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首页> 外文期刊>Journal of pediatric endocrinology & metabolism: JPEM >Precocious puberty: clinical and endocrine profile and factors indicating neurogenic precocity in Indian children.
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Precocious puberty: clinical and endocrine profile and factors indicating neurogenic precocity in Indian children.

机译：性早熟：临床和内分泌特征以及指示印度儿童神经源性早熟的因素。

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The objective of this study was to evaluate the clinical and endocrine profile of patients with precocious puberty followed up in a tertiary care hospital. Records of 140 patients (114 girls, 26 boys) with precocious puberty were reviewed. Clinical features including age of onset, stage of pubertal development, presenting symptoms, features suggestive of CNS involvement and family history were analyzed. Endocrine investigations included basal and GnRH-stimulated levels of LH and FSH as well as 17OHP, DHEA, hCG and thyroid profile. Abdominal and pelvic ultrasonography and CNS imaging were correlated with clinical features. Girls outnumbered boys in this series (4.4:1). Neurogenic central isosexual precocious puberty (CIPP) was more common in boys (10 out of 18, 55.6%) than girls (16 out of 77, 20.8%). The most common cause of neurogenic CIPP was hypothalamic hamartoma present in five girls and four boys. Other causes of neurogenic CIPP included neurotuberculosis, pituitary adenoma, hydrocephalus, post radiotherapy, CNS tumors and malformations. Peripheral precocious puberty (PPP) was secondary to adrenal causes in boys and ovarian cysts in girls. Benign variants of precocious puberty, such as premature thelarche and premature adrenarche, were present in 23 and six girls, respectively. Hypothyroidism was present in four girls and McCune-Albright syndrome in one girl. Girls with neurogenic CIPP had a lower age of onset as compared to idiopathic CIPP (3.6 +/- 2.7 years vs 5.4 +/- 2.5 years, p = 0.014). The lowest age of onset was seen in girls with hypothalamic hamartoma (1.6 +/- 0.9 years). Forty-seven girls with CIPP (seven neurogenic and 40 idiopathic) presented after the age of 6 years. Features of CNS involvement, in the form of seizures, mental retardation, raised intracranial tension or focal neurological deficits, were present in seven girls (43.8%) and four boys (40%), and gelastic seizures were present in three children. Girls with CIPP had greater bone age advancement (3.4 +/- 1.5 years) and negative height standard deviation for bone age (-2.7 +/- 1.5) than those with PPP (1.9 +/- 1.6 years and -1.3 +/- 1.3) and premature thelarche (0.4 +/- 0.4 years and -0.8 +/- 0.8). Patients with neurogenic CIPP had significantly higher levels of baseline and GnRH-stimulated levels of LH and FSH and LH:FSH ratio than those with idiopathic CIPP. Occurrence of neurogenic CIPP in seven girls with an age of onset after 6 years emphasizes the need for CNS imaging in these girls contrary to the current recommendations. The fact that 65.6% cases of idiopathic CIPP presented after the age of 6 years raises the possibility that these patients may be physiological variants of normal puberty. Pointers to neurogenic CIPP included early age of onset in girls, clinical features of CNS involvement, and elevated basal and stimulated LH levels and LH:FSH ratio.
机译：这项研究的目的是评估在三级保健医院随访的性早熟患者的临床和内分泌情况。回顾了140例性早熟患者的记录（114例女孩，26例男孩）。临床特征包括发病年龄，青春期发育阶段，出现的症状，提示中枢神经系统受累的特征和家族史。内分泌检查包括基础和GnRH刺激的LH和FSH水平以及17OHP，DHEA，hCG和甲状腺特征。腹部和盆腔超声检查以及中枢神经系统成像与临床特征相关。在这个系列中，女孩人数超过男孩（4.4：1）。神经源性中枢等性性早熟（CIPP）在男孩中更为普遍（18名中有10名，占55.6％）比女孩（77名中的16名，占20.8％）更常见。神经源性CIPP的最常见原因是下丘脑错构瘤，出现在五个女孩和四个男孩中。神经源性CIPP的其他原因包括神经结核，垂体腺瘤，脑积水，放疗后，中枢神经系统肿瘤和畸形。周围性早熟（PPP）是继发于男孩的肾上腺病因和女孩的卵巢囊肿。性早熟的良性变体，如早熟的lar虫和早熟的肾上腺，分别存在于23和6个女孩中。甲状腺功能减退症出现在四名女孩中，而McCune-Albright综合征出现在一名女孩中。与特发性CIPP相比，具有神经源性CIPP的女孩的发病年龄较低（3.6 +/- 2.7岁与5.4 +/- 2.5岁，p = 0.014）。下丘脑错构瘤女孩的发病年龄最低（1.6 +/- 0.9岁）。 6岁以后出现47例具有CIPP的女孩（七个神经源性和40个特发性）。中枢神经系统受累的特征有癫痫，智力低下，颅内张力升高或局灶性神经功能缺损，其中有七个女孩（43.8％）和四个男孩（40％），以及三个孩子出现了全弹性发作。与PPP的女孩（1.9 +/- 1.6岁和-1.3 +/- 1.3）相比，CIPP的女孩具有更大的骨龄进展（3.4 +/- 1.5岁）和负的身高标准偏差（-2.7 +/- 1.5）。 ）和早熟（0.4 +/- 0.4年和-0.8 +/- 0.8）。神经源性CIPP患者的基线水平和GnRH刺激的LH和FSH水平以及LH：FSH比水平明显高于特发性CIPP患者。发生神经性CIPP的7名发病年龄在6岁后的女孩中，强调了这些女孩中枢神经系统成像的需要，与目前的建议相反。 6岁以后出现65.6％的特发性CIPP病例的事实增加了这些患者可能是正常青春期的生理变异的可能性。指向神经性CIPP的指标包括女孩发病年龄早，中枢神经系统受累的临床特征以及基础和刺激的LH水平和LH：FSH比升高。

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