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MALE PSEUDOHERMAPHRODITISM WITH 17 α ‐HYDROXYLASE DEFICIENCY
Author(s) -
Abad L.,
Parrilla J. J.,
Marcos J.,
Gimeno F.,
Bernal A. Loapez
Publication year - 1980
Publication title -
bjog: an international journal of obstetrics and gynaecology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.157
H-Index - 164
eISSN - 1471-0528
pISSN - 1470-0328
DOI - 10.1111/j.1471-0528.1980.tb04491.x
Subject(s) - male pseudohermaphroditism , pseudohermaphroditism , medicine , endocrinology
Summary A case of male pseudohermaphroditism with 17 α ‐hydroxylase deficiency is reported in a 23‐year‐old woman presenting with primary amenorrhoea and a history of bilateral inguinal hernia repair. She was tall, had hypoplastic external genitalia with a blood pressure of 220/140 mm Hg. Her karyotype was XY. Acute adrenal failure occurred following exploratory laparotomy. After treatment with gluco‐corticoids and oestrogens, the hormone and electrolyte profiles returned to normal and the blood pressure fell. The biochemical implications of this enzyme deficiency are discussed.