Otto Kuchel, M.D.; Pavel Hamet, M.D.; Wojciech Nowaczynski, D.SC.; Marcel Cadotte, M.D.; Jacques Genest, M.D., F.A.C.P.
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Fifteen to 25% of patients with gonadal dysgenesis have "essential" hypertension. The deficiency of 17-hydroxylation is another rare form of hypertension associated with hypergonadotropic hypogenitalism. Two new varieties of this association are described.
1. A syndrome of severe hypertension affecting three siblings (two sisters, one brother) is associated with hypogenitalism (ovarian agenesis and cryptorchism, respectively), decreased hearing, renal insufficiency, and decreased platelets adhesiveness. At autopsy of one sister a marked thymic hyperplasia and hypoplasia of several organs were found. Adrenals presented hyperplasia of zona glomerulosa and produced excessive deoxycorticosterone and compound "S". Plasma aldosterone and compound "S" secretion rate are
Kuchel O, Hamet P, Nowaczynski W, et al. Hypertension and Hypogenitalism: Two New Varieties of This Clinical Association.. Ann Intern Med. 1970;72:788–789. doi: https://doi.org/10.7326/0003-4819-72-5-788_4
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Published: Ann Intern Med. 1970;72(5):788-789.
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