All issues > Volume 27(5); 1984

Case Report

J Korean Pediatr Soc. 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

¹Department of Pediatrics, Hanyang Universityt College of Medicine, Seoul, Korea

Abstract

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.

Keywords :Adrenogenital syndrome; Congenital adrenal hyperplasia