DOCSLIB.ORG

Absent End-Diastolic Velocity in the Umbilical Artery and Its Clinical Significance

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Absent End-Diastolic Velocity in the Umbilical Artery and Its Clinical Significance Chapter 25 Absent End-Diastolic Velocity in the Umbilical Artery and Its Clinical Significance Dev Maulik, Reinaldo Figueroa Among the characteristics of the umbilical arterial prognosis. With the further increase of impedance, Doppler waveform, the end-diastolic velocity is of the end-diastolic velocity eventually becomes absent. primary hemodynamic and clinical significance. As Such a development, though rare, is ominous and re- discussed in Chap. 10, the end-diastolic velocity dem- sults in a profoundly adverse perinatal outcome. An onstrates an impressive continuous increase through- example of absent end-diastolic velocity (AEDV) is out the gestation which is attributable to an ever-in- shown in Fig. 25.1. Occasionally, further hemody- creasing decline of the fetoplacental flow impedance. namic deterioration occurs, resulting in reversal of It results in a concomitant decrease in the pulsatility the end-diastolic velocity (Fig. 25.2). The impressive of the waveform and is reflected in the Doppler in- amount of information [1±26] now available on the dices such as the systolic/diastolic (S/D) ratio and the clinical significance of the absent and reversed end- resistance index (RI), both of which progressively de- diastolic velocity in the umbilical artery is appraised cline with the advancing gestation. These changes are in this chapter. prognostically reassuring. In contrast, any decline in the end-diastolic velocity with the consequently rising Doppler indices indicates rising impedance in the fe- toplacental vascular bed and signifies a worsening Fig. 25.2. Progressive disappearance of the end-diastolic frequency shift in the umbilical arterial Doppler waveforms from a pregnancy complicated with severe fetal growth re- striction at 33 weeks' gestation. Top left: Presence of the end-diastolic frequency shift, although the Doppler indices Fig. 25.1. Example of absent end-diastolic velocity in the were high for the gestational age (systolic/diastolic ratio 5; umbilical artery. Top: Color Doppler-directed pulsed Dop- resistance index 0.8). Top right: Absence of the end-diastol- pler interrogation of the umbilical vessels. Bottom: umbili- ic frequency shift. Bottom left: Spontaneous deceleration cal arterial Doppler waveforms. Note that there is no no- with prolongation of the diastolic phase and the appear- ticeable loss of low frequency shift information, as the ance of umbilical venous pulsation. Bottom right: Progres- high-pass filter was set at 50 Hz sion to the reversal of the end-diastolic frequency shift 376 D. Maulik, R. Figueroa Table 25.1. Incidence of absent and reversed end-diastolic velocity in high- and low-risk populations Study, No. of Risk category Doppler type High-pass filter AEDV first author patients (Hz) No. % Johnstone [7] 380 High PW, CW 150 24 6.30 Beattie [27] 2,097 Low CW 200 6 0.29 Huneke [14] 226 High CW 200 18 8.00 Malcolm [15] 1,000 High PW 100 25 2.50 Wenstrom [17] 450 High PW 100 22 4.90 Weiss [19] 2,400 Unselected PW 50 51 2.10 Battaglia [23] a 46 Very high PW 100 26 56.20 Pattinson [22] 342 Very high PW, CW 150 120 34.50 200 Rizzo [25] 6,134 High PW 100 192 3.10 Karsdorp [24] a 459 Very high ?PW Lowest 245 53.40 CW, continuous wave; PW, pulsed wave; AEDV, absent end-diastolic velocity. a Fetuses with congenital anomalies and dyskaryosis were clearly excluded. The other studies either included these fe- tuses or are unclear about it. Incidence in this manner, 245 developed AREDV, for an inci- dence of 53.4%. In comparison, Beattie and Dornan [27] found that only 6 of 2,097 singleton pregnancies The frequency with which absent or reversed end- developed AREDV for an incidence of 0.29%. The ac- diastolic velocity (AREDV) is encountered in the um- tual rate might even be lower if we consider that the bilical artery varies according