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成人β-地中海贫血症的医学并发症管理方法。

How I manage medical complications of β-thalassemia in adults.

机构信息

Department of Internal Medicine, American University of Beirut Medical Centre, Beirut, Lebanon; and.

Department of Clinical Sciences and Community, University of Milan, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Ca' Granda Foundation Maggiore Policlinico Hospital, Milan, Italy.

出版信息

Blood. 2018 Oct 25;132(17):1781-1791. doi: 10.1182/blood-2018-06-818187. Epub 2018 Sep 11.

Abstract

The complex pathophysiology in β-thalassemia can translate to multiple morbidities that affect every organ system. Improved survival due to advances in management means that patients are exposed to the harmful effects of ineffective erythropoiesis, anemia, and iron overload for a longer duration, and we started seeing new or more frequent complications in adult compared with younger patients. In this article, we highlight particular aspects of managing adult patients with β-thalassemia, using our own experience in treating such patients. We cover both transfusion-dependent and nontransfusion-dependent forms of the disease and tackle specific morbidities of highest interest.

摘要

β-地中海贫血的复杂病理生理学可导致多种影响每个器官系统的疾病。由于管理方面的进步,患者的生存率得到提高,这意味着他们会在更长的时间内受到无效红细胞生成、贫血和铁过载的有害影响,与年轻患者相比,我们开始在成年患者中看到新的或更频繁的并发症。在本文中,我们将利用我们在治疗此类患者方面的经验,重点介绍β-地中海贫血成年患者的管理的具体方面。我们涵盖了依赖输血和不依赖输血两种疾病形式,并解决了最受关注的具体疾病。

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