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重型地中海贫血患者糖尿病自然史及管理的新概念

New concept in natural history and management of diabetes mellitus in thalassemia major.

作者信息

Chatterjee Ratna, Bajoria Rekha

机构信息

Institute for Women's Health, University College Hospital, University College London, London, UK.

出版信息

Hemoglobin. 2009;33 Suppl 1:S127-30. doi: 10.3109/09553000903347880.

Abstract

Diabetes Mellitus is a major endocrinopathy, which occurs due transfusional haemosiderosis and is found in 20-30% of adult patients with beta-thalassaemia worldwide, accounting for significant morbidity. It is multifactorial with iron loading being the dominant cause and its management poses a clinical challenge. Diabetes in thalassaemia patients is distinct from type 2 diabetes. It is peculiar in many aspects including its pathophysiology and occurs due to insulin resistance as well as islet cell insufficiency. This article reviews the natural history of diabetes in this presentation with emphasis on prevention monitoring and management. Use of MRI techniques may be useful for future monitoring as well as biochemical monitoring to prevent complications of diabetes. Early intervention with intensified chelation may reverse pancreatic function and structural changes as evident from MRI.

摘要

糖尿病是一种主要的内分泌病,因输血性含铁血黄素沉着症而发生,在全球20%-30%的成年β地中海贫血患者中可见,会导致显著的发病率。它是多因素的,铁负荷是主要原因,其管理带来了临床挑战。地中海贫血患者的糖尿病不同于2型糖尿病。它在许多方面都很特殊,包括其病理生理学,是由胰岛素抵抗以及胰岛细胞功能不全引起的。本文回顾了这种情况下糖尿病的自然史,重点是预防、监测和管理。使用MRI技术可能对未来的监测以及生化监测有用,以预防糖尿病并发症。强化螯合的早期干预可能会逆转胰腺功能和MRI显示的结构变化。

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