to the risk category of high-pass filter setting was 200 Hz, which is relatively the obstetric population, the time of gestation at high for umbilical arterial Doppler insonation. which the observation is made, and the Doppler ex- amination technique. For high-risk pregnancies the incidence varies from 2.1% to 56.0% (Table 25.1). Technical Considerations Such a wide range may be explained by the differing definitions of high-risk pregnancy used by the inves- As alluded to above and discussed elsewhere in this tigators and by the level of the high-pass filter used. book, the procedure used for Doppler measurement For example, the basis for high-risk categorization of may affect the measured magnitude of the end-dia- a pregnancy may range from clearly defined clinical stolic frequency shift. It is apparent from the basic criteria, such as hypertension, to ill-defined group- principles of the Doppler shift that shifted frequen- ings of various clinical conditions. In contrast to that cies can only be underestimated, not overestimated. in the high-risk population, the incidence of AREDV There are two technical sources of this problem: (1) may be as low as 0.29% in an obstetric population the threshold setting of the high-pass filter; and (2) with a low prevalence of pregnancy complications. the angle of insonation between the Doppler beam The following two examples illustrate this point. In and the flow axis. The high-pass filter (see Chap. 3) probably the largest reported series on AREDV, Kars- eliminates from the Doppler signal the low-fre- dorp et al. [24] used well-defined criteria for select- quency/high-amplitude frequency component and is ing the population for a multicenter study. Only pa- used to remove signals generated by movement of the tients with hypertension or fetal growth restriction vascular wall or other adjacent tissues. This filter, (or both) were included. Hypertension was defined as however, also removes low-frequency components a diastolic pressure of 110 mmHg by a single mea- generated from the slow-moving blood flow as en- surement or 90 mmHg by two or more measure- countered during the end-diastolic phase of the cardi- ments. Also included were patients with hypertension ac cycle. Thus end-diastolic frequencies are removed plus proteinuria; the latter was defined as urinary from the umbilical Doppler waveform. A relatively protein loss of more than 300 mg in 24 h. Intrauterine high setting of the filter therefore leads to a false di- growth restriction (IUGR) was defined as the abdom- agnosis of AEDV. It is strongly recommended that inal circumference measuring less than the 5th per- the high-pass filter should be at the lowest possible centile for gestational age based on local population- setting, which may not exceed 100 Hz. The second specific nomograms. The lowest possible high-pass consideration is related to the angle of insonation, filter threshold was used. Of the 459 patients selected which is inversely related to the magnitude of the es- a Chapter 25 Absent End-Diastolic Velocity in the Umbilical Artery and Its Clinical Significance 377 timated Doppler shift because of the cosine function Table 25.2. Absent and reverse end-diastolic velocity in of the angle in the Doppler equation (see Chap. 2). A the umbilical artery and adverse perinatal outcome larger angle therefore leads to a lower frequency mea- Perinatal outcome Mean Range surement, which leads to disappearance of the end- diastolic frequency even in the presence of end-dia- Death (%) 45 17±100 stolic flow. Gestational age (weeks) 31.6 29±33 Birth weight (g) 1,056 910±1,481 Absent End-Diastolic Velocity Small-for-gestational age (%) 68 53±100 Cesarean section for fetal 73 24±100 and Adverse Perinatal Outcome distress (%) Apgar score <7 at 5 min (%) 26 7±69 There is an ominous association between the AREDV Admission to neonatal 84 77±97 in the umbilical artery and adverse perinatal outcome intensive care unit (%) (Table 25.2). The latter includes not only morbid Congenital anomalies (%) 10 0±24 states, such