The Mauriac Syndrome

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Abstract
A ten-year-old girl, treated for diabetes mellitus with single daily doses of short acting insulin preparations for three years, presented with short stature, truncal obesity, round facies, and hepato megaly : the Mauriac syndrome. She had elevated levels of plasma cortisol suggesting hyperadreno corticism secondary to the poor diabetic control. The urinary excretion of 17 hydroxycorticoids and 17 ketosteroids were normal, however. The hypothalamic-hypophyseal-adrenal cortex axis was functionally normal. She had a normal growth hormone response to the administration of arginine monochloride. On adequate control of the diabetes mellitus, all the manifestations disappeared.