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持续性非卧床腹膜透析患者单核细胞酯酶缺乏的家族性

Familiality of monocyte esterase deficiency in patients on continuous ambulatory peritoneal dialysis.

作者信息

Hull D R, Corr B R, Markey G M, Alexander H D, Morris T C

机构信息

Haematology Department, Belfast City Hospital, Northern Ireland.

出版信息

Ren Fail. 1998 Jul;20(4):607-12. doi: 10.3109/08860229809045153.

Abstract

The purpose of this study was to determine whether or not peripheral blood monocyte esterase deficiency occurring in patients on CAPD was a familial characteristic. The peripheral blood monocyte esterase status of 74 patients on CAPD was determined by a naphthyl acetate esterase staining of cytospin preparations of their mononuclear cells following separation over ficoll. The peripheral blood of first degree relatives and spouses of monocyte esterase deficiency patients was similarly investigated for the deficiency. Three patients bad monocyte esterase deficiency and familiality of the defect was demonstrated in two of their families. The third family was incompletely investigated because of lack of consent. The monocyte esterase deficiency demonstrated in this cohort of patients did not result from their renal failure but was a familial characteristic.

摘要

本研究的目的是确定接受持续性非卧床腹膜透析(CAPD)治疗的患者出现的外周血单核细胞酯酶缺乏是否为家族性特征。对74例接受CAPD治疗的患者,通过对其经淋巴细胞分离液分离后的单核细胞进行醋酸萘酯酶染色,来测定外周血单核细胞酯酶状态。对单核细胞酯酶缺乏患者的一级亲属和配偶的外周血进行类似的缺乏情况调查。3例患者存在单核细胞酯酶缺乏,其中2个家族证实了该缺陷具有家族性。由于未获得同意,第三个家族未进行全面调查。该组患者中所显示的单核细胞酯酶缺乏并非由肾衰竭所致,而是一种家族性特征。

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