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[以血尿和严重贫血为表现的遗传性出血性毛细血管扩张症]

[Hereditary hemorrhagic telangiectasia presenting with hematuria and severe anemia].

作者信息

Paz A, Goren E, Segal M

机构信息

Urology Dept., Assaf Harofeh Medical Center, Zrifin.

出版信息

Harefuah. 1995 Jul;129(1-2):24-5, 79, 78.

PMID:7557703
Abstract

A patient with hereditary hemorrhagic telangiectasia was admitted with hematuria and severe anemia after mild recurrent episodes of epistaxis. Telangiectasias were found in the skin and buccal and nasal mucosa. No defect in the coagulation mechanism was found; thrombocyte count and function were normal. On cystoscopy, tortuous engorged vessels, some actively bleeding, were seen in the trigonal mucosa. Biopsy showed enlarged vessels in the lamina propria. Electrocoagulation of the bleeding vessels stopped hematuria, but 6 months later it recurred. This time Nd-YAG laser was used to stop the bleeding after electrocoagulation was ineffective.

摘要

一名遗传性出血性毛细血管扩张症患者,在轻度反复鼻出血后出现血尿和严重贫血而入院。在皮肤、颊黏膜和鼻黏膜发现有毛细血管扩张。未发现凝血机制缺陷;血小板计数及功能正常。膀胱镜检查时,在三角区黏膜可见迂曲、充血的血管,有些正在出血。活检显示固有层血管扩张。对出血血管进行电凝止血后血尿停止,但6个月后复发。此次在电凝无效后使用Nd-YAG激光止血。

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