dysfunction, 2035 causes, 2177 Endothelial glycocalyx, 894 dietary guidelines, 354–356 Endothelin, 192, 891, 2106 Estimated GFR (eGFR), 616 Endothelin-1, 995, 1958–1959, 2057–2058 ESWL, 1853 Endothelium, 2238 Ethanol, 2032 End-stage kidney disease (ESKD), 806, 2694 Ethnicity, 1916 2708 Index

Etiologies of Fanconi syndrome, 1361 External validity, 476 acquired forms of, 1376–1377 Extracellular and intracellular fluid compartments, 363–365 acute tubulointerstitial nephritis with uveitis Extracellular matrix, 994, 1135–1136 syndrome, 1376 Extracellular matrix (ECM) ankylosing spondylitis, 1376–1377 in kidney, 2181 anorexia nervosa, 1377 protein expression, 2182 autoimmune interstitial nephritis, 1377 Extracellular water (ECW) spaces, 182 distal renal tubular acidosis, 1376 Extracorporeal circuit volumes, 2159 Legionella pneumonia, 1377 Extracorporeal membrane oxygenation (ECMO), 2160 membranous nephropathy, 1377 Extra-skeletal calcification, 2233 multiple myeloma, 1376 Exudation, 719 paroxysmal nocturnal hemoglobinuria, 1376 Exudative glomerulonephritis, 965 Sjögren syndrome, 1376 Eye, 1366, 1455 acute tubulointerstitial nephritis with uveitis (TINU) syndrome, 1361 F anorexia nervosa, 1361 Fabrication/forgery, 495 autoimmune interstitial nephritis, 1361 Fabry disease, 791–792 autosomal dominant Fanconi syndrome, macrosomia, Facilitative glucose transporters, 1185–1186 young onset diabetes mellitus, 1361, 1375 F-actin, 814, 815 autosomal recessive Fanconi syndrome, NaPi-IIa gene Factor B, 930 mutations, 1361, 1375 Factor D, 930 cystinosis, 1361, 1367 Factor H, 907, 936 Dent disease, 1361–1364 Factor-H related proteins, 936 Dent 2 disease, 1363 Factor I, 930, 936 distal renal tubular acidosis, 1361 Factor XIII, 1013 Fanconi-Bickel syndrome, 1361, 1370–1371 Fadrozole, 2057 Fanconi syndrome, EHHADH mutations, 1361, 1375 Failure to thrive, 1358 Galactosemia, 1361, 1367–1369 False-negative rate, 492 glycogen storage disease type I, 1369–1370 False positive haematuria, 597 glycogen storage disease type I (von Gierke disease), False-positive rate, 492 1361 Falsification/fraud, 495 hereditary fructose intolerance, 1361, 1369 Familial amyloidosis, 792 idiopathic Fanconi syndrome, 1361, 1375–1376 Familial hematuria, 706, 720, 736, 779 Lowe syndrome, 1361, 1364–1365 Familial hyperaldosteronism lysinuric protein intolerance, 1361, 1374 type I, 1968–1969 membranous nephropathy, 1361 type II, 1969 microvillous inclusion disease, 1361, 1374–1375 type III, 1969 mitochondriopathies, 1361, 1365–1367 Familial hypocalciuric hypercalcaemia (FHH), 1222 myeloma, 1361 Familial hypomagnesemia with hypercalciuria and nephrotic syndrome, 1361 nephrocalcinosis (FHHNC), 1228, 1843 renal transplantation, 1361 with severe ocular involvement, 1229 Sjögren syndrome, 1361 Familial incidence, 963 Tyrosinemia, 1361 Familial juvenile megaloblastic anemia, 794 Tyrosinemia I, 1371 Familial Mediterranean fever, 792 Wilson disease, 1361, 1372–1374 Familial renal glycosuria, 1187–1188 Etiology, 2145–2152 Familial renal iminoglycinuria, 1169 European guidelines, 2231 Familial Wilms' tumor, 1880 European Renal Association-European Dialysis and Family history, 2003 Transplant Association, 2683 Fanconi-Bickel syndrome, 320, 1186, 1188, 1285, 1361, European Society for Paediatric Nephrology (ESPN) 1370–1371 activities of, 2683–2684 Fanconi, de Toni, 1330 historical development of, 2681–2682 Fanconi renotubular syndrome 3, 1285 strategic plan of, 2683 Fanconi Syndome Index (FSI), 1334 Everolimus, 2565 Fanconi syndrome, 405, 1282, 1660, 1834 Evidence-based, 1008 Fanconi syndrome, EHHADH mutations, 1361, 1375 Exercise, 2034–2035 Fats, 2244 Exogenous antigens, 894 Fc receptor I (FcαRI/CD89), 990 Experimental designs, 483–484 FDA, 2227 External genitalia, 99 Febuxostat, 1847 Index 2709

Fecal potassium excretion, 236 Formin INF2, 823 Fenoldopam, 2058, 2154 Formin mDia2, 816 Ferric gluconate, 2226 Fractional excretion of sodium (FENa), 201 Ferritin, 2224 Fractional excretion of urea, 2149 Ferroportin, 2222 Fractures, 2232 Ferumoxytol, 2226 Frasier syndrome, 736, 818, 1872, 1879–1880 Fetal programming, 2173 Frasier syndromes, 818 Fetal urine analysis, 106 Free thiol, 1344 Fetal urine uropathy, 1758 Free water clearance, 202 Fexofenadine, 2278 Frequently relapsing nephrotic syndrome (FRNS), 2692 FGF-2, 915 Frothy urine, 597 FGF23, 195, 2228 Frozen flavored oral rehydration solutions, 382 Fiber, 351 Fructose, 2038–2039 Fibrillar type III, 819 FSGS, 820 Fibrin, 917–918 Full house, 948 Fibrinolytic cascade, 918 Fungal infections, 2580 Fibrin-stabilizing factor, 1013 Furosemide, 2154 Fibroblast growth factor 23 (FGF23), 1004, 2350–2351 FXYD2, 1231 Fibroblast growth factor (FGF), 279, 915, 2227 Fibroblasts, 995 Fibrocystin (FC), 1106 G Fibrogenesis, 2179, 2181 GABA transporter branch, 1162 Treatments, 2191–2192 Galactose, 866 Fibromuscular dysplasia (FMD), 1971 Galactose-deficient IgA1, 993 Fibronectin, 990 Galactosemia, 1285, 1361, 1367–1369 Fibronectin glomerulopathy, 795 Galactosylation, 989 Fibrosis, 993 β 1,3-galactosyltransferase, 990 Ficolins, 935 Galloway-Mowat syndrome (GMS), 822 Filling disorders, 1805–1806 GalNAc, 990 First use syndrome, 2444 Ganciclovir, 1669 Fish oil/omega 3 fatty acids, 1009 GAP catalytic domain, 816 Fistula stenosis, 2447 Gastric tube placement, 1856 Flow-mediated dilation, 2237 Gastrointestinal disorders, 2589–2590 Fluid and electrolytes, 1340 Gastrointestinal manifestations, 1456 Fluid and electrolyte therapy Gastrostomy, 2244, 2250 pediatric intensive care unit, 390–391 Gauze pad, 713 renal dysfunction, 389–394 GBM, 887 Fluid balance, 2142, 2240–2242 Genealogy-driven methodology, 1838 Fluid intake, 968 Gene mutation, 706 Fluid management, 2153 Generalized aminoaciduria, 1187, 1330, 1357–1358, 1370 Fluid overload, 2145, 2147 Gene therapy, 1400 Fluid replacement therapy, 376 Genetic factors, 986–987, 1451, 1555–1556 volume depletion assessment, 376–377 Genetic hypercalciuric stone-forming rat (GHS) Fluid-responsive, 2146 model, 1827 Fluid secretion, 1132–1133 Genetics, 1917–1918 cAMP mediated secretion, 1133 Genetic screening, 742 cilia, 1134–1135 Genetic testing, 706 transport, 1133–1134 Genital pain, 1789 Fluid spaces, 362 Genitourinary Development Molecular Atlas Project Foam cells, 719, 722, 736 (GUDMAP), 1756 Focal, embolic and non-supurative glomerulonephritis Genitourinary system, reconstruction and with bacterial endocarditis, 971 regeneration of, 572 Focal segmental glomerulosclerosis (FSGS), 840, 1530 Genome-wide association studies (GWAS), 428, 430, Focal segmental glomerulosclerosis, post transplantation, 437, 987, 1839, 1918 2527–2528 Genome-wide SNP arrays, 431 Folate, 2223 Genomics, 500–504 Food and Drug Administration Modernization Act of 1997 end-stage renal disease, 510–512 (FDAMA), 2040 hemolytic uremic syndrome, 506–508 Foot process, 840 nephrotic syndrome, 504, 505 2710 Index

Genomics (cont.) Glycation end-products, 1552 next-generation sequencing, 512–517 Glycemic control, 1553–1554 polycystic kidney disease, 508–509 Glyceraldehyde 3-phosphate dehydrogenase (GAPDH), 962 primary IgAN nephropathy, 509–510 Glycine, 1157 Gentamicin, 2279 Glycocalyx, 942 GFR, 197–198, 735 Glycogen storage disease type I, 1361, 1369–1370, 1834 GH-IGF system, 1550 Glycoglycinuria, 1181 Giant cells, 720 Glycosuria, 1182–1183 Gibbs-Donnan effect, 183 Glyoxylate, 1390, 1400 Gitelman syndrome, 1215–1221 Gonadal toxicity, 860 clinical manifestations, 1217–1219 Gonadotropin releasing hormone (GnRH), 647 genetics, 1215–1216 Goodpasture syndrome, 896 pathophysiology, 1216–1217 Gordon's syndrome, 1292, 1970 quality of life, 1219 Grafts and shunts, 2447 GLEPP1. See Protein tyrosine phosphatase receptor Granulocyte colony stimulating factor (GCSF), 2561 type O (PTPRO) Granulomatosis, 733–734 Glomerular basement membrane (GBM), 707, 708, Granulomatosis with polyangiitis, 720 711, 715, 717, 719, 721–722, 725, 728, Granulomatous Polyangiitis (GPA), 1439 736, 740, 778, 966 GR-9b, 857 Glomerular capillary tuft development, 13 G339R founder mutations, 1333 Glomerular capillary wall, 758 Gross hematuria, 1851, 1891–1892 Glomerular density, 1007 Group C streptococcus, 961 Glomerular disease, 706, 717, 722, 2219 Growth, 2244–2247 Glomerular filtration, 1657 Growth and kidney disease, 2173 Glomerular Filtration Barrier, 41–46 Growth factors, 888, 914, 1550–1551 Glomerular filtration rate (GFR), 614, 708, 722, 964, 1703, Growth failure and nutrition, 2242–2247 1913, 2268, 2688 Growth hormone, 648, 1341, 2243 estimation equation, 2211 Growth hormone binding protein (GHBP), 649 evaluation, 2208–2212 Growth hormone(GH) secretion, 271 limitations, 2210 Growth hormone therapy, 2368 normal ranges, 2209 Growth retardation, 857, 1358, 1476, 2357–2358 Glomerular filtration rate (GFR) method, 2484 Growth retardation, in chronic acidosis, 271–272 Glomerular hyperfiltration, 1524, 1526 Growth velocity, 640 Glomerular injury, 884 Guatemala, 2633 Glomerular proteinuria, 622 Guibaud-Vainsel syndrome or marble brain disease, 1291 Glomerular tubular balance, 66 Guillain-Barre syndrome, 1977 Glomerulogenesis, 12–13 Gut, 1366 Glomerulonephritis, 971, 2012 Glomerulopathy, 706, 708, 712, 715, 720, 734, 737, 738, H 740, 741, 1530 Haber Weiss reaction, 921 Glomerulosclerosis, 1597 Haiti, 2633 Glomerulotubular cross-talk, 995 Half-life, 2226 Glucocorticoid/immunosuppressive, 1000 Haplotype analysis, 461 Glucocorticoid-remediable aldosteronism (GRA), HapMap Project, 428, 430 1968–1969 Hartnup disease, 1179–1180 Glucocorticoid remediable hyperaldosteronism, Δ HCO3 Ratio, 413 2066–2067 Healthcare interventions, 972 Glucocorticoids, 2065 Hearing loss, 1289 Glucose-galactose malabsorption, 1187–1188 Heart, 1366 Glucose transport development, 67–69 Heart lesions, 972 Glucosuria, 1339, 1359 Heat-shock proteins (Hsps), 2120 Glucuronidation, 1663 Heavy metals, 1286, 1681 GLUT1 (SLC2A1), 1185 Height, 2242 GLUT2, 1284 Height SDS, 2247 GLUT2 (SLC2A2), 1185, 1188 Hematologic complications, 2590 Glutaraldehyde, 714 Hematopoietic stem cell transplantation (HSCT), 1512 Glutathione peroxidase, 922 Hematopoietic system, 1455 Glycans, 990 Hematoxylin and eosin stain, 713–714 Index 2711

