输血依赖型β地中海贫血患者的肝病:代谢功能障碍相关性脂肪性肝病的新作用

IF 2.5 Q2 GASTROENTEROLOGY & HEPATOLOGY World Journal of Hepatology Pub Date : 2024-05-27 DOI:10.4254/wjh.v16.i5.671
N. Fragkou, E. Vlachaki, Ioannis Goulis, E. Sinakos
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引用次数: 0

摘要

在这篇社论中,我们强调了代谢功能障碍相关性脂肪性肝病(MASLD)在未来输血依赖性β地中海贫血(TDBT)患者肝病方面可能发挥的作用。脂肪性肝病的特征是肝脏中脂肪过度积聚(肝脂肪变性),并伴有心脏代谢因素。MASLD 的发生与胰岛素抵抗密切相关,其发病率的增加与糖尿病(DM)和肥胖症在全球的流行同步。TDBT 患者需要终身定期输血以确保生存。通过这些输血,大量的铁被积累起来,造成转铁蛋白饱和,导致游离铁分子循环,对重要器官(主要是肝脏和心肌)造成损害。过去,这些患者发生肝病的主要机制是铁对肝脏的毒性作用和慢性丙型肝炎,目前已找到了现代有效的治疗方法,并取得了成功。在治疗和监测这些患者方面取得的更多进展导致死亡人数减少,预期寿命延长。生存期的延长使他们更容易受到疾病的侵袭,直到最近,这些疾病主要与非地中海贫血症的普通人群有关,如肥胖症和糖尿病。关于地中海贫血症在这一人群中的发病率或发病风险因素的文献资料不足。不过,最近一项针对 45 名大量输血的 beta 型地中海贫血患者的研究(Padeniya 等人,BJH)表明,脂肪变性的存在是影响肝脏弹性成像值的一个因素,因此也是影响肝纤维化的一个因素。这些研究结果表明,未来在 TDBT 患者肝病领域的研究应侧重于 MASLD 对这些患者的发生、风险因素和影响。
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Liver disease in patients with transfusion-dependent β-thalassemia: The emerging role of metabolism dysfunction-associated steatotic liver disease
In this Editorial, we highlight the possible role that metabolism dysfunction-associated steatotic liver disease (MASLD) may play in the future, regarding liver disease in patients with transfusion-dependent β-thalassemia (TDBT). MASLD is characterized by excessive accumulation of fat in the liver (hepatic steatosis), in the presence of cardiometabolic factors. There is a strong correlation between the occurrence of MASLD and insulin resistance, while its increased prevalence parallels the global epidemic of diabetes mellitus (DM) and obesity. Patients with TDBT need regular transfusions for life to ensure their survival. Through these transfusions, a large amount of iron is accumulated, which causes saturation of transferrin and leads to the circulation of free iron molecules, which cause damage to vital organs (primarily the liver and myocardium). Over the past, the main mechanisms for the development of liver disease in these patients have been the toxic effect of iron on the liver and chronic hepatitis C, for which modern and effective treatments have been found, resulting in successful treatment. Additional advances in the treatment and monitoring of these patients have led to a reduction in deaths, and an increase in their life expectancy. This increased survival makes them vulnerable to the onset of diseases, which until recently were mainly related to the non-thalassemic general population, such as obesity and DM. There is insufficient data in the literature regarding the prevalence of MASLD in this population or on the risk factors for its occurrence. However, it was recently shown by a study of 45 heavily transfused patients with beta-thalassemia (Padeniya et al , BJH), that the presence of steatosis is a factor influencing the value of liver elastography and thus liver fibrosis. These findings suggest that future research in the field of liver disease in patients with TDBT should be focused on the occurrence, the risk factors, and the effect of MASLD on these patients.
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来源期刊
World Journal of Hepatology
World Journal of Hepatology GASTROENTEROLOGY & HEPATOLOGY-
CiteScore
4.10
自引率
4.20%
发文量
172
期刊最新文献
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