as fetal growth restriction, developmental anomalies, and abnormal chromosomes, but also a Aneuploidy (%) 6.4 0±18 substantial increase in perinatal deaths. In addition, there is a significant association with pregnancy com- plications, such as hypertensive disease of pregnancy and oligohydramnios. Perinatal Mortality One of the most remarkable features of umbilical ar- terial absent and reverse flow is the catastrophic in- crease of deaths of fetuses in utero and of neonates. From a total of 1,126 cases of AREDV reviewed in this chapter, 193 were stillborn and 312 died during the neonatal period. These data translate into a 170/ 1,000 stillbirth rate and 280/1,000 neonatal mortality rate, respectively ± hence a 450/1,000 perinatal mor- tality rate. Most deaths are attributable to obstetric complications, such as growth restriction and hyper- tension with the underlying pathology of chronic in utero respiratory and nutritional deprivation, but they Fig. 25.3. Umbilical arterial Doppler velocimetry showing an agonal pattern. Note that reverse end-diastolic velocity also may be attributed to the higher frequency of was present for most of the cardiac cycle (60%±70%). This anomalies and aneuploidy encountered in these in- pattern signifies that fetoplacental perfusion, which is nor- fants, as well as prematurity. It is difficult to correct mally present for the duration of the cardiac cycle, was the mortality figures for congenital malformations seen in this case for only a fraction of that time and chromosomal abnormalities, as most studies do not explicitly report this information. However, it ap- pears that the corrected perinatal mortality is ap- Perinatal Morbidity proximately 340/1,000 births. When the end-diastolic Not only is there a high rate of perinatal loss, the sur- flow in the umbilical artery is reversed, the outcome viving fetuses and infants demonstrate signs of pro- is abysmal.
Load more

Recommended publications
  • Management and Investigation of Neonatal Encephalopathy: 2017 Update Kathryn Martinello,1 Anthony R Hart,2 Sufin Yap,3 Subhabrata Mitra,1 Nicola J Robertson1
    Review Arch Dis Child Fetal Neonatal Ed: first published as 10.1136/archdischild-2015-309639 on 6 April 2017. Downloaded from Management and investigation of neonatal encephalopathy: 2017 update Kathryn Martinello,1 Anthony R Hart,2 Sufin Yap,3 Subhabrata Mitra,1 Nicola J Robertson1 1Department of Neonatology, ABSTRACT definite aetiological diagnosis is known, and Institute for Women’s Health, This review discusses an approach to determining the hypoxic-ischaemic encephalopathy (HIE) where University College London, UK 2 cause of neonatal encephalopathy, as well as current clear diagnosis of hypoxia-ischaemia is known to Department of Neonatal and ’ Paediatric Neurology, Sheffield evidence on resuscitation and subsequent management have led to the neonate s clinical state. Children’s Hospital NHS of hypoxic-ischaemic encephalopathy (HIE). Foundation Trust, Sheffield, UK Encephalopathy in neonates can be due to varied 3 DETERMINING THE AETIOLOGY OF NE Department of Inherited aetiologies in addition to hypoxic-ischaemia. A Metabolic Diseases, Sheffield The initial stages of managing NE will be the same Children’s Hospital NHS combination of careful history, examination and the for most babies, with good resuscitation and sup- Foundation Trust, Sheffield, UK judicious use of investigations can help determine the portive management. However, as the picture cause. Over the last 7 years, infants with moderate to evolves and investigations return, clinicians should fi Correspondence to severe HIE have bene ted from the introduction of consider the aetiology of NE as this could lead to Professor Nicola J Robertson, routine therapeutic hypothermia; the number needed to specific treatments, aid with prognosis and recur- Institute for Women’s Health, treat for an additional beneficial outcome is 7 (95% CI University College London, 74 rence risk counselling, and assist with the evalu- Huntley Street, London WC1E 5 to 10).
