Steinberg Martin H, Brugnara Carlo
Boston University School of Medicine, Boston, Massachusetts 02118, USA.
Expert Opin Investig Drugs. 2002 May;11(5):645-59. doi: 10.1517/13543784.11.5.645.
Sickle cell disease pathophysiology results from sickle haemoglobin polymerisation and its effects on the sickle erythrocyte and the vasculature. Many of the abnormalities of sickle cell disease are secondary to the damage caused by the polymer and the injured red cell. Pharmacological treatment of the disease is focused on the inhibition of sickle haemoglobin polymerisation, prevention or repair of red cell dehydration and interruption of the interaction of sickle cells with the endothelium.
镰状细胞病的病理生理学源于镰状血红蛋白的聚合及其对镰状红细胞和脉管系统的影响。镰状细胞病的许多异常情况继发于聚合物和受损红细胞所造成的损害。该疾病的药物治疗重点在于抑制镰状血红蛋白的聚合、预防或修复红细胞脱水以及阻断镰状细胞与内皮细胞的相互作用。
