[11beta-hydroxylase deficiency: improvement of final height with growth hormone and gonadotropin-releasing hormone analog].
Steroid 11beta-hydroxylase deficiency is the second most common cause of congenital adrenal hyperplasia. 11beta-hydroxylase intervenes in cortisol synthesis and its deficiency leads to accumulation of adrenal androgens--producing prenatal virilization and, subsequently, hyperandrogenism--as well as 11-deoxycorticosterone, leading to the development of hypertension. We describe a 7-year-old girl who was referred for pubarche and accelerated skeletal maturation due to 11beta-hydroxylase deficiency. Because the patient's predicted height was below her target height, the combination of gonadotropin-releasing hormone analog and growth hormone was added to oral glucocorticoid therapy. With this therapeutic strategy, the patient's predicted height improved significantly and the girl reached a final height in agreement with her target height at the age of 13 years and 6 months.
Orío Hernández
M
M
Servicio de Endocrinología Pediátrica, Hospital Universitario Infantil La Paz, Madrid, España. yeyaorio@yahoo.es
Yebra Yebra
J
J
Bezanilla López
C
C
Gracia Bouthelier
R
R
D000284:Administration, Oral; D000312:Adrenal Hyperplasia, Congenital / Q000175:diagnosis / Q000188:drug therapy*; D000367:Age Factors; D001827:Body Height; D002648:Child; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D007987:Gonadotropin-Releasing Hormone / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000627:therapeutic use*; D013006:Growth Hormone / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D011379:Prognosis; D013252:Steroid 11-beta-Hydroxylase / Q000378:metabolism; D013997:Time Factors; D016896:Treatment Outcome
D005938:Glucocorticoids; D007987:Gonadotropin-Releasing Hormone; D013006:Growth Hormone; D013252:Steroid 11-beta-Hydroxylase