Nonketotic hyperglycinemia in two siblings with neonatal seizures.

Seizures are a common problem in neonates. Differential diagnoses include infection, trauma, hypoxia and congenital metabolic disorders. Among these, congenital metabolic disorder is less familiar to general pediatricians. We report two patients with nonketotic hyperglycinemia (NKH), a rare and lethal congenital metabolic disease. Transient hyperammonemia and transient hypouricemia, uncommon features found in NKH, were detected in one patient. High doses of sodium benzoate and dextromethorphan failed to modify the clinical course. Neuropathology denoted characteristic diffuse vacuolization and changes in reactive and gliotic astrocytes. The clinical course, biochemical findings, diagnostic approaches and diagnostic tests are discussed in detail. Recent modalities of treatment are reviewed. Because of its rarity and rapidly progressive course, it maybe underdiagnosed resulting in death before being recognized. Awareness of the possibility of congenital metabolic disorder in early neonatal catastrophe will increase the diagnostic rate.

Oral ibuprofen and indomethacin for treatment of patent ductus arteriosus in premature infants: a randomized trial at Ramathibodi Hospital.

BACKGROUND: Ibuprofen given intravenously to premature newborn infants is a proven treatment for patent ductus arteriosus (PDA). The efficacy of ibuprofen is comparable to indomethacin in many clinical trials with fewer renal side effects. However, the intravenous form of ibuprofen is not available in Thailand, whereas, the oral suspension form is widely used for antipyretic treatment in children. Therefore, the authors investigated the possibilities of using oral ibuprofen for the treatment of PDA in premature newborn infants. OBJECTIVE: To assess whether oral ibuprofen at 10 mg/kg/dose daily for 3 days was as effective as indomethacin to treat symptomatic PDA in premature infants and to compare the side effects of oral ibuprofen to indomethacin. SUBJECTS AND METHOD: Eighteen premature infants with gestational ages less than 34 weeks born at Ramathibodi Hospital who developed symptomatic PDA were randomly assigned to receive three doses of either indomethacin (oral or intravenous administration 0.2 mg/kg/dose for three doses given at 12 hourly intervals or oral ibuprofen (10 mg/kg/dose for three doses given at 24 hourly intervals). The rates of ductal closure, infants' clinical courses, side effects and complications were recorded. RESULTS: Birth weight, gestational age, gender, age onset and number of infants who had respiratory distress syndrome were similar in both groups, PDA was closed in 7 of 9 infants given ibuprofen (78%) and in 8 of 9 infants given indomethacin (89%) (p > 0.05). The mean plasma concentration of ibuprofen was 28.05 microg/ml at 1 hour after the third dose. Neonates in the ibuprofen group had more urine output. However, the increment of serum BUN and creatinine were not significantly different in both groups. There were no significant differences in duration of ventilator support as well as number of patients with bronchopulmonary dysplasia, intraventricular hemorrhage, necrotizing enterocolitis and death in both groups. CONCLUSION: Oral ibuprofen therapy is as effective as indomethacin for the treatment of PDA in premature infants and seems to have fewer renal side effects.

Oral erythromycin for treatment of feeding intolerance in preterm infants: a preliminary report.

Feeding intolerance is a common problem in preterm infants resulting in a prolonged hyperalimentation which is associated with an increased risk of serious and sometimes even life threatening complications, including cholestasis jaundice, liver impairment, nutritional deficiency, biochemical rickets and catheter-related septicaemia. Erythromycin, a commonly used macrolide antibiotic, has been reported as having potent prokinetic properties and enhancing gastrointestinal motor activity. The authors, therefore, conducted a preliminary study of oral erythromycin for the treatment of feeding intolerance in preterm infants to evaluate the safety and efficacy of this drug. AIM: To evaluate the safety and efficacy of oral erythromycin as a prokinetic agent in promoting enteral feeding in preterm infants with feeding intolerance. METHOD: Preterm infants, gestational age (GA) < or = 36 wk, who met the feeding intolerance criteria, were enrolled in the study. Inclusion criteria included infants who received enteral feeding less than half of full feeding or less than 75 ml/kg/day by day 14 post-natal age or gastric residual > or = 50 per cent of a given amount of feeding, more than 2 consecutive feeds by day 7 post-natal age. All patients received oral erythromycin ethylsuccinate 12 mg/kg every six hours for 2 days, then 3 mg/kg every six hours for 5 days. The times taken to establish full enteral feeding after the drug treatment and time to stop hyperalimentation were recorded. Potential adverse effects of erythromycin were assessed. Response to treatment was defined as decreased gastric residual < 30 per cent of a similar amount of the previous feed and was able to continue to full feeding. RESULTS: Ten preterm infants were enrolled in this study with a mean GA of 30.8 weeks (26-35), mean birth weight of 1,489 g (range 900-2,560 g) and mean age at entry of 9.2 days (range 7-12 days). Nine of 10 infants responded to treatment within 24 hours. The average time to establish full enteral feeding after the drug treatment was 6.6 days (range 4-10 days). None of the infants developed adverse effects such as vomiting, diarrhea, or pyloric stenosis. CONCLUSION: The preliminary data indicates that oral erythromycin is effective and safe in facilitating enteral feeding in preterm infants with feeding intolerance. Infants can achieve full feeding within a week after treatment, and this may shorten the course of hyperalimentaiton. Further randomized controlled trials are warranted.

Vitamin A status in premature infants.

BACKGROUND: Premature infants are at risk of vitamin A deficiency due to inadequate transplacental transport, inadequate storage and increased tissue utilization. Previous studies reported a significant decrease in serum vitamin A levels in premature infants at birth compared to those of full term infants. OBJECTIVE: To determine serial changes of plasma vitamin A status during the first month of life in 19 healthy, very low birth weight premature infants. METHOD: Subjects were fed with premature infant formula and received multivitamin supplementation. Plasma vitamin A concentrations were measured at 7, 14, and 30 days of age. RESULTS: Plasma vitamin A levels at 7,14 and 30 days of age were 24.63 +/- 6.08, 30.97 +/- 5.26 and 30.68 +/- 7.14 microg/dl, respectively. Plasma vitamin A levels at age 7 days were significantly lower than those at 14 and 30 days of life (p < 0.001). Three infants out of 19 (16%) had low plasma vitamin A (<20 microg/dl) at 7 days. At 14 and 30 days of age, all infants had normal plasma vitamin A levels. CONCLUSION: The results suggested that healthy premature infants were prone to subclinical vitamin A deficiency during the first week of life which could be treated by adequate enteral feeding and routine multivitamin supplementation. A high dose of vitamin A supplementation was not necessary in healthy premature infants.