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FAMILIAL CONGENITAL ADRENAL SYNDROME

FAMILIAL CONGENITAL ADRENAL SYNDROME Bulloch and Sequeira in 19051 established the relationship between tumor of the adrenal cortex and pseudohermaphroditism. As early as 1756, however, Cooke described a case of tumor of the adrenal gland in a girl of 7 years of age. She was very obese and had an abnormal growth of hair on the face and pubic region. No attempt was made to point out the part of the gland involved. In 1910 Apert2 studied a series of cases of tumor of the adrenal cortex in females and concluded that the varying symptomatology depended on the age at which the disease developed. He proposed the following classification: Type 1. Hyperplasia of the embryonal period. In this type the internal organs are female, but outwardly there are masculine forms, such as undeveloped breasts, a hypospadiac penile-like clitoris and a vulva-like cul-de-sac. Type 2. Hyperplasia of the fetal period. Here the anomaly http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American journal of diseases of children American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1936 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
ISSN
0096-8994
eISSN
1538-3628
DOI
10.1001/archpedi.1936.04140020051006
Publisher site
See Article on Publisher Site

Abstract

Bulloch and Sequeira in 19051 established the relationship between tumor of the adrenal cortex and pseudohermaphroditism. As early as 1756, however, Cooke described a case of tumor of the adrenal gland in a girl of 7 years of age. She was very obese and had an abnormal growth of hair on the face and pubic region. No attempt was made to point out the part of the gland involved. In 1910 Apert2 studied a series of cases of tumor of the adrenal cortex in females and concluded that the varying symptomatology depended on the age at which the disease developed. He proposed the following classification: Type 1. Hyperplasia of the embryonal period. In this type the internal organs are female, but outwardly there are masculine forms, such as undeveloped breasts, a hypospadiac penile-like clitoris and a vulva-like cul-de-sac. Type 2. Hyperplasia of the fetal period. Here the anomaly

Journal

American journal of diseases of childrenAmerican Medical Association

Published: Aug 1, 1936

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