Hematuria, 781, 963, 973, 1532–1535 Heterogenous nucleation, 1830, 1837 Hemodialysis, 1341 Heterologous immunity, 2461, 2473–2474 for acute kidney injury, 2453–2454 Heteromeric Amino Acid Transporters (HAT), blood-borne viruses, 2451–2452 SLC3/SLC7 family of, 1166–1167 CKD-mineral and bone disorder, 2453 Hexosamine pathway, 1552–1553 Complications, 2443–2445 Heyman nephritis, 907 drug prescription, 2453 Hinge region, 990 factors affecting prescription, 2439–2443 Hinman's syndrome, 1810–1811 machine and blood and dialysate circuits, 2436–2439 Histidinuria, 1180 nutrition, 2452 Histologic classification, 1457–1460 outcomes, 2454 Histomorphometric, 2232 principles, 2435–2436 HIV, 2617 vascular access, 2445–2448 HLA-DQA1, 825 Hemodialysis (HD), 1396 Holding manoeuvres, 1789 Hemodialysis adequacy, 2450–2451 HOMA-IR, 2238 Hemodialysis or peritoneal dialysis, 969 Home blood pressure, 1909–1910 Hemodynamic factors, 1550 Homeostasis, 219 Hemofilters, 2158 Homogenous nucleation, 1837 Hemoglobin, 2221, 2225 Honduras, 2633 Hemolysis, 1524, 1527 Hong Kong, 2641 Hemolytic uremic syndrome (HUS), 708, 726–727, 1513, Hormonal abnormalities, 2035 1587, 2145 Hormonal contraception, 1456 clinical manifestations, 1493–1495 Horseshoe kidney, 129, 680, 682 epidemiology, 1492 Hospitalization, 726, 2227, 2251 long term complications, 1496 Human ESCs/iPSCs into embryonic renal stem/progenitor Hemolytic uremic syndrome, post transplantation, cells, 540–545 2528–2530 Human subjects, 496 Hemopexin, 852 Hungry bone syndrome, 2367 Hemophilus parainfluenza, 986 HUS, 715, 720, 721, 740–741 Hemorrhagic cystitis, 860 Hyaline, 722, 726 Henderson-Hasselbalch equation, 248 Hyaline casts, 610 Henoch-Schönlein nephritis (HSN), 984 Hyaluronan, 2383 Henoch-Schönlein purpura (HSP), 707, 727, 740, 984 Hydralazine, 2049 Heparin, 2159 Hydrochlorothiazide (HCTZ), 2046 Heparin anticoagulation, 2156 Hydrogen, 2242 Heparin induced thrombocytopenia, 2159 Hydronephrosis, 99–101 Heparin-warfarin, 1010 Hydrostatic perfusion pressure, 364 Hepatic receptors, 189 Hydroxychloroquine, 1473 Hepatitis C positivity, 729 Hydroxyurea (HU), 1535 Hepatocellular carcinoma, 1371 Hyperaldosteronism, 265, 404, 2016, Hepatocyte transplantation, 1400 2060, 2070 Hepatosplenomegaly, 606 Hypercalcemia, 2017, 2234, 2368 Hepcidin, 2222, 2227 Hypercalciuria, 272, 1205, 1360, 1364 Hereditary acro-osteolysis with nephropathy, 794 Hyperfiltration, 1529, 1547–1548 Hereditary aminoacidurias, 1169 Hyper IgM syndrome (HIM), 2467 Hereditary angiopathy with nephropathy, aneurysms and Hyperinsulinemia, 1965 cramps (HANAC Syndrome), 788 Hyperkalemia, 236, 1527, 2144, 2153 Hereditary fructose intolerance, 1285, 1361, 1369 causes in children, 394–395, 397 Hereditary glycosuria, 1187–1188 decreased effective circulating volume, 397 Hereditary hypophosphatemic rickets with hypercalciuria definition, 394 (HHRH), 1844 evaluation in children, 398–399 Hereditary lecithin-cholesterol acyltransferase (LCAT) renin-angiotensin-aldosterone system, 397–398 deficiency, 793 treatment in children, 399–401, 407 Hereditary nephritis, 706 unexplained in children, 398 Hereditary spherocytosis, 1289 Hyperleptinemia, 1965 Hesitancy, 1789 Hyperlipidemia, 840, 2235 Heterogeneity in macrophages, 903 Hypernatermia Heterogeneity of cell types, 2194 increased potassium intake, 395 2712 Index

Hypernatremia Hypomagnesemia/magnesium deficiency Causes, 212 management, 1236 in children, 375 Hyponatremia, 207–210 Definition, 374–376 Asymptomatic, 387–388 diagnosis and evaluation, 210–211 decreased volume status and urine Na20 mEq/L, 373 severe, 388–389 definition, 372 treatment, 211 etiology, 373 Hyperosmolality effect, 224 normal/expanded volume and urine Na20 mEq/L, 374 Hyperoxaluria, 1390. See also Primary hyperoxaluria type pseudohyponatremia, 373 1 (PH1); Primary hyperoxaluria type 2 (PH2) Hypophosphatemia, 1359, 1379, 2352, 2369 antibiotic use, 1829 Hypophosphatemic nephrolithiasis/osteoporosis 1, 1844 bariatric surgery, 1830 Hypophosphatemic rickets, 1334, 1336 cystic fibrosis, 1829 Hyposthenuria, 1527 dietary, 1827 Hypothermia, 2160 enteric, 1827 Hypothyroidism, 1456, 1976 primary, 1827 Hypouricemia, 1594–1595 Hyperparathyroidism, 2223, 2232, 2235 Hypovolemia, 843, 2441 Hyperphosphatemia, 2153, 2237, 2352 Hypoxia, 2190 Hyperprostaglandin E syndrome, 227 Hypoxia inducible factors (HIFs), 17 Hypersensitive immune reactions, 896 Hypersensitivity reaction, 721 Hypertension, 599, 964, 1003, 1531, 1535, 1724, I 2145, 2174 Icodextrin, 2395 causes of, 2017–2018 Idiopathic Fanconi syndrome, 1361, 1375–1376 diagnosis, 1998–2000 Idiopathic hyperaldosteronism, 1975 endocrine abnormalities, 2013–2017 Idiopathic hypercalciuria management, 1734 bone mineral density, 1825 primary hypertension, 1997, 2004 definition, 1824 renal transplantation complication, 2581–2582 dietary phosphorous intake, 1826 screening evaluation, 2003–2004 GHS model, 1827 secondary hypertension, 1997 risk factor, 1824 severity and duration, 2000–2001 Idiopathic infantile hypercalcemia (IIH), 300 Hypertension (HTN), 1907, 2025 Idiopathic membranous nephropathy, 1060 Prevalence, 1912–1913 Idiopathic nephrotic syndrome, 840 Hypertension regulation, 2192 Idiopathic renal hypouricemia, 1848 Hypertensive Athletes, 2070–2073 Ifosfamide, 1377, 1674–1675, 2148 Hypertensive emergency, 2076–2080 IF positivity, 740 Hyperthyroidism, 1456, 1976, 2070 IgA, 710, 714, 727, 734, 737, 740, 991 Hypertonic urethral sphincter, 1790 IgA1, 988 Hypertriglyceridemia, 2237 IgA2, 988 Hyperuricosuria, 1360, 1594–1595, 1830–1831 IgA-binding-regions, 986 Hypoalbuminemia, 840 IgA/C3, 1004 Hypoallergenic diet, 356–357 IgA-dominant glomerulonephritis, 960 Hypocalcemia, 2144 IgA glomerulonephritis, 901 Hypocalciuria, 1216–1217, 1231 IgA nephropathy, 706, 708, 713, 727, 734, 737, 740, Hypochloremic metabolic alkalosis, 264 742, 2690 Hypocitraturia, 1830 IgA vasculitis, 984 Hypogonadism, 1347 Ig deposits, 966 Hypokalemia, 238, 263, 1339 IGF, 2243 causes in children, 401–405 IgG, 710, 714, 727, 730, 733, 741 causes of children, 402 IgM, 711, 714, 722, 726, 728, 734, 736, 740, 996 chronic, 401 IgM-associated nephropathy, 850 definition, 401 IL-4, 910 unexplained in children, 406 IL-6, 909 Hypokalemia and acid-base disorders, 406 IL-8, 909 Hypokalemic alkalosis, 1213 IL-10, 910 Hypomagnesemia, 1216–1217 IL-11, 910 with impaired brain development, 1234–1235 IL-12, 910 with secondary hypocalcemia, 1232–1234 IL-17, 897 Index 2713

