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促性腺激素释放激素类似物治疗先天性肾上腺皮质增生症合并中枢性性早熟儿童。

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

作者信息

Güven Ayla, Nurcan Cebeci Ayşe, Hancili Suna

机构信息

Göztepe Education and Research Hospital, Pediatric Endocrinology Clinic, İstanbul, Türkiye.

出版信息

Hormones (Athens). 2015 Apr-Jun;14(2):265-71. doi: 10.14310/horm.2002.1555.

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.

摘要

目的

先天性肾上腺皮质增生症(CAH)患儿可能并发中枢性性早熟(CPP),这可能会影响最终身高。我们旨在评估促性腺激素释放激素类似物(GnRHa)治疗对CAH患儿生长的影响。

设计

12例CAH患儿纳入随访研究。8例患者接受了GnRH刺激试验。GnRHa治疗每4周给药3.75mg;3例患者剂量增至7.5mg。治疗期间监测患者的骨龄、生长速度和体重指数。

结果

诊断时的中位实足年龄和骨龄分别为6.8(3.5)岁和11(1.2)岁。中位随访时间为4.4(4.9)年。诊断时与末次随访之间(p=0.005)以及GnRHa治疗开始时与末次随访之间(p=0.004),骨龄与实足年龄的中位比值存在显著差异。治疗第1年、第2年和第3年末的中位生长速度分别为4(2.5)cm、3.4(5.2)cm和5.5(5.5)cm。第2年生长速度与诊断时的中位骨龄(rho:-0.758,p=0.004)和治疗开始时的中位骨龄(rho:-0.876,p<0.001)呈负相关。

结论

对于并发CPP的CAH患儿,尤其是骨龄与实足年龄没有极度超前的患儿,应考虑使用GnRHa治疗以增加线性生长并减少骨龄进展。

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