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[多脾综合征(作者译)]

[The syndrome of polysplenia (author's transl)].

作者信息

Zach M, Beitzke A, Gypser G, Sager W

出版信息

Rofo. 1977 May;126(5):454-60. doi: 10.1055/s-0029-1230615.

Abstract

A strong tendency to symmetrical development of the organ systems manifests itself in the syndrome of Polysplenia. These symmetrical organs show the anatomy of leftsided structures. For this reason the syndrome is also referred to as the "syndrome of bilateral leftsidedness". It encompasses complex abnormalities of situs, typical cardiac and vascular deformities as well as extracardiac malformations. Polysplenia is the leading symptom. Diagnosis is rarely made intra vitam. Certain typical deformities can give hints to this diagnosis, which is finally proved by szintigraphic and arteriographic visualization of multiple spleens. A typical case is reported and subsequently discussed according to the picture of this syndrome in the literature.

摘要

多脾综合征表现出器官系统对称发育的强烈倾向。这些对称器官呈现左侧结构的解剖特征。因此,该综合征也被称为“双侧左侧化综合征”。它包括复杂的内脏位置异常、典型的心脏和血管畸形以及心外畸形。多脾是主要症状。生前很少能做出诊断。某些典型畸形可为此诊断提供线索,最终通过多个脾脏的闪烁扫描和动脉造影显像得以证实。本文报告了一个典型病例,并根据文献中该综合征的表现进行了讨论。

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