Effects of liver transplantation on long-chain polyunsaturated fatty acid status in infants with biliary atresia.

BACKGROUND: The long-chain polyunsaturated fatty acid (LC-PUFA) status of infants with untreated biliary atresia (BA) is known to be poor and is correlated to the severity of the liver disease. Liver transplantation (LT) markedly increases survival of patients with BA but the extent to which this reverses poor LC-PUFA status is not known. METHODS: To explore this question, the erythrocyte (red blood cell, RBC) phospholipid content of eight infants with BA who underwent LT was determined 2 months after an initial portoenterostomy, immediately before LT, and 6 and 12 months after LT. Before LT, all infants were fed a protein hydrolysate formula containing medium-chain triglycerides and essential fatty acids. Afterward, they were fed a normal diet for age. The RBC phospholipid content at each time point was compared with that of 28 age-matched control infants. RESULTS: Just before LT, median RBC phospholipid content of C20:4n-6, C20:5n-3, and C22:6n-3 was 25%, 48%, and 30% lower, respectively, than that observed in age-matched control infants. After LT, the RBC phospholipid content of most fatty acids reached normal values by 6 months. However, that of C20:4n-6 and C22:6n-3 contents remained 5% and 15% lower, respectively, than in normal control infants. Twelve months after LT, C20:4n-6 content remained lower than in normal children, but that of C22:6n-3 did not differ. The ratio of C20:3n-6/C20:4n-6, a reflection of delta-5 desaturase activity, was abnormal compared with normal children before LT (0.17 vs. 0.10, P < 0.009) but normalized by 6 months after LT (0.11 vs. 0.10, not significant). CONCLUSIONS: These data show that the abnormal LC-PUFA status of children with BA improves after LT but is not entirely reversed within a year after surgery. They suggest that the abnormal status before LT may be secondary, in part, to low delta-5 desaturase activity. The extent to which a different pre- and/or post-LT diet can prevent PUFA deficiency and/or hasten recovery of PUFA status remains to be determined.

Neurenteric cyst: antenatal diagnosis and therapeutic approach.

We hereby report the second case of antenatally diagnosed "neurenteric cyst" in the literature. The patient we describe presented as a neonatal respiratory distress syndrome (NRDS) which did not respond to a vigorous resuscitation. Anomaly of the antenatal ultrasonography and of the initial chest x-ray added to the non-response to therapy, led us to an urgent thoracic scan and to a lifesaving thoracotomy. We think that every posterior mediastinal cystic mass, with or without vertebral malformations, diagnosed antenatally, should raise the possibility of neurenteric cyst. The perinatal management of the newborn to be, will thus be simplified. This will lead to a better outcome.

[Hydrocarbon poisoning in children]. / Intoxications aux hydrocarbures chez l'enfant.

Carbohydrate poisoning is among serious children intoxications, made possible by inadequate stocking; boys, one to five years old, are particularly at risk in a series of 43 children examined and admitted in Hôtel-Dieu de France Hospital over a 10-year period. Outcome is constantly good, after a short hospital care; clinical exam and chest X-ray are of particular value on admission. Some cases may need home care, whereas others should undertake hospital day care; treatment is discussed and long-term follow-up is being stressed on.