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

机译:促性腺激素释放激素类似物治疗先天性肾上腺增生并发中枢性性早熟的儿童

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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
机译:目的:儿童中枢性早熟(CPP)可并发先天性肾上腺皮质增生(CAH),这可能会损害最终身高。我们旨在评估促性腺激素释放激素类似物(GnRHa)治疗对CAH儿童生长的影响。设计:对十二名患有CAH的儿童进行了随访研究。 8例患者接受了GnRH刺激试验。每4周以3.75 mg的剂量给予GnRHa治疗;三名患者的剂量必须增加到7.5 mg。治疗期间监测患者的骨龄,生长速度和体重指数。结果:诊断时的中位年龄和骨龄分别为6.8(3.5)岁和11(1.2)岁。中位随访时间为4.4(4.9)年。在诊断和最后一次就诊之间(p = 0.005)以及开始GnRHa治疗和最后一次就诊之间(p = 0.004),发现了骨龄与时间年龄的中位数比率存在显着差异。在治疗的第一年,第二年和第三年结束时,中位生长速度分别为4(2.5)cm,3.4(5.2)cm和5.5(5.5)cm。在诊断时(rho:-0.758,p = 0.004)和开始治疗时(rho:-0.876,p),第二年生长速度与中位骨龄成反比。

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