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[尿道下裂:见解与挑战]

[Hypospadias : Insights and challenges].

作者信息

Rübben I, Stein R

机构信息

Klinik und Poliklinik für Urologie, Kinderurologie und Uroonkologie, Universitätsklinikum Essen, Hufelandstr. 55, 45122, Essen, Deutschland.

Zentrum für Kinder- und Jugendurologie des Klinikums Mannheim GmbH, Universitätsklinikum, Medizinische Fakultät Mannheim, Universität Heidelberg, Heidelberg, Deutschland.

出版信息

Urologe A. 2017 Oct;56(10):1256-1265. doi: 10.1007/s00120-017-0498-x.

Abstract

Disorders of the ventral tubularization of the urethra, such as the hypospadias, are among the second most frequent congenital childhood malformations. An increasing incidence has been observed suggesting a doubling in the US, which could not be documented for the European area. The underlying causes of this congenital defect remain unidentified. Genetic risk constellations or environmental influences, in particular by so-called endocrine disrupting chemicals (EDCs), are discussed as triggering factors. Boys after in vitro fertilization are more likely to have hypospadias than in nonreproductive-assisted pregnancies. Animal models (especially mice) elicited causal relationships between prenatal hormonal exposure (estrogens, progesterone) and antiandrogens such as flutamide, finasteride, antiandrogenic fungicides (vinclozolin) and phthalates and the formation of hypospadias. An aesthetic and/or functional deficit are indications for surgical correction. The indications and the complications of hypospadias surgery must be in detail and realistically discussed with the parents and patients. Recent publications demonstrated that the risk of complications increases with the increase of the follow-up time. High-volume centers with extensive experience have a positive effect on the complication rate. Competent follow-up to adult age should be ensured.

摘要

尿道腹侧管状化障碍,如尿道下裂,是儿童期第二常见的先天性畸形。据观察,其发病率呈上升趋势,在美国发病率可能翻倍,但欧洲地区尚无相关记录。这种先天性缺陷的根本原因仍不明晰。遗传风险组合或环境影响,特别是所谓的内分泌干扰化学物质(EDCs),被认为是触发因素。与非辅助生殖妊娠相比,体外受精后的男孩患尿道下裂的可能性更高。动物模型(尤其是小鼠)揭示了产前激素暴露(雌激素、孕酮)以及抗雄激素药物如氟他胺、非那雄胺、抗雄激素性杀菌剂(乙烯菌核利)和邻苯二甲酸盐与尿道下裂形成之间的因果关系。美观和/或功能缺陷是手术矫正的指征。必须与家长和患者详细且实际地讨论尿道下裂手术的指征和并发症。最近的出版物表明,并发症风险会随着随访时间的增加而增加。经验丰富的大型医疗中心对降低并发症发生率有积极作用。应确保对成年患者进行充分的随访。

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