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Abnormal low ratio of cholic acid to chenodeoxycholic acid in a cholestatic infant with severe hypoglycemia
1Department of Pediatrics and Child Health, Kurume University School of Medicine, Japan.
Insights
A premature infant experienced severe hypoglycemia and cholestasis due to underdeveloped bile ducts and pituitary issues. This case highlights immature bile acid metabolism in neonates.
Area of Science:
- Neonatology
- Pediatric Endocrinology
- Hepatology
Background:
- Premature infants are susceptible to metabolic and liver complications.
- Hypoglycemia and cholestasis can indicate underlying endocrine or hepatic dysfunction.
Observation:
- A premature infant presented with severe hypoglycemia and cholestasis.
- The infant also had intracranial hemorrhage, sepsis, and was diagnosed with idiopathic panhypopituitarism.
- Abnormal serum and biliary bile acid profiles were noted, including a low cholic acid to chenodeoxycholic acid ratio.
Findings:
- Hypoplasia of interlobular bile ducts was identified as the cause of cholestasis.
- Idiopathic panhypopituitarism contributed to the infant's complex medical condition.
- Abnormal bile acid profiles suggest immature enterohepatic circulation and hepatic 12 alpha-hydroxylation.
Implications:
- This case underscores the importance of evaluating bile acid metabolism in neonates with unexplained cholestasis and endocrine disorders.
- Findings suggest that physiological immaturity in bile acid pathways can manifest as severe liver dysfunction in premature infants.
- Further research into neonatal bile acid metabolism is warranted to improve diagnosis and management of related conditions.
Abstract:
We report a premature infant with severe hypoglycemia (serum glucose: 6 mg/dl) and cholestasis (serum total bile acids: 211.55 mumol/L) caused by hypoplasia of the interlobular bile ducts. This patient had developed intracranial hemorrhage and sepsis while undergoing treatment for hypoglycemia. As a result of endocrine evaluation, we made a diagnosis of idiopathic panhypopituitarism, congenital absence or hypoplasia of the pituitary gland. Moreover, we found abnormal bile acid profiles: The ratio of cholic acid to chenodeoxycholic acid was abnormally low in serum (0.04) and in biliary bile (0.33). However, 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestan-26-oic acid and bile alcohols were not detected. We therefore suspected that the severe cholestasis and abnormal bile acid profiles in the serum and biliary bile in this patient were related to physiologic immaturity of the enterohepatic circulation of bile acids and immaturity of hepatic 12 alpha-hydroxylation.