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
|
|