Bleeding score in type 1 von Willebrand disease patients using the condensed MCMDM-1 vWD validated questionnaire

Anil Pathare, F. Al Hajri, S. Al Omrani, N. Al Obaidani, B. Al Balushi, K. Al Falahi

Research output: Contribution to journalArticle

Abstract

Background: Assessment of the severity of bleeding symptom has led to the evolution of bleeding assessment tools which are now validated. Aims: To administer the condensed molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease VWD (MCMDM-1 vWD) questionnaire to the Omani type 1 vWD patients and correlate it with the laboratory parameters. Methods: Patients and controls were personally interviewed and the condensed MCMDM-1 vWD questionnaire administered by a single investigator. Bleeding score (BS) was calculated, based on the presence or absence of the bleeding symptoms according to a standard validated questionnaire in both the patients and the controls. Results: The median age of the patient cohort was 27 (range, 7-49) years with 60.87% of females. The median time to administer condensed MCMDM-1 BS questionnaire was 11 minutes (interquartile range-IQR;7,16). Overall, bleeding from the oral cavity was the most predominant symptom (63%). The median BS was 5 (IQR;1,8) although individual scores ranged between 0 and 29. However, there was no statistically significant difference in BS between genders (males: median 4; IQR 1,6 and females: median 5, IQR 1,10) (P >.05, Kruskal-Wallis test) The Spearman's correlation value of BS was weak with FVIII:C levels and von Willebrand Ristocetin co-factor activity; very weak with von Willebrand Antigen level, and moderate with vonWillebrand Collagen Binding activity being -0.29, −0.28, −0.14 and −0.43, respectively. Conclusion: The BS reflects the severity of bleeding among the vWD patients. Although the BS was abnormal, it did not correlate significantly with the surrogate laboratory parameters [P >.05].

Original languageEnglish
Pages (from-to)515-520
Number of pages6
JournalInternational Journal of Laboratory Hematology
Volume40
Issue number5
DOIs
Publication statusPublished - Oct 1 2018

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Type 1 von Willebrand Disease
Biomarkers
Ristocetin
Hemorrhage
Collagen
Surveys and Questionnaires
von Willebrand Factor

Keywords

  • bleeding score
  • BS
  • MCMDM-1
  • Questionnaire
  • vWD

ASJC Scopus subject areas

  • Hematology
  • Clinical Biochemistry
  • Biochemistry, medical

Cite this

Bleeding score in type 1 von Willebrand disease patients using the condensed MCMDM-1 vWD validated questionnaire. / Pathare, Anil; Al Hajri, F.; Al Omrani, S.; Al Obaidani, N.; Al Balushi, B.; Al Falahi, K.

In: International Journal of Laboratory Hematology, Vol. 40, No. 5, 01.10.2018, p. 515-520.

Research output: Contribution to journalArticle

Pathare, Anil ; Al Hajri, F. ; Al Omrani, S. ; Al Obaidani, N. ; Al Balushi, B. ; Al Falahi, K. / Bleeding score in type 1 von Willebrand disease patients using the condensed MCMDM-1 vWD validated questionnaire. In: International Journal of Laboratory Hematology. 2018 ; Vol. 40, No. 5. pp. 515-520.
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abstract = "Background: Assessment of the severity of bleeding symptom has led to the evolution of bleeding assessment tools which are now validated. Aims: To administer the condensed molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease VWD (MCMDM-1 vWD) questionnaire to the Omani type 1 vWD patients and correlate it with the laboratory parameters. Methods: Patients and controls were personally interviewed and the condensed MCMDM-1 vWD questionnaire administered by a single investigator. Bleeding score (BS) was calculated, based on the presence or absence of the bleeding symptoms according to a standard validated questionnaire in both the patients and the controls. Results: The median age of the patient cohort was 27 (range, 7-49) years with 60.87{\%} of females. The median time to administer condensed MCMDM-1 BS questionnaire was 11 minutes (interquartile range-IQR;7,16). Overall, bleeding from the oral cavity was the most predominant symptom (63{\%}). The median BS was 5 (IQR;1,8) although individual scores ranged between 0 and 29. However, there was no statistically significant difference in BS between genders (males: median 4; IQR 1,6 and females: median 5, IQR 1,10) (P >.05, Kruskal-Wallis test) The Spearman's correlation value of BS was weak with FVIII:C levels and von Willebrand Ristocetin co-factor activity; very weak with von Willebrand Antigen level, and moderate with vonWillebrand Collagen Binding activity being -0.29, −0.28, −0.14 and −0.43, respectively. Conclusion: The BS reflects the severity of bleeding among the vWD patients. Although the BS was abnormal, it did not correlate significantly with the surrogate laboratory parameters [P >.05].",
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AU - Pathare, Anil

AU - Al Hajri, F.

AU - Al Omrani, S.

AU - Al Obaidani, N.

AU - Al Balushi, B.

AU - Al Falahi, K.

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AB - Background: Assessment of the severity of bleeding symptom has led to the evolution of bleeding assessment tools which are now validated. Aims: To administer the condensed molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease VWD (MCMDM-1 vWD) questionnaire to the Omani type 1 vWD patients and correlate it with the laboratory parameters. Methods: Patients and controls were personally interviewed and the condensed MCMDM-1 vWD questionnaire administered by a single investigator. Bleeding score (BS) was calculated, based on the presence or absence of the bleeding symptoms according to a standard validated questionnaire in both the patients and the controls. Results: The median age of the patient cohort was 27 (range, 7-49) years with 60.87% of females. The median time to administer condensed MCMDM-1 BS questionnaire was 11 minutes (interquartile range-IQR;7,16). Overall, bleeding from the oral cavity was the most predominant symptom (63%). The median BS was 5 (IQR;1,8) although individual scores ranged between 0 and 29. However, there was no statistically significant difference in BS between genders (males: median 4; IQR 1,6 and females: median 5, IQR 1,10) (P >.05, Kruskal-Wallis test) The Spearman's correlation value of BS was weak with FVIII:C levels and von Willebrand Ristocetin co-factor activity; very weak with von Willebrand Antigen level, and moderate with vonWillebrand Collagen Binding activity being -0.29, −0.28, −0.14 and −0.43, respectively. Conclusion: The BS reflects the severity of bleeding among the vWD patients. Although the BS was abnormal, it did not correlate significantly with the surrogate laboratory parameters [P >.05].

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