IL-18, 910 INF2, 815, 823 IL-1β, 909 Infantile nephropathic cystinosis, 1333 Imaging techniques, 709 Infants, 1999 Imanitib mesylate, 1378 Infecting bacteria in community acquired and hospital Imerslund-Grasbeck syndrome, 794 acquired, 972 Iminoglycinuria, 1180 Infecting organisms, 974 Immune complex disease, 736 Infection-related stones, 1825 Immune complex glomerulonephritis, 973 Infectious complications, 1475 Immune complex, IgAN, 985 Infective endocarditis (hospital and community Immune complex-mediated glomerulonephritis, 893 acquired), 971 Immune deposits, 974 INF-γ, 910 Immune-mediated glomerular injury, 884 Inflammation, 2180–2181, 2185, 2222 Immune system, 884 Informed consent, 496 Immunoabsorption, 868 Inherited complement deficiencies, 1466 Immunodysregulation, polyendocrinopathy, enteropathy, Inherited hyperoxaluria, 1390 X-linked (IPEX) syndrome, 2472 Inherited renal parenchymal conditions, 1835 Immunoglobulin A nephropathy (IgAN), 946–947, 984 Initiation, 893 Immunoglobulins, 736 Injury Immunohistochemistry, 714 initiating progression, 2178–2179 Immunological injury, 463 Innate host immune systems, 1697 Immunostaining, 721, 736, 741 Insensible losses, 2153 Immunosuppressants, 1010 Insensible water losses, 367 Immunosuppression, pediatric renal transplantation In situ hybridization, 742 antiproliferative agents, 2560–2563 In situ immune complexes, 884, 896, 960 calcineurin inhibitors, 2558–2560 Insulin, 194–195, 222 induction therapy (see Induction therapy) Insulin-dependent diabetes mellitus, 841 mTOR inhibitors, 2563–2565 Insulin dependent diabetes mellitus (IDDM), 1494 steroids, 2557–2558 Insulin growth factor(IGF)-I, 271 treatment of rejection, 2565–2566 Insulin-like growth factor, 2243 Immunosuppressive medications, 1974 Insulin resistance, 1966 Impaired growth, 1336 Integrin α 3 subunit, 824 Impaired respiratory function, 1347 β4-integrin gene ITGB4, 824 Imperforate anus, 1799 Intensive blood glucose control, 1557–1558 Incidence, 492, 840 Intercalated cell, 253 Incidence, AKI, 2141 α-intercalated cells, 253 Incipient nephropathy, 1547 Interference mRNA (RNAi), 1400 Incomplete emptying, 1789 Interferon, 911 Incontinence, 1780, 1787 Interleukin-1, 994 diagnostic approach, 1812–1814 Interleukin-6, 994 Independent variables, 474 Interleukin-8, 995 India, 2644–2645 Interleukin-18, 2150 Indian hedgehog (Ihh), 283 Interleukin-2 receptor (IL-2r), 2556–2557 Indications for biopsy, 965 Interleukins, 888, 894, 909–910 Indinavir, 1671 Intermittency, 1789 Indomethacin, 867, 1208, 1214, 1221, 1341 Intermittent hemodialysis (IHD), 2156, 2280 Indonesia, 2646–2647 Intermittent incontinence, 1788 Induced pluripotent stem cells Internal jugular, 2250 Human, 540–545 Internal validity, 476 Mouse, 535–540 International Pediatric Nephrology Association Inducible costimulatory molecule, 899 (IPNA), 2687 Inducible T cell costimulator (ICOS) pathway, 2468–2469 International Study of Kidney Disease in Children, 1001 Induction therapy, 1467, 1471 Interquartile range, 488 Alemtuzumab, 2556 Interrupted innervation, 1794 co-stimulation blockade, 2557 Interstitial apatite, 1836 goal of, 2553 Interstitial fibrosis, 998, 1410, 1421, 1751 interleukin-2 receptor, 2556–2557 Interstitial lymphocyte infiltration, 998 OKT3, 2555 Interstitial nephritis, 971, 973 polyclonal lymphocyte depleting antibodies, Intima, 721 2554–2555 Intra-abdominal abscesses, 975 2714 Index

Intracellular calcium, 2112–2113 Ketogenic diet, 1833 Intradialytic hypotension, 2444 Ketorolac, 1853 Intramembranous ossification, 280 Ki-67, 849 Intraperitoneal pressure, 2386–2387 Kidney, 580–582, 1366 Intrauterine growth retardation, 1557 Kidney damage, 1696 Intravascular extension, 1892 Kidney development, 4, 527–530 Intravascular volume contraction, 263 Kidney disease impact, 389–390 Intravenous fluids Kidney Disease Improving Global Outcomes (KDIGO), composition of, 384 1015, 2221, 2280 Intravenous methylprednisolone, 1010 Kidney Disease Outcomes Quality Initiative's (KDOQI) Intravenous therapy clinical practice guideline, 2212 choice and volume, 383–384 Kidney embryology, 4 colloid solutions and volume resuscitation, 383, 386 Kidney injury molecule-1 (KIM-1), 2150 parenteral, 383 Kidney malformations, 135 Intussusception, 1012 Kidney regeneration, 531, 533, 545, 546 Inverted formin 2, 815–816 Kidney regeneration, developmental approaches, Iohexol, 621, 2208 582–583 IQ, 2248 Kidney transplantation, 1397 IQGAP1, 812 Kidney vasculature Iraq, 2651 Development, 38 Iron, 2222, 2224 vasculogenesis and angiogenesis, 38–39 Iron sucrose, 2226 Kinins, 194 Ischemia, 1656, 1661 Korotkoff sounds, 1908 Ischemia reperfusion injury (IRI), 1130–1131 Kuwait, 2652–263 Ischemic thrombosis of bone, 1476 ISKDC, 855 Isolated cystinuria, 1177 L Isolated dominant hypomagnesemia, 1230–1232 Labetalol, 2049 Isolated glycinuria, 1181 Laboratory examinations, 2143 Isolated kidney transplantation, 1397 Lactate, 264 Isolated proximal RTA, 1282 LAMB3, 824–825 Isolated recessive hypomagnesemia, 1232 Laminin, 778, 990 ItalKid Project, 2215 Laminin β 2 gene mutations (Pierson syndrome), 762–763 Italkid study, 2218 Laminins, 819 ITGA3, 824–825 Lanthanum, 2234 Laparoscopic ureteral re-implantation, 1735 Laser capture microdissection (LCM), 741 J Late non-responders, 855 Japanese Pediatric IgAN Treatment Study Group, 1010 Latent period, 963 Japanese Society for Pediatric Nephrology, 2687 Latin American Association of Pediatric Nephrology. Japanese Study Group of Kidney Disease in Children See Associaciation Latinoamericano de (JSKDC), 2694 Nefrologica Pediatrica (ALANEPE) Japanese Study Group of Renal Disease in Children Latinoamericano de Trasplante Renal Pediatrico (JSRDC), 2692 (RELATREP), 2636 Joints and muscles, 1453–1454 Lazy bladder, 1790 Jordan, 2652 L-cystine, 1158 LDL, 2240 Lead, 1378 K Lead (Pb), 1682 Kaplan Meier analysis, 490 Lebanon, 2653 Kappa statistic, 489 Lectin pathway, 935, 962, 1007 Kawasaki Disease (KD), 1432 Left ventricular hypertrophy, 1924 KCNJ10, 1230 Left ventricular hypertrophy (LVH), 2581 KDIGO, 863 Legionella pneumonia, 1377 KDIGO classification, 2211 Leptin, 1965 K/DOQI stages, 2212 Lesch-Nyhan syndrome, 1594, 1847 Kearns–Sayre syndrome (KSS), 305 Leukopenia, 860, 2591–2592 Kelley–Seegmiller syndrome, 1594 Leukotriene, 919 Keratopathy, 1348 Levamisole, 857 Index 2715

Liddle's syndrome, 265, 1970, 2067 Magnetic resonance imaging (MRI), 1730 Light chain cast nephropathy, 720 Magnetic resonance urography (MRU), 678 Light chain deposition disease, 715 Maintenance electrolyte therapy, 371–372 Light chains, 741 Maintenance therapy, 366–368, 1471 Light microscopy (LM), 709, 725 Major histocompatibility complex (MHC), Likelihood ratios (LR), 493 987, 2458–2459 Linkage analysis, 434 Maladaptation, 2178 Linkage and candidate gene studies, 1838–1839 Malaria, 2616 Linkage-based, 987 Malaysia, 2647 Lipases, 2113 Malformation, 157 Lipids, 2185 Malignancies, 841 Lipomeningocele, 1798 Malnutrition, 2223 Lipoprotein glomerulopathy, 793–794 Mammalian target of rapamycin (mTOR) pathway, Lipoxins, 921 1131–1132 Liver, 1366 Mannan-binding lectin pathway, 989 Liver transplantation, 1397 Mannitol, 190 Liver-type fatty acid binding protein, 2152 Mannose-binding lectin (MBL), 935 LMW Proteinuria, 1360, 1362 Mann-Whitney test, 490 LMX1B, 816 MAPK/ERK, 994 Loading dose, 2270 Masked hypertension, 1914, 2000, 2236 Local acidification, 1831 Massive proteinuria, 840 Long-term outcomes, 2145 Mass spectrometry, 433 Loop diuretics, 968, 2046 Mass transfer area coefficient (MTAC), Loss of heterozygosity at 11p15, 1876 2388–2390 Low birth weight, 1556 Masugi, 893 Lower urinary tract development, 21–26 Matrix metalloproteinase 2, 918 Lower urinary tract (LUT) disorders, 1791 Matrix production, 887 Lower urinary tract (LUT) malfunctions, 1789 Maturity-onset diabetes of the young type 3 Lowes Syndrome, 1285 (MODY 3), 1187 lowe syndrome, 1361, 1364–1365, 1840 MBL-associated serine proteases (MASPs), 935 Low purine diet, 2039 MCD, 717, 719, 722, 724, 726 Low renin hypertension, 1236 MCP-1, 904, 912 L-thyroxine, 1341 Mean, 488 Luer-lock catheter, 2391 Mean corpuscular volume (MCV), 2224 Lupus nephritis, 905 Mechanoreceptors, 189 LUT dysfunction (LUTD), 1805–1810 Median, 488 Treatment, 1808–1809 Medical care standards, 1350 LVH, 2236 Medical expulsive therapy, 1854 Lymphoma, 693 Medication history, 2003 Lymphomatous disorders, 841 Medullary cystic kidney disease, 1093 Lyonization, 736 Medullary sponge kidney, 1289, 1835 Lysinuric protein intolerance, 1172, 1177–1178, MEFV, 987 1361, 1374 Megacystis microcolon intestinal hypoperistalsis Lysis, 999 syndrome (MMIHS), 1769 Lysosomal integral membrane protein (LIMP-2), 822 Megalin, 896, 1658–1659 Lysosomal membrane transport, 1330 Definition, 1361 Lysosomal storage diseases, 1349 in Dent disease patients, 1356 Lysosomes, 1332 in Lowe syndrome patients, 1364 Lytic TCC, 942 recycling of, 1362 role of, 1355, 1361 structure of, 1356 M Megaureter, 680 Machines, 2158–2159 Melamine-containing kidney stones, 1833 Macrophage migration inhibition factor, 903 MELAS syndrome, 305, 1591 Macrophages, 899, 901 Melatonin, 1954 Macroscopic hematuria, 1003 Membrane attack complex (MAC), 905, 935, 989 Magnesium, 2038 Membrane cofactor protein (CD46), 936 Magnesium metabolism disorders, 1226–1236 Membrane cofactor protein (MCP), 907, 1506 Magnetic resonance angiography (MRA), 2007 Membrane transport principals, 64–65 2716 Index

