The topics of neonatal encephalopathy and cerebral palsy, as well as hypoxic-ischemic encephalopathy, are of paramount importance to anyone who ventures to deliver infants. Criteria sufficient to define an acute intrapartum hypoxic event as sufficient to cause cerebral palsy have been advanced previously by both The American College of Obstetricians and Gynecologists (ACOG) and the International Cerebral Palsy Task Force. ACOG convened a task force that over the past 3 years reviewed these criteria based upon advances in scientific knowledge. In this review, we cover the slow but steady progression toward defining the pathogenesis and pathophysiology of neonatal encephalopathy and cerebral palsy. Four essential criteria are also advanced as prerequisites if one is to propose that an intrapartum hypoxic-ischemic insult has caused a moderate to severe neonatal encephalopathy that subsequently results in cerebral palsy. Importantly, all four criteria must be met: 1) evidence of metabolic acidosis in fetal umbilical cord arterial blood obtained at delivery (pH less than 7 and base deficit of 12 mmol/L or more), 2) early onset of severe or moderate neonatal encephalopathy in infants born at 34 or more weeks' gestation, 3) cerebral palsy of the spastic quadriplegic or dyskinetic type, and 4) exclusion of other identifiable etiologies, such as trauma, coagulation disorders, infectious conditions, or genetic disorders. Other criteria that together suggest intrapartum timing are also discussed.

Purpose: To assess the occurrence of two mutations associated with susceptibility to aminoglycoside ototoxicity.Methods: Genetic analysis of anonymized, residual diagnostic specimens. Results: One occurrence of the A1555G mutation and seven occurrences of the 961delT ϩ C(n) nucleotide change were found. Two previously unreported sequence changes, T961G and 956 -960insC, were also found in six and five specimens, respectively. Conclusions: Genetic susceptibility to aminoglycoside ototoxicity may be more common than previously suspected.Further study of the 961delT ϩ C(n) mutation is recommended to confirm its role in aminoglycoside ototoxicity and assess penetrance and variability with and without exposure to aminoglycoside antibiotics. Genet Med 2002:4(5):336 -345.

SUMMARY A transient form of hypertrophic cardiomyopathy has been previously described in infants of diabetic mothers (IDMs). The purpose of this study was to determine the incidence, natural history and pathologic features of this cardiomyopathy in symptomatic and asymptomatic IDMs.We studied 47 IDMs for evidence of cardiomyopathy. Among 24 symptomatic IDMs, five had marked septal hypertrophy with echocardiographic features suggesting left ventricular outflow obstruction and five had hypertrophy of the right ventricular free wall. With the exception of mild septal hypertrophy, these abnormalities resolved during the first 6 months of life, and echocardiograms in the first-degree family members were normal. Of 23 asymptomatic IDMs, three had septal hypertrophy and two had right ventricular free wall hypertrophy; none of the asymptomatic IDMs had evidence of outflow obstruction.One symptomatic IDM died, and autopsy revealed a hypertrophic septum that distorted both ventricular cavities. Microscopic examination revealed hypertrophic fibers and occasional areas of cellular disarray in the septum.Despite the clinical and pathologic similarities of the cardiomyopathy in IDMs to the hypertrophic cardiomyopathies in older children and adults, its transient and nonfamilial nature suggest that it is a separate disease. We speculate that it is a manifestation of the generalized organomegaly in IDMs.

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The Palivizumab Outcomes Registry collected data on 19,548 high-risk infants who received > or =1 dose of palivizumab and followed prospectively from 2000 through 2004. Ninety-one children with cystic fibrosis (CF) were identified who received palivizumab off label. None of the infants with CF who received prophylaxis was hospitalized as a result of respiratory syncytial virus lower respiratory tract infection. Evaluations of palivizumab use in infants with CF could be warranted.