malabsorption of fat - soluble vitamins is known during 
 cholestasis , while that of fat - soluble hormones has not been reported . we report a case of 
 congenital hypopituitarism with cholestasis whose hypoglycemia and hypothyroidism were not 
 improved by conventional doses of hydrocortisone and l - thyroxine . the patient , a male infant , was born to unrelated healthy parents by emergency caesarean 
 section because of a low level of estriol ( data not available ) in maternal urine at 39 wk of 
 gestation . his birth weight and height were 3.3 
 kg ( + 0.5 sd ) and 48.8 cm ( 0.7 sd ) , respectively . hypoglycemia disappeared with a 
 continuous infusion of glucose ( 4 mg / kg / min ) . conjugated hyperbilirubinemia , liver 
 dysfunction and hepatomegaly were found at one and a half months of age . at three and a half 
 months of age he had convulsions with hypoglycemia ( 5 mg / dl ) after a six - hour fasting . he 
 was diagnosed as having acth , tsh and gh deficiency at four months of age from the 
 laboratory findings shown in table 1table 1 laboratory findings when hypopituitarism was diagnosed . sept - optic - dysplasia was suspected because of nystagmus and bilateral optic 
 nerve hypoplasia . hydrocortisone ( 10 mg / m / day ) and l - thyroxine ( 6.5 g / kg / day ) were started at 
 four months of age . in spite of replacement therapy , hypoglycemia and the hypothyroid state 
 persisted at five months of age , when fat - soluble vitamins were relatively low with 
 cholestasis : serum vitamin e level , 2.3 mg / l ( normal 39 mg / l ) ; serum 25 hydroxyvitamin d 
 level , 15 ng / ml ( normal 1580 ng / ml ) . the doses of hydrocortisone and l - thyroxine needed to 
 be increased to a maximum of 20 mg / m / day and 14 g / kg / day , respectively , to keep 
 normal blood glucose levels and normal thyroxine levels ( table 2table 2 laboratory findings and doses of therapy ) . in contrast sodium valproate ( 20 mg / kg / day ) , which was started due to 
 seizure without hypoglycemia , was not increased to keep the appropriate level ( around 50 
 g / ml ) . blood glucose levels after six - hour fasting rose above 60 mg / dl with hydrocortisone ( 20 
 mg / m / day ) at five and a half months of age , one month before start of growth 
 hormone treatment ( see below ) . serum cortisol levels ( 2 h after taking hydrocortisone 
 orally , when the levels are supposed to be in the highest range ) were not extremely high 
 ( around 8 g / dl ) and obesity was not noted at five and a half months of age . similarly , a 
 euthyroid state was eventually attained with l - thyroxine ( 14 g / kg / day ) at seven months of 
 age . height had been 2 sd since one month of age . at six and a half months of age growth 
 hormone treatment serum cortisol level ( 2 h after taking hydrocortisone orally ) increased up to 15 g / dl 
 without change of dose ( 20 mg / m / day ) at seven months of age , when cholestasis 
 had improved : direct bilirubin level , 0.9 mg / dl ; bile acid level , 85 mol / l . the dose of 
 hydrocortisone could be reduced to 10 mg / m / day at seven months of age . the dose of l - thyroxine could be 
 decreased similarly at seven months of age ( table 
 2 ) . the dose of l - thyroxine was 3.5 g / kg / day at nine months of age when 
 cholestasis was completely resolved . fat - soluble hormones such as hydrocortisone and l - thyroxine were monitored during 
 cholestasis in a patient with congenital hypopituitarism . the dose of these hormones had to 
 be changed according to the condition of 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 ) . relatively high doses of l - thyroxine ( 8 to 10 
 g / kg / day ) were needed until six months of age in five cases . in these cases the doses of 
 hydrocortisone tended to be high ( 10 to 20 mg / m / day ) . one of the reasons why hydrocortisone and l - thyroxine should be increased in cholestasis is 
 because supplements of fat - soluble vitamins are needed . insufficiency of bile acids 
 secretion in the intestine causes difficulty for the absorption of fat - soluble substances . serum bile acid levels , an established marker for cholestasis ( 16 ) , were roughly correlated with the dose of hydrocortisone and 
 l - thyroxine in our case . in conclusion , the dose of hydrocortisone and l - thyroxine should be increased during 
 cholestasis in patients with hypopituitarism .
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 g / kg / day ) ) were needed to resolve hypoglycemia and hypothyroidism during 
 cholestasis . 
 the doses could be reduced to 10 mg / m2/day and 3.5 g / 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 .