    [Show full text]
  • Various Outcomes of Idiopathic Grade IV Intraventricular Haemorrhage in Term Newborns at Two Years of Age. Das S1, Bhattacharya M2, Chatterjee K3, Sarkar N4, Aich B5
    Bangladesh Journal of Medical Science Vol. 17 No. 02 April’18 Case report: Various outcomes of Idiopathic Grade IV Intraventricular Haemorrhage in term newborns at two years of age. Das S1, Bhattacharya M2, Chatterjee K3, Sarkar N4, Aich B5 Bangladesh Journal of Medical Science Vol. 17 No. 02 April’18. Page : 316-318 DOI: http://dx.doi.org/10.3329/bjms.v17i2.35893 Introduction: duration of NICU stay was 15 days. Intraventricular Haemorrhage (IVH) generally Case1 (corresponds to Figure1) - At 2 years of age, occurs in infants <32 weeks and/or <1500 grams. he was developing right sided spastic hemiparetic Incidence of IVH in term neonates is 3.5-5% 1,2 . cerebral palsy. Right sided limbs exhibited 50% of IVH in term neonates is primarily caused by hypertonia, brisk deep tendon reflexes, ankle clonus trauma and asphyxia; a minority of haemorrhages and persistence of cortical thumb. The child showed is caused by extension of bleed from Subdural, early hand preference, dwarfing and dyspraxia of Subarachnoid and Intraparenchymal haemorrhage affected limbs. Electroencephalogram (EEG), Visual or caused by vascular lesions, coagulopathies or Evoked Potential (VEP), Brainstem Auditory Evoked tumours. 25% of cases have no significant risk Response (BAER) and Fundoscopy were normal factors. Most of the germinal matrix has regressed at 2 years of age. Developmental assesment was by term, so most haemorrhages (35%) arise from the done by Developmental Assesment Scale For Indian posterior tufts at the glomus in choroid plexus, 24% Infants (DASII) which is based on Bayley Scale of from Thalamus, 17% from residual Germinal Matrix Infant Development (BSID) II norms.
    [Show full text]
  • Mid-Trimester Preterm Premature Rupture of Membranes (PPROM): Etiology, Diagnosis, Classification, International Recommendations of Treatment Options and Outcome
    J. Perinat. Med. 2018; 46(5): 465–488 Review article Open Access Michael Tchirikov*, Natalia Schlabritz-Loutsevitch, James Maher, Jörg Buchmann, Yuri Naberezhnev, Andreas S. Winarno and Gregor Seliger Mid-trimester preterm premature rupture of membranes (PPROM): etiology, diagnosis, classification, international recommendations of treatment options and outcome DOI 10.1515/jpm-2017-0027 neonates delivered without antecedent PPROM. The “high Received January 23, 2017. Accepted May 19, 2017. Previously pub- PPROM” syndrome is defined as a defect of the chorio- lished online July 15, 2017. amniotic membranes, which is not located over the inter- nal cervical os. It may be associated with either a normal Abstract: Mid-trimester preterm premature rupture of mem- or reduced amount of amniotic fluid. It may explain why branes (PPROM), defined as rupture of fetal membranes sensitive biochemical tests such as the Amniosure (PAMG-1) prior to 28 weeks of gestation, complicates approximately or IGFBP-1/alpha fetoprotein test can have a positive result 0.4%–0.7% of all pregnancies. This condition is associ- without other signs of overt ROM such as fluid leakage with ated with a very high neonatal mortality rate as well as an Valsalva. The membrane defect following fetoscopy also increased risk of long- and short-term severe neonatal mor- fulfils the criteria for “high PPROM” syndrome. In some bidity. The causes of the mid-trimester PPROM are multi- cases, the rupture of only one membrane – either the cho- factorial. Altered membrane morphology including marked rionic or amniotic membrane, resulting in “pre-PPROM” swelling and disruption of the collagen network which is could precede “classic PPROM” or “high PPROM”.
