Zaparackaite Indre, Barauskas Vidmantas
Clinic of Paediatric Surgery, Kaunas University of Medicine, Eiveniu 2, 3007 Kaunas, Lithuania.
Medicina (Kaunas). 2003;39(2):105-13.
Congenital genital anomalies are a very complex pathology. In order to clarify its causes it is important to revert to the genetic conditions and regularities of embriological development. The genital disturbances are mostly determined by chromosomal or endocrinic disorders or by impaired biochemical processes. Clinical problems arise when the genetical sex is in discrepancy with ambiguous genitalia. True hermaphroditism, congenital adrenal hyperplasia, testicular feminization and gonadal dysgenesis are the most common syndromes. Diagnostic criteria applied are similar for all (establishment of karyotype, investigation of hormones and their derivates, genital ultrasound and endoscopy, if needed - radiological examination), but medical and surgical treatment is applied to each patient individually.
先天性生殖器异常是一种非常复杂的病理学现象。为了阐明其病因,追溯胚胎发育的遗传条件和规律很重要。生殖器紊乱大多由染色体或内分泌紊乱或生化过程受损所决定。当遗传性别与生殖器模糊不一致时就会出现临床问题。真两性畸形、先天性肾上腺皮质增生症、睾丸女性化和性腺发育不全是最常见的综合征。所有综合征适用的诊断标准相似(确定核型、检测激素及其衍生物、必要时进行生殖器超声和内窥镜检查以及放射学检查),但对每个患者的药物和手术治疗都是个体化的。