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The Correlation between the Bone Age and Predicted Adult height, Final Adult Height in the Children with Congenital Adrenal Hyperplasia, Especially 21-Hydroxylase Deficiency

Journal of the Korean Pediatric Society 1994;37(3):300-308.
Published online March 15, 1994.
The Correlation between the Bone Age and Predicted Adult height, Final Adult Height in the Children with Congenital Adrenal Hyperplasia, Especially 21-Hydroxylase Deficiency
Sei Won Yang, Hyung Ro Moon
Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea
선천성 부신피질 과형성을 가진 소아에서 성장의 예후에 관한 연구 -21-hydroxylase 결핍을 중심으로-
양세원, 문형로
서울대학교 의과대학 소아과학교실
Abstract
We studied the correlation between the bone age and the predicted adult height, final adult height in the 69 children (30 salt losing form and 39 non-salt losing form) diagnosed as 21-hydroxylase deficiency, retrospectively. The results were as belows; 1) The bone age was similar to the chronological age in the children with salt-losing form (5.3±3.5 years, respectively), but the bone age was much more advanced, compared to the chronological age in the children with non-salt losing from (7.8±2.9 and 12.0±3.5 years, respectively) (Table 1. P<0.001) 2) In the children with salt-losing form, the height SDS for the chronological age and bone age, were -0.3±1.3 and -1.1±2.6, respectively, which has no difference. In the children with non-salt losing form, the height SDS for the bone age, were much lower than the height SDS for the chronological age (-2.1±1.3 and 1.5±1.5, respectively) (Table 2. P<0.001). 3) The incidence of true precocious puberty is significantly higher in the children with non-salt losing from (26 children) than in the children with salt losing from (6 children) (p<0.05). 4) All children with salt losing form received hydrocortisone and mineralocorticoid within the first month of life, but the average age at the first hydrocortisone therapy in the children with non-salt losing form was 5.0±3.3(0.1∼13.1) years of age. The dosages of hydrocortisone were similar in two forms (24.3±7.6 mg/m2 in salt losing form and 24.9±6.4 mg/m2 in non-salt losing form). 5) The predicted adult height, obtained by BP method, was shortest among three methods predicting adult height (RWT; 181.5±14.6 cm, TW; 169.2±13.2 cm, BP; 151.6±9.3 cm) (Fig. 1. P<0.001). 7) Seventeen children with 21-hydroxylase deficiency attained 152.1±8.8cm of final adult height (152.2±1.5 cm in 12 salt losing form and 152±1.4 cm in 5 non-salt losing form). These final adult heights were closest to the predicted adult height, obtained by BP method, compared to other two methods (Fig. 2. P<0.05). 8) Among 7 children, whose mid-parental height could be obtained, one child could reach within the target height and other 6 children reached far below the target height (Fig. 3. P<0.005). In conclusion, to attain normal growth and normal final adult height, it is suggested that meticulous control should be needed for adequate suppression of adrenal androgen and mineralocorticoid should be given to all children who have high level of plasma renin activity in the children with 21-hydroxylase deficiency, with regular follow-up of laboratory tests and growth parameter. Additionally, even if patient has non-salt losing form, the diagnosis should be made and adequate hormonal therapy should be given as soon as after birth.
Key Words: 21-hydroxylase, Predicted Adult Height, Final Adult Height


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