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强直性肌营养不良中的中性粒细胞功能缺陷。

Defective neutrophil function in myotonic dystrophy.

作者信息

Seay A R, Ziter F A, Hill H R

出版信息

J Neurol Sci. 1978 Jan;35(1):25-30. doi: 10.1016/0022-510x(78)90099-0.

DOI:10.1016/0022-510x(78)90099-0
PMID:624959
Abstract

Polymorphonuclear leukocytes (PMNs), obtained from 10 patients with myotonic dystrophy and 39 age-matched controls, were tested for chemotactic activity, chemiluminescence (CL), and phagocytosis. PMNs from 8 of the 10 patients had depressed chemotactic indexes (30 +/- 23) as compared to controls (61 +/- 18) (P less than 0.0003), and 6 of the 10 patients' PMNs produced lower peaks in CL (less than 90%) than those of controls. Phagocytosis and bactericidal activity were evaluated in 7 patients and were normal. Control PMNs incubated in patient serum produced normal chemotactic activity and CL. These results indicate defects in 2 PMN membrane-associated activities in myotonic dystrophy and suggest that the PMN is affected by the generalized membrane abnormality underlying myotonic dystrophy.

摘要

从10例强直性肌营养不良患者和39例年龄匹配的对照者中获取多形核白细胞(PMN),检测其趋化活性、化学发光(CL)和吞噬作用。10例患者中有8例的PMN趋化指数(30±23)低于对照者(61±18)(P<0.0003),10例患者中有6例的PMN产生的CL峰值低于对照者(<90%)。对7例患者的吞噬和杀菌活性进行了评估,结果正常。在患者血清中孵育的对照PMN产生正常的趋化活性和CL。这些结果表明强直性肌营养不良患者的PMN存在两种膜相关活性缺陷,并提示PMN受强直性肌营养不良潜在的全身性膜异常影响。

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Defective neutrophil function in myotonic dystrophy.强直性肌营养不良中的中性粒细胞功能缺陷。
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引用本文的文献

1
Abnormal regulation of monocyte insulin-binding affinity after glucose ingestion in patients with myotonic dystrophy.强直性肌营养不良患者摄入葡萄糖后单核细胞胰岛素结合亲和力的异常调节。
Proc Natl Acad Sci U S A. 1981 Apr;78(4):2567-71. doi: 10.1073/pnas.78.4.2567.
2
Lymphocyte capping in myotonic dystrophy.强直性肌营养不良中的淋巴细胞帽化。
J Neurol Neurosurg Psychiatry. 1983 Jan;46(1):90-2. doi: 10.1136/jnnp.46.1.90.