| Adrenaogenital Syndrome with Congenital Adrenal Hyperplasia. |
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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)
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문경래1, 박영봉1, 김상기1, 김진헌1, 나창수1, 서재홍2, 황호원2 |
1조선대학교 의과대학 소아과학교실
2조선대학교 의과대학 병리학교실 |
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| Abstract |
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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.
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| Key Words:
Adrenogenital syndrome, Salt losing form |
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