| THE CASES OF ADRENOGENITAL SYNDROME DUE TO CONGENITAL VIRILIZING ADRENAL HYPERPLASIA
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Yong Eun Moon, Kook Hoon Ko, Eui Sun Lim |
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Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea |
| 先天性 副腎性器症候群의 3例 |
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文 永殷, 高充 勸, 杯 宣善 |
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延世大醫大 小兒科學敎室 |
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| Abstract |
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Adrenogenital syndrome of the congenital type is a familial condition in which excessive androgen secretion occurs in homozygous offspring as a result of an inborn error in metabolism. The excessive secretion of androgen originates during the gestational period and continues throughout life if not treated. Accurate clinical and anatomical descriptions of the disease have been available since the early years of this century. However, it is only since the discovery by Wilkins and associates ef the efficacy of cortisone in controlling this abnormal sscretion, thus enabling the9&individuals to lead a near-normal life, that interest in this condition has become widespread.
The abnormal secretion of androgen with its virilizing effects exerts a profound influence on sexual development and somatic growth. In the female there occurs the syndrome of female pseudohermaphroditism, and, in the male, macrogenitosomia precox. This report is a presentation of three cases of female pseudohermaphroditism due to congenitai virilizing adrenal hyperplasia observed ia the last year. Casa 1 and 2 are sibling. The diagnosis and management are discussed.
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