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Three Cases of female Pseudohermaphroditism with Congenital Adrenal Hyperplasia.

Journal of the Korean Pediatric Society 1984;27(3):297-302.
Published online March 31, 1984.
Three Cases of female Pseudohermaphroditism with Congenital Adrenal Hyperplasia.
Yaung Sook Choi, Shin Chul Jun, Hie Ju Park, Chan Yung Kim
Department of Pediatrics, Medical College of Busan National University Busan Korea
先天性 副腎過形成症에 의한 女性 假性半陰陽 3例
崔楊被, 全信哲, 朴喜柱, 金贊榮
益山大學校 醫科大學 小兒科學敎室
Abstract
We have experienced three cases of female pseudohermaphroditism with congenital adrenal hyperplasia. First case was salt-losing type and the other two cases were non salt-losing type. First patient, 2 months old female infant, was admitted to hospital because of vomiting, diarrhea, dehydration and ambiguous sex. In electrolyte study, she had hyponatremia and hyperkalemia. The other two cases, 2 Years 10 Months old female and 4 Years old female, were complaints of ambiguous sex and accelerated growth. Their 24 Hours urinary 17-ketosteroid were increased and sex chromatin of all three cases were positive. These three patient were considered to have 21-hydroxylase defect. A brief review of related literatures was made.
Key Words: Female pseudohermaphroditism, Congenital adrenal hyperplasia, 17-ketosteroid, 21-hydroxylase defect


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