Sharma V K, Howden C W
Department of Internal Medicine, University of South Carolina School of Medicine, Columbia, USA.
Dig Dis. 1998 May-Jun;16(3):169-74. doi: 10.1159/000016861.
Hereditary hemorrhagic telangiectasia (HHT) or Osler-Rendu-Weber (ORW) disease is an autosomal-dominant disorder that frequently presents with gastrointestinal bleeding which may be a diagnostic and therapeutic challenge. Hepatic involvement in this disease is increasingly recognized and poses another therapeutic challenge. With advances in genetic screening and diagnostic procedures, and the increasing awareness of the condition by physicians and patients, this disease is being diagnosed more often. This article reviews the available literature on gastrointestinal and hepatic manifestations of HHT, and the various diagnostic and therapeutic modalities available for its management.
遗传性出血性毛细血管扩张症(HHT)或奥斯勒-伦杜-韦伯(ORW)病是一种常染色体显性疾病,常表现为胃肠道出血,这可能是一个诊断和治疗上的挑战。肝脏受累在这种疾病中越来越受到重视,也带来了另一个治疗挑战。随着基因筛查和诊断程序的进步,以及医生和患者对该疾病认识的提高,这种疾病的诊断越来越频繁。本文综述了关于HHT胃肠道和肝脏表现的现有文献,以及可用于其治疗的各种诊断和治疗方法。