镰状细胞疾病肾损害

Kidney complications in sickle cell disease

镰状细胞疾病(SCD)是一种由编码血红蛋白多肽的基因发生点突变后引起的遗传病。患者红细胞内出现病态的血红蛋白S(HbS),在氧张力低的毛细血管,HbS形成凝胶结构,导致红细胞扭曲成僵硬的镰刀状。镰状红细胞不能通过毛细血管,加上HbS的凝胶化使血液的黏滞度增大,阻塞毛细血管,引起局部组织器官缺血。肾脏是SCD常见受累器官,严重影响患者预后。该文综述了近年SCD肾损害的研究概况与进展。

Sickle cell disease （ SCD） is an inherited anemia disorder due to the mutation of p-globin gene encoding hemoglobin peptide. SCD patients present with homozygous inheritance of hemoglobin S（HbS）. HbS can form gel structure within the capillary with low oxygen tension, which leads to rigid sickle mass twisted by the red blood cells. The sickle red blood cells fail to penetrate the capillary. In addition, the gelation of HbS increases the blood viscosity, blocks capillary vessels and provokes ischemia of topical tissues and organs. Kidney is the common organ affected by SCD and significantly influences the clinical prognosis. This paper summarizes the recent research progress on the kidney injuries caused by SCD.