    [Show full text]
  • Hyperbilirubinemia and Kernicterus Jesus Peinado PGY2 Merle Ipson MD March 2009 Hyperbilirubinemia
    Hyperbilirubinemia and Kernicterus Jesus Peinado PGY2 Merle Ipson MD March 2009 Hyperbilirubinemia Most common clinical condition requiring evaluation and treatment in the NB Most common cause of readmission in the 1 st week Generally a benign transitional phenomenon May pose a direct threat of brain damage May evolve into kernicterus Kernicterus 1. Choreoathetoid cerebral palsy 2. High-frequency central neural hearing loss 3. Palsy of vertical gaze 4. Dental enamel hypoplasia (result of bilirubin-induced cell toxicity) Kernicterus Originally described in NB with Rh hemolytic disease Recently reported in healthy term and late preterm Reported in breast-fed infants w/out hemolysis Most prevalent risk factor is late preterm Late Preterm Infant Relatively immature in their capacity to handle unconjugated bilirubin Hyperbilirubinemia is more prevalent, pronounced and protracted Eightfold increased risk of developing TSB > 20 mg/dl (5.2%) compared to term (0.7%) Pathobiology Increased bilirubin load in the hepatocyte Decreased erythrocyte survival Increased erythrocyte volume Increased enterohepatic circulation Decreased hepatic uptake from plasma Defective bilirubin conjugation Bilirubin Metabolism Bilirubin Metabolism Bilirubin Metabolism How bilirubin Damages the Brain Determinants of neuronal injury by bilirubin 1. Concentration of unconjugated bilirubin 2. Free bilirubin 3. Concentration of serum albumin 4. Ability to bind UCB 5. Concentration of hydrogen ion 6. Neuronal susceptibility Intracellular Calcium Homeostasis
    [Show full text]
  • Subset of Alphabetical Index to Diseases and Nature of Injury for Use with Perinatal Conditions (P00-P96)
    Subset of alphabetical index to diseases and nature of injury for use with perinatal conditions (P00-P96) SUBSET OF ALPHABETICAL INDEX TO DISEASES AND NATURE OF INJURY FOR USE WITH PERINATAL CONDITIONS (P00-P96) Conditions arising in the perinatal period Conditions arising—continued - abnormal, abnormality—continued Note - Conditions arising in the perinatal - - fetus, fetal period, even though death or morbidity - - - causing disproportion occurs later, should, as far as possible, be - - - - affecting fetus or newborn P03.1 coded to chapter XVI, which takes - - forces of labor precedence over chapters containing codes - - - affecting fetus or newborn P03.6 for diseases by their anatomical site. - - labor NEC - - - affecting fetus or newborn P03.6 These exclude: - - membranes (fetal) Congenital malformations, deformations - - - affecting fetus or newborn P02.9 and chromosomal abnormalities - - - specified type NEC, affecting fetus or (Q00-Q99) newborn P02.8 Endocrine, nutritional and metabolic - - organs or tissues of maternal pelvis diseases (E00-E99) - - - in pregnancy or childbirth Injury, poisoning and certain other - - - - affecting fetus or newborn P03.8 consequences of external causes (S00-T99) - - - - causing obstructed labor Neoplasms (C00-D48) - - - - - affecting fetus or newborn P03.1 Tetanus neonatorum (A33) - - parturition - - - affecting fetus or newborn P03.9 - ablatio, ablation - - presentation (fetus) (see also Presentation, - - placentae (see also Abruptio placentae) fetal, abnormal) - - - affecting fetus or newborn
    [Show full text]
  • Motor Deficits Are Triggered by Reperfusion-Reoxygenation Injury As Diagnosed by MRI and by a Mechanism Involving Oxidants
    5500 • The Journal of Neuroscience, April 18, 2012 • 32(16):5500–5509 Neurobiology of Disease Motor Deficits Are Triggered by Reperfusion-Reoxygenation Injury as Diagnosed by MRI and by a Mechanism Involving Oxidants Alexander Drobyshevsky,1 Kehuan Luo,1 Matthew Derrick,1 Lei Yu,1 Hongyan Du,2 P. V. Prasad,3 Jeannette Vasquez-Vivar,4 Ines Batinic-Haberle,5 and Sidhartha Tan1 1Department of Pediatrics, 2Center on Outcomes, Research and Education, and 3Radiology, NorthShore University Healthcare Research Institute, Evanston, Illinois 60201, 4Department of Biophysics, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, and 5Department of Radiation Oncology, Duke University Medical Center, Durham, North Carolina 27710 The early antecedents of cerebral palsy (CP) are unknown but are suspected to be due to hypoxia-ischemia (H-I). In our rabbit model of CP, the MRI biomarker, apparent diffusion coefficient (ADC) on diffusion-weighted imaging, predicted which fetuses will develop postnatalhypertonia.SurvivingH-Ifetusesexperiencereperfusion-reoxygenationbutasubpopulationmanifestedacontinueddeclineof ADC during early reperfusion-reoxygenation, which possibly represented greater brain injury (RepReOx). We hypothesized that oxida- tive stress in reperfusion-reoxygenation is a critical trigger for postnatal hypertonia. We investigated whether RepReOx predicted postnatal neurobehavior, indicated oxidative stress, and whether targeting antioxidants at RepReOx ameliorated motor deficits, which included testing of a new superoxide dismutase mimic (MnTnHex-2-PyP). Rabbit dams, 79% gestation (E25), were subjected to 40 min uterine ischemia. Fetal brain ADC was followed during H-I, immediate reperfusion-reoxygenation, and 4–72 h after H-I. Endpoints were postnatal neurological outcome at E32, ADC at end of H-I, ADC nadir during H-I and reperfusion-reoxygenation, and area under ADC curve during the first 20 min of reperfusion-reoxygenation.
