de Jong P E, de Jong-van den Berg L T, Sewrajsingh G S, Schouten H, Donker A J, Statius van Eps L W
Nephron. 1981;29(3-4):138-41. doi: 10.1159/000182331.
Sickle cell nephropathy is characterized by severe defects in renal medullary functions. In contrast, both proximal tubular secretion and reabsorption are found to be elevated. Since proximal tubular cells are involved in reabsorption and catabolism of beta 2-microglobulin (beta 2-m), we studied serum concentration and urinary excretion of this low molecular weight protein in sickle cell nephropathy. Serum beta 2-m concentration was higher in sickle cell anaemia (SCA) patients compared to control persons. beta 2-m excretion, however, was normal and beta 2-m clearance was not significantly decreased. Fractional beta 2-m excretion was significantly lower and therefore tubular reabsorption of beta 2-m was increased in the SCA patients. There was no correlation between serum beta 2-m concentration and the tubular reabsorption of beta 2-m and phosphate. These findings are further evidence of a particular function of the proximal tubule in sickle cell nephropathy.
镰状细胞肾病的特征是肾髓质功能严重缺陷。相比之下，近端肾小管的分泌和重吸收均升高。由于近端肾小管细胞参与β2-微球蛋白（β2-m）的重吸收和分解代谢，我们研究了镰状细胞肾病患者中这种低分子量蛋白质的血清浓度和尿排泄情况。与对照组相比，镰状细胞贫血（SCA）患者的血清β2-m浓度更高。然而，β2-m排泄正常，且β2-m清除率未显著降低。SCA患者的β2-m排泄分数显著降低，因此β2-m的肾小管重吸收增加。血清β2-m浓度与β2-m和磷酸盐的肾小管重吸收之间无相关性。这些发现进一步证明了近端小管在镰状细胞肾病中的特殊功能。
