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患有出血时间延长的氮血症犬的血管性血友病因子未发生改变。

von Willebrand factor is not altered in azotemic dogs with prolonged bleeding time.

作者信息

Brassard J A, Meyers K M

机构信息

Department of Molecular and Experimental Medicine, Scripps Research Institute, La Jolla, California.

出版信息

J Lab Clin Med. 1994 Jul;124(1):55-62.

PMID:8035104
Abstract

The effects of azotemia on von Willebrand factor (vWf) plasma concentration, structure, and function were studied by utilizing canine models for both uremic bleeding and type I vWf deficiency (vWd). Seventy-five percent to 80% renal mass reduction in eight mixed-breed dogs induced marked azotemia (blood urea nitrogen [BUN] 103 +/- 7 mg/dl [mean +/- SEM]; creatinine 5.8 +/- 1 mg/dl) and prolonged mean buccal mucosal bleeding time (BMBT) from 1.8 +/- 0.2 minutes to 7.0 +/- 0.4 minutes. The mean vWf plasma concentration increased from 0.88 +/- 0.11 U/ml to 1.26 +/- 0.14 U/ml. The pre- and postsurgical sodium dodecyl sulfate-agarose gel electrophoresis multimeric patterns were similar in all dogs. Administration of cryoprecipitate from pooled azotemic mixed-breed dog plasma to five Doberman pinschers with type I vWd increased the mean plasma vWf from 0.14 +/- 0.01 U/ml to 0.48 +/- 0.04 U/ml and decreased the BMBT from 7.1 +/- 0.6 minutes to 3.14 +/- 0.09 minutes. After renal mass reduction, five type I vWd Dobermans developed marked azotemia (BUN 79 +/- 8.6 mg/dl; creatinine 3.7 +/- 0.6 mg/dl) and prolonged BMBT (16.1 +/- 3.6 minutes). Findings in the eight azotemic mixed-breed dogs indicated that (1) vWf plasma levels were normal to increased in azotemic dogs; (2) vWf structure and multimeric distribution were not altered in canine azotemia; and (3) vWf was functional when placed in a non-azotemic environment. The prolongation of the BMBT in azotemic vWd dogs indicated that factors other than alteration of vWf function were responsible for the prolonged BMBT in canine azotemia.

摘要

利用犬类尿毒症出血模型和I型血管性血友病因子缺乏症(vWd)模型,研究了氮质血症对血管性血友病因子(vWf)血浆浓度、结构和功能的影响。八只杂种犬肾质量减少75%至80%,导致明显的氮质血症(血尿素氮[BUN]103±7mg/dl[平均值±标准误];肌酐5.8±1mg/dl),平均口腔黏膜出血时间(BMBT)从1.8±0.2分钟延长至7.0±0.4分钟。vWf血浆平均浓度从0.88±0.11U/ml增加至1.26±0.14U/ml。所有犬术前和术后十二烷基硫酸钠-琼脂糖凝胶电泳多聚体模式相似。将混合的氮质血症杂种犬血浆冷沉淀给予五只患有I型vWd的杜宾犬,使血浆vWf平均水平从0.14±0.01U/ml增加至0.48±0.04U/ml,并使BMBT从7.1±0.6分钟缩短至3.14±0.09分钟。肾质量减少后,五只I型vWd杜宾犬出现明显的氮质血症(BUN 79±8.6mg/dl;肌酐3.7±0.6mg/dl),BMBT延长(16.1±3.6分钟)。八只氮质血症杂种犬的研究结果表明:(1)氮质血症犬的vWf血浆水平正常或升高;(2)犬类氮质血症中vWf结构和多聚体分布未改变;(3)vWf置于非氮质血症环境中时具有功能。氮质血症vWd犬的BMBT延长表明,除vWf功能改变外,其他因素也导致了犬类氮质血症中BMBT延长。

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