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在西非某地区医院的实践中,通过十二烷基硫酸钠-聚丙烯酰胺凝胶电泳(SDS-PAGE)定义的贫血患者红细胞膜蛋白异常情况。

Red cell membrane protein abnormalities as defined by sds-page among patients with anemia in a West African region hospital practice.

作者信息

Dosunmu Adedoyin, Uche Ebele, Osikomaiya Bodunrin, Ismail Ayobami, Akinbami Akinsegun, Akanmu Alani

机构信息

Department of Hematology and Blood Transfusion, Lagos State University College of Medicine, Lagos, Nigeria.

Department of Hematology and Blood Transfusion, General Hospital, Gbagada, Lagos, Nigeria.

出版信息

Caspian J Intern Med. 2020 May;11(3):283-289. doi: 10.22088/cjim.11.3.283.

Abstract

BACKGROUND

Erythrocytes require an ability to deform and withstand shear stress while negotiating microcirculation. These properties are largely due to their excess surface area per volume and the characteristics of the membrane's protein. Deficiencies of these proteins are associated with chronic hemolysis.

METHODS

This was a cross-sectional study aimed at determining the prevalence of red cell membrane protein abnormalities as determined by sodium dodecyl sulphate polyacrilamide gel electrophoresis (SDS-PAGE) among patients with anemia attending the outpatient clinics of the hospital.

RESULTS

A total of 823 participants were recruited into the study with a mean age of 34±14 years. There were 410 (49.8%) participants with hematocrit ≥ 36% and 413 with hematocrit ≤ 35.9% of which 192 participants (23.3%) had abnormal red cell indices. Following SDS-PAGE, 21 (10.9%) of the 192 participants had deficient PAGE tracing. Abnormal spectrin band was observed in 17 (81%) of the 21 participants. The hematocrit was significantly lower while the reticulocyte count and red cell distribution width were higher in participants with red cell membrane abnormalities.

CONCLUSION

One in ten patients with mild anemia and abnormal red cell indices in clinical practice may be having hereditary red cell membrane protein defect. Presence of raised reticulocyte count, family history of mild anemia, increased red cell distribution width and red cell morphology may be used to screen for membrane deficiency.

摘要

背景

红细胞在通过微循环时需要具备变形能力并承受剪切应力。这些特性很大程度上归因于其单位体积的表面积过大以及膜蛋白的特性。这些蛋白的缺乏与慢性溶血相关。

方法

这是一项横断面研究,旨在确定在该医院门诊就诊的贫血患者中,通过十二烷基硫酸钠聚丙烯酰胺凝胶电泳(SDS-PAGE)测定的红细胞膜蛋白异常的患病率。

结果

共有823名参与者被纳入研究,平均年龄为34±14岁。血细胞比容≥36%的参与者有410名(49.8%),血细胞比容≤35.9%的有413名,其中192名参与者(23.3%)红细胞指数异常。SDS-PAGE检测后,192名参与者中有21名(10.9%)PAGE条带缺失。21名参与者中有17名(81%)观察到血影蛋白条带异常。红细胞膜异常的参与者血细胞比容显著降低,而网织红细胞计数和红细胞分布宽度较高。

结论

在临床实践中,每十名轻度贫血且红细胞指数异常的患者中可能有一名存在遗传性红细胞膜蛋白缺陷。网织红细胞计数升高、轻度贫血家族史、红细胞分布宽度增加和红细胞形态等情况可用于筛查膜蛋白缺乏。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f8ab/7442468/0d50a61e772a/cjim-11-283-g001.jpg

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