All issues > Volume 12(4); 1969

Case Report

J Korean Pediatr Soc. 1969;12(4):205-212. Published online April 30, 1969.

Two Cases of Congenital Adrenogenital Syndrome

Yung Cho Kwon¹, Chong Koo Yun¹, Chang Soo Rha¹, Hyung Ro Moon¹, Byung Hee Lee²

¹Dept, of Pediatrics, College of Medicine, Seoul, National University
²Dept, of Pediatrics, Young Dung Po City Hospital, Seoul, Korea

Abstract

The authors report two cases of congenital adrenogenital syndome. The patient in case I was a girl born with ambiguious sex, and manifested virilization and accelerated growth. The height and bone age on admission at age of 3 years and 10 mounths was 103. 5 cm. and 6 years and 9 months, respectively. The vaginogram revealed normal internal genital organs. The blood pressure and electrolytes studies were normal. The 24 hours urinary 17-Ketosteroids was 8. 72 mg. prior to treatment. She was discharged on cortisone acetate 25 mg. daily after cliteridectomy. She was missed during the second years of follow-up. When she was again seen at the outpatient department two years after the discharge, physical examination revealed acne and pubic hair. Her bone age and urinary 17-Ketos-teroids was 13 years and 14. 4 mg/24 hrs, respectively. The patient in case II, a 50 day-old male infant was admitted for the second time to the hospital in salt-losing crisis. His phalus was unusually large and his nipples as well as phalus showed increased pigmentation. He responded poorly to fluid therapy and showed signs of hemorrhagic renal infarction during the first hospitalization. On each admission, the urinary 17-Ketosteroids determination revealed 2. 7 mg/24 hrs and 4. 7 mg/24 hrs. He expired at home 3 days after the second discharge. Both patients were considered to have 21-hydroxylation defect, the latter “salt-loser” and former “non salt-loser” from the clinical evidence.

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