We present four patients with typical neonatal onset non-ketotic hyperglycinemia (NKH) who developed hydrocephalus requiring shunting in early infancy. Brain imaging revealed acute hydrocephalus, a megacisterna magna or posterior fossa cyst, pronounced atrophy of the white matter, and an extremely thin corpus callosum in all. The three older patients had profound developmental disabilities. This suggests that the development of hydrocephalus in NKH is an additional poor prognostic sign.
Gastric lactobezoars (GLBs) are the most common form of bezoars in neonates and consist of aggregations of undigested milk constituents. GLB can present with a variety of intra-abdominal clinical symptoms, and occasionally, extra-abdominal symptoms. Conservative management, with a period of bowel rest and intravenous fluids, is the most common treatment regimen for uncomplicated GLB. Surgical measures are reserved for the rare complications of obstruction and/or perforation. Although limited, utilization of the protein-cleaving enzyme N-acetylcysteine has been described for the disintegration of GLB in toddlers. In this paper, we discuss the first documented use of N-acetylcysteine for a neonatal GLB. Supporting literature, the infant's unusual presentation, and details of the treatment regimen are discussed.
Objective: Intraventricular hemorrhage (IVH) occurs in up to 25% of very low birth weight (VLBW) preterm neonates. Previous studies found that indomethacin administered in the first 6 h of life reduces the incidence of severe IVH in VLBW neonates and decreases cerebral blood flow, suggesting a decrease in cerebral oxygen delivery. Using near-infrared spectroscopy (NIRS), we monitored cerebral oxygenation before, during and after slow indomethacin infusion in extremely low birth weight (ELBW) neonates to determine whether indomethacin decreases cerebral oxygen saturation and increases cerebral oxygen extraction.Study Design: Twenty-seven ELBW neonates less than 30 weeks gestational age treated with indomethacin for IVH prophylaxis were monitored for arterial oxygen saturation (SaO 2 ) and NIRS-determined regional cerebral oxygen saturation (rSO 2 ). At 30 to 60 s intervals, SaO 2 , rSO 2 and mean arterial pressure (MAP) were recorded using a VitalSync. Average fractional cerebral oxygen extraction was calculated for the hour before indomethacin infusion, during the infusion and 2 h after infusion.Result: Fractional cerebral oxygen extraction increased from baseline after indomethacin administration from 0.23±0.11 to 0.25±0.10 (P ¼ 0.034).
Conclusion:Fractional cerebral oxygen extraction increased 9% with indomethacin 0.1 mg kg À1 given over 1 to 2 h. However, the clinical implications of this small increase in extraction, likely representative of decreased cerebral perfusion, are unknown but may be harmful to the developing brain.
There is no difference in the effect of two formulations of isotretinoin on spine bone density after 6 months of treatment. BMD increased and the small change in spine Z-score over treatment disappeared after height adjustment. Mean positive Z-scores and HAZ in the study were likely due to the exclusion of low and inclusion of high Z-score subjects.
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