PDF(Pubmed)
Abstract：
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.
摘要：
在这篇社论中，我们强调了代谢功能障碍相关的脂肪变性肝病（MASLD）在未来可能发挥的作用，关于输血依赖性β地中海贫血（TDBT）患者的肝病。MASLD的特征是肝脏中脂肪的过度积累（肝性脂肪变性），在存在心脏代谢因子的情况下。MASLD的发生与胰岛素抵抗有很强的相关性,而其患病率的增加与糖尿病（DM）和肥胖症的全球流行相似。TDBT患者需要定期输血以确保其生存。通过这些输血,积累了大量的铁，这导致转铁蛋白饱和，并导致自由铁分子的循环，对重要器官（主要是肝脏和心肌）造成损害。过去，这些患者肝病发展的主要机制是铁对肝脏和慢性丙型肝炎的毒性作用，已经找到了现代有效的治疗方法，导致成功的治疗。这些患者在治疗和监测方面的进一步进展导致死亡人数减少，并增加他们的预期寿命。这种增加的存活率使他们容易受到疾病发作的影响，直到最近主要与非地中海贫血人群有关，如肥胖和DM。文献中关于MASLD在该人群中的患病率或其发生的危险因素的数据不足。然而,最近，一项对45例大量输血的β-地中海贫血患者的研究表明了这一点(Padeniya等人，BJH),脂肪变性的存在是影响肝脏弹性成像价值的一个因素，因此肝纤维化。这些结果表明，未来在肝病领域的研究应该集中在TDBT患者的发生，风险因素，以及MASLD对这些患者的影响。
