HYPERTENSIVE FORM OF CONGENITAL ADRENOCORTICAL HYPERPLASIA

Abstract

ABSTRACT A case of the hypertensive type of congenital adrenocortical hyperplasia (Eberlein-Bongiovanni's syndrome) is described. The patient was followed up for 12 years. The urinary steroid pattern was characterized by the presence of tetrahydro-11-deoxycortisol and tetrahydro-11-deoxycorticosterone. The urinary excretion of pregnanetriol was only moderately increased. Adequate cortisone treatment was started 11 years after hypertension was demonstrated. Treatment had no effect on the blood pressure but the excretion of steroids became normal. The patient was 14 years old when first seen by the authors. She then showed virilism. Menstruation occurred for the first time after 10 days of cortisone treatment. Laparotomy revealed polycystic ovarian changes of the type seen in Stein-Leventhal's syndrome. Wedge resection of the ovaries was followed by normal menstruations without any cortisone treatment. The relationship between abnormal secretion of steroids by the ovaries or the adrenal cortex and polycystic changes of the ovaries is discussed.