Insulin-like growth factor-1 (IGF-1) in children with postoperative biliary atresia: a cross-sectional study.

Biliary atresia is the leading cause of chronic infantile cholestasis which eventually leads to cirrhosis. Re-establishment of biliary drainage by Kasai portoenterostomy and liver transplantation for end-stage liver disease has favorably altered the clinical outcome. However, growth failure, one of the major complications of chronic liver disease, remains a major problem. The aim of the study is to evaluate growth, nutritional status and serum growth factor IGF-1 in children with biliary atresia after Kasai operation and at comparing these data between the groups with successful and unsuccessful operation. Fifty-four children with postoperative biliary atresia were evaluated for their clinical outcome, height, blood biochemistry related nutritional status and serum IGF-1. Height and serum IGF-1 were expressed as standard deviation score (SDS) to minimize the influence of age. With 44.4% of the enrolled patients the operation had been unsuccessful and jaundice persisted. The mean age of children with jaundice in comparison with the jaundice free groups was not significantly different (42.0 and 49.9 months, p = 0.458). In jaundice-free patients, hematocrit, serum albumin, calcium and phosphorus were normal and significantly higher. In the successful Kasai group, the height SDS and serum IGF-1 SDS were within the normal range and significantly higher (height SDS 0.2 +/-1.0 vs. -0.9 +/- 1.2, p < 0.01 and IGF-1 SDS 0.5 +/- 2.2 vs. -1.3 +/- 1.0, p < 0.01). The mean IGF-1 SDS in the failed Kasai group was less than -1. Children with good outcome of postoperative biliary atresia showed better growth, better nutritional status and higher serum IGF-1 levels when compared to those with unsuccessful operation.

Galactosemia in Thai patient at Phramongkutklao Hospital: a case report.

Galactosemia is a rare autosomal recessive disorder of galactose metabolism, which occurs as a consequence of a deficiency of one of these three enzymes: galactokinase, galactose-1-phosphate uridyltransferase, and uridine diphosphate galactose-4-epimerase, leading to elevated level of galactose and its metabolites in blood. The presented case was a 2-month-old, Thai female infant with persistent cholestatic jaundice, bilateral posterior subcapsular cataracts, and hepatomegaly. Laboratory investigations showed slightly elevated serum aminotransferase, and increased urinary excretion of galactose, galactitol and galactonate (by urine gas chromatography/mass spectrometry). These findings indicated an error in galactose metabolism. Soy-based formula was introduced to the patient. Clinical and laboratory results were improved after a few months of treatment. Genetic counseling was provided to the family for 25% of recurrence risk. Prenatal diagnosis is not established in Thailand.