Abstract:We report a female infant with congenital hypopituitarism. All anterior pituitary hormones were deficient, and the anterior pituitary gland was undetectable on magnetic resonance images. Antidiuretic hormone secretion was appropriate, and the posterior lobe was localized at the end of the stalk, in the sella. These observations suggest that the posterior pituitary gland may develop independently of the anterior lobe.
“…At least thirty-two cases of congenital hypopituitarism with cholestasis have been reported
(2,3,4,5,6,7,8,9,10,11,12,13,14,15). The doses
of replacement therapy were mentioned in 11 cases (3,
5, 7, 8, 10, 11).…”
A patient with congenital hypopituitarism associated with cholestasis is reported here.
Large doses of fat-soluble hormones (hydrocortisone (20 mg/m2/day) and
L-thyroxine (14 μg/kg/day)) were needed to resolve hypoglycemia and hypothyroidism during
cholestasis. The doses could be reduced to 10 mg/m2/day and 3.5 μg/kg/day,
respectively, after improvement of cholestasis. Sodium valproate, which is a water-soluble
drug, did not need any dose adjustments during cholestasis. Adjustment of fat-soluble
hormone doses during cholestasis should be considered in patients with cholestasis.
“…At least thirty-two cases of congenital hypopituitarism with cholestasis have been reported
(2,3,4,5,6,7,8,9,10,11,12,13,14,15). The doses
of replacement therapy were mentioned in 11 cases (3,
5, 7, 8, 10, 11).…”
A patient with congenital hypopituitarism associated with cholestasis is reported here.
Large doses of fat-soluble hormones (hydrocortisone (20 mg/m2/day) and
L-thyroxine (14 μg/kg/day)) were needed to resolve hypoglycemia and hypothyroidism during
cholestasis. The doses could be reduced to 10 mg/m2/day and 3.5 μg/kg/day,
respectively, after improvement of cholestasis. Sodium valproate, which is a water-soluble
drug, did not need any dose adjustments during cholestasis. Adjustment of fat-soluble
hormone doses during cholestasis should be considered in patients with cholestasis.
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