Document Detail

Inverse relation between plasma concentration of von Willebrand factor and CrEDTA clearance in systemic sclerosis.
MedLine Citation:
PMID:  8035386     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Microvascular abnormalities involving endothelial cell dysfunction occur as an early event in systemic sclerosis. We studied plasma concentrations of von Willebrand factor (vWf), a substance released from injured endothelial cells, and its relation to pulmonary and renal dysfunction. METHODS: vWf was determined by immunoelectrophoresis. Renal function was assessed by CrEDTA clearance, an accurate measure of glomerular filtration rate and pulmonary function by spirometry and carbon monoxide diffusing capacity. Pulmonary pressure was measured by Doppler cardiography. RESULTS: In 22 patients with scleroderma, vWf concentrations related inversely to CrEDTA clearance. In contrast, no relationship between plasma vWf and pulmonary function was found; 9/10 patients with pulmonary hypertension and 9/12 patients with normal pulmonary pressure showed elevated levels of vWf. However in 5 patients with pulmonary hypertension without radiographic evidence of pulmonary fibrosis there was a strong correlation between plasma vWf and pulmonary pressure (rho = 0.90) which fell short of statistical significance due most likely to small population size. CONCLUSION: Renal but not pulmonary dysfunction was associated with elevated plasma levels of vWf.
A Scheja; J Eskilsson; A Akesson; F A Wollheim
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Journal of rheumatology     Volume:  21     ISSN:  0315-162X     ISO Abbreviation:  J. Rheumatol.     Publication Date:  1994 Apr 
Date Detail:
Created Date:  1994-08-16     Completed Date:  1994-08-16     Revised Date:  2010-03-24    
Medline Journal Info:
Nlm Unique ID:  7501984     Medline TA:  J Rheumatol     Country:  CANADA    
Other Details:
Languages:  eng     Pagination:  639-42     Citation Subset:  IM    
Department of Rheumatology, University Hospital, Lund, Sweden.
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MeSH Terms
Biological Markers
Edetic Acid / pharmacokinetics*
Glomerular Filtration Rate
Hypertension, Pulmonary / complications,  physiopathology
Metabolic Clearance Rate
Microcirculation / injuries
Middle Aged
Scleroderma, Systemic / blood*,  complications,  physiopathology
von Willebrand Factor / metabolism*
Reg. No./Substance:
0/Biological Markers; 0/von Willebrand Factor; 60-00-4/Edetic Acid

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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