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Factors Affecting the Presence of Menarche and Final Adult Height in Adult Female Patients with Congenital Adrenal Hyperplasia of 21-Hydroxylase Deficiency

Journal of the Korean Pediatric Society 2001;44(1):75-82.
Published online January 15, 2001.
Factors Affecting the Presence of Menarche and Final Adult Height in Adult Female Patients with Congenital Adrenal Hyperplasia of 21-Hydroxylase Deficiency
Seong Yong Lee, Chae Kyoung Song, Min Ho Jung, Jin Soon Hwang, Sei Won Yang
Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea
21-Hydroxylase 결핍이 원인인 선천성 부신피질 과형성을 가진 성인 여자 환자에서 초경의 유무 및 최종 성인키에 영향을 미치는 인자에 대한 연구
이성용, 송채경, 정민호, 황진순, 양세원
서울대학교 의과대학 소아과학교실
Abstract
Purpose
: Amenorrhea and short stature are serious complications in patients with congenital adrenal hyperplasia of 21-hydroxylase deficiency(CAH). We analysed several factors influencing the presence of menarche and final adult height in these patients.
Methods
: We retrospectively reviewed medical records of 24 female patients with CAH. We analysed possible factors affecting the menarche and final adult height, such as clinical type, predicted adult height, age at treatment, age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP.
Results
: The occurrence rate of menarche were significantly higher in patients with the simple virilizing form rather than the salt losing form(P<0.05). No significant differences were found in age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP between menarche group and amenorrhea group. The age at treatment was younger in amenorrhea group(P<0.05). There were no significant differences in the proportion of patients who attained predicted adult height between two clinical types. No significant differences were found in age at treatment, age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP between the group of patients who attained predicted adult height and the group of patients who didn`t. Final adult height did not show any correlation with age at treatment, age at onset of puberty, BMI, and plasma levels of DHEA and 17-OHP but showed significant correlation with mid-parental height(r=0.426, P=0.01).
Conclusion
: Our data suggest that the presence of menarche in patients with CAH depends on the degree of prenatal exposure to adrenal androgen regardless of the degree of postnatal control and age at onset of puberty. Additionally, it seems likely that mid-parental height determines the final adult height.
Key Words: 21-Hydroxylase deficiency, Amenorrhea, Final adult height


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