中间型和轻型β0地中海贫血中的铁过载与溶酶体稳定性：血清铁蛋白与血清N-乙酰-β-D-氨基葡萄糖苷酶水平之间的相关性

Iron overload and lysosomal stability in beta zero-thalassaemia intermedia and trait: correlation between serum ferritin and serum N-acetyl-beta-D-glucosaminidase levels.

作者信息

Frigerio R, Mela Q, Passiu G, Cacace E, La Nasa G, Perpignano G, Carcassi U E

出版信息

Scand J Haematol. 1984 Sep;33(3):252-5. doi: 10.1111/j.1600-0609.1984.tb02224.x.

DOI:10.1111/j.1600-0609.1984.tb02224.x

Abstract

Increased iron storage represents a characteristic condition in patients with beta zero-thalassaemia intermedia. Iron overload is an important factor in cellular damage. Recent studies have shown an enhanced lysosomal fragility due to increased iron storage. 13 patients with beta-thalassaemia intermedia, aged 17-44 years, were studied. Both serum ferritin and serum N-acetyl-beta-D-glucosaminidase levels were evaluated in all subjects studied. A significant linear correlation (P less than 0.05) between serum ferritin and serum N-acetyl-beta-D-glucosaminidase levels were found.

摘要

铁储存增加是中间型β0地中海贫血患者的一个特征性情况。铁过载是细胞损伤的一个重要因素。最近的研究表明，由于铁储存增加，溶酶体脆性增强。对13例年龄在17至44岁之间的中间型β地中海贫血患者进行了研究。对所有研究对象的血清铁蛋白和血清N-乙酰-β-D-氨基葡萄糖苷酶水平进行了评估。发现血清铁蛋白与血清N-乙酰-β-D-氨基葡萄糖苷酶水平之间存在显著的线性相关性（P<0.05）。