TY - JOUR
T1 - True precocious puberty complicating congenital adrenal hyperplasia
T2 - Treatment with a luteinizing hormone-releasing hormone analog
AU - Pescovitz, Ora Hirsch
AU - Comite, Florence
AU - Cassorla, Fernando
AU - Dwyer, Andrew J.
AU - Poth, Merrily A.
AU - Sperling, Mark A.
AU - Hench, Karen
AU - McNemar, Ann
AU - Skerda, Marilyn
AU - Loriaux, D. Lynn
AU - Cutler, Gordon B.
PY - 1984/5
Y1 - 1984/5
N2 - 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 have seen four such children (three boys and one girl) who had the diagnosis of CAH made between the ages of 3 and 6 yr. These patients were treated with standard doses of hydrocortisone and fludrocortisone. A diagnosis of true precocious puberty was made because of testicular enlargement in the boys, breast development in the girl, progressive pubic hair development, rapid growth, and rapid bone age maturation. Plasma steroid levels were elevated for age, and gonadotropin levels were within the normal pubertal range, both basally and in response to LHRH stimulation. We treated these children with daily sc injections of a LHRH analog (LHRHα) for 6-18 months in addition to the standard hydrocortisone and fludrocortisone therapy for CAH. LHRHα significantly decreased basal plasma LH and FSH, peak LH and FSH responses to native LHRH, and testosterone levels. Testis size decreased in the males, and breast development regressed in the female. LHRHα therapy led to significant decreases in linear growth rate, ulnar growth rate, and rate of bone age advancement. These results suggest that LHRHα is an effective adjunct to hydrocortisone and fludrocortisone in the treatment of true precocious puberty complicating CAH.
AB - 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 have seen four such children (three boys and one girl) who had the diagnosis of CAH made between the ages of 3 and 6 yr. These patients were treated with standard doses of hydrocortisone and fludrocortisone. A diagnosis of true precocious puberty was made because of testicular enlargement in the boys, breast development in the girl, progressive pubic hair development, rapid growth, and rapid bone age maturation. Plasma steroid levels were elevated for age, and gonadotropin levels were within the normal pubertal range, both basally and in response to LHRH stimulation. We treated these children with daily sc injections of a LHRH analog (LHRHα) for 6-18 months in addition to the standard hydrocortisone and fludrocortisone therapy for CAH. LHRHα significantly decreased basal plasma LH and FSH, peak LH and FSH responses to native LHRH, and testosterone levels. Testis size decreased in the males, and breast development regressed in the female. LHRHα therapy led to significant decreases in linear growth rate, ulnar growth rate, and rate of bone age advancement. These results suggest that LHRHα is an effective adjunct to hydrocortisone and fludrocortisone in the treatment of true precocious puberty complicating CAH.
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U2 - 10.1210/jcem-58-5-857
DO - 10.1210/jcem-58-5-857
M3 - Article
C2 - 6368580
AN - SCOPUS:0021270578
SN - 0021-972X
VL - 58
SP - 857
EP - 861
JO - Journal of Clinical Endocrinology and Metabolism
JF - Journal of Clinical Endocrinology and Metabolism
IS - 5
ER -