Etiology and clinical features of isosexual precocious puberty in Taiwanese girls: twenty-three years' experience in National Taiwan University Hospital.
The aim of this study was to elucidate the etiology and clinical features of Taiwanese girls with isosexual precocious puberty. 460 girls with precocious puberty were enrolled in this study. 284 of them had a GnRH test and 179 girls with gonadotropin-dependent precocious puberty (GDPP) underwent brain MRI. Our results showed 7% of these 460 girls had gonadotropin-independent precocious puberty (GIPP). The other 93% patients had GDPP and 96% of them were idiopathic. Functional ovarian cyst and hypothalamic hamartoma were leading causes of GIPP and organic GDPP, respectively. The presence of undetectable basal serum FSH and LH levels had a specificity of 95% in girls with GIPP. Among girls with GDPP, those aged < 5 years had a higher percentage of detected CNS lesions than older girls (8% vs 3%). Girls with organic CNS lesions also had more advanced bone age SDS (6.9 +/- 3.3 vs 3.3 +/- 1.9), larger uterine volume (13.8 +/- 12.1 vs 6.6 +/- 8.1 cm3), higher basal serum estradiol (33.5 +/- 17.1 vs 26.8 +/- 18.2 pg/ml), and higher peak LH level after GnRH stimulation (45.2 +/- 36.2 vs 25.8 +/- 27.3 U/l) than those with idiopathic GDPP. In conclusion, GIPP is uncommon in Taiwanese girls with precocious puberty. Functional ovarian cyst and hypothalamic hamartoma are leading causes of GIPP and organic GDPP, respectively. The presence of neurological deficit, younger age at onset of puberty, presence of menstruation, rapid advance of bone age, markedly enlarged uterus, high serum estradiol, and high peak LH level after GnRH stimulation are suggestive of organic GDPP.
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机译：这项研究的目的是阐明台湾同性性早熟女孩的病因和临床特征。这项研究纳入了460名性早熟的女孩。他们中的284位接受了GnRH测试，并有179位依赖促性腺激素依赖性性早熟（GDPP）的女孩接受了脑部MRI检查。我们的研究结果表明，在这460名女孩中，有7％患有非促性腺激素依赖性性早熟（GIPP）。其他93％的患者患有GDPP，其中96％为特发性患者。功能性卵巢囊肿和下丘脑错构瘤分别是GIPP和器质性GDPP的主要原因。 GIPP女孩中存在无法检测到的基础血清FSH和LH水平的特异性为95％。在患有GDPP的女孩中，年龄小于5岁的女孩中枢神经系统病变的检出率要高于年龄较大的女孩（8％对3％）。患有中枢神经系统病变的女孩的骨龄SDS也更高（6.9 +/- 3.3 vs 3.3 +/- 1.9），子宫体积更大（13.8 +/- 12.1 vs 6.6 +/- 8.1 cm3），基础血清雌二醇更高（33.5 +/- 17.1 vs. 26.8 +/- 18.2 pg / ml），并且GnRH刺激后的LH峰值水平较高（45.2 +/- 36.2 vs 25.8 +/- 27.3 U / l）。总之，GIPP在性早熟的台湾女孩中并不常见。功能性卵巢囊肿和下丘脑错构瘤分别是GIPP和器质性GDPP的主要原因。神经功能缺损的存在，青春期发病的年龄较小，月经的存在，骨龄的快速发展，子宫的明显增大，血清雌二醇的高水平以及GnRH刺激后LH峰值的高均提示了有机GDPP。
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