Accelerated conversion of dehydroepiandrosterone sulfate to estrogen in a patient with Crow-Fukase syndrome and diabetes mellitus

About 28% of patients with the Crow-Fukase syndrome exhibit glucose intoler ance which may be induced by low serum levels of dehydroepiandrosterone (DH EA). We report a patient with the Crow-Fukase syndrome who exhibited non-in sulin dependent diabetes mellitus (NIDDM) worsened prior to admission. He r eceived the DHEA sulfate (DHEA-S) infusion test to evaluate aromatase activ ity. This patient exhibited an increase in aromatase activity measured by t he conversion of the intravenously loaded DHEA-S to estrogen, and low serum levels of DHEA and DHEA-S, These abnormalities returned to nearly normal d uring the administration of prednisolone, 60 mg per day. No adverse effect on his diabetes was observed during the corticosteroid treatment. Five cont rol patients with diabetes but without the Crow-Fukase syndrome showed no i ncrease in the conversion of DHEA-S to estrogen, which suggests that aromat ase activity is normal in diabetes. The increase in aromatase activity in our patient may have led to a low ser um concentration of DHEA that in turn caused glucose intolerance and a dete rioration of the diabetes prior to admission. Glucocorticoid therapy may be beneficial in Crow-Fukase syndrome to improve the distorted metabolism of DHEA with no adverse effect on the diabetes.