Vaziri N D, Gonzales E, Barton C H, Chen H T, Nguyen Q, Arquilla M
Department of Medicine, University of California, Irvine.
J Lab Clin Med. 1991 Feb;117(2):152-6.
Plasma and urine concentrations of factor XIII and its circulating substrates (fibronectin, fibrinogen, and alpha 2-antiplasmin) were measured in a group of 36 patients with nephrotic syndrome. The results were compared with those obtained in a group of 32 normal volunteers (control group) and 12 patients with end-stage renal disease (ESRD). A mild but significant reduction in plasma level and an abnormal urinary excretion of alpha 2-antiplasmin was found in the nephrotic group. Plasma concentrations of factor XIII, fibronectin, and fibrinogen were significantly elevated in patients with nephrosis. In contrast, patients with ESRD showed no significant difference in the plasma concentrations of either factor XIII, fibronectin, or alpha 2-antiplasmin and only a modest elevation of fibrinogen when compared with normal controls. No significant correlation was found between serum creatinine concentration and plasma levels of factor XIII and its circulating substrates in the nephrotic group. No measurable quantities of factor XIII and only small quantities of fibronectin were found in the urine of patients with nephrosis. Elevation of plasma factor XIII, fibronectin, and fibrinogen concentrations in the nephrotic group is considered to be the result of a combination of increased synthesis and possibly contracted intravascular distribution of these macromolecular proteins in the face of their negligible urinary losses. The presence of the observed abnormalities in the nephrotic group and their absence in the non-nephrotic ESRD group tends to exclude renal failure as a cause of these abnormalities. Although the clinical significance of these abnormalities is uncertain, they can potentially contribute to the thrombophilic diathesis and platelet hyperaggregability in nephrotic syndrome.
对36例肾病综合征患者测定了血浆和尿液中凝血因子XIII及其循环底物（纤连蛋白、纤维蛋白原和α2 -抗纤溶酶）的浓度。将结果与32名正常志愿者（对照组）和12例终末期肾病（ESRD）患者的结果进行比较。肾病组发现血浆水平轻度但显著降低，α2 -抗纤溶酶尿排泄异常。肾病患者血浆中凝血因子XIII、纤连蛋白和纤维蛋白原浓度显著升高。相比之下，与正常对照组相比，ESRD患者血浆中凝血因子XIII、纤连蛋白或α2 -抗纤溶酶的浓度无显著差异，仅纤维蛋白原有适度升高。肾病组血清肌酐浓度与血浆中凝血因子XIII及其循环底物水平之间未发现显著相关性。肾病患者尿液中未检测到可测量量的凝血因子XIII，仅发现少量纤连蛋白。肾病组血浆凝血因子XIII、纤连蛋白和纤维蛋白原浓度升高被认为是这些大分子蛋白合成增加以及可能因尿中损失可忽略不计而导致血管内分布收缩的综合结果。肾病组中观察到的异常情况存在，而非肾病ESRD组中不存在这些异常情况，这倾向于排除肾衰竭是这些异常情况的原因。尽管这些异常情况的临床意义尚不确定，但它们可能会导致肾病综合征中的血栓形成倾向和血小板高聚集性。
