Hypertension With Adrenal, Genital, Renal Defects, and Deafness

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Abstract
In 1955, Eberlein and Bongiovanni1described the deficiency of the steroid 11βhydroxylase in the hypertensive form of virilizing adrenal hyperplasia. More recently, Biglieri and co-workers2brought attention to a different type of hypertension associated with hypogenitalism and absence of 17α-hydroxylation of adrenocortical steroids. Turner's syndrome may be also associated with hypertension.3The association of hypertension with hereditary renal disease (Alport's syndrome) is rare, and it is only in the later stages that hypertension appears in patients with this syndrome who also suffer from nephropathy and deafness.4 We report here another form of severe hypertension associated with an abnormal steroidogenesis, hypogenitalism (ovarian dysgenesis or cryptorchidism), renal failure, and deafness in three siblings. Subjects Three children of a family of five children of French Canadian extraction are described in this report. The remaining two siblings are healthy; they have normal blood pressure and sexual development. The 54-year-old mother