All issues > Volume 29(6); 1986

Original Article

J Korean Pediatr Soc. 1986;29(6):604-612. Published online June 30, 1986.

Congenital adrenal Hyperplasia.

Byoung Hai Ahn¹, Sei Won Yang¹, Hyung Ro Moon¹

¹Department of Pediatrics, College of Medicine, Seoul National University

Abstract

Clinical data of twenty-eight children with congenital adrenal hyperplasia who were admitted to the Seoul National University Hospital from June 1979 to July 1984 were analyzed retrospectively. Male to female ratio was 1 : 1.3. The average age at diagnosis was 3.2 years, 1.3 months for eleven salt-losers and all were 3 months or younger, 4.6 years for seventeen nonsaltlosers. Chief complaints at initial presentation in salt-losers included vomiting/diarrhea, coma, tachypnea/dyspnea, ambiguous genitalia, skin pigmentation and failure to thrive in decreasing order. On the other hand, chief complaints in nonsalt-losers were ambiguous genitalia in females and somatic or sexual precocity in males. Among nonsalt-losers twelve children were above the 90th percentile iii height on admission. Bone ages were advanced in all children beyond infancy. Among salt-losers eight children were below the 25th percentile in height on admission. Urinary 17-ketosteroid levels were raised in all cases except one, and mean values were 3.5mg/24hrs in patients under 2 weeks of age, 3.8 mg/24 hrs in patients from 2 weeks〜 1 year, 3.5 mg/24 hrs in patients from 1 〜5 years, and 18.8 mg/24 hrs after 5 years of age. Mean, serum sodium and potassium concentrations in salt-losers were 114.9 mEq/L and 7.0 mEq/L, respectively. Steroid doses were guided by urinary 17-ketosteroid, height velocity, and bone age. There were two salt-losers in whom 9a-fluorohydrocortisone was discontinued after one and two years of therapy, respectively. Clitoroplasty or clitoridectomy was carried out in ten of fourteen nonsalt-losing female children at ages varying from 2 to 15 years. Vaginoplasty was performed simultaneously in seven patients. The mean duration of follow-up was 1 year and 11 months in ten of seventeen nonsalt-losers and the average annual height increment was 4.2 cm/year. The mean duration of follow-up was 1 year and 9 months in eight of eleven salt-losers and the average annual height increment was 19.5 cm/year during infancy and 11.8 cm/year beyond infancy. Menarche occurred at 12.5 years and 13.5 years in two nonsalt-losers in whom steroid therapy was started after 10 years of age.

Keywords :Congenital adrenal hyperplasia