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A quantitative anal...
A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD)
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Tosetto, A (author)
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Rodeghiero, F (author)
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Castaman, G (author)
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Goodeve, A (author)
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Federici, AB (author)
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Batlle, J (author)
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Meyer, D (author)
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Fressinaud, E (author)
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Mazurier, C (author)
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Goudemand, J (author)
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Eikenboom, J (author)
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Schneppenheim, R (author)
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Budde, U (author)
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Ingerslev, J (author)
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Vorlova, Z (author)
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Habart, D (author)
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- Holmberg, Lars (author)
- Lund University,Lunds universitet,Pediatrik, Lund,Sektion V,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Paediatrics (Lund),Section V,Department of Clinical Sciences, Lund,Faculty of Medicine
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- Lethagen, Stefan (author)
- Lund University,Lunds universitet,Institutionen för kliniska vetenskaper, Malmö,Medicinska fakulteten,Department of Clinical Sciences, Malmö,Faculty of Medicine
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Pasi, J (author)
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Hill, F (author)
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Peake, I (author)
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(creator_code:org_t)
- Elsevier BV, 2006
- 2006
- English.
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In: Journal of Thrombosis and Haemostasis. - : Elsevier BV. - 1538-7933 .- 1538-7836. ; 4:4, s. 766-773
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Abstract
Subject headings
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- Background: A quantitative description of bleeding symptoms in type 1 von Willebrand disease (VWD) has never been reported. Objectives: The aim was to quantitatively evaluate the severity of bleeding symptoms in type 1 VWD and its correlation with clinical and laboratory features. Patients and methods: Bleeding symptoms were retrospectively recorded in a European cohort of VWD type 1 families, and for each subject a quantitative bleeding score (BS) was obtained together with phenotypic tests. Results: A total of 712 subjects belonging to 144 families and 195 controls were available for analysis. The BS was higher in index cases than in affected family members (BS 9 vs. 5, P < 0.0001) and in unaffected family members than in controls (BS 0 vs. -1, P < 0.0001). There was no effect of ABO blood group. BS showed a strong significant inverse relation with either von Willebrand ristocetin cofactor (VWF:RCo), von Willebrand antigen (VWF:Ag) or factor VIII procoagulant activity (FVIII:C) measured at time of enrollment, even after adjustment for age, sex and blood group (P < 0.001 for all the four upper quintiles of BS vs. the first quintile, for either VWF:RCo, VWF:Ag or FVIII:C). Higher BS was related with increasing likelihood of VWD, and a mucocutaneous BS (computed from spontaneous, mucocutaneous symptoms) was strongly associated with bleeding after surgery or tooth extraction. Conclusions: Quantitative analysis of bleeding symptoms is potentially useful for a more accurate diagnosis of type 1 VWD and to develop guidelines for its optimal treatment.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Kardiologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cardiac and Cardiovascular Systems (hsv//eng)
Keyword
- von Willebrand disease diagnosis
- von Willebrand disease
- bleeding score
- inherited bleeding disorders
Publication and Content Type
- art (subject category)
- ref (subject category)
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- By the author/editor
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Tosetto, A
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Rodeghiero, F
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Castaman, G
-
Goodeve, A
-
Federici, AB
-
Batlle, J
-
show more...
-
Meyer, D
-
Fressinaud, E
-
Mazurier, C
-
Goudemand, J
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Eikenboom, J
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Schneppenheim, R
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Budde, U
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Ingerslev, J
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Vorlova, Z
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Habart, D
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Holmberg, Lars
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Lethagen, Stefan
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Pasi, J
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Hill, F
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Peake, I
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show less...
- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Cardiac and Card ...
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Journal of Throm ...
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Lund University