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Congenital adrenal Hyperplasia.

Journal of the Korean Pediatric Society 1986;29(6):604-612.
Published online June 30, 1986.
Congenital adrenal Hyperplasia.
Byoung Hai Ahn, Sei Won Yang, Hyung Ro Moon
Department of Pediatrics, College of Medicine, Seoul National University
선천성 부신피질과형성
안병해, 양세원, 문형로
서울대학교 의과대학 소아과학교실
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.
Key Words: Congenital adrenal hyperplasia


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