Membranoproliferative glomerulonephritis, 950, Metabolic diseases, 708 973, 1003 Metabolic dysfunction, 2186 animal models, 1040–1041 Metabolic memory, 1554 characterization, 1036 Metabolic syndrome, 2059–2060 clinical outcomes, 1047–1048 Metabolomics, 441 clinical presentations, 1043–1045 Meta-iodobenzylguanidine (MIBG), 676 complement system, 1037–1040 Metanephrines, 2015 diagnosis, 1045 Metanephros, 7–8 differential diagnosis, 1045–1046 Methicillin-resistant Staphylococcus aureus, 969 in patients with, 1040–1043 Methioninuria, 1180 treatment, 1046–1047 Methotrexate, 1675 Membranoproliferative glomerulonephropathy Methylprednisolone, 855 (MPGN), 1531 Methylprednisolone pulse therapy, 863 Membranoproliferative glomerulophritis, post Metoprolol, 2049 transplantation, 2530 Mexico, 2633 Membranoproliferative GN, 970 Michaelis-Menten elimination, 2274 Membranous nephropathy, 951–952, 1361, 1377 Microalbuminuria, 1529, 1724, 1925, 2145 Membranous nephropathy (MN) Microalbuminuria (MA), 1547, 1548 clinical features, 1060 screening recommendations, 1557 histopathology, 1056–1060 Microangiopathic hemolytic anemia (MAHA), 1531 incidence, 1056 Microcirculation, 916 pathogenesis of idiopathic MN, 1060–1062 Microcoria, 818 pathogenesis of secondary MN, 1062–1064 Microembolization, 972 treatment, 1064–1071 MicroRNAs (miRNAs), 993, 1553 Memory, 2248 Microscopic polyangiitis, 720, 733 Mendelian disease, 426 Microscopic Polyangiitis (MPA), 1437 Mendelian disorders, 236 Microthrombi formation, 1500 Mendelian genetics, 426–427 Microvesicles, 944 Meningocele, 1797 Microvillous inclusion disease, 1361, 1374–1375 Mercaptoacetyltriglycine (MAG3), 2005, 2006 Miction, 1781 Mercaptopropionyl glycine (MPG), 1176 Micturition, 602 Mercury, 1378 Middle aortic syndrome, 2011 Mesangial cell, 994 MIM173900, 1117 Mesangial cells, 886 MIM173910, 1117 Mesangial deposits, 988 Mineral and bone disorder. See Chronic kidney disease Mesangial hypercellularity, 998 mineral and bone disorder (CKD-MBD) Mesangial interposition, 1001 Mineralization, 2228 Mesangial proliferative glomerulonephritis, 973 Mineralocorticoids, 233 Mesangial proliferative GN, 970 Minimal change, 840 Mesangial waists, 967 Minimal-change disease (MCD), 706, 840 Mesangiocapillary junctions, 996 Minimal glomerular changes (MCD), 810 Mesangiolysis, 922 Minoxidil, 2049 Mesangium, 887, 992 Misconduct, 495 Mesoderm embryonic layer, 279 Misdirected repair, 2178 MEST score, 1000 Mitochondrial cytopathies, 795 Meta-analyses, 1015 Mitochondrial DNA, 726 Metabolic acidosis, 249, 260–261, 1359, 1528 Mitochondrial hypomagnesemia, 1235–1236 Metabolicacidosis, 258 Mitochondrial respiratory chain, 1357, 1377 Metabolic acidosis Mitochondriopathies, 820, 1361, 1365–1367 causes, 410 Mitrofanoff's catheterizable stoma, 1802 in children, 410 Mixed acid-based disorder, 412–413 description, 409 Mixed chimerism, 2482 respiratory compensation, 410 Mizoribine, 1011 Metabolicalkalosis, 258 Mode, 488 Metabolic alkalosis, 261, 263, 265, 414 Modification of Diet in Renal Disease (MDRD) trial, 2219 in children, 412 Molecular structure of amino acid transporters, 1160 description, 411 Mongolia, 2641–2642 development, 412 Monoamino transporter branch, 1162 respiratory compensatory response, 412 Monocyte chemoattractant protein (MCP-1), 995, 1551 Index 2717

Monocyte/macrophage, 994 Necrotizing vasculitis, 1975 Monogenic disorders, 1839 Needle biopsy, 712, 734 Monogenic lupus, 1466 Negative predictive value, 493 Monomeric IgA, 988 Neoadjuvant chemotherapy, 1888 Monosymptomatic enuresis, 1803–1804 Neonatal AKI, 2143 Treatment, 1804 Neonatal HTN, 2074–2076 Morphogenesis, 4 Neonatal lupus, 1465–1466 Morphometry, 714, 715 Neonatal severe primary hyperparathyroidism (NSHPT), Mouse ESCs/iPSCs into embryonic renal stem/progenitor 1222, 1224–1225 cells, 535–540 Neonatal ureteral ligation, 1752 MPGN type II, 907 NEPH1, 812 mRNA, 725 Nephrin, 814 M-type phospholipase A2 receptor antibodies, 1061 Nephrin molecule, 756 Multicystic dysplastic kidney (MCDK), 103, 109, Nephritis associated plasmin receptor (NAPlr), 962 126–128, 682 Nephritogenic streptococci, 961 Multidisciplinary, 2250 Nephrocalcinosis, 272, 691, 1205, 1211, 1392, 1834 Multi-hit mechanism, 993 Nephrogenic diabetes insipidus (NDI) Multiple endocrine neoplasia type 2 (MEN 2), 2015 clinical manifestations, 1307–1309 Multiplex ligation dependent probe amplification definition, 1307 (MLPA), 1507 diagnostic procedures, 1309 Multisystemic involvement, 1346 differential diagnosis, 1309 Muronab-CD3, 2555 electrolyte imbalance, 1307–1308 Muscle, 1366 failure to thrive, 1308 Musculoskeletal complications, 2586–2589 in females, 1317 linear growth, 2587–2588 genetics, 1312 Mutation, in sodium bicarbonate cotransporter, 1283 genotype-phenotype correlations in X-linked Mutations in EHHADH, 1285 NDI, 1314 Mutations in the phospholipase C epsilon 1 (PLCE1) hereditary forms of, 1317 gene, 763 history, 1307 Mutations, in transcription factor HNF1, 1285 Hydronephrosis, 1308 Myanmar, 2647–2648 Hydroureter, 1308 Mycobacterium tuberculosis infection, 2579 hypernatremic dehydration, 1308 Mycophenolate mofetil (MMF), 861, 866, 1010, Megacystis, 1308 1068–1067, 1468–1469 molecular machinery, 1310 Mycophenolic acid, 2561–2563 plasma vasopressin levels, 1309 Myeloma, 1361 psychological development, 1308 Myelomeningocele, 1797 trabeculated bladder wall, 1308 Myeolodysplasia, 1797 treatment, 1318–1321 Myocardial hibernation, 2444 vasopressin test, 1309 MYO1E, 813 Nephrogenic mesenchyme, 1871 Myofibroblasts, 995 Nephrogenic syndrome, 199 Myoglobin, 720 Nephrolithiases, 1396 Nephrolithiasis, 272, 1724, 2626 Nephronal factors, 2108 N Nephron formation, 8–10 Na+-amino acid co-transporter, 1167–1168 Nephron hypertrophy, 2189 N-acetylcysteine, 1680, 2154 physiological consequences, 2190 Na+-dependent transport systems for amino acids, 1157 Nephron loss, 2191 + Na /HCO3 cotransporter (NBC), 252 Nephron number, 1920 Nail-patella syndrome (NPS), 720, 789–791, Nephronophthisis, 814 816, 819–820 Nephronophthisis and medullary cystic kidney disease Na+/K+ ATPase, 251–252 animal models, 1091 Na+/K+ ATPase maturation, 65–66 diagnosis, 1093–1094 National Institute of Health and Care Excellence disease mechanisms, 1082–1084 (NICE), 1708 extrarenal clinical manifestations, 1091–1093 Naturally derived materials, 577 natural history, 1079–1082 NBC1, 1283 pathology and histopathology, 1082 Nebivolol, 2049, 2057 prognosis and therapy, 1095 Necrosis, 917, 1659 Nephron progenitors/cap mesenchyme, 10–11 2718 Index

Nephrons, 1309–1312 Normal growth Nephron segmentation, 12 childhood phase, 638 Nephropathic cystinosis, 1330 infancy phase, 637 Nephrosialidosis, 792 puberty, 638 Nephrosis, 840 Normalized protein catabolic rate (nPCR), 655, 2451 Nephrotic syndrome, 604, 607, 960, 974, 975, 1003, 1065, Normal miction, physiology of, 1784 1361, 2622–2624 Normal physiology of avp on the distal nephrons, diet modification, 358 1309–1312 Nephrotoxicity, 861 Normal voiding frequency, 1788 Nephrotoxic medication, 2144 North American Pediatric Renal Trials and Collaborative Nervous system, 1454–1455 Studies (NAPRTCS), 2214, 2553, 2557, Nesiritide, 2154 2559, 2560 Neuraminidase (sialidase), 963 Not otherwise specified, 849 Neurocognitive, 2247 NPHP1, 1084–1085 Neurofibromatosis, 2015 NPHP3, 1088 Neurogenic bladders, 1789, 1797 NPHP4, 1088–1089 Management, 1797 NPHP5, 1089 Neurologic abnormalities, 2017–2018 NPHP6/CEP290, 1089–1090 Neurologic complications, 2592 NPHP7/GLIS2, 1090 Neuromodulation, 1802, 1808 NPHP2/inversin, 1085–1088 Neuro-muscular physiology, of bladder, NPHP9/NEK8, 1090 1783–1784 NPHP8/RPGRIP1L, 1090 Neurophysins, 190 NPHS1, 756–757, 811 Neutral endopeptidase, 1061–1062 NPHS2, 760–761, 810 Neutrophil extracellular traps, 900 NPHS3, 763 Neutrophil gelatinase-associated lipocaclin (NGAL), NSAIDs, 1209, 1678–1679 1498, 2150 Nuclear factor-kappaB, 995 Neutrophils, 899 Nuclear inclusions, 721 Newborn screening test, 1339 Null hypothesis, 474 New Zealand, 2673 Nutrition, 2160, 2244–2247 Next generation sequencing (NGS), 428, 431, 435–436, Nutritional consultations, 1340 440, 785, 816 NHANES, 2221 NHANES study, 2221 O Nicardipine, 2052 Obese children, 1997 Niche, stem cell, 554–555 Obesity, 1810, 1915, 2001, 2035, 2060, 2174, 2238, 2586 NIDUS, 2437 hypertension, 1965–1966 Nifedipine, 2050 Observational designs, 475 Nitric oxide (NO), 194, 891, 922, 1959–1960, 2107 Obstruction of upper respiratory airways, 1810 NKCC2 (type I BS), 1204 Obstructive sleep apnea (OSA), 1977, 2060–2061 NO, 903 Occludin and claudin, 65 NO2, 922 Oceania, 2677–2678 Nocturia, 1789 Ochoa's syndrome, 1810–1811 Nocturnal enuresis, 2615 OCT. See Organic cation transporter family (OCT) Non-carbonic acid, 268 Oculo-cerebro-renal syndrome of Lowe, 1284 Non-glomerular disease, 2219 Odds ratio, 490 Non-infectious HUS, 1503 O-glycosylation sites, 990 Non-lytic TCC, 942 1,25(OH)2D, 2350 Nonmuscle myosin heavy chain IIA (NMMHC-IIA), 791 OKT4, 992 Non-nephrotic proteinuria, 960 OKT8, 992 Non-neurogenic neurogenic bladder (NNBB), 1810 Oligohydramnios, 105 Nonparametric statistical-tests, 488 sequence, 1109 Nonprobability sample, 485 Oligonephronia, 2064 Non-radioactive iothalamate, 621 Oliguria, 598, 964 Non-steroidal anti-inflammatory agents, 841 Oman, 2653 Non-steroidal anti-inflammatory drugs (NSAIDs), Omapatrilat, 2057 921, 2144 Oncogenic osteomalacia (OOM), 312–314, 320 Norepinephrine, 2154 One-compartment model, 2270 Normal aging, 718 Open biopsy, 712 Index 2719

