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遗传性出血性毛细血管扩张症(奥斯勒-韦伯-伦杜病)的腹腔镜检查结果

Laparoscopic findings in hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease).

作者信息

Solis-Herruzo J A, Garcia-Cabezudo J, Santalla-Peciña F, Duran-Aguado A, Olmedo-Camacho J

出版信息

Endoscopy. 1984 Jul;16(4):137-9. doi: 10.1055/s-2007-1018558.

Abstract

The laparoscopic findings observed in eight patients with hereditary haemorrhagic telangiectasia are reported. Clinical signs or laboratory data suggestive of liver involvement were present in all cases and constituted the main indication for laparoscopic examination. Characteristic vascular lesions distributed at random were found in four cases. Numerous oval, round or polygonal areas of 0.5 to 1.5 cm in diameter were observed on the external surface of the liver. They were of a reddish or pink colour, flat or slightly raised, and made up of a dense vascular meshwork. In one patient macronodular cirrhosis was associated with the vascular lesions. In the remaining four cases laparoscopic examination did not show striking vascular changes, although the liver biopsy revealed a fatty liver in three cases and micronodular cirrhosis with intense inflammatory activity, steatosis and Mallory bodies in one case. In these cases the accompanying hepatic lesions could probably not be aetiopathogenically related to Osler's disease.

摘要

报告了8例遗传性出血性毛细血管扩张症患者的腹腔镜检查结果。所有病例均有提示肝脏受累的临床体征或实验室数据,这构成了腹腔镜检查的主要指征。4例发现随机分布的特征性血管病变。在肝脏外表面观察到许多直径为0.5至1.5厘米的椭圆形、圆形或多边形区域。它们呈红色或粉红色,扁平或略有隆起,由密集的血管网组成。1例患者的大结节性肝硬化与血管病变相关。其余4例腹腔镜检查未显示明显的血管变化,尽管肝活检显示3例为脂肪肝,1例为伴有强烈炎症活动、脂肪变性和马洛里小体的小结节性肝硬化。在这些病例中,伴随的肝脏病变可能与奥斯勒病在病因学上无关。

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