    [Show full text]
  • 6/11/2019 1 “Lab Called… Your CBC Clotted”
    6/11/2019 Neonatal Lab Interpretation Tanya Hatfield, MSN, RNC - NIC UCSF Benioff Children’s Outreach Services “Lab called… your CBC clotted” 2 Objectives ▪ Interpret lab values ▪ Discuss jaundice of the newborn ▪ Understand specific hematologic problems 3 1 6/11/2019 CBC - Hematopoiesis 4 Erythrocytes - RBCs ▪ Main protein is hemoglobin ( Hgb ) ▪ RBC function is to protect Hgb ▪ Hgb function is oxygen/CO2 transport Reticulocytes ▪ Inversely proportional to GA at birth ▪ Falls quickly to less than 2% by 7 days ▪ Elevated early Retic Ct may indicate bleeding, hemolysis or chronic blood loss 5 Kenner, C. (2014). Comprehensive neonatal nursing care (Fifth ed., pp. 334 - 338) Components of CBC ▪ Main protein is hemoglobin (Hgb) ▪ RBC function is to protect Hgb ▪ Hgb function is oxygen/CO2 transport Reticulocytes ▪ Inversely proportional to GA at birth ▪ Falls quickly to less than 2% by 7 days ▪ Elevated early Retic Ct may indicate bleeding, hemolysis or chronic blood loss 6 Kenner, C. (2014). Comprehensive neonatal nursing care (Fifth ed., pp. 334 - 338) 2 6/11/2019 RBC Indices ▪ Mean corpuscular volume (MCV): size and volume ▪ Mean corpuscular hemoglobin (MCH): average amount (weight) of hemoglobin ▪ Mean corpuscular hemoglobin concentration (MCHC): average concentration ▪ Nucleated RBC: circulating pre - reticulocyte 7 Kenner, C. (2014). Comprehensive neonatal nursing care (Fifth ed., pp. 334 - 338) Anemia ▪ Differential • ↓ Erythrocyte production ‒ infection, nutritional deficiencies, leukemia, bone marrow failure, anemia of prematurity
    [Show full text]
  • Clinical and CSF Studies in Newborn Infants with Neurological Abnormalities
    Arch Dis Child: first published as 10.1136/adc.49.5.351 on 1 May 1974. Downloaded from Archives of Disease in Childhood, 1974, 49, 351. Clinical and CSF studies in newborn infants with neurological abnormalities S. W. DE SOUZA and R. D. G. MILNER From the Department of Child Health, University of Manchester and St. Mary's Hospital, Manchester De Souza, S. W., and Milner, R. D. G. (1974). Archives ofDisease in Childhood, 49, 351. Clinical and CSF studies in newborn infants with neurological abnormalities. Thirty-four infants with abnormal neurological signs of no specific aetiology and 49 infants without abnormal neurological signs were selected in the newborn period. After a detailed neurological examination, the hyperexcitability syndrome was diagnosed in 18 and the apathy syndrome in 16 infants. The apathy syndrome was associated with apnoeic attacks, absent sucking, swallowing, and Moro responses as well as pneumonia. The hyperexcitability syndrome was associated with a history of fetal distress, a very poor condition at birth, and also absent sucking and swallowing responses. 3 infants with the apathy syndrome died. The majority of abnormal infants had either blood-stained or xanthochromic CSF. Cisternal puncture was helpful in diagnosing intracranial haemorrhage and was associated with subsequent clinical improvement in 6 infants. Though there was an association between apathy and hyperexcitability syndromes in the newborn period and subsequent neurological abnormalities during the first year, these abnormalities were present only in a minority. This study shows that the clinical diagnosis of the apathy or hyperexcitability syndrome in the newborn period has diagnostic and prognostic significance.
