{"id":407,"date":"2017-10-24T08:30:58","date_gmt":"2017-10-24T12:30:58","guid":{"rendered":"https:\/\/elearning.wfh.org\/resource\/mcmdm-1-mvw\/"},"modified":"2022-09-21T15:17:38","modified_gmt":"2022-09-21T19:17:38","slug":"mcmdm-1-mw","status":"publish","type":"resource","link":"https:\/\/elearning.wfh.org\/fr\/resource\/mcmdm-1-mw\/","title":{"rendered":"MCMDM-1 mW"},"content":{"rendered":"<h3>Molecular and Clinical Markers for the Diagnosis and Management of Type 1 (MCMDM-1) VWD Bleeding Questionnaire<\/h3>\n<p class=\"\"><strong>Authors\/developers:<\/strong><br class=\"\" \/>Tosetto A, Rodeghiero F, Castaman G, Goodeve A, Federici AB, Batlle J, Meyer D, Fressinaud E, Mazurier C, Goudemand J, Eikenboom J, Schneppenheim R, Budde U, Ingerslev J, Vorlova Z, Habart D, Holmberg L, Lethagen S, Pasi J, Hill F, Peake I.<\/p>\n<p class=\"\"><a href=\"https:\/\/www1.wfh.org\/docs\/en\/Resources\/Assessment_Tools_MCMDM-1VWD.pdf\" target=\"_blank\" rel=\"noopener\">Scoring sheet<\/a><\/p>\n<h6><em>N.B. These scores are posted with the permission of the developers.<\/em><\/h6>\n\t\t<div data-elementor-type=\"section\" data-elementor-id=\"7934\" class=\"elementor elementor-7934\" data-elementor-post-type=\"elementor_library\">\n\t\t\t\t\t<section class=\"elementor-section elementor-top-section elementor-element elementor-element-3a68fc18 elementor-section-boxed elementor-section-height-default elementor-section-height-default\" data-id=\"3a68fc18\" data-element_type=\"section\">\n\t\t\t\t\t\t<div class=\"elementor-container elementor-column-gap-no\">\n\t\t\t\t\t<div class=\"elementor-column elementor-col-100 elementor-top-column elementor-element elementor-element-9e93620\" data-id=\"9e93620\" data-element_type=\"column\">\n\t\t\t<div class=\"elementor-widget-wrap elementor-element-populated\">\n\t\t\t\t\t\t<div class=\"elementor-element elementor-element-67fb01f5 elementor-widget elementor-widget-spacer\" data-id=\"67fb01f5\" data-element_type=\"widget\" data-widget_type=\"spacer.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t<div class=\"elementor-spacer\">\n\t\t\t<div class=\"elementor-spacer-inner\"><\/div>\n\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-44b434f elementor-widget elementor-widget-html\" data-id=\"44b434f\" data-element_type=\"widget\" data-widget_type=\"html.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t<style>\r\n\r\n\/* Style the tab *\/\r\n.popTab {\r\n  float: left;\r\n  border: 1px solid #ccc;\r\n  background-color: #f1f1f1;\r\n  width: 35%;\r\n  height: 500px;\r\n}\r\n\r\n\/* Style the buttons inside the tab *\/\r\n.popTab button {\r\n  display: block;\r\n  background-color: inherit;\r\n  color: black;\r\n  padding: 15px 10px;\r\n  width: 100%;\r\n  border: none;\r\n  border-radius: 0;\r\n  outline: none;\r\n  text-align: left;\r\n  cursor: pointer;\r\n  transition: 0.3s;\r\n  font-size: 1.3em;\r\n}\r\n\r\n\/* Change background color of buttons on hover *\/\r\n.popTab button:hover {\r\n  background-color: #ddd;\r\n}\r\n\r\n\/* Create an active\/current tablink class *\/\r\n.popTab button.active {\r\n  background-color: #ccc;\r\n  border: none!important;\r\n}\r\n\r\n\/* Style the tab content *\/\r\n.popContent {\r\n  float: left;\r\n  padding: 0px 1em;\r\n  border: 1px solid #ccc;\r\n  width: 65%;\r\n  border-left: none;\r\n  height: 500px;\r\n  overflow-y:auto;\r\n}\r\n\r\n.popContent table tr {\r\n    \/*pointer-events: none;*\/\r\n\r\n}\r\n\r\n.popContent table td {\r\n    border-top: none;\r\n    border-left: none;\r\n    border-right: none;\r\n    border-bottom: 1px solid #eee;\r\n    background-color: white!important;\r\n    padding-left: 0;\r\n}\r\n\r\n<\/style>\r\n\r\n\r\n<div class=\"popTab\">\r\n   <button class=\"popLinks active\" onclick=\"openTab(event, 'description')\">Description<\/button>    \r\n   <button class=\"popLinks\" onclick=\"openTab(event, 'utility')\">Utility<\/button>\r\n   <button class=\"popLinks\" onclick=\"openTab(event, 'administration')\">Administration<\/button>\r\n   <button class=\"popLinks\" onclick=\"openTab(event, 'psychometrics')\">Psychometrics<\/button>\r\n   <button class=\"popLinks\" onclick=\"openTab(event, 'references')\">References<\/button>\r\n \r\n  \r\n<\/div>\r\n\r\n<div id=\"description\" class=\"popContent\">\r\n  <h3>Description<\/h3>\r\n  <p>The MCMDM-1 VWD bleeding questionnaire is an expert-administered questionnaire studied primarily in the research setting to distinguish between types of VWD and to assess bleeding pattern and severity. The target population is patients with VWD and their family members.<\/p>\r\n\r\n\r\n  <p><strong>Contact person:<\/strong><br>\r\n  F Rodeghiero:\r\n<a href=\"mailto:rodeghiero@hemato.ven.it\">rodeghiero@hemato.ven.it<\/a>\r\n<\/p>\r\n\r\n<p class=\"\"><strong>Date of this review: <\/strong>August 19, 2013<br>\r\n<strong>Updated: <\/strong>April 2014<\/p>\r\n\r\n\r\n<\/div>\r\n\r\n\r\n<div id=\"utility\" class=\"popContent\" style=\"display:none\">\r\n  <h3>Utility<\/h3>\r\n  \r\n  <p><strong>Overall utility:<\/strong><\/p>\r\n  <p>Useful in the research setting for assessment of VWD and differentiating between types of VWD.<\/p>\r\n\r\n  <p><strong>Limitations:<\/strong><\/p>\r\n   <ul>\r\n \t<li>Time to complete is the main limitation of this questionnaire<\/li>\r\n \t<li>Requires a skilled professional to administer<\/li>\r\n<\/ul>\r\n\r\n\r\n<\/div>\r\n\r\n\r\n<div id=\"administration\" class=\"popContent\" style=\"display:none\">\r\n  <h3>Administration<\/h3>        \r\n   <ol style=\"margin-bottom:1em\">\r\n \t<li><strong>Time to complete:<\/strong> 40 minutes.<\/li>\r\n    <li><strong>Equipment\/space required:<\/strong> None.<\/li>\r\n    <li><strong>Training required:<\/strong>\u00a0Expert-administered; no training specific to the administration of the scoring tool is required.<\/li>\r\n    <li><strong>Cost:<\/strong> None<\/li>\r\n    <li><strong>Scoring\/scaling and interpretation of results:<\/strong>\r\n    <ul>\r\n \t  <li>Scale: \u20131 to 4 for each of 12 symptoms, HOWEVER, maximum score for cutaneous bleeding: 2; maximum score for gastrointestinal tract bleeding: 3; score of \u20131 is assigned for lack of bleeding on 2 or more challenges for each of post-surgery bleeding, post-partum hemorrhage, and bleeding post-tooth extraction<\/li>\r\n \t  <li>Possible range of total score: \u20133 to +45<\/li>\r\n \t  <li>Differentiating features: to attempt to improve sensitivity over the <a style=\"color: #e31837;\" href=\"https:\/\/www1.wfh.org\/docs\/en\/Resources\/Assessment_Tools_Other_Scores.pdf\" target=\"_blank\" rel=\"noopener\">Vicenza score<\/a>, the range of possible scores for a symptom was increased, with a score of \u20131 possible in the absence of bleeding after significant hemostatic challenges<\/li>\r\n    <\/ul>\r\n  <\/ol>\r\n\r\n<\/div>\t\r\n\r\n<div id=\"psychometrics\" class=\"popContent\" style=\"display:none\">\r\n  <h3>Psychometrics<\/h3>        \r\n  \r\n  <p><strong>Psychometric properties:<\/strong><\/p>\r\n  \r\n  <ol style=\"margin-bottom:1em\">\r\n    <li>\r\n      Construct validity:<br>\r\n      <u>Convergent validity<\/u><br>\r\n \t  <ul>\r\n \t    <li>Bleeding score (BS) was greater in index cases &gt; affected family members &gt; unaffected family members &gt; controls or type 3 VWD &gt; type 2 VWD &gt; type 1 VWD &gt; control.