Open lithotomy, 1853 Papillary necrosis (PN), 1528–1529, 1532–1535 Open/modified open biopsy, 709 Papillorenal syndrome, 817 Ophthalmic manifestation, 1336 Paracalcitol, 2220 Opsonization, 938 Paraformaldehyde, 713 Oral and esophageal lesions, 2589 Paragangliomas, 1975 Oral contraceptive, 2073 Paraguay, 2633 Oral cysteamine therapy, 1342, 1349 Parametric statistical tests, 488 Oral fluids, composition, 380 Paraquat, 1378 Oral rehydration Parasitic infections, 2579–2580 for previously healthy, well-nourished children, 379 Parathyroidectomy, 1225, 2367–2368 schemes, 381 Parathyroid hormone (PTH), 292–293, 301, 632, and serum sodium abnormalities, 381 648, 2228, 2231, 2353–2354 Oral rehydration solutions (ORS), 382–383 Parathyroid tumors, 295–296 Oral rehydration therapy Parental consanguinity, 601 electrolyte solutions, 377 Parenteral intravenous therapy, 383 rapid rehydration, 386 Paricalcitol, 2235 Organic acid transport, 70 Partial lipodystrophy, 950 Organic anion transporter (OAT), 1657 Partial nephrectomy, 1893 induction, 70 Partial pressure of carbon dioxide (PaCO2), 256 Organic anion-transporting polypeptide (OATP) Partial remission, 1472 family, 1657 Parvovirus B19, 840 Organic cation transporter family (OCT), 713, 1657 Passive transport, 65 Organ transplantation, 1396–1399 Pathogenesis of lupus nephritis, 1456–1457 Oscillometric BP, 1908 Pathophysiology, 2148 Oscillometric method, 1999 Patient history, 1790 Osmium, 715 PAX2, 817 Osmoprotective molecule, 200–201 PAX6, 818 Ossification, 279 PCNA, 849 Osteitis fibrosa cystica, 2228, 2355 PDSS2, 821 Osteoclasts, 283 Pearson's correlation coefficient, 490 Osteocytes, 282 Pediatric CKD. See also Chronic kidney disease in Osteodystrophy, 1341, 2227 children (CKiD) Osteoid, 282 transition to ESRD, 2249–2251 Osteomyelitis, 975 Pediatric intensive care unit, 390–391 Osteonecrosis, 2369 Pediatric kidney transplantation, complication rate, 2593 Osteopenia, 1348 Pediatric Mizoribine Study Group, 2693 Osteopetrosis, 1290 Pediatric peritoneal dialysis Osteoporosis, 272, 857 adequacy, 2403–2411 Overactivation, 928 encapsulating peritoneal sclerosis, 2416–2417 Overflow proteinuria, 622 equipment, 2390–2397 Oxalate, 1390 history, 2382–2383 Oxalobacter formigenes, 1829 infectious complications, 2411–2414 Oxalosis, 708 maintenance, 2397–2399 Oxford classification, 1000–1001 membrane function assessment, 2387–2390 Oxidant stress, 994 non infectious complications, 2414–2416 Oxidative metabolism and ROS generation, 2190 prescription, 2399–2403 Oxidative phosphorylation treatment outcomes, 2417–2419 ATP production, 1365 Pediatric renal transplantation, 574 definition, 1366 contraindications, correctable or temporary, inhibition of, 1357 2507–2509 in respiratory chain, 1365 demographics, 2504–2507 Oxidative stress (OS), 1552, 1661–1662, 1674, 2185 donor selection, 2513–2516 graft dysfunction, 2517–2519 incidence and frequency, 2502–2504 P indications and contraindications, 2507–2509 Pakistan, 2645 long term outcomes, 2531–2534 Palestine, 2653–2654 mortality, 2534 p-aminohippurate (PAH), 1657 recipient preparation, 2511–2513 Panama, 2633 recurrent diseases after transplantation, 2527–2531 2720 Index

Pediatric renal transplantation (cont.) Physical therapies, 1348 rejection, 2521–2527 Pierson syndrome, 725, 762, 788–789, 818 renal transplant biopsy, 2520 PKD1 gene, 1104, 1117 surgical considerations and intraoperative PKD2 gene, 1104, 1117 management, 2516–2517 Placental ischemia, 1978 transition to adult care, 2534–2535 Plagiarism, 495 urologic complications, 2519–2520 Plasma cells, 721, 738, 741 PedsQL, 2249 Plasma exchange, 868, 1015, 1471 Pegylated, 2226 Plasmapheresis, 974 Pelvic electromyography, 1791 Plasma potassium concentration, 222 Pelvic stimulation, 1787 Plasma protease cascades, 928 Pelvic ultrasound, 1791 Plasma protein binding, 2274 Penicillin, 968 Plasma protein insudation, 718, 722 Peptidome, 441 Plasma renin activity, 964, 2007 Pericarditis, 1454 Plasmin-like activity, 962 Perihilar variant, 849 Plasminogen, 918 Periodic acid-Schiff (PAS) stains, 714, 739 activator inhibitor, 918 Perioperative prophylactic antibiotics, 2157 activator inhibitor-1, 914 Periostin, 286 activator receptor, 918 Peripheral adrenergic neuron antagonists, 2048 Platelet-derived growth factor (PDGF), 914, 994 Peripheral tolerance, 2481 Platelets, 916–917 Perirenalurinoma, 99 Platinum, 1378 Peritoneal dialysis (PD), 393, 1396, 2156, 2157, 2288 PLCE1, 812 Peritoneal equilibration test (PET), 2387–2388 PLCε1 gene mutations (NPHS3), 763–764 Peritoneal membrane, 2383–2384 PLCG2, 825 Peritubular capillary endothelia, 15 Pluripotent stem cells, 533–534 Perlman syndrome, 1880 directed differentiation of, 534–535 Peroxisome proliferator-activated receptor γ Pneumococcal antigen, 975 (PPARγ), 1971 Pneumococcal vaccine, 2250 Personal dialysis capacity (PDC) test, 2390 Pneumocystis, 2579 Personal history, 2003 Podocin, 814, 819 Peru, 2633 Podocin gene mutations (NPHS2), 760–761 pH, 248, 256 Podocytes, 755, 840, 886, 889–890, 1549 PHA1, renal vs. generalized forms, 1247 actin cytoskeleton, 826 Pharmacologic Treatment, 2041 depletion, 994 Phase II clinical trials, 483 dysregulation, 840 Phase III trials, 483 foot process effacement, 867 Phase I trial, 483 terminal differentiation, 13 Phase IV clinical trials, 483 Podocytopathy, 840 Phenocopies, 1103 Pollakiuria, 599 Phenotypic subtypes, 1174 Polyarteritis nodosa, 721 Phenytoin, 2274 Polycitra, 1340 Pheochromocytoma, 1975, 2013–2015, 2068 Polyclonal B cell responses, 960 Philippines, 2648–2649 Polycystic kidney and hepatic disease 1 (PKHD1) gene, Phosphate, 250, 1340 1106–1107 additives, 355 Polycystic kidney disease (PKD) buffer system, 249 ADPKD (see Autosomal dominant polycystic kidney transport development, 70–73 disease (ADPKD)) Phosphate-binding agents, 2362–2363 ARPKD (see Autosomal recessive polycystic kidney Phosphate-independent mechanisms, 2350 disease (ARPKD)) Phospholipase A2 receptor (PLA2 R) antibodies, 1061 cellular pathophysiology, 1126–1127 Phospholipase C epsilon1, 812–813 cyst formation, 1127–1128, 1136 Phospholipids, 2113 cystic phenotype, 1136–1138 Phosphorus, 351–353, 2228, 2231 definition, 1103 absorption, 357 differential diagnosis of, 1104–1106 handling, developmental changes in, 346 fluid secretion (see Fluid secretion) intake for children with CKD, 2233 molecular pathogenesis, 1124–1126 Recommended minimum intake, 347 Polycystin-1 (PC1), 1104, 1117 Physical activity, 1915–1916 Polycystin-2 (PC2), 1104, 1117 Index 2721

Polydipsia, 1358 Potter phenotype. See Oligohydramnios, sequence Polyductin. See Fibrocystin(FC) Power Doppler mode, 670 Polyhydramnios, 1205 Pre-dialysis CKD, 2690 Polymeric IgA, 988 Prednisolone, 855, 1010 Polymorphisms, Ig, 987 Prednisone, 855 Polyol pathway, 1552 Preeclampsia, 720 Polyoma virus, 741–742 Pre-emptive, 2251 Polysaccharide, 989 Pregnancy, 2018 Polytypic PTLD, 741 Pregnancy-induced HTN, 1978–1979 Polyuria, 370, 598, 1339, 1358, 1360, 1532, 2153 Prehypertension, 1908 Poor appetite, 1337 Premature infants, 646 Population-based data, 1855 Prematurity, 1919–1920 Population parameters, 485 PRENATAL Hydronephrosis, 106–108 Portal hypertension, 1348 Prepubertal duration, 1555 Portosystemic shunting, 1006 Pre-renal classification, 2147 Positional cloning, 434 Prevalence, 492 Positional instillation of contrast (PIC) cystography, 1729 Prevention, 2152–2153 Positive predictive value, 493 Preventive treatment, 968 Postconcussion syndrome (PCS), 2030 Primary distal renal tubular acidosis, 1833 Posterior reversible leukoencephalopathy, 969 Primary glomerular injury, 2179 Posterior urethral valves (PUV), 101, 105, 1795–1796 Primary glucocorticoid resistance, 1970 bladder and urethra development, 1769 Primary hyperaldosteronism (PAL), 1975–1976 characterization, 1769 Primary hyperoxaluria clinical presentation and diagnosis, 1770 AGXT mutations, 1849 genetics, 1770 combined kidney and liver transplantation, 1851 management, 1770–1771 description, 1849 pathophysiology, 1770 diagnosis, 1850 Postinfectious glomerulonephritis, 734–735 hemodialysis, 1850 Post-micturition dribble, 1789 prevalence, 1849 Post-renal classification, 2146 pyridoxine treatment, 1850 Postscarlatinal glomerulonephritis, 960 types, 1849 Post streptococcal acute glomerulonephritis, 905 Primary hyperoxaluria type 1 (PH1) Post-streptococcal glomerulonephritis (PSGN), 945–946, combined liver-kidney transplantation, 1397–1399 960–961, 2616 diagnosis, 1392–1393 Posttransplantation lymphoproliferative disease, 868 dialysis, 1396 Post-transplant complications, 2592 infantile form of, 1391 Post transplant diabetes mellitus (PTDM), 2560, kidney transplantation, 1397 2583–2584 liver transplantation, 1397 Posttransplant lymphoproliferative disorder (PTLD), 2556 mutation analysis, 1394 Post-traumatic stress disorder (PTSD), 2030–2031 oxalate burden, 1393–1394 Potassium, 2038, 2241 pathophysiology, 1390–1392 concentration, 394 post-transplantation, 1398–1399 decreased dietary intake, 402 preemptive liver transplantation, 1398 distribution, 394 prenatal and preimplantation diagnosis, 1394 endocrine effects, 403 supportive measures, 1394–1395 eneteral supplementation, 407 urological aspects, 1396 extrarenal losses, gastrointestinal tract, 403 Primary hyperoxaluria type 2 (PH2) hyperkalemia, 394–401 clinical presentation, 1400–1401 random urinary levels, 398 diagnostic test, 1401 renal losses, 404–405 genetics, 1400 therapies redistribution, 400 metabolic derangement, 1400 transcellular redistribution, 402–403 treatment and prognosis, 1401 transcellular redistribution of, 396 Primary hyperoxaluria type 3 (PH3) urgency supplementation, 407 clinical presentation, 1401–1402 urine quantification, 405–406 diagnostic test, 1402 Potassium chloride supplements, 407 genetics, 1401 Potassium phosphate, 2159 metabolic derangement, 1401 Potassium-sparing diuretics, 2046 treatment and prognosis, 1402 Potassium supplementation, 1215, 1921 Primary hyperparathyroidism (PHPT), 318, 1976 2722 Index

