外周性性早熟患儿的病因及预后随访

Etiology and prognosis of peripheral precocious puberty in children

摘要:

目的：探讨外周性性早熟的病因及预后。方法：应用简化的促性腺激素释放激素（GnRH）激发试验测定卵泡刺激素（FSH）及黄体生成素（LH）、雌二醇（E2）水平，并采用B超检查及骨龄检测等方法对125例外周性性早熟患儿进行病因诊断。随访病例102例，随访时间3个月至7.5年。结果：125例患儿的病因分布为：摄入外源性性激素80例，卵巢囊肿11例，McCuneAlbright 综合征11例，先天性肾上腺皮质增生症（CAH）5例，卵巢畸胎瘤、男性化肾上腺肿瘤、女性化肾上腺肿瘤、垂体柄肿瘤各1例，另有14例患儿病因尚不能确定。预后：摄入外源性性激素者随访72例，均在1～6个月性征消退；11例卵巢囊肿患儿中，8例1~4个月性征自行消退，但其中1例2年3个月后转为中枢性性早熟；1例囊肿切除术后性征消退；卵巢畸胎瘤者术后性征消退；McCuneAlbright 综合征及CAH者治疗后临床症状减轻，7例转为中枢性性早熟；2例肾上腺肿瘤术后性征消退；1例垂体柄肿瘤术后1年死亡。结论：外周性性早熟病因多样，详细的病史、体检、辅助检查有助于早期明确诊断，不同病因预后不同。

Abstract:

OBJECTIVE: To study the causes and prognosis of peripheral precocious puberty. METHODS: The levels of follicle stimulating hormone (FSH), luteinizing hormone (LH) and estradiol (E2) were detected by a simplified gonadotrophin-releasing hormone (GnRH) stimulation test. The etiologies of 125 children with peripheral precocious puberty were explored by ultrasound scans and bone age assessment. A total of 102 cases were followed up for 3 months to 7.5 years. RESULTS: The etiological distribution of these children was as follows: exogenous hormones intake (n=80), ovarian cyst (n=11), McCune-Albright syndrome (n=11), congenital adrenal hyperplasia (CAH) (n=5), ovarian teratoma (n=1), masculine adrenal tumor (n=1), feminine adrenal tumor (n=1), and handle pituitary tumor (n=1). The causes in 14 cases were unknown. Follow-up showed that the sexual characteristics of 72 cases due to exogenous hormones intake subsided after 1-6 months. Of 11 cases with ovarian cysts, the sexual characteristics subsided spontaneously in 8 cases after 1 to 4 months, but one case was transformed to central precocious puberty after 2 years and three months. One child with ovarian cysts underwent an operation and than the sexual characteristics subsided. The sexual characteristics of the patient who had an ovarian teratoma subsided after operation. The clinical symptoms of children with McCune-Albright syndrome or CAH were alliaviated partly after treatment, and 7 cases were transformed to central precocious puberty. The clinical symptoms of 2 cases of adrenal tumors subsided after operation. One child with handle pituitary tumor died one year after operation. CONCLUSIONS: Varied causes may contribute to peripheral precocious puberty and therefore must be carefully identified through history taking, physical examination, and auxiliary examinations. The prognosis may differ for patients with different etiologies of peripheral precocious puberty.