<sup>1,2,3\u00a0<\/sup><\/li>\r\n \t    <li>Higher BS was associated with lower VWF:RCo, longer PFA-100 closure times, lower VWF:Ag, lower FVIII:C, lower platelet count.<sup>4,5,6,7<\/sup><\/li>\r\n \t    <li>In subjects with either a clinical bleeding history or a hemostatic laboratory abnormality, almost half (46%) of those with a BS \u22654 had an underlying coagulation disorder (platelet secretion defect, VWD, coagulation factor deficiency, defect of fibrinolysis, secondary coagulation disorders).<sup>8<\/sup><\/li>\r\n      <\/ul>\r\n      <u>Predictive validity<\/u>\r\n      <ul>\r\n \t    <li>Was shown in that an increased BS was associated with increased skin bleeding time, increased need for prophylaxis, increased visits for bleeding episodes requiring treatment, decreased health related quality of life, and higher risk of post-operative bleeding.<sup>6,9,10,11<\/sup><\/li>\r\n      <\/ul>\r\n      <u>Group differences<\/u>\r\n      <ul>\r\n \t    <li>When used in unaffected family members and control group.<sup>1,2<\/sup><\/li>\r\n \t    <li>No discriminant validity was demonstrated between BS in those with a BS \u22654 and various patterns of platelet secretion defects or number of agonists that elicited a reduced response.<sup>8<\/sup><\/li>\r\n        <\/ul>\r\n      <\/li>\r\n \t  <li>Criterion validity:\r\n \t    <ul>\r\n \t      <li>Increased BS quintiles had a strong inverse association with VWF:Ag, FVIII:C, and VWF:RCo levels.<sup>1<\/sup><\/li>\r\n        <\/ul>\r\n      <\/li>\r\n \t  <li>Reliability:\r\n \t    <ul>\r\n \t      <li>No studies identified.<\/li>\r\n        <\/ul>\r\n \t  <\/li>\r\n \t  \r\n \t<li>Responsiveness\/sensitivity:\r\n \t<ul>\r\n \t  <li>Responsiveness: Clear trend of increasing BS with age in index cases and affected family members, with no trend in controls or unaffected family members.<\/li>\r\n \t  <li>Using a cut off BS \u22652, in comparing index cases and affected family members to controls and unaffected family members, there was a statistically significant increased likelihood ratio of having VWD (based on laboratory cut offs).<sup>1\u00a0<\/sup><\/li>\r\n    <\/ul>\r\n  <\/ol>\r\n  \r\n  <p><strong>Languages studied:<\/strong> English<\/p>\r\n\r\n  <p><strong>Groups tested with this measure:<\/strong><\/p>\r\n  <ul style=\"margin-bottom:1em\">\r\n \t<li>Type 1 VWD (index cases)<\/li>\r\n \t<li>Affected and unaffected family members of index cases<\/li>\r\n \t<li>Controls with no history of bleeding problems<\/li>\r\n \t<li>Type 2A, 2B, 2M VWD<\/li>\r\n \t<li>Type 3 VWD<\/li>\r\n \t<li>Type 3 VWD obligate carriers<\/li>\r\n \t<li>Adults referred for a bleeding disorder evaluation<\/li>\r\n <\/ul>\r\n\r\n  <p><strong>Age: <\/strong>Children and adults<\/p>\r\n\r\n  <p><a href=\"\/resource\/glossary-of-psychometric-properties\/\" target=\"_blank\" rel=\"noopener\"><button>Glossary of Psychometric Properties<\/button><\/a><\/p>\r\n\r\n<\/div>\r\n\r\n\r\n<div id=\"references\" class=\"popContent\" style=\"display:none\">\r\n  <h3>References<\/h3>        \r\n  <ol>\r\n   <li>Tosetto A et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD). J Thromb Haemost 2006; 4: 766-773.