King’s College London School of Medicine, Molecular Haematology, London, UK.
Haematologica. 2012 Feb;97(2):201-5. doi: 10.3324/haematol.2011.050336. Epub 2011 Oct 11.
Studies have questioned whether renal dysfunction in sickle cell disease is linked to hemolysis-associated vasculopathy. We have investigated renal function and markers of hemolysis in a cohort of 424 adult African-British patients with sickle cell disease. While significant associations were found in HbSS and HbSβ(0) (sickle cell anemia) patients with and without controlling for covariates between hemolytic markers and albuminuria, the associations were not significant in patients with HbSC. Estimated glomerular filtration rate, a marker of renal function, correlated significantly with reticulocyte count and bilirubin. Alpha thalassemia, present in 34% of the sickle cell anaemia patients, had a protective effect against albuminuria in this group. Altogether, the incidence of hyperfiltration was 71% and microalbuminuria 37%, making nephropathy a common complication of sickle cell anemia.
研究质疑镰状细胞病中的肾功能障碍是否与溶血性血管病变有关。我们调查了 424 名成年非洲裔英国镰状细胞病患者的肾功能和溶血标志物。在 HbSS 和 HbSβ(0)（镰状细胞贫血）患者中，尽管在控制了协变量后，溶血标志物与蛋白尿之间存在显著相关性，但在 HbSC 患者中，相关性不显著。肾小球滤过率估计值是肾功能的标志物，与网织红细胞计数和胆红素显著相关。34%的镰状细胞贫血患者存在α地中海贫血，这对该组的蛋白尿有保护作用。总的来说，高滤过的发生率为 71%，微量白蛋白尿为 37%，使肾病成为镰状细胞贫血的常见并发症。