    [Show full text]
  • Unexpected Neurological Symptoms of Ruxolitinib: a Case Report
    Case Report J Hematol. 2020;9(4):137-139 Unexpected Neurological Symptoms of Ruxolitinib: A Case Report Francesca Furiaa, d, Maria P. Canevinia, b, Augusto B. Federicib, c, Maria C. Carraroc Abstract ease or can evolve from PV or essential thrombocythemia (ET), is characterized by progressive anemia, marrow fibrosis, Ruxolitinib is a highly potent JAK2 inhibitor approved for the treat- and extramedullary hematopoiesis which becomes prominent ment of myelofibrosis (idiopathic or post-polycythemia vera or post- especially in the spleen, with different degrees of splenomeg- essential thrombocythemia) and, more recently, for polycythemia aly. Constitutional symptoms (night sweats and weight loss), vera with an inadequate response to or intolerant of hydroxyurea. pruritus, fatigue, and sequelae of splenomegaly are common The most common adverse events of ruxolitinib include immunosup- [4]. Apart from allogeneic stem-cell transplantation, which is pression with an increased risk of reactivation of silent infections and the only curative treatment, only few therapies are available increased non-melanoma skin cancer. The known neurological side for the treatment of MF [5]. effects of ruxolitinib are dizziness and headache, but no neurologi- The main goal of therapy in PV is to prevent thrombotic cal paroxysmal episodes have been recorded. This report deals with events while avoiding iatrogenic harm and minimizing the risk an 80-year-old outpatient woman with polycythemia vera turned into of transformation to post-PV MF or acute myeloid leukemia myelofibrosis who experienced neurological episodes of hypoesthe- (AML) [6]. sia and weakness of right arm and leg during ruxolitinib treatment. Most patients receive low-dose aspirin and undergo phle- botomy [7], with goal of maintaining hematocrit values of less Keywords: Ruxolitinib; Polycythemia vera; Myelofibrosis than 45%.
    [Show full text]
  • Transient Tone Abnormalities in “High Risk” Infants and Cognitive Outcome at Five Years
    R E S E A R C H P A P E R Transient Tone Abnormalities in “High Risk” Infants and Cognitive Outcome at Five Years SUDHA CHAUDHARI, MANGALMURTI BHALERAO, ANJALI CHITALE, BHARATI PATIL, ANAND PANDIT AND MAHENDRA HOGE From the Division of Neonatology, Department of Pediatrics, KEM Hospital, Pune 411 011, Maharashtra, India. Correspondence to: Dr Sudha Chaudhari, Consultant, Division of Neonatology, Department of Pediatrics, KEM Hospital, Pune 411 011, India. [email protected] Received: March 20, 2009; Initial review: April 24, 2009; Accepted: August 24, 2009. Objective: To identify transient tone abnormalities and Results: Out of 190 high risk infants, 113 were normal HR determine its prevalence in “high risk” infants and their and 67 (35.2%) were labeled as TTA. Ten infants with cognitive outcome at 5 years. cerebral palsy had abnormal tone throughout the first year. Design: Prospective cohort observational study. Controls had normal tone throughout the follow-up period. Although there was no difference in the IQ of the TTA group Setting: High risk infants discharged from a level II (98.5 + 12.4) and the normal HR (99.1+13.1) group, it was neonatal unit in a 12 month period, and followed upto 5 significantly less (P=0.04) than that of controls (106.1 + years. 9.1). Preschool inventory in TTA children showed poor Methods: High risk infants and normal controls were language development (P=0.014). assessed for abnormalities of tone using the method Conclusion: Many of the tone abnormalities detected at 6 described by Amiel-Tison at 3, 6, 9, 12 months.