<\/li>\r\n \t<li>Eikenboom J, et al. Linkage analysis in families diagnosed with type 1 von Willebrand disease in the European study, molecular and clinical markers for the diagnosis and management of type 1 VWD. J Thromb Haemost 2006; 4: 774-782.<\/li>\r\n \t<li>Akin M et al. An evaluation of DDAVP infusion test with PFA-100 and vWF activity assays to distinguish vWD types in children. Clin Appl Thromb Hemost 2011; 17: 441-448.<\/li>\r\n \t<li>Castaman G et al. The impact of bleeding history, von Willebrand factor and PFA-100 on the diagnosis of type 1 von Willebrand disease: results from the European study MCMDM-1VWD. Br J Haematol 2010; 151: 245-251.<\/li>\r\n \t<li>de Wee EM et al. Effect of fibrinolysis on bleeding phenotype in moderate and severe von Willebrand disease. Haemophilia 2012; 18: 444-451.<\/li>\r\n \t<li>Federici AB et al. Efficacy and safety of highly purified, doubly virus-inactivated VWF\/FVIII concentrates in inherited von Willebrand\u2019s disease: results of an Italian cohort study on 120 patients characterized by bleeding severity score. Haemophilia 2010; 16: 101-110.<\/li>\r\n \t<li>Federici AB et al. Clinical and molecular predictors of thrombocytopenia and risk of bleeding in patients with von Willebrand disease type 2B: a cohort study of 67 patients. Blood 2009; 113(3): 526-34.<\/li>\r\n \t<li>Lotta LA et al. Prevalence of disease and relationships between laboratory phenotype and bleeding severity in platelet primary secretion defects. PLoS ONE 2013; 8: 1-6.<\/li>\r\n \t<li>Shamsakhzari S Hemorrhagic symptoms and bleeding risk in obligatory carriers of type 3 von Willebrand disease in southern Iran. Blood Coagul Fibrinolysis 2011; 22: 325-330.<\/li>\r\n \t<li>de Wee EM et al. Health-related quality of life among adult patients with moderate and severe von Willebrand disease. J Thromb Haemost 2010; 8: 1492-1499.<\/li>\r\n \t<li>Castaman G et al. Different bleeding risk in type 2A and 2M von Willebrand disease: a 2-year prospective study in 107 patients. J Thromb Haemost 2012; 10: 632-638.<\/li>\r\n \t<li>Rydz N, James P The evolution and value of bleeding assessment tools. J Thromb Haemost 2012; 10: 2223-2229.<\/li>\r\n  <\/ol>\r\n\r\n<\/div>\r\n\r\n\r\n\r\n\r\n\r\n\r\n<script>\r\nfunction openTab(evt, country) {\r\n  var i, popContent, popLinks;\r\n  popContent = document.getElementsByClassName(\"popContent\");\r\n  for (i = 0; i < popContent.length; i++) {\r\n    popContent[i].style.display = \"none\";\r\n  }\r\n  popLinks = document.getElementsByClassName(\"popLinks\");\r\n  for (i = 0; i < popLinks.length; i++) {\r\n    popLinks[i].className = popLinks[i].className.replace(\" active\", \"\");\r\n  }\r\n  document.getElementById(country).style.display = \"block\";\r\n  evt.currentTarget.className += \" active\";\r\n}\r\n<\/script>\r\n   \t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<div class=\"elementor-element elementor-element-297dea56 elementor-widget elementor-widget-spacer\" data-id=\"297dea56\" data-element_type=\"widget\" data-widget_type=\"spacer.default\">\n\t\t\t\t<div class=\"elementor-widget-container\">\n\t\t\t\t\t<div class=\"elementor-spacer\">\n\t\t\t<div class=\"elementor-spacer-inner\"><\/div>\n\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/div>\n\t\t\t\t\t<\/div>\n\t\t<\/section>\n\t\t\t\t<\/div>\n\t\t\n","protected":false},"featured_media":23154,"template":"","format":[],"old-keyword":[],"topic":[3994],"serie":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v23.4 - 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