Peiffer Kathleen M Z
Drexel University, Nurse Anesthesia Program, Philadelphia, PA, USA.
AANA J. 2009 Apr;77(2):115-8.
Hereditary hemorrhagic telangiectasia (HHT), Osler-Weber-Rendu Syndrome, is an uncommon disease but may be present in many people who remain undiagnosed. It is an autosomal dominant disorder characterized by multiple arteriovenous malformations (AVMs) and telangiectases that affect multiple organ systems. Hereditary hemorrhagic telangiectasia patients have a propensity for bleeding, especially from the oropharynx, nasopharynx and gastrointestinal tract, as well as from rupture of AVMs of other organ systems. Anesthetic care of patients with HHT involves very specific interventions with regard to control of bleeding, maintaining adequate oxygenation, and balancing hemodynamic values to optimize perfusion without compromising anesthetic depth.
遗传性出血性毛细血管扩张症(HHT),又称奥斯勒-韦伯-伦杜综合征,是一种罕见疾病,但可能存在于许多未被诊断的人群中。它是一种常染色体显性疾病,其特征是影响多个器官系统的多发性动静脉畸形(AVM)和毛细血管扩张。遗传性出血性毛细血管扩张症患者有出血倾向,尤其是来自口咽、鼻咽和胃肠道,以及其他器官系统的动静脉畸形破裂处。对HHT患者的麻醉护理涉及控制出血、维持充足氧合以及平衡血流动力学参数以优化灌注同时不影响麻醉深度等非常具体的干预措施。