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梨状腹综合征的发病机制:前列腺发育不全导致的功能性尿道梗阻。

Pathogenesis of the prune-belly syndrome: a functional urethral obstruction caused by prostatic hypoplasia.

作者信息

Moerman P, Fryns J P, Goddeeris P, Lauweryns J M

出版信息

Pediatrics. 1984 Apr;73(4):470-5.

PMID:6231520
Abstract

Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic experience with seven cases of prune-belly syndrome is reviewed. Findings indicate that the urogenital anomalies can be attributed to a functional urethral obstruction which in turn is the result of prostatic hypoplasia. The histology of the abdominal wall is that of atrophy-ie, the degeneration of already formed muscle--and not of primitive muscle. This observation supports the theory that the abdominal muscle hypoplasia is a nonspecific lesion, resulting from fetal abdominal distension of various causes. Transient fetal ascites may be an important feature of the prune-belly syndrome.

摘要

腹肌缺如、泌尿系统异常和隐睾是梅干腹综合征的三大主要特征,该综合征也被称为三联征综合征或伊格尔-巴雷特综合征。其病因尚不清楚,发病机制仍存在争议。本文回顾了7例梅干腹综合征的临床和病理经验。研究结果表明,泌尿生殖系统异常可归因于功能性尿道梗阻,而这又是前列腺发育不全的结果。腹壁的组织学表现为萎缩,即已形成的肌肉发生退化,而非原始肌肉的情况。这一观察结果支持了以下理论,即腹肌发育不全是一种非特异性病变,由各种原因导致的胎儿腹部膨胀引起。短暂性胎儿腹水可能是梅干腹综合征的一个重要特征。

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