Bhattad Venugopal Brijmohan, Bowman Jennifer N, Panchal Hemang B, Paul Timir K
East Tennessee State University, Johnson City, TN, USA.
J Investig Med High Impact Case Rep. 2017 Feb 1;5(1):2324709617692833. doi: 10.1177/2324709617692833. eCollection 2017 Jan-Mar.
Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare genetic blood disorder that leads to abnormal bleeding due to absent capillaries and multiple abnormal blood vessels known as arteriovenous malformations. A feature of HHT is high-output heart failure due to multiple arteriovenous malformations. High-output heart failure can lead to recurrent epistaxis Kiesselbach area syndrome (REKAS), further exacerbating heart failure through increased blood loss and resultant anemia. We report a patient with HHT who presented with high-output heart failure contributing to REKAS. In patients with REKAS, we propose if anemia is present, REKAS can be avoided by correcting the anemia by increasing the hemoglobin level to greater than 9 to 10 g/dL. This decreases hyperdynamic circulation and reduces pressure in the blood vessels of the nose.
遗传性出血性毛细血管扩张症（HHT），也称为奥斯勒-韦伯-伦杜综合征，是一种罕见的遗传性血液疾病，由于缺乏毛细血管以及存在多个称为动静脉畸形的异常血管而导致异常出血。HHT的一个特征是由于多个动静脉畸形导致的高输出量心力衰竭。高输出量心力衰竭可导致复发性鼻小柱区综合征（REKAS），通过失血增加和由此导致的贫血进一步加重心力衰竭。我们报告了一名患有HHT的患者，其出现高输出量心力衰竭导致REKAS。对于患有REKAS的患者，我们建议如果存在贫血，可通过将血红蛋白水平提高到大于9至10 g/dL来纠正贫血，从而避免REKAS。这会减少高动力循环并降低鼻腔血管压力。
