Journal of the Korean Pediatric Society 2003;46(4):400-403.
Published online April 15, 2003.
A Case of True Precocious Puberty Complicating Congenital Adrenal Hyperplasia
Su Jin Kim1, Ju Suk Lee2, Su Yung Kim1
1Department of Pediatrics, College of Medicine, Pusan National University, Pusan, Korea
2Department of Pediatrics, College of Medicine, Sungkyunkwan University, Samsung Medical Center, Masan, Korea
진성 성조숙증으로 전환된 선천성 부신 과형성증 1례
김수진1, 이주석2, 김수영1
1부산대학교 의과대학 소아과학교실
2성균관대학교 의과대학 마산삼성병원 소아과
Correspondence: 
Su Yung Kim, Email: suyung@pusan.ac.kr
Abstract
Congenital adrenal hyperplasia(CAH) is a recognized cause of precocious pseudopuberty. Some children with CAH also develop true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis. We review a case of CAH who eventually developed central precocious puberty nine months after initial treatment with corticosteroid. A 3-year-old boy visited complaining of rapid growth, a large penis and frequent penile erections. This patient was diagnosed with CAH with elevated 17-OH progesterone and cortical hypertrophy of adrenal gland on CT scan. His gonadotropin levels were within the normal prepubertal range. Even on treatment with corticosteroid he grew rapidly and had testicular enlargement, pubic hair development and rapid bone maturation. At second admission, his gonadotropin levels were elevated both basally and in response to LHRH stimulation, suggesting that the CAH led to early activation of pubertal gonadotropin secretion(true precocious puberty). He was treated with monthly depot injections of a LHRH analog in addition to the hydrocortisone. His second sexual characteristics regressed gradually and rate of linear growth and bone maturation decreased.
Key Words: True precocious puberty, Congenital adrenal hyperplasia


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