    [Show full text]
  • Depression at Birth in Term Infants Exposed to Maternal Chorioamnionitis: Does Neonatal Fever Play a Role?
    Original Article Depression at Birth in Term Infants Exposed to Maternal Chorioamnionitis: Does Neonatal Fever Play a Role? Lina F. Shalak, MD have birth depression, than infants with lesser degree of temperature Jeffrey M. Perlman, MB elevation after birth. Within the NICU group, the extent of temperature Gregory L. Jackson, MD elevation was not associated with worse neurological outcomes. Abbot R. Laptook, MD Journal of Perinatology (2005) 25, 447–452. doi:10.1038/sj.jp.7211326 Published online 28 April 2005 OBJECTIVES: (1) To determine the incidence and the time course of elevated temperature following delivery in term infants with clinical chorioamnionitis (CHORIO) and (2) to determine if the extent of INTRODUCTION temperature elevation at birth is associated with increased likelihood of The presence of maternal fever has been associated with an NICU Admissions, birth depression, or with short-term neurological increased need of resuscitation at birth, lower Apgar scores, a abnormalities. higher incidence of seizures in the newborn and increased 1–3 DESIGN/METHODS: mortality. Moreover an association between clinical chorioamnionitis (CHORIO) and cerebral palsy has been described The infants were divided into two groups based on the median admission both in term and preterm infants.4–6 However, the mechanisms rectal temperature of 37.81C for the cohort. Depression at birth was that link these two conditions remain ill defined, especially for defined as either the need of positive pressure ventilation for >2 minutes, infants born at term. Since hyperthermia during and following intubation, or Apgar score <6 at 5 minutes. Neurological examination hypoxic insults in experimental animals enhances neuronal death, and assessment of encephalopathy (Sarnat staging) was performed at maternal fever associated with clinical CHORIO has been proposed birth and daily thereafter, by one investigator blinded to temperature as a potential mechanism of injury.7 However, the role of an findings.
    [Show full text]
  • Incidence of Obstructive Sleep Apnea Is Higher Among Children Who
    VOLUME 25, NUMBER 1 2016 Incidence of Obstructive Sleep Apnea KEY INSIGHTS Is Higher Among Children Who Have ■ Cerebral palsy is among the most common disabilities that can lead Cerebral Palsy and Epilepsy to development of obstructive sleep apnea (OSA). John Garcia, MD, Beverly Wical, MD, and Jennifer Maytum, DNP ■ The likelihood of OSA increases with more severe forms of cerebral palsy, or when cerebral palsy is combined Obstructive sleep apnea (OSA) is a sleep disorder that occurs when the throat with an epilepsy diagnosis. muscles relax during sleep, blocking airflow in the nose and mouth. Although largely associated with adults, the disorder also affects children, particularly ■ Other groups at heightened risk for developing OSA include children those who have complex medical conditions causing hypertonia or hypotonia who have neuromuscular conditions (abnormal muscle tone). or craniofacial anomalies. ■ The Pediatric Sleep Questionnaire A number of childhood-onset disabilities can affect muscle tone, leading to (PSQ) is an effective tool for providers development of OSA. Cerebral palsy is among the most common, particularly to assess patients for sleep problems. when the condition is severe or combined with an epilepsy diagnosis. ■ Treatment for OSA may be medical Routinely assessing cerebral palsy patients for sleep disorders can facilitate or surgical depending on the child’s accurate diagnosis and successful treatment of OSA in this population. age, condition and other factors. Heightened Risk Factors Children who have cerebral palsy typically have hypertonia (high or tight muscle tone) in their extremities and hypotonia (low or loose muscle tone) in their midline. This tone combination is the opposite of typically developing children, who have relatively loose extremities and a rigid midline.
    [Show full text]