Primary hypertension, 1997, 2004–2005 Pulmonary metastasis, 1892 Principal cell, 253 Pulmonary receptors, 189 Probability sample, 485 Pulse steroid therapy, 974 Prognosis, 969 Purposive sampling, 485 acute TIN, 1420 Putative transmembrane Crumbs homolog 2 protein, 813 in children and in adults, 969 Pyridoxine, 1395, 1399 factors, 1461–1463 Programmed death 1 (PD-1), 2469–2470 Progressive osseous heteroplasia (POH), 309 Q Pro-inflammatory cytokines, 903 Qatar, 2654 Proliferation, 887 Quality of life, 2249 Properdin, 931 Quetiapine, 403 Prophylactic antibiotic treatment, 1832 Quinine, 1511 Prorenin, 1956 Quota sampling, 485 Prostaglandins, 193–194, 891, 904, 919 Protease-activated receptor-2, 919 Proteasomes, 986 R Protein, 353–354, 2160 RAC1, 823 buffer system, 249 Race, 1916 intake, 1556 Radiation therapy, 1895 Protein b0,+AT, 1174 Radiographic evaluation, 1884–1888 Protein-losing enteropathy, 1012, 1014 Radiolucent kidney stones, 1844 Protein tyrosine kinase (PTK), 229 Radionuclide cystography (RNC), 676 Protein tyrosine phosphatase receptor type O Radionuclide imaging, 2005 (PTPRO), 813 Radionuclide renal scintigraphy, 1761–1763 Proteinuria, 622, 964, 973, 1723, 1730, 2174, 2218 Radiosensitivity, 1880 Proteome analysis, 431–434 Randall´s plaques and plugs, 1836–1837 Proton–coupled imino acid transporters (PAT), 1167 Randomized controlled Proto-oncogene, 295, 1131 clinical trials, 482–483 Proximal and distal RTA, 1274 trial, 1008 Proximal convoluted tubule (PCT), 225 Range, 488 Proximal renal tubular acidosis (Type II RTA), 1282 RANTES, 904, 912 Proximal tubular defect, 1333 Rapidly progressive, 1003 Proximal tubular epithelial cells, 995 Rapidly progressive glomerulonephritis (RPGN), Proximal tubule, 198, 1276 707, 1014 dysfunction, 1529 Rapid rehydration, 386 NaCl transport development, 75 Rarefaction, 1952 transport, 66–67 rBAT/SLC3A1, 1173 water transport, 77–78 Reabsorbing bicarbonate, in proximal tubule, 1277 Proximal tubulear acidification development, 73–75 Reactive oxygen molecules (ROM), 2111 Prune belly syndrome (PBS), 1769, 1795 Reactive oxygen species (ROS), 900, 921, 1661, 2461 P-450 s, 1663 Recall bias, 480 Pseudohermaphroditism, 736, 818 Receiver operator characteristic (ROC) curves, 494 Pseudohyperaldosteronism, 238, 265 Receptors, 2243 Pseudohyperkalemia, 236, 395 Recessive oncogene, 295 Pseudohypoaldosteronism (PHA), 397, 1292 Recombinant human erythropoietin (rHuEPO) Pseudohypoaldosteronism type II, 1970–1971, 2068 therapy, 2221 Pseudohyponatremia, 373 Recurrence, in renal allograft, 1017 Pseudohypoparathyroidism (PHP), 308–310 Recurrence, of IgAN, 988 Pseudotumor cerebri, 1337 Red blood cell, 706 Pseudotumors, 1888 Red blood cell distribution width (RDW), 2224 Psychological disorders, 1809–1810 Red urine, 597 PTH. See Parathyroid hormone (PTH) Reference BP values, 1908 PTH levels, 1232 Reflection coefficient, 65 PTHrP, 283 Reflux nephropathy (RN), 1702, 1716, 2012–2013 Puberty, 638, 1554 in adults, 1725 Pudendal reflex, 1784 Regenerative medicine, 571 Pulmonary edema, 969 Regulated upon Activation, Normal T cell Expressed and Pulmonary involvement, 1454 Secreted (RANTES ), 995 Index 2723

Regulation of amino acid transport, 1158–1160 Renal osteodystrophy, 2350, 2354–2357 Relative risk, 480–481, 490 Renal parenchymal disease, 2012–2013 Removal of the infected shunt, 975 Renal phosphate transport regulation, 71 Renal agenesis, 109, 118, 163–164 Renal potassium excretion, 225 BRA, 120 Renal potassium wasting, 406 URA, 118, 120 Renal progression, 2217 Renal angina index (RAI), 2150 Renal replacement therapy, 2154–2156, 2219 Renal arteriography, 2006 Renal reserve, 2161 Renal artery stenosis (RAS), 063, 2693 Renal scarring, 2178–2188 Renalase, 1961, 2056 mechanism of renal injury, 1721–1722 Renal autoregulation, 1961 recent randomized studies, 1733 Renal biopsy, 840, 845, 1213 risk factors, renal injury, 1722 Renal blood flow, 964 Renal stem cells Renal cancer stem cells, 555 clinical application of, 557–558 Renal cell carcinoma (RCC), 555–556 kidney development, 527–530 Renal colic, 1175 Renal stroma, 13–14 Renal diet Renal structural injury, 2146 developmental physiology, 342–347 Renal sympathetic denervation, 2040 etiology of renal disease, 347 Renal sympathetic nerve activity (RSNA), 1953 prevention of cardiovascular disease and Inflammation, Renal transplantation, 1361, 1476, 2009 348–354 in pediatrics, 2574 Renal ectopia, 109 urinary tract infection (UTI), 2574–2575 and fusion anomalies, 129–130 Renal transplantation complication Renal excretion abnormalities, intrinsic/acquired anemia, 2590–2591 glomerular or tubular dysfunction, 396–397 bacterial infections, 2574–2580 Renal failure, 1335 BK virus (BKV), 2578–2579 Renal Fanconi syndrome, 1333 cardiovascular complications, 2580–2581 Renal function, 1925–1926 Cytomegalovirus infections, 2575–2576 Renal handling of drugs dyslipidemias, 2584–2586 CKD and dialyzability, 2277–2280 electrolyte abnormalities, 2592–2594 drug removal and dosing during CRRT, 2281–2288 Epstein-Barr virus infections and PTLD, 2576–2577 drug removal during intermittent hemodialysis, fungal infections, 2580 2280–2281 gastrointestinal complaints, 2589–2592 Renal hypoplasia/dysplasia (RHD), 2064 hypertension, 2581–2583 autosomal dominant renal-coloboma syndrome neurologic complications, 2592 (RCS), 122 obesity, 2586 BRHD, 120 parasitic infections, 2579–2580 clinical presentation in utero, 115–118 pneumocystis, 2579 defects in ureteric bud branching, 121–122 post-transplant diabetes mellitus, 2583–2584 definition of, 115 pulmonary complications, 2594 dysregulation of the nephron progenitor pool, 122–123 transplant renal artery stenosis, 2582–2583 environmental causes, 124–125 Varicella infections, 2577–2578 lower tract anomalies, 124 Renal tubular acidosis (RTA), 397, 404, 629 polymorphic gene variants, 125 Renal tubular cell hyperplasia, 1128–1129 subtle renal hypoplasia, 125–126 AKI/IRI, 1130–1131 syndromic genes, 123 apoptosis and proto-oncogenes, 1131 URHD, 120 arachidonic acid metabolites, 1132 Renal imaging recommendations, 1736 cAMP mediated proliferation and intracellular calcium, Renal injury, biomarkers, 1730 1129–1130 Renal injury risk factors c-Src, 1130 bladder bowel dysfunction, 1722–1723 EGFR, 1129 delayed treatment, 1723 mTOR pathway, 1131–1132 young age, 1723 PC1, Stat3 and Src, 1130 Renal interstitial hydrostatic pressure (RIHP), 1961 Renal tubular dysplasia (RTD), 123–124 Renal/intrinsic classification, 2146 Renal tubular plugging, 1836 Renal involvement, in type 2 diabetic patients, 1559–1560 Renal tubulointerstitial injury, 2189 Renal ischemia, 1526 Renal tubulopathies, 404–405 Renal medullary carcinoma (RMC), 1533 Renal ultrasound, 1413, 2004, 2148 Renal metabolism, 1662–1663 Renal vascular development, 14–17 2724 Index

