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慢性铁过载的最佳管理策略。

Optimal management strategies for chronic iron overload.

作者信息

Barton James C

机构信息

Southern Iron Disorders Center, Birmingham, Alabama 35209, USA.

出版信息

Drugs. 2007;67(5):685-700. doi: 10.2165/00003495-200767050-00004.

Abstract

Iron overload is characterised by excessive iron deposition and consequent injury and dysfunction of target organs, especially the heart, liver, anterior pituitary, pancreas and joints. Iron overload disorders are common worldwide and occur in most major race/ethnicity groups. Physiological mechanisms to excrete iron are very limited. Thus, all patients with iron overload need safe and effective treatment that is compatible with their co-existing medical conditions. Treatments for iron overload include phlebotomy and erythrocytapheresis that remove iron predominantly as haemoglobin, and chelation therapy with drugs that bind excess iron selectively and increase its excretion. The most important potential benefits of therapy are preventing deaths due to cardiac siderosis and hepatic cirrhosis. Preventing iron-related injury to endocrine organs is critical in children. Successful treatment or prevention of iron overload increases quality of life and survival in many patients. This article characterises the major categories of iron overload disorders, tabulates methods to evaluate and treat iron overload, and describes treatment options for iron overload disorders. Research needed to advance knowledge about treatment of iron overload is proposed.

摘要

铁过载的特征是铁过度沉积以及由此导致的靶器官损伤和功能障碍,尤其是心脏、肝脏、垂体前叶、胰腺和关节。铁过载疾病在全球范围内都很常见,在大多数主要种族/民族群体中都会发生。机体排泄铁的生理机制非常有限。因此,所有铁过载患者都需要安全有效的治疗,且这种治疗要与他们并存的疾病状况相适应。铁过载的治疗方法包括放血疗法和红细胞单采术,它们主要通过血红蛋白来去除铁,以及使用能选择性结合过量铁并增加其排泄的药物进行螯合治疗。治疗最重要的潜在益处是预防因心脏铁沉着症和肝硬化导致的死亡。预防铁对内分泌器官的损伤对儿童至关重要。成功治疗或预防铁过载可提高许多患者的生活质量和生存率。本文阐述了铁过载疾病的主要类别,列出了评估和治疗铁过载的方法,并描述了铁过载疾病的治疗选择。还提出了推进铁过载治疗知识所需的研究。

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