EFFECT OF THE MENARCHE ON HEPATOMEGALY IN AN APUBESCENT GIRL WITH A SYNDROME RESEMBLING GLYCOGEN DISEASE*

Abstract

A patient whose symptoms and findings resembled glycogen disease was presented. Her hepatomegaly disappeared with the onset of spontaneous ovarian function. Urinary hormone assays indicate that retarded statural and sexual development are secondary to the disturbance in glycogen metabolism rather than due to a primary endocrinopathy. Recent investigations which involve the pancreas in the patho-genesis of the disease are cited and discussed. Review of the literature and study of this patient indicates that when patients reach sexual maturity the prognosis is good.