Abstract

This poorly understood disease has been studied in 27 girls and two boys. Metabolic aberrations included electrocardiographic abnormalities, hypovitaminosis A, hypercarotenemia, abnormal oral glucose tolerance curves, decreased xylose excretion, decreased excretion of luteinizing (LH) and follicle-stimulating hormones (FSH), decreased serum thyroxine levels, absence of diurnal variation of plasma concentrations of glucocorticoids, elevation of blood urea nitrogen, bone marrow hypoplasia, and leukopenia. With restoration of proper nutrition, most of these abnormalities (except FSH and LH) were corrected. An effective method for inpatient treatment is described. There were no deaths during this period of study.

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