简化促性腺激素释放激素兴奋试验在女性同性性早熟症诊断中的应用

目的 探讨简化促性腺激素释放激素(GnRH)兴奋试验用于女性同性性早熟症发病原因的诊断及指导治疗的价值。方法 对42例女性同性性早熟患者施行简化GnRH兴奋试验,其中38例患者平均随访26(3～78)个月,观察患者的临床表现及病情发展情况。结果 42例患者对GnRH兴奋试验反应分为黄体生成激素(LH)优势型、卵泡刺激素(FSH)优势型及无反应型3类。其中LH优势型14例(33％,14/42),包括下丘脑错构瘤1例、特发性性早熟13例;14例中生长过速或骨成熟过早各10例。FSH优势型13例(31％,13/42),包括生长过速2例、骨成熟过早1例。无反应型15例(36％,15/42),8例为外周性性早熟症,包括卵巢颗粒泡膜瘤1例、自主性功能性卵巢滤泡囊肿2例、McCune-Albright综合征2例、外源性性早熟症3例;15例中生长过速4例、骨成熟过早5例。FSH优势型13例及无反应型的其余7例,未发现明确的发病原因,考虑为一过性性早熟症或乳房早发育。结论 简化GnRH兴奋试验有助于女性同性性早熟症发病原因的诊断,及客观判断下丘脑-垂体-卵巢轴是否被激活,较临床指标更为可靠。

Clinical application of simplified gonadotropin releasing hormone stimulating test in management of female isosexual precocious puberty

Objective To explore the clinical application of simplified gonadotropin releasing hormone (GnRH) test in the management of female isosexual precocious puberty(IPP). Methods Simplified GnRH stimulating test was performed in 42 girls with IPP. Thirty-eight of them were followed-up for a mean of 26(3-78)months. Results Fourteen cases showed luteinizing hormone(LH)-predominant response after GnRH stimulation, 13 showed follicle stimulating hormone (FSH)-predominant response and 15 showed no response. In the LH-predominant group, one had hypothalamic harmatoma, the other 13 were idiopathic type. Ten of them had acceleration of growth and bone prematurity. There were 8 peripheral IPP in the no response group, i.e. ovarian granulose-theca tumor 1, autonomous ovarian follicular cysts 2, McCune-Albright syndrome 2, and exogenous IPP 3. Among them, 1/2 had growth acceleration and 3/4 had bone prematurity. The remaining 7 of the no-response group and 13 with FSH-predominant response had no known etiology and were considered as transient IPP or premature thelarche based on no progression of pubertal development during follow-up. One fourth of them also showed acceleration of growth and bone prematurity. Conclusion Simplified GnRH test is helpful to objectively evaluate activation of the hypothalamic-pituitary-ovary axis, and is more reliable than those clinical features in differential etiological diagnosis of female IPP.