Congenital Adrenocortical Hyperplasia With Cushing's Syndrome

Abstract

Cushing's syndrome in infants is a rare disorder, usually caused by adrenocortical carcinoma. Clinical and pathological features of a fatal case occurring in a female infant 8 weeks of age are reported. Initial abnormalities were glycosuria, granulocytosis, and failure to thrive. The marked retardation of skeletal maturation, with bone age interpreted as less than term, suggests a prenatal onset of the endocrine disorder. The infant died after adrenal exploration. Both adrenal glands were hyperplastic and nodular while generalized wasting, sparing heart and kidneys, characterized the anatomic changes generally. An unexpected preponderance of acidophils was discovered in cell counts of the anterior pituitary.