Renal vein thrombosis, 694, 721 Scarring, biochemical events, 2185–2187 Renin, 263, 1213, 2000 Scarring process, cell biology, 2183 Renin-angiotensin-aldosterone axis, 191–192 Schimke immuno-osseous dysplasia, 820 Renin-angiotensin-aldosterone system (RAAS), Schimmelpenning-Feuerstein-Mims syndrome, 317 397, 1954, 2036 School-screening, 1003 Renin-angiotensin system (RAS), 987, 994, 1756, 2105 Screening, 1895–1896 Reninoma, 1976 laboratory evaluation, 2003–2004 Renin-producing tumors, 2015–2016 SCr monitoring, 2152 Renovascular disease, 2005, 2008–2012 Secondary active transport, 64 Renovascular thrombosis, 2148 Secondary enuresis, 598 Repeat renal biopsies, 1461 Secondary hyperoxaluria, 1390 Repetitive infusions, 385 Secondary hypertension, 1997 Reprogrammed somatic cells, 575–576 Secondary membranous nephropathy, 1059–1060 Residual renal function (RRF), 2280 Secondary MN, 1064 Respiratory acidosis, 260–261, 263 Secondary nephrotic syndromes, 764–765 in children, 409 Secondary spongiosa, 283 Respiratory alkalosis, 265 Segmental glomerulosclerosis, 1000 in children, 410 Sensitivity, 492 Retina, 1924–1925 Sensorineural deafness, 607, 781 retinal changes, 2001 Sequences, 157 retinal drusen, 950 Serine, 990 Retinoblastoma, 1871 Serology, 733 Retinopathy, 1348, 2003 Serum anion gap, 259–260, 410–411 Retrograde intraflagellar transport-A protein IFT139, 814 Serum β2 microglobulin levels, 2691 Reverse transcription polymerase chain reaction Serum creatinine, 2689, 2692 (RT-PCR), 742 Serum osmolality, 184, 368 Rhabdomyolysis, 720, 1495 Serum sickness, 960, 962 Rheos Baroreflex Hypertension Therapy System, 2040 Serum sodium Rheumatoid factors, 992 Serum sodium, abnormalities, kidney injury, rhGH, 2245–2246 391–394 RHOA, 823 2003 Seventh Report of the Joint National Committee Rho family GTPases, 823 (JNC 7), 1998 Rho-GDP dissociation inhibitor α (Rho-GDIα), 823 Severe hypernatremia, 388–389 rHuEPO, 2225 Seyffart's Directory of Drug Dosage in Kidney Risk, injury, failure, loss, end-stage kidney disease Disease, 2268 (RIFLE), 2140 SGLT-1, 67, 1183 risperidone, 403 SGLT1 (SLC5A1), 1183 Rituximab (RTX), 852, 862, 866, 868, 1070, 1469–1470 SGLT2 (SLC5A2), 1184 for Childhood-Onset Refractory Nephrotic Syndrome SGLT3 (SLC5A4), 1184 (RCRNS) Study Group, 2694 SGLT4 (SLC5A8), 1184 RIVUR trial, 1705 SGLT5 (SLC5A10), 1184 ROMK (type II BS), 1204 Shiga toxin, 1490, 1501 RRT modalities, 2154 mediated cell injury mechanisms, 1496–1500 Runx2, 285 mediated HUS, 1500–1501 Shigella dysenteriae, 1501–1502 Short run creatinine level, 722 S Sickle cell disease (SCD), 2616 Sacral agenesis, 1799 Sickle cell nephropathy (SCN) Salt intake, 1920–1921 clinical features, 1529–1533 Salt-sensitive hypertension, 183 pathogenesis, 1524–1529 Sample size, 486 treatment, 1534–1539 Sample statistics, 485 Sickle cell trait (SCT), 1538–1539 Sampling bias, 484 Sieving coefficient, 65 Sampling scheme, 485–486 Signal transducer and activator of transcription 3 Sanjad-Sakati syndrome, 306 (STAT3), 1130 Saudi Arabia, 2654–2655 Simple random sampling, 485 Saving Young Lives (SYL) program, 2608, 2678 Simpson-Golabi-Behmel syndrome, 1880 SCARB2, 822 Singapore, 2649 Index 2725

Single nucleotide polymorphisms (SNP), 430–431, Somatic cell nuclear transfer (SCNT), 573 987, 1698 Sotos syndrome, 1880 Single photon emission computed tomography South-East Asian ovalocytosis (SAO), 1289 (SPECT), 676 South Korea, 2642–2643 Single vessel vasculitis, 1431 Sox9, 285 Sirolimus, 2563–2564 Spastic diplegia, 1801 Sjogren's syndrome, 1290, 1361, 1835 SPEB/zymogen, 962 Skeletal mineralization, 2356 Specific evaluation, 2004–2008 Skin, 1366 Specificity, 492 leison, 1454 Speech therapies, 1348–1349 SLC5A1, 1183 Sphincters, 1783 SLC6A18, 1181 Sphygmomanometer, 1999 SLC6A19, 1179 Spina bifida occulta, 1799 SLC6A20, 1181 Spinal cord injury, 1799, 1832 SLC7A9, 1174 Spinal dysraphisms, 1797 SLC12A3, 1215 Spironolactone, 1221 SLC36A2, 1181 Sports drinks, 2034 SLC1 family, 1160–1161 Spraying, 1789 SLC6 family, 1162 Sri Lanka, 2646 SLC7 family, 1166–1167 Staghorn calculus, 1831 SLC 16 (TAT) family, 1167 Staging, pediatric renal tumors, 1882 SLC36 family, 1167 Standard permeability analysis (SPA), 2388 SLC 38 (SNAT) family, 1167–1168 Standard therapy, 1706 SLC3/SLC7 family of Heteromeric Amino Acid Staphylococcus aureus, 986 Transporters (HAT), 1166–1167 Statins, 1558 Sleep disordered breathing, 1922 Statistical inference, 475 Sleeping disorders, 1810 Statistical significance, 491–492 Slit diaphragm, 717 Statistical tests, 488–491 components, 755 Statistical validity, 476 Slow low efficiency daily dialysis (SLEDD), 2157 STEC HUS, 1491, 1495 SMARCAL1, 820 STEC infection diagnosis, 1492–1493 Smoking, 1556, 1922 Stem cell transplant, 2144 cessation, 1559 Stents, 2006 SNF2 subfamily, 820 Steroid-resistant nephrotic syndrome (SRNS), 806, Social functioning, 2249 840, 2693 Social stress, 1788 Steroids, 2557–2558 Socioeconomic status, 1922 resistant idiopathic nephrotic syndrome, 864 Sodium, 2038, 2241 Steroid-sensitive NS (SSNS), 825 abnormality evaluation, 202–203 1st generation immunometric assays (IMA), 2354 bicarbonate, 260 Stone analysis, 1852 in body fluid homeostasis, 187–189 Stone precursor renal papillary lesions, 1836 consumption, 1967 Stop dialysate flow method, 2451 deficit, 203–207 Straining, 1789 efferent mechanisms, 191–197 Stratified random sampling, 485 excess, 206–207 Streptococcal M types, 961 hydrogen ion exchangers, 250 Streptococcal pharyngitis clinical scores, 967 hypernatremia, 372 Streptococcal pyrogenic exotoxin B (SpeB), hyponatremia, 372–376 946, 962 laboratory assessment, 201–202 Streptococcus pneumonia, 842 osmolality regulation, 372 infections, 1502 requirement, 184–185 Streptococcus zooepidemicus, 961 sensor mechanisms, 189–190 Stress reduction, 2032 serum sodium, 372 Structural malformations of urinary tract, transport, 65 1831–1832 Soluble intercellular adhesion molecule-1 [ICAM-1] 996 Structural variants, 430–431 Soluble urokinase receptor, 840, 851 Study population, 484 Solutions, 2159 Subclinical nephritis, 963 Solvent drag, 65 Subepithelial humps, 946 2726 Index

Succinylcholine, 224 Tenofovir, 1670 Sulfation, 1663 Terminal complement complexes (TCC), 936 Superantigen-induced immune reactivity, 970 Terminal galactose, 990 Superantigens T cell activation, 960 Terminal pathway, 935–941 Superoxide anion, 921 Tertiary active transport, 64 Superoxide dismutase, 922 Tetracycline, 2232 51Cr EDTA, 618–619 Tetraspanin (CD37), 990 99mTc-diethylenetriaminepentaacetic acid (DTPA), 1763 TGF-ß, 1550 99mTc-dimercaptosuccinic acid (DMSA), 1763 Th1, 897 99mTc DTPA, 619 Th2, 897 Supportive therapies, 1348–1349 Th17, 897 Suppressor of cytokine signaling (SOS), 648 Thailand, 2649–2650 Surrogate markers, 1008 Thalassemia, 2615–2616 Susceptibility alleles, 426–428 T helper type 2 cells (Th2 cells), 2470 Sutures, 282 Theophyilline, 2154 Swallowing difficulty, 1346 Therapeutic cloning, 573 Swan neck, 1334 Therapeutic efficacy, 708 Swedish Reflux Trial, 1732 Therapeutic trials, 2150 Sympathetic nervous system (SNS) activation, 1964–1965 Thiazide diuretics, 2046 Symptomatic hyponatremia, 386–387 Thick ascending limb developmental transport, 78 Synaptopodin, 725, 819 Thick ascending limb of the loop of Henle (TALH), Syndrome, 157 226–228 Synechiae, 2179 Thin basement membrane nephropathy, Synsorb Pk, 1501 786–787 Synthetic polymer, 577 Thirst, 196 Syria, 2655 Threonine, 990 Systematic sampling, 485 Threshold, 66 System b0,+ 1172 Thrombocytopenia, 2591–2592 Systemic involvement, 1393 Thrombomodulin (THBD), 942, 1507 Systemic lupus erythematosus (SLE), 707, 715, 723, 731, Thromboplastin, 709 740, 947–948, 1062, 1512, 2626 Thrombosis, 844, 1475 Systemic vasculitis, 1975, 2012 Thrombotic microangiopathies (TMA), 948 Systolic function, 2237 Thrombotic microangiopathy (TMA), 726, 891, 916, 1482, 1504, 1512, 1531 Thrombotic thrombocytopenia purpura (TTP), 726, 1490, T 1502–1503 Tachycardia, 604 Thromboxan, 919 Tacrolimus (FK506), 740, 865, 1470, 2559–2560 Thyroglobulin, 714, 741, 2555 Taiwan, 2643 Thyroid disorders, 2016 Takayasu arteritis (TA), 1442–1444, 2626 Thyroid hormone, 1960 Targeted enrichment systems, 429 Tickover mechanism, 930 Target organ damage, 1923–1924 Tight junction, 65 Target population, 484 Tip variant, 849 T cell activation Tissue factor, 916, 918–919 4BB1, 2470 Tissue molds, 713 CD154/CD40 pathway, 2467–2468 Tissue oxygen, 2222 CD28/CTLA-4/B7-1/B7-2 pathway, 2465–2467 Tissue re-engineering ICOS/ICOSL Pathway, 2468–2469 bladder, 579 OX40/OX40L pathway, 2469 kidney, 581–582 PD-1/PD-L1/PD-L2 pathway, 2469–2470 urethra, 578–579 responses/immune deviation, 2470, 2472 Titratable acid, 250, 1278, 2242 T-cell immunodeficiency, 820 Titratable acid concentration, 1275 T-cell receptor, 896 T lymphocytes, 1408, 1452–1453 T cell subsets, 893 TMV, 2232 Technetium 99 m-diethylene triamine pentaacetic acid TNF-α,911 (99mTc-DTPA), 673 Toll-like receptors (TLRs), 903–905, 2461 Technetium 99 m dimercaptosuccinic acid renal Toluidine blue, 715 scan, 1729 Tonsillectomy, 1011 Tenckhoff catheter, 2382 Tonsils, 991 Index 2727

