All issues > Volume 46(4); 2003

Case Report

J Korean Pediatr Soc. 2003;46(4):400-403. Published online April 15, 2003.

A Case of True Precocious Puberty Complicating Congenital Adrenal Hyperplasia

Su Jin SJ Kim¹, Ju Suk JS Lee², Su Yung SY 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

Correspondence Su Yung SY 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.

Keywords :True precocious puberty, Congenital adrenal hyperplasia