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遗传性贮存池病对尿毒症出血的增强作用。

Potentiation of uremic bleeding by hereditary storage pool disease.

作者信息

Berty R M, Zeigler Z R, Bruns F J

机构信息

Department of Medicine, Montefiore University Hospital, University of Pittsburgh School of Medicine, PA.

出版信息

Am J Kidney Dis. 1992 Apr;19(4):326-30. doi: 10.1016/s0272-6386(12)80448-3.

DOI:10.1016/s0272-6386(12)80448-3
PMID:1562020
Abstract

This study demonstrates that specific bleeding tests can separate the thrombocytopathy of uremia alone from the bleeding disorders caused by uremia superimposed on preexisting platelet dysfunction. The case history of a uremic patient with exaggerated bleeding tendencies is presented. The findings in this patient are compared with the clinical characteristics and platelet function studies of nine other patients with chronic renal failure. The index and other uremic patients were similar except for the clinical bleeding and results of platelet function studies. The patient's nonocclusive bleeding time and measured blood loss during bleeding time tests were increased compared with the other uremic controls. In addition, her platelet aggregation in response to collagen was lower than that of the other uremic subjects. Repeat studies following renal transplantation were consistent with hereditary storage pool disease. An underlying platelet disorder may potentiate the hemostatic defects of uremia. The diagnosis should be suspected in patients with frequent and severe bleeding manifestations. Renal transplantation led to control of clinical bleeding.

摘要

本研究表明,特定的出血试验能够将单纯的尿毒症血小板病与尿毒症叠加既往存在的血小板功能障碍所引起的出血性疾病区分开来。本文介绍了一名出血倾向严重的尿毒症患者的病例史。将该患者的检查结果与其他九名慢性肾衰竭患者的临床特征及血小板功能研究结果进行了比较。除了临床出血情况及血小板功能研究结果外,该指标患者与其他尿毒症患者相似。与其他尿毒症对照相比,该患者的非闭塞性出血时间及出血时间试验期间的实测失血量增加。此外,她对胶原的血小板聚集反应低于其他尿毒症受试者。肾移植后的重复研究结果符合遗传性储存池病。潜在的血小板疾病可能会加重尿毒症的止血缺陷。对于有频繁且严重出血表现的患者应怀疑有此诊断。肾移植使临床出血得到了控制。

相似文献

1
Potentiation of uremic bleeding by hereditary storage pool disease.遗传性贮存池病对尿毒症出血的增强作用。
Am J Kidney Dis. 1992 Apr;19(4):326-30. doi: 10.1016/s0272-6386(12)80448-3.
2
Skin bleeding time for the evaluation of uremic platelet dysfunction and effect of dialysis.皮肤出血时间评估尿毒症血小板功能障碍及透析的影响。
Clin Appl Thromb Hemost. 2012 Mar-Apr;18(2):185-8. doi: 10.1177/1076029611427438. Epub 2012 Feb 12.
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Plasma and platelet von Willebrand factor defects in uremia.尿毒症患者血浆和血小板中血管性血友病因子缺陷
Am J Med. 1988 Dec;85(6):806-10. doi: 10.1016/s0002-9343(88)80025-1.
4
[Case of uremic platelet dysfunction].[尿毒症血小板功能障碍病例]
Nihon Jinzo Gakkai Shi. 2005;47(7):834-8.
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Reduced platelet thromboxane formation in uremia. Evidence for a functional cyclooxygenase defect.尿毒症患者血小板血栓素生成减少。环氧化酶功能缺陷的证据。
J Clin Invest. 1983 Mar;71(3):762-8. doi: 10.1172/jci110824.
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Evaluation of acquired platelet dysfunctions in uremic and cirrhotic patients using the platelet function analyzer (PFA-100 ): influence of hematocrit elevation.使用血小板功能分析仪(PFA-100)评估尿毒症和肝硬化患者获得性血小板功能障碍:血细胞比容升高的影响。
Haematologica. 1999 Jul;84(7):614-9.
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Bleeding time in uremia: a useful test to assess clinical bleeding.尿毒症患者的出血时间:一项评估临床出血情况的有用检查。
Am J Hematol. 1979;7(2):107-17. doi: 10.1002/ajh.2830070203.
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Deranged platelet calcium homeostasis in diabetic patients with end-stage renal failure. A possible link to increased cardiovascular mortality?终末期肾衰竭糖尿病患者血小板钙稳态紊乱。这与心血管死亡率增加有关联吗?
Diabetes Care. 1996 Oct;19(10):1062-6. doi: 10.2337/diacare.19.10.1062.
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Platelet aggregometry cannot identify uremic platelet dysfunction in heart failure patients prior to cardiac surgery.血小板聚集试验无法在心脏手术前识别心力衰竭患者的尿毒症性血小板功能障碍。
J Clin Lab Anal. 2017 Sep;31(5). doi: 10.1002/jcla.22084. Epub 2016 Oct 31.
10
Uremic platelet dysfunction: past and present.尿毒症血小板功能障碍:过去与现在。
Curr Hematol Rep. 2005 Sep;4(5):359-67.

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A Morphometric Analysis of Platelet Dense Granules of Patients with Unexplained Bleeding: A New Entity of Delta-Microgranular Storage Pool Deficiency.不明原因出血患者血小板致密颗粒的形态计量分析:δ-微颗粒储存池缺乏的一种新类型
J Clin Med. 2020 Jun 4;9(6):1734. doi: 10.3390/jcm9061734.