Akihiko Kimura, Ken Yuge, Shigenori Yukizane, Masayoshi Kage, Hiroshi Nittono, Reijiro Mahara, Takao Kurosawa, Masahiko Tohma

Abnormal Low Ratio of Cholic Acid to Chenodeoxycholic Acid in a Cholestatic Infant with Severe Hypoglycemia

  • Gastroenterology
  • Pediatrics, Perinatology and Child Health

SummaryWe report a premature infant with severe hypoglycemia (serum glucose: 6 mg/dl) and cholestasis (serum total bile acids: 211.55 μmol/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, 3α,7α,12α‐trihydroxy‐5β‐chole‐stan‐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 12α‐hydroxylation.

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