Total body water Tuberculosis, 2616–2617 effective circulating volume, 365–366 Tubular amino acid transport, 1156–1157 extracellular and intracellular fluid compartments, maturation of, 1168–1169 363–365 Tubular atrophy, 996, 998, 1000 Toxicity, 740 Tubular cells, 1656 Trabecular, 2228 Tubular, dysgenesis. See Renal tubular dysplasia (RTD) Trabecular BMD, 2231 Tubular fluid, 2241 Tracking, of blood pressure, 1923 Tubular potassium transport developmental aspects, 83–84 Tranexamic acid, 1535 Tubular proteinuria, 622 Trans-differentiation, 849 Tubular reabsorption of phosphate (TRP), 627 Transferrin, 2224 Tubular resistance to aldosterone, 1293 Transferrin receptor (CD71), 990 Tubular secretory pathways, 1657 Transforming growth factor, 914–915 Tubulin-specific chaperone cofactor E (TBCE), 306 Transforming growth factor-β (TGF-β), 994 Tubulointerstitial disease, 1526–1528 Transforming growth factor-beta-1 (TGFb1), 1496 Tubulointerstitial injury, 2179 Transient neonatal hyperphenylalaninemia, 1235 Tubulointerstitial nephritis (TIN), 1595 Transient receptor potential cation channel, subfamily C, acute TIN, 1411–1415 member 6, 815 etiologic classification of, 1415 Transient receptor potential vanilloid 5 (TRPV5), 1839 prognosis, 1420 Transient/unsustained hypertension, 2000 chronic TIN, 1420–1423 Transition, 2249 etiologic classification of, 1422 Transplantation, 2625–2626 treatment and prognosis, 1421 Transplantation immunobiology classification of, 1408 allograft rejection, 2474–2480 clinical presentation of, 1412 allorecognition (see Allorecognition) definition, 1407 anergy, 2482 renal tubular dysfunction in, 1413 apoptosis and immune regulation, 2481 serum and urine abnormalities, 1414 clinical tolerance and chimerism, 2482–2483 Tumor necrosis factor (TNF), 911, 994, 996 clonal deletion, 2481 Tunnelled catheters, 2447–2448 heterologous immunity and transplant rejection, Turner syndrome, 646, 2012 2473–2474 Turnover, bone, 2231 immunoregulation/regulatory T cells, 2472–2473 Twin studies, 1838 immunosuppressive agents, 2483–2484 Type A or type O classic renal glycosuria, 1188 T cell activation, co-stimulation/co-inhibition of Type A, SLC7A9, 1174 (see T cell activation) Type B, SLC7A9, 1174 Transplant models, 463–464 Type 2 diabetes mellitus, 735 Transplant rejection, 721 Type I and type II RTA, 1275 Transplant renal artery stenosis (TRAS), 1974, Type Ic hearing loss, 1288 2582–2583 Type I collagen, 283 Transport amino acid, 69–70 Type II hypersensitivity, 905 Transport processes, proximal tubule, 1355–1357 Type III BS, 1211 Transport system Bo, 1179 Type III collagen fibrils, 816 Transtubular potassium gradient (TTKG), 398 Type III collagen nephropathies, 789–791 Treatment, 967–969, 1175 Type III hypersensitivity, 905 membranous nephropathy, 1472–1473 Type III Renal Tubular Acidosis, 1290 and prognosis, chronic TIN, 1421 Type III RTA, 1275 relapse, 1472 Type IV BS, 1210 of shunt nephritis, 974–975 Type IV collagen, 708, 714, 733, 736, 741, 778 Treg, 899 Type IV Renal Tubular Acidosis, 1291–1293 Treponema antigens, 975 Type IV RTA, 1275 Trichrome stain, 714, 721 Type 1 pseudohyperaldosteronism, 265 Tricyclic antidepressants, 1804–1805 Type 2 pseudohyperaldosteronism, 265 Triglycerides, 2240 Tyrosinemia, 1285, 1361 Tris-hydroxymethyl aminomethane (THAM), 261 Tyrosinemia I, 1371 Trisomy-21, 646 TRPC6, 810, 815 TRPM6, 1233 U TTC21B, 814 Ultrafiltration, 2385–2386, 2435 t-test, 489 Ultrasonography, 2004 2728 Index

Ultrasound Urinary supersaturations, 1836 absence of side effects, 668 Urinary tract infection (UTIs), 600, 1696, application, 670 2574–2575, 2615 hydronephrosis, 667 Urine ammonium, 1275 technology, 97 Urine anion gap (UAG), 411 Unilateral hydronephrosis, 108–109 Urine concentration, 1656 Unilateral hydroureteronephrosis, 99 Urine examination, 2148 Unilateral renal agenesis (URA), 118–120 Urine osmolal gap (UOG), 411 Unilateral renal hypoplasia/dysplasia (URHD), 121 Urine osmolality, 632 Unilateral ureteral obstruction, 1751 Urine output, 2146 Uninhibited bladder contraction, 1789–1790 Urine potassium quantification, 405–406 United Arab Emirates, 2656 Urodynamic examinations, 1791–1793 Unmet metabolic needs, 2190 Urodynamics, 1786 untoward, 2394 Urofacial syndrome, 1810 Upper respiratory tract, infections in, 989 Uroflowmetry, 1791 Upregulation of type I interferon, 1466 Urogenital sinus malformation, 1796, 1797 Upshaw-Schulman syndrome, 1503 Urolithiases, 1392 Urea kinetic modelling, 2450 Urolithiasis, 691, 1172, 1593 Urea reduction ration, 2156 Urolithiasis, pediatric Urea-splitting, 1831 ample fluid intake, 1855 Uremic toxins, 649 clinical features, 1851 Ureteral ectopia, 1796 dianostic evaluation, 1851–1853 Ureter malformations, 1795 dietary, 1856–1857 Ureteroceles, 680, 1796 epidemiology, 1822–1824 Ureteropelvic junction obstruction (UPJO), 102–103, 680 genealogy studies, 1838 characterization, 1763 genome-wide association study, 1839 Clinical Presentation, 1765 incidence, 1822–1823 genetics, 1765 lifetime prevalence, 1822 long-term outcome, 1767 linkage and candidate gene studies, management, 1765–1767 1838–1839 markers, 1767 obesity, 1822 Ureterovesical junction obstruction (UVJO) oxalobacter formigenes, 1823 characterization, 1767 pathogenesis, 1835–1837 clinical presentation, 1768 pharmacotherapy, 1857–1858 diagnosis and management, 1768–1769 risk factors, 1824–1835 pathogenesis, 1767–1768 sodium and fructose intake, 1823 Ureterovesical obstruction (UVJ), 104 twin studies, 1838 Urethra, 578–579 Uropathogenic bacteria, 1697 Urethral abnormalities, 684 Urticaria, 1012 Urethral dilatation, 1802 Uruguay, 2633 Urethral instability, 1789–1790 Use and Acceptance of Oral Rehydration Solutions, Urethral pain, 1789 382–383 Urgency, 1789 USRDS, 2227, 2244 Uric acid, 1004, 1922–1923, 1967, 2004, 2175 urolithiasis, 1834 Uricosuria, 1360 V Uridine diphosphate glucuronosyltransferases Vaccination, 1475 (UDPGTs), 1663 Vaccines, 2250 Urinalysis, 624–625 Vacuolar myopathy, 1332, 1346 Urinary albumin excretion, 1557 Validity, 476 Urinary albumin excretion rate (AER), 1547 Valproic acid, 1286, 1377 Urinary calculi, 1175 Vancomycin, 1655, 1667–1168, 2274, 2279 Urinary concentrating ability derangements, water Vaptans, 210 homeostasis, 370 Variable vessel vasculitis, 1431 Urinary concentration and dilution, 81 Variance, 488 Urinary control development, 1784, 1786 Varicella-zoster virus (VZV), 2577–2578 Urinary macromolecular inhibitors, 1837 Variegated electron-dense subepithelial deposits, 734 Urinary retention, 1789 Vascular access, 2156, 2158 Index 2729

Vascular endothelial growth factor (VEGF), 819, von Gierke disease, 1361 915, 1551 von Hippel-Lindau disease, 2015 Vascular permeability factor/endothelial growth factor Vulnerable populations, 496 (VPF/VEGF), 994 Vasculitis, 901 syndromes, 1431 W Vasculogenesis and angiogenesis, 38 WAGR syndrome, 818, 1879 kidney vasculature, 38–41 Water Vaso-occlusion, 1524 abnormality evaluation, 202–203 Vasopressin, 2058 balance disturbances, 207–212 effect, in hospitalized children, 368 in body fluid homeostasis, 186–189 VATER association, 167–168 efferent mechanisms, 196 V-ATPase, 251 efffector mechanisms, 197–201 Vβ2(+) T cells, 970 laboratory assessment, 202 VEGF. See Vascular endothelial growth factor (VEGF) requirement, 185–186 VEGF-A, 890 sensor mechanisms, 189–191 Venezuela, 2634 Water and electrolyte requirements Ventriculoatrial or centriculoperitoneal shunts, 974 in infants and children with chronic kidney Vesicoamniotic shunt (VAS), 108 disease, 371 Vesicostomy, 1802 maintenance electrolyte therapy, 371–372 Vesicoureteral reflux, 684–685, 2013 maintenance therapy, 366–368 Vesicoureteral reflux (VUR), 105, 1716 nephrogenic diabetes insipidus, 370–371 after UTI, 1718 serum osmolality, 368 anatomy, 1716–1717 urinary concentrating ability derangements, water clinical presentation, 1718–1719 homeostasis, 370 embryology, 1716–1717 water homeostasis in acutely ill children, perioperative grading, 1717–1718 period, 368–369 incidence, 1716 Water deprivation test, 631–632 International grading system, 1717 Water diuresis, 370 management, 1716 WDR73, 823 management in children, AUA guidelines, 1737 Weak stream, 1789 natural history, 1717 Wegener Granulomatosis(WG), 1439–1442 prenatal hydronephrosis, 1718–1719 Weight loss, 2037 prevalence, 1716 Wetting, 598–599 published guidelines, 1736–1737 Whitaker test, 1763 sibling reflux, 1719 White coat HTN (WCH)/White coat hypertension (WCH), Vietnam, 2650 1913, 2061 Viral gastroenteritis, 377 WHO. See World Health Organization (WHO) Viral infections, 844 Whole genome sequencing (WGS), 428 Visual-motor, 2248 Wilcoxan signed rank test, 490 Visual processing, 1337 William's syndrome (WS), 298–300, 2000 Vitamin B12, 2224 Wilms' tumor, 556–557, 818, 1870 Vitamin D, 2220 gene, 1872 deficiency in patients with CKD, 2235 genetics, 1871–1872 therapy, 2364, 2366 histology, 1881 25(OH)vitamin D, 2352 nephron progenitor cells, 1877–1879 Vitamin D-dependent rickets (VDDR), 315–317 suppressor genes, 1876 Vitamin D receptor (VDR), 293 treatment, 1880–1881 Vitronectin, 936 Wilms' tumor suppressor gene (WT1), 761–762 Voiding cystourethrography (VCUG), 1728 Wilson disease, 1285, 1361, 1372, 1835 Voiding cysturethrogram (VCUG), 1760 With-no-lysine-kinases, 229 Voiding dysfunction and constipation, 1733–1734 with no lysine kinases (WNKs), 1292 Voiding frequency, 1786 Wnt, 280 Voiding postponement, 1789 World anti-doping agency, 2071 Voiding urosonography (VUS), 670 World Health Organization (WHO), 379, 732 Volume Depletion Assessment, 376–377 classification, 1458 Volumetric BMD, 2233 Worse kidney function, diagnosis, 2174 Vomiting, 1337 WT1, 817–818 2730 Index

WT1(+KTS) and WT1(–KTS) variants, 818 Y WT1 gene, 1872 Yemen, 2656 WTX, 1876 y+LAT-1 (SLC7A7), 1178 W138X founder mutations, 1333

Z X Zinc finger family, 817 Xanthinuria, 1594 Zinc finger transcription factor, 818 Xanthogranulomatous pyelonephritis, 1831 Zona occludens, 725 Xenobiotics, 1655 Zymogen precursor (zSPEB), 962 X-linked hypophosphatemia (XLH), 314 X-linked recessive hypoparathyroidism, 302 Xylene, 714