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Adrenaogenital Syndrome with Congenital Adrenal Hyperplasia.

Journal of the Korean Pediatric Society 1985;28(1):78-84.
Published online January 31, 1985.
Adrenaogenital Syndrome with Congenital Adrenal Hyperplasia.
Kyeong Rae Moon1, Yeong Bong Park1, Sang Gi Kim1, Jin Heon Kim1, Chang Soo Ra1, Jae Hong Seo2, Ho Won Hwang2
1Department of Pediatrics,College of Medicine, Chosun University
2Department of Pathology,College of Medicine, Chosun University
선천성 부신과형 성증에 의한 부신성 기증후군 1례(Salt losing 형)
문경래1, 박영봉1, 김상기1, 김진헌1, 나창수1, 서재홍2, 황호원2
1조선대학교 의과대학 소아과학교실
2조선대학교 의과대학 병리학교실
Abstract
Adrenogenital syndrome is caused by a defect in biosynthesis of adrenal corticoids as a result of deficiency in one of the essential enzymes, most commonly 21-hydroxylase. About 50% of 21-hydroxylase deficiency have salt losing type as a result of aldosterone deficiency. We have experienced a casa of adrenogenital syndrome associated with congenital adrenal hyperplasia (salt losing form) in a 3 months old female infant with severe dehydration and malnutrition due to persistent vomiting and diarrhea since shortly after the birth. And the patient was expired at the age of 4 month. Serum electrolytes revealed hyponatremia and hyper kalemia. Urinary 17-ketosteroid level was markedly elevated and 17-OHCS level was slightly decreased. She was diagnosed as a salt losing form of adrenogenital syndrome. We reported, here, this case with autopsy findings and literatures were briefly reviewed.
Key Words: Adrenogenital syndrome, Salt losing form


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