Desai M, Colaco M P, Choksi C S, Ambadkar M C, Vaz F E, Gupte C
Division of Pediatric Endocrinology, Bai Jerbai Wadia Hospital for Children, Bombay.
Indian Pediatr. 1993 May;30(5):607-23.
Eighty children (58 girls and 22 boys) with isosexual precocity seen in the past eight years were evaluated clinically and investigated to identify the underlying cause. Of these, 50% (29 girls and 11 boys) had centrally mediated true precocious puberty (TPP). The girls could be classified into five major groups (I) Central precocious puberty 29-subclassified into idiopathic (ITPP, 15) and organic or neurogenic (NTTP, 14), (II) Premature thelarche (PT, 20), (III) Premature menarche (PM, 2), (IV) Premature adrenarche (PA, 5), and, (V) Others: hypothyroid (n = 1), and McCune Albright Syndrome (n = 1). ITPP as a cause of precocity in girls was seen less often (52%) and NTPP more often (48%) compared to most Western series, with tubercular meningitis as the cause in 31% and hypothalamic hamartomas in 10%. Though the LH and estradiol levels were significantly higher (p < 0.05) in TPP, compared to PT, these were not helpful in differentiating because of considerable overlap. LH-predominant-response (LH/FSH ratio > 1) to LHRH testing was seen in TPP. Amongst the 22 boys, 11 (50%) had TPP, ITPP in 27% and NTPP in 73%. Hamartomas (n = 4) and TBM (n = 3) contributed equally to NTPP; pineal tumor was seen in one. The adrenal (n = 7) and testicular (n = 2) causes together involved 41% of the boys with precocity, congenital adrenal hyperplasia (CAH) CAH, 11-beta hydroxylase being the commonest cause. Of the 6 boys witdeficiency was found in four and nonsalt losing form of 21-hydroxylase deficiency in 2. Testicular and adrenal tumors and testotoxicosis were noted in one case each. The etiologic factors were more varied in boys.
对过去八年中诊治的80例同性性早熟儿童（58例女孩和22例男孩）进行了临床评估并调查以确定潜在病因。其中，50%（29例女孩和11例男孩）患有中枢性真性性早熟（TPP）。女孩可分为五大组：（I）中枢性性早熟29例，再分为特发性（ITPP，15例）和器质性或神经源性（NTTP，14例）；（II）乳房早发育（PT，20例）；（III）初潮过早（PM，2例）；（IV）肾上腺功能早现（PA，5例）；（V）其他：甲状腺功能减退（n = 1）和McCune-Albright综合征（n = 1）。与大多数西方系列报道相比，ITPP作为女孩性早熟的病因较少见（52%），NTPP较常见（48%），其中结核性脑膜炎占31%，下丘脑错构瘤占10%。虽然TPP患者的促黄体生成素（LH）和雌二醇水平显著高于PT患者（p < 0.05），但由于有相当大的重叠，这些指标无助于鉴别诊断。TPP患者对促性腺激素释放激素（LHRH）试验表现为以LH为主的反应（LH/促卵泡生成素（FSH）比值>1）。在22例男孩中，11例（50%）患有TPP，其中ITPP占27%，NTPP占73%。错构瘤（n = 4）和结核性脑膜炎（n = 3）在NTPP病因中所占比例相同；1例患有松果体瘤。肾上腺（n = 7）和睾丸（n = 2）病因共占男孩性早熟病例的41%，先天性肾上腺皮质增生症（CAH），11-β羟化酶缺乏症是最常见的病因。在6例患有CAH的男孩中，4例为失盐型，2例为非失盐型21-羟化酶缺乏症。睾丸和肾上腺肿瘤以及睾丸中毒症各有1例报道。男孩的病因更为多样。
