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Isolation of Neonatal Extrahepatic Cholangiocytes
Published on: June 5, 2014
Cholestasis secondary to panhypopituitarism in an infant
Daniel DeSalvo1, John F Pohl, Don P Wilson
1Department of Pediatrics, The Children's Hospital at Scott and White, Texas A&M Health Science Center, 2401 S. 31st St., Temple, TX 76508, USA.
Insights
Infant cholestasis can signal panhypopituitarism, a rare endocrine disorder. Prompt diagnosis and treatment of pituitary malfunction resolved hypoglycemia and cholestasis in a rare case, highlighting growth hormone
Area of Science:
- Pediatric Endocrinology
- Hepatology
- Endocrinology
Background:
- Infantile cholestasis necessitates comprehensive evaluation to rule out hepatic, endocrine, and metabolic etiologies.
- Timely diagnosis is crucial for preventing hepatic and neurologic complications in infants.
Observation:
- A rare case of infant cholestasis associated with panhypopituitarism, hypoglycemia, and bile duct paucity is presented.
- Liver biopsy revealed small bile ducts and atrophied biliary cells with reduced lumen size.
Findings:
- Panhypopituitarism was identified as the cause of cholestasis and hypoglycemia in this infant.
- Treatment of the underlying pituitary disorder led to the resolution of both hypoglycemia and cholestasis.
Implications:
- Panhypopituitarism should be considered in infants presenting with cholestasis and hypoglycemia.
- Growth hormone deficiency may play a role in the development of biliary duct abnormalities.
Abstract:
Cholestasis occurring in infancy should be evaluated completely to exclude hepatic as well as endocrine or metabolic causes. A rapid diagnosis should be made to ensure that hepatic and neurologic complications are prevented. We describe a rare case of infant cholestasis due to panhypopituitarism resulting in hypoglycemia and liver biopsy findings consistent with bile duct paucity. Existing bile ducts were noted to be small, and electron microscopy demonstrated diminutive and atrophied biliary cells with a diminished bile duct lumen size. Hypoglycemia and cholestasis resolved with treatment of the underlying panhypopituitarism. Panhypopituitarism should be considered in any infant who presents with cholestasis, hypoglycemia, and other manifestations of pituitary malfunction. Growth hormone deficiency may affect bile duct formation as demonstrated in this patient.
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