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Adrenogenital Syndrome with Congenital Adrenal Hyperplasia.

Journal of the Korean Pediatric Society 1984;27(5):511-515.
Published online May 31, 1984.
Adrenogenital Syndrome with Congenital Adrenal Hyperplasia.
Myoung Sung Moon, Kwang Nam Kim, Woo Gill Lee
Department of Pediatrics, Hanyang Universityt College of Medicine, Seoul, Korea
선천성 부신증식증에 의한 부신성기증후군 1례
문명성, 김광남, 이우길
한양대학교 의과대학 소아과학교실
When the adrenogenital syndrome is associated with congenital adrenal hyperplasia, it is caused by an inborn defect in the biosynthesis of adrenal corticoids. Especially, infant with congenital adrenal hyperplasia caused by a 21-hydroxylation. defect in steroidogenesis often present with salt-losing crisis, hyponatremia and dehydration. Vomiting is prominent and disturbances in cardiac rate and rhythm may occur. Without treatment, collapse and death usually occur within a few weeks. The authors experienced a case of adrenogenital syndrome in a 6 months old female infant with complaints of abnormality in external genitalia at birth, poor feeding and poor weight gain. So, we reported this case with literature review.
Key Words: Adrenogenital syndrome, Congenital adrenal hyperplasia

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