This paper presents the case of a boy developing symptoms of adrenal insufficiency soon after birth, followed by unmistakable evidences of the changes in the development associated with androgens. Other reports bring out the fact that the picture of increased androgenic activity with or without adrenocortical insufficiency is associated with hyperplasia and persistence of the fetal (androgenic)...

Adrenocortical hyperplasia is uncommon in childhood. It occurs as the adrenogenital syndrome (congenital virilism in girls) which has now been shown to be due to a congenital defect in the synthesis of cortisol by the adrenal cortex. In the United States its incidence is once in every 58,000 live births (Grumbach, 1956) and it has a recessive mode of inheritance. The disease is more easily diag...

A combination of Turner syndrome (TS) and classical congenital adrenal hyperplasia (CAH) is rare. A one-day-old newborn was referred to our hospital with ambiguous genitalia. The parents were third-degree relatives. The infant's weight was 3350g (50-75p), and the head circumference was 34.5cm (50p). The gonads were nonpalpable. Presence of a 3 cm phallus, one urogenital opening into the perineu...

Should hirsute women be investigated? Most only need careful clinical evaluation. First, they need to be examined to determine whether they are hirsute or hypertrichotic, and for the degree of hair growth to assess the most appropriate form of treatment. Second, they need to be clinically evaluated for signs and symptoms of virilism to determine the extent of investigation needed. If virilism i...

Congenital virilizing adrenal hyperplasia miiay present a varied clinical picture. Patients have been described who have only excessive virilization (1); others have an associated Addison-like electrolyte disturbance (2, 3), with hypoglycemia (4), or with hypertension (5-9). Recent studies (1012) indicate that the manifestation of virilism may result from defects in the biosynthesis of cortisol...

The administration of cortisone to patients with virilism associated with adrenal hyperplasia results in a marked decrease in urinary 17-ketosteroid excretion (1-3) whereas no significant fall occurs in those patients whose virilism is due to an adrenal tumor (4, 5). The administration of cortisone inhibits pituitary adrenocorticotropin secretion causing secondary atrophy of the adrenals (6, 7)...

Ideas have a life-cycle: they stimulate understanding in their youth but become stale and inhibitory with age. The same is true of the names of diseases which spring from these ideas. Following on some of our own recent studies, and in particular in the course of presenting them at seminars and lectures, it became apparent that the term "idiopathic hirsuties" has now outlived its usefulness let...