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An Autopsy Case of 17-Hydroxylase Deficiency with Malignant Hypertension

The First Department of Internal Medicine and The Department of Pathology, School of Medicine, Nagasaki University, Nagasaki, Japan

Address requests for reprints to: Dr. Isao Morimoto, The First Department of Internal Medicine, School of Medicine, Nagasaki University, Sakamoto-machi, Nagasaki 852, Japan.

This is the first autopsy case of male 17-hydroxylase deficiency with malignant hypertension. The subject had hypertension, hypokalemic alkalosis, and pseudohermaphroditism. At age 21, 17-hydroxylase deficiency was diagnosed by low urinary excretion of 17-hydroxysteroids, low secretion rate of cortisol, and low plasma testosterone level in association with high urinary excretion of pregnanediol and high plasma progesterone and corticosterone. Urinary excretion of aldosterone and PRA were suppressed, and p lasma ACTH was elevated. Hypertension and hypokalemic alkalosis were normalized with dexamethasone therapy. After missing 5 yr of followup, malignant hypertension developed, and PRA and aldosterone were elevated. Histological examination revealed some characteristic arteriolar lesions as in malignant nephrosclerosis. Juxtaglomerular hyperplasia and an increase of renin granules were observed, which reflected high PRA. Abnormal histological findings of endocrine organs were observed in the breast, the pituitary gland, the adrenal glands, and the testis. (J Clin Endocrinol Metab 56: 915, 1983)

Received July 27, 1982.

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