The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study
(2005) In Journal of Thrombosis and Haemostasis 3(12). p.2619-2626- Abstract
- Objective: The aim of this study was the validation of the criteria defining a significant mucocutaneous-bleeding history in type 1 von Willebrand disease (VWD). Subjects and methods: To avoid selection bias, 42 obligatory carriers (OC) of type 1 VWD were identified from a panel of 42 families with type 1 VWD enrolled by 10 expert centers. OC were identified by the presence of an offspring and another first degree relative with type 1 VWD (affected subjects, AFF). A standardized questionnaire was administered to evaluate hemorrhagic symptoms at the time of first examination, using a bleeding score ranging from 0 (no symptom) to 3 (hospitalization, replacement therapy, blood transfusion). Sensitivity, specificity, diagnostic likelihood... (More)
- Objective: The aim of this study was the validation of the criteria defining a significant mucocutaneous-bleeding history in type 1 von Willebrand disease (VWD). Subjects and methods: To avoid selection bias, 42 obligatory carriers (OC) of type 1 VWD were identified from a panel of 42 families with type 1 VWD enrolled by 10 expert centers. OC were identified by the presence of an offspring and another first degree relative with type 1 VWD (affected subjects, AFF). A standardized questionnaire was administered to evaluate hemorrhagic symptoms at the time of first examination, using a bleeding score ranging from 0 (no symptom) to 3 (hospitalization, replacement therapy, blood transfusion). Sensitivity, specificity, diagnostic likelihood ratios, positive and negative predictive values for the diagnosis of type 1 VWD were calculated from the data collected in OC and in 215 controls. Results: Having at least three hemorrhagic symptoms or a bleeding score of 3 in males and 5 in females was very specific (98.6%) for the bleeding history of type 1 VWD, although less sensitive (69.1%). None of the misclassified OC had life-threatening bleeding episodes after diagnosis. Conclusions: We suggest that the use of a standardized questionnaire and bleeding score may be useful for the identification of subjects requiring laboratory evaluation for VWD. (Less)
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https://lup.lub.lu.se/record/894399
- author
- organization
- publishing date
- 2005
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- inherited bleeding disorders, von Willebrand disease, von Willebrand, disease diagnosis
- in
- Journal of Thrombosis and Haemostasis
- volume
- 3
- issue
- 12
- pages
- 2619 - 2626
- publisher
- Wiley-Blackwell
- external identifiers
-
- pmid:16359502
- wos:000233623700005
- scopus:29244439008
- pmid:16359502
- ISSN
- 1538-7933
- DOI
- 10.1111/j.1538-7836.2005.01663.x
- language
- English
- LU publication?
- yes
- additional info
- The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Emergency medicine/Medicine/Surgery (013240200)
- id
- f9b1e645-de63-4daa-98a2-1fc97e43e842 (old id 894399)
- date added to LUP
- 2016-04-01 12:13:56
- date last changed
- 2022-05-14 19:28:04
@article{f9b1e645-de63-4daa-98a2-1fc97e43e842, abstract = {{Objective: The aim of this study was the validation of the criteria defining a significant mucocutaneous-bleeding history in type 1 von Willebrand disease (VWD). Subjects and methods: To avoid selection bias, 42 obligatory carriers (OC) of type 1 VWD were identified from a panel of 42 families with type 1 VWD enrolled by 10 expert centers. OC were identified by the presence of an offspring and another first degree relative with type 1 VWD (affected subjects, AFF). A standardized questionnaire was administered to evaluate hemorrhagic symptoms at the time of first examination, using a bleeding score ranging from 0 (no symptom) to 3 (hospitalization, replacement therapy, blood transfusion). Sensitivity, specificity, diagnostic likelihood ratios, positive and negative predictive values for the diagnosis of type 1 VWD were calculated from the data collected in OC and in 215 controls. Results: Having at least three hemorrhagic symptoms or a bleeding score of 3 in males and 5 in females was very specific (98.6%) for the bleeding history of type 1 VWD, although less sensitive (69.1%). None of the misclassified OC had life-threatening bleeding episodes after diagnosis. Conclusions: We suggest that the use of a standardized questionnaire and bleeding score may be useful for the identification of subjects requiring laboratory evaluation for VWD.}}, author = {{Rodeghiero, F and Castaman, G and Tosetto, A and Batlle, J and Baudo, F and Cappelletti, A and Casana, P and De Bosch, N and Eikenboom, JCJ and Federici, AB and Lethagen, Stefan and Linari, S and Srivastava, A}}, issn = {{1538-7933}}, keywords = {{inherited bleeding disorders; von Willebrand disease; von Willebrand; disease diagnosis}}, language = {{eng}}, number = {{12}}, pages = {{2619--2626}}, publisher = {{Wiley-Blackwell}}, series = {{Journal of Thrombosis and Haemostasis}}, title = {{The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study}}, url = {{http://dx.doi.org/10.1111/j.1538-7836.2005.01663.x}}, doi = {{10.1111/j.1538-7836.2005.01663.x}}, volume = {{3}}, year = {{2005}}, }