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Anterior pituitary hormone effects on hepatic functions in infants with congenital hypopituitarism
Wikrom Karnsakul1, Pairunyar Sawathiparnich, Saroj Nimkarn
1Department of Pediatrics, Section of Gastroenterology and Nutrition, West Virginia University School of Medicine, Morgantown, WV 26506-9214, USA. wkarnsakul@hsc.wvu.edu
Insights
Congenital hypopituitarism can cause neonatal cholestasis in infants. Glucocorticoid and thyroid hormone replacement therapy helped resolve cholestasis and hepatosplenomegaly, but further monitoring is advised.
Area of Science:
- Pediatric Endocrinology
- Neonatal Medicine
- Hepatology
Background:
- Congenital hypopituitarism is a rare cause of neonatal cholestasis.
- The impact of anterior pituitary hormones on liver function in neonates is not well understood.
Purpose of the Study:
- To investigate the role of anterior pituitary hormones in neonatal cholestasis.
- To evaluate the effects of hormone replacement therapy on cholestasis and liver function in infants with congenital hypopituitarism.
Main Methods:
- Retrospective review of medical charts of eight infants with congenital hypopituitarism and neonatal cholestasis.
- Analysis of endocrinological investigations, eye examinations, and MRI findings.
- Assessment of treatment outcomes following hormone replacement therapy.
Main Results:
- Eight infants (4 male, 4 female) presented with cholestatic jaundice and subsequently developed anterior pituitary hormone deficiencies.
- Common clinical signs included hypoglycemia, ocular abnormalities, and microphallus; septo-optic dysplasia was prevalent (5/8 cases).
- Cholestasis and hepatosplenomegaly resolved in most infants after glucocorticoid and thyroid hormone replacement, though transaminase levels remained elevated. Cortisol deficiency and hypoglycemia were universal. Hyperlipidemia persisted in one case.
Conclusions:
- Glucocorticoid and thyroid hormones are crucial for resolving cholestasis and hepatosplenomegaly in infants with congenital hypopituitarism.
- Persistently elevated transaminases and hyperlipidemia post-treatment may necessitate long-term follow-up or growth hormone therapy.
- Growth hormone deficiency treatment was limited by factors including hypoglycemia resolution, infant age, and financial constraints.
Background:
Congenital hypopituitarism is an uncommon cause of neonatal cholestasis. Little is known about the effect of anterior pituitary hormone on hepatic functions.
Methods:
A retrospective review of the medical charts of eight infants with congenital hypopituitarism and neonatal cholestasis was performed. The results of endocrinological investigations, eye examinations, and magnetic resonance imaging were used to classify these infants.
Results:
Eight infants (4 male and 4 female; mean age, 1.7 weeks) who presented with cholestatic jaundice subsequently (mean age, 7.6 weeks) developed isolated or multiple anterior pituitary hormone deficiencies. Persistent hypoglycemia, ocular abnormalities, and microphallus were often clinical signs prompting further endocrinological and radiological investigations. Septo-optic dysplasia was prevalent, occurring in five cases. Cholestasis and hepatosplenomegaly resolved within a mean of 9.7 and 10 weeks, respectively, in the majority of cases after replacement of glucocorticoid and thyroid hormones. However, transaminase levels remained high after hormone replacement. Cortisol deficiency and hypoglycemia were noted in all cases, often following stress. Hyperlipidemia persisted in one case after the resolution of cholestasis and after corticosteroid and thyroid hormone replacement therapy. Growth hormone deficiency was not corrected due to the absence of hypoglycemia after corticosteroid hormone, an infant's age, and/or a lack of financial resources.
Conclusions:
In our series, it appears that glucocorticoid and thyroid hormones play a significant role in the resolution of cholestasis and hepatosplenomegaly. A persistently elevated transaminase level and hyperlipidemia after corticosteroid and thyroid hormone replacement may indicate the need for long-term follow-up and/or growth hormone therapy.
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