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The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study

Rodeghiero, F ; Castaman, G ; Tosetto, A ; Batlle, J ; Baudo, F ; Cappelletti, A ; Casana, P ; De Bosch, N ; Eikenboom, JCJ and Federici, AB , et al. (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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organization
publishing date
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
2020-12-08 05:21:22
@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},
  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},
}