The discriminant power of bleeding history for the diagnosis of type 1 von Willebrand disease: an international, multicenter study
| Autor: | Francesco Baudo, Javier Batlle, Jcj Eikenboom, Giancarlo Castaman, Francesco Rodeghiero, Augusto B. Federici, N. de Bosch, A. Cappelletti, Alberto Tosetto, P Casana, Stefan Lethagen, S. Linari, Arun Srivastava |
|---|---|
| Rok vydání: | 2005 |
| Předmět: |
Adult
Male Heterozygote congenital hereditary and neonatal diseases and abnormalities Pediatrics medicine.medical_specialty Blood transfusion Offspring medicine.medical_treatment Hemorrhage Sensitivity and Specificity Severity of Illness Index Sex Factors Predictive Value of Tests Surveys and Questionnaires hemic and lymphatic diseases Positive predicative value Severity of illness Von Willebrand disease medicine Humans First-degree relatives Medical History Taking Family Health business.industry Case-control study Hematology medicine.disease von Willebrand Diseases Case-Control Studies Predictive value of tests Female business Algorithms |
| Zdroj: | Journal of Thrombosis and Haemostasis. 3:2619-2626 |
| ISSN: | 1538-7836 |
| DOI: | 10.1111/j.1538-7836.2005.01663.x |
| Popis: | 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) |
| Databáze: | OpenAIRE |
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