Gonadotropin releasing hormone analog treatment in children with congenital adrenal hyperplasia complicated by central precocious puberty

Abstract

OBJECTIVE: Congenital adrenal hyperplasia (CAH) can be complicated by central precocious puberty (CPP) in children, which may compromise final height. We aimed to evaluate the effect of gonadotropin-releasing hormone analog (GnRHa) therapy on growth in children with CAH. DESIGN: Twelve children with CAH were enrolled in a follow-up study. Eight patients underwent the GnRH stimulation test. GnRHa-treatment was administered at 3.75 mg every 4 weeks; the dose had to be increased to 7.5 mg in three patients. Bone age, growth velocities and body mass index of the patients were monitored during treatment. RESULTS: Median chronologic age and bone age at diagnosis were 6.8 (3.5) years and 11 (1.2) years, respectively. Median follow-up was 4.4 (4.9) years. A significant difference was found in the median ratio of bone age to chronological age between diagnosis and last visit (p=0.005) and between the beginning of GnRHa treatment and last visit (p=0.004). Median growth velocity was 4 (2.5) cm, 3.4 (5.2) cm and 5.5 (5.5) cm at the end of the first, second and third years of the therapy, respectively. Second-year growth velocity was inversely correlated with median bone age at diagnosis (rho:-0.758, p=0.004) and at the initiation of therapy (rho:-0.876, p<0.001). CONCLUSION: GnRHa therapy should be considered for augmentation of linear growth and diminishment of bone age advancement in children with CAH complicated by CPP, particularly in children who do not have extremely advanced bone age for chronological age.

Central precocious puberty complicating congenital adrenal hyperplasia: North Indian experience

“…However, the treatment outcomes appear to be different as compared to patients treated in the developed countries. [45] Although our patients showed slowing down of BA advancement, the HV did not decrease significantly after GnRHa therapy in contrast with data from developed countries. [45] Additionally, the improvement in PAH barely reached statistical significance after 3 years of therapy.…”

Congenital adrenal hyperplasia due to 21-hydroxylase deficiency in South Africa

“…The hydrocortisone suppresses the production of adrenal androgens and stimulates the release of gonadotrophins if hypothalamic maturation is already present. [20,21] All our patients with CPP are receiving treatment with a GnRH analogue. In SA, considerable limitations exist in optimally managing this condition.…”

Peripheral precocious puberty including congenital adrenal hyperplasia: causes, consequences, management and outcomes

“…Several small case series of children with CAH and CPP showed regression in breast development and testicular volume, decreased BA advancement and linear growth velocity and improved growth potential with GnRHa treatment (8,20). Treatment should be started early and appears to be more beneficial in those whose BA is not significantly advanced (21).…”