Acute fatty liver of pregnancy associated with short-chain acyl-coenzyme A dehydrogenase deficiency.

There is a correlation between pregnancy complications such as acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency. We diagnosed another fatty acid beta-oxidation defect, short-chain acyl-coenzyme A dehydrogenase deficiency, in an infant when evaluating him because his mother had acute fatty liver of pregnancy. Other beta-oxidation defects, in addition to LCHAD deficiency, should be considered in children born after pregnancies complicated by liver disease.

Early cirrhosis in survivors with Jeune thoracic dystrophy.

Two siblings with Jeune thoracic dystrophy had persistent evidence of hepatic dysfunction; one had cirrhosis. Liver disease, a significant problem in patients who survive this condition, may be progressive in some.

Evaluation of the effect of a fiber-enriched formula on infant colic.

Because infants with colic appear to have abdominal pain similar to that of adults with irritable bowel syndrome, who may benefit from the addition of fiber to their diet, we tested whether fiber added to infant formula would alleviate colic. Twenty-seven normal, term infants (aged 2 to 8 weeks; 14 girls) with colic, defined as crying plus fussing for more than 3 hours a day for at least 3 days of a 6-day baseline period, were enrolled. Infants were randomly assigned in 9-day periods to a sequence of placebo (Isomil formula) followed by fiber-supplemented formula (Isomil plus soy polysaccharide) (n = 12) or the reverse (n = 15). Daily diaries of crying, fussing, sleeping, formula, intake, and stooling were kept. Twenty-two infants completed three lactulose breath hydrogen tests at the end of the baseline period and after each study period. The crossover trial was followed by 30 to 35 days of use of the study formula chosen by the parents as most beneficial but unknown to the investigators. Growth was monitored throughout. Serum cholesterol, calcium, phosphate, albumin, iron, and zinc concentrations were measured at the conclusion. There were no significant differences in average daily time spent by the infants in fussing and crying during ingestion of the fiber-supplemented formula. However, parents of 18 of 27 infants chose fiber-supplemented formula as most beneficial in ameliorating symptoms of colic. While the infants were consuming fiber-supplemented formula, stool frequency increased, and breath hydrogen excretion increased significantly, in response to lactulose. Growth and serum biochemical measurements were normal in all infants. Supplementation of infant formula with the level of soy polysaccharide used in this study may have reduced crying and fussing in some infants but did not affect colicky behavior in the majority of infants, who continued to cry and fuss excessively.

Suppression of gastric acid secretion by intravenous administration of famotidine in children.

This study was designed to establish appropriate dosing requirements for intravenous use of famotidine, a new H2-receptor antagonist, in pediatric patients. Eighteen children, 2 to 69 months of age (mean +/- SD: 31 +/- 23 months), were treated with intravenously administered famotidine to prevent bleeding of the upper gastrointestinal tract. Continuous intragastric pH monitoring was carried out to ascertain the effectiveness of various intravenous doses. An initial dose of 0.4 mg/kg was given and repeated only after the intragastric pH had dropped to less than 4.0 for 2 hours. Increased doses were given (0.8, 1.2, and 1.6 mg/kg) if the previous dose did not raise the intragastric pH to greater than 4.0 for greater than or equal to 6 hours. Sixteen patients achieved an intragastric pH greater than or equal to 4.0 for greater than or equal to 6 hours, 13 with a dose of 0.4 mg/kg, and three with a dose of 0.8 mg/kg. The mean duration of continuous pH greater than 4.0 per dose was 9.0 +/- 7.5 hours. Ten patients received two or more intravenous doses of famotidine; the mean duration of pH greater than 4.0 after the second dose was less than that after the first (14.9 +/- 8.0 hours vs 6.9 +/- 3.2 hours, p less than 0.01). We conclude that intravenous famotidine therapy raises gastric pH to greater than 4.0 for approximately 9 hours in most children. Prolonged intravenous use of famotidine rapidly leads to a decreased duration of efficacy, necessitating intragastric pH monitoring to assess the effectiveness of treatment.