Fragkou Nikolaos, Vlachaki Efthimia, Goulis Ioannis, Sinakos Emmanouil
4 Department of Internal Medicine, Hippokratio Hospital, Aristotle University of Thessaloniki, Thessaloniki 54642, Greece.
2 Department of Internal Medicine, Hippokratio Hospital, Aristotle University of Thessaloniki, Thessaloniki 54642, Greece.
World J Hepatol. 2024 May 27;16(5):671-677. doi: 10.4254/wjh.v16.i5.671.
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 , 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.
在这篇社论中,我们强调了代谢功能障碍相关脂肪性肝病(MASLD)在未来可能对输血依赖型β地中海贫血(TDBT)患者的肝脏疾病所起的作用。MASLD的特征是在存在心脏代谢因素的情况下,肝脏中脂肪过度积累(肝脂肪变性)。MASLD的发生与胰岛素抵抗之间存在很强的相关性,而其患病率的增加与全球糖尿病(DM)和肥胖症的流行情况平行。TDBT患者需要终生定期输血以确保生存。通过这些输血,大量铁被积累,这导致转铁蛋白饱和并导致游离铁分子循环,从而对重要器官(主要是肝脏和心肌)造成损害。过去,这些患者肝脏疾病发展的主要机制是铁对肝脏的毒性作用和丙型肝炎,现已找到针对这些疾病的现代有效治疗方法,从而实现了成功治疗。对这些患者治疗和监测方面的进一步进展导致了死亡率的降低和预期寿命的增加。这种生存率的提高使他们易患一些疾病,而这些疾病直到最近主要与非地中海贫血的普通人群有关,如肥胖症和DM。关于该人群中MASLD的患病率或其发生的危险因素,文献中的数据不足。然而,最近一项对45例重度输血的β地中海贫血患者的研究(帕德尼亚,《英国血液学杂志》)表明,脂肪变性的存在是影响肝脏弹性成像值进而影响肝纤维化的一个因素。这些发现表明,未来在TDBT患者肝脏疾病领域的研究应聚焦于MASLD在这些患者中的发生情况、危险因素及其影响。