Anterior pituitary hormone effects on hepatic functions in infants with congenital hypopituitarism.
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.
Karnsakul
Wikrom
W
Department of Pediatrics, Section of Gastroenterology and Nutrition, West Virginia University School of Medicine, Morgantown, WV 26506-9214, USA. wkarnsakul@hsc.wvu.edu
Sawathiparnich
Pairunyar
P
Nimkarn
Saroj
S
Likitmaskul
Supawadee
S
Santiprabhob
Jeerunda
J
Aanpreung
Prapun
P
D002779:Cholestasis / Q000209:etiology*; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D013006:Growth Hormone / Q000172:deficiency; D006529:Hepatomegaly / Q000209:etiology*; D020249:Hormone Replacement Therapy; D006801:Humans; D006854:Hydrocortisone / Q000172:deficiency; D007003:Hypoglycemia / Q000209:etiology; D007018:Hypopituitarism / Q000150:complications* / Q000151:congenital / Q000188:drug therapy; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000473:pathology / Q000503:physiopathology*; D008297:Male; D010908:Pituitary Hormones, Anterior / Q000172:deficiency*; D013163:Splenomegaly / Q000209:etiology; D013963:Thyroid Hormones / Q000627:therapeutic use; D000637:Transaminases / Q000097:blood
D005938:Glucocorticoids; D010908:Pituitary Hormones, Anterior; D013963:Thyroid Hormones; D013006:Growth Hormone; D000637:Transaminases; D006854:Hydrocortisone