Platelet von Willebrand Factor in Patients with von Willebrand Disease Type I: Preliminary Report

Thomas, Kathy B.; Zieger, Barbara; Jessat, U.; Zehenter, A.; Grohmann, A.; Sutor, A. H.

doi:10.1007/978-3-642-79009-6_39

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“…The method used in the present study for the separation and detection of vWF multimers is capable of detecting the ultrahigh molecular weight multimers if present in the sample, such as those found in normal platelet lysates. 34 It is also conceivable that during the acute phase of HUS vascular damage, often involving endothelial cell swelling and widening of the subendothelial space, may promote the attachment of normal, well-functioning, high molecular weight vWF multimers to the exposed subendothelial components including collagen fibers.…”

Section: Discussionmentioning

confidence: 99%

Function of von Willebrand Factor in Children with Diarrhea-Associated Hemolytic-Uremic Syndrome (D+ HUS)

Sutor¹,

Thomas²,

Prüfer³

et al. 2001

Semin Thromb Hemost

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Reports on von Willebrand factor (vWF) in hemolytic-uremic syndrome (HUS) are not unequivocal. Because of potential pathogenic implications, we examined the ability of vWF to bind to collagen in vitro, which reflects its function. Plasma vWF antigen (vWF:Ag) and collagen-binding activity (vWF:CBA) were measured by enzyme-linked immunosorbent assay in children with (1) diarrhea-associated (D+) HUS (n = 27), (2) chronic renal insufficiency (CRI) (n = 8), (3) gastroenteritis (GE) not associated with HUS (n = 15), (4) immune thrombocytopenia (ITP) (n = 40) and from controls (n = 35). Structural vWF was evaluated by multimer analysis. Children with D+ HUS had vWF:Ag of 2.53 and vWF:CBA of 1.98 U/mL. The corresponding values for patients with ITP were 1.35 and 1.82 U/mL, with CRI 1.55 and 1.55 U/mL, and with GE 1.68 and 2.10 U/mL; all values were higher than in controls (1.04 and 1.16 U/mL). The mean ratio of vWF:CBA to vWF:Ag ratio in controls was 1.13; only children with HUS had a dysfunctional vWF, as indicated by a low ratio of 0.78; the ratio was elevated in children with ITP (1.36) and GE (1.27) and was normal in those with CRI (1.06). No ultralarge molecular multimers of vWF were detected in any group, including HUS. The very high concentration of plasma vWF:Ag in HUS probably reflects endothelial cell damage or irritation. In contrast to all other groups, only children with HUS had a dysfunctional vWF, caused either by a primary (due to enterohemorrhagic Escherichia coli) or secondary (due to consumption of functionally active vWF) process. This abnormality was not obvious as structural anomaly by multimer analysis.

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Section: Discussionmentioning

confidence: 99%