Incidence of von Willebrand disease in Denmark, 1995‐2016: A cohort study

Laursen, Anne Sofie Dam; Rasmussen, Torben Riis; Chiu, Gretchen R.; Brouwer, Emily; Poulsen, Lone Hvitfeldt; Mikkelsen, Ellen M

doi:10.1111/hae.14257

Cited by 4 publications

(5 citation statements)

References 19 publications

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“…Like haemophilia, 9 the prevalence at birth shows promise in estimating the 'true' prevalence of VWD. 24,25 However, due difficulty in diagnosing type 1 VWD, social/cultural issues and the variability in VWD prevalence (Table 6), we are not able to identify the 'true' prevalence of VWD that would aid in healthcare planning at this moment. Enhancing awareness, diagnostic capabilities of VWD, and better data collection systems will go a long way towards addressing the 'true' prevalence of VWD.…”

Section: Discussionmentioning

confidence: 96%

“…However, we are not certain if the type 3 VWD prevalence will continue to grow resulting in the difficulty of establishing a 'true' prevalence. Like haemophilia, 9 the prevalence at birth shows promise in estimating the 'true' prevalence of VWD 24,25 . However, due difficulty in diagnosing type 1 VWD, social/cultural issues and the variability in VWD prevalence (Table 6), we are not able to identify the 'true' prevalence of VWD that would aid in healthcare planning at this moment.…”

Section: Discussionmentioning

confidence: 99%

“…Comparison of prevalence of von Willebrand disease (VWD) for all types by method/source and income classification.Note 1: The prevalence at birth estimates for Denmark were determined by dividing the annual number of people born with VWD (see Supplemental TableS3in the Supporting Information of24 by the annual number of live births (https://www.statbank.dk/statbank5a) for 2007−2016. N-number of countries, Income Classifications: High income countries (HIC), upper middle income countries (UMIC), lower middle income countries (LMIC), and low income countries (LIC).…”

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confidence: 99%

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Reported prevalence of von Willebrand disease worldwide in relation to income classification

et al. 2023

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Introduction:The diagnosis of von Willebrand disease (VWD) is complex and challenging, especially when diagnostic resources are limited. This results in a lack of consistency in identifying and reporting the number of people with VWD and variations in the VWD prevalence worldwide. Aim:To analyze the reported prevalence of VWD worldwide in relation to income classification.Methods: Data on the VWD prevalence from the World Federation of Hemophilia Annual Global Survey, national registries of Australia, Canada, and the United Kingdom, and the literature were analysed. The income level of each country was classified according to the World Bank. Results:The mean VWD prevalence worldwide was 25.6 per million people. The VWD prevalence for high-income countries (HIC) of 60.3 per million people was significantly greater (p < .01) than upper middle (12.6), lower middle (2.5) and low (1.1) income countries. The type 3 VWD prevalence for HIC of 3.3 per million people was significantly greater (p < .01) than lower middle (1.3) and low income (0.7) countries. The reported VWD prevalence was greater among females than males. Conclusion:The reported VWD prevalence varied considerably across and within income classifications. The variability of type 3 VWD prevalence was less than the VWD prevalence (all types). The variability in detection and diagnosis of type 1 VWD presents a challenge in forming a consistent prevalence value across countries and income classifications.

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Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Reported prevalence of von Willebrand disease worldwide in relation to income classification

et al. 2023

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“…1 Depending on the population studied, the prevalence of VWD has been reported to vary between 1% and less than 0.01% of people. [2][3][4] Similarly, the prevalence of hemophilia carriership is 1 in 3,000 and that of rare, severe inherited bleeding disorders is 1 per 500,000 to 1 per 2 million. 5,6 Although the range of the various inherited bleeding disorders is discussed briefly, this review focuses primarily on the maternal and fetal implications of VWD and hemophilia, with the objective of providing clinicians a practical clinical approach to the management of these conditions during pregnancy and the postpartum period.…”

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confidence: 99%

Von Willebrand Disease, Hemophilia, and Other Inherited Bleeding Disorders in Pregnancy

Pacheco

Saade

James

2023

Obstetrics &Amp; Gynecology

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Inherited bleeding disorders, which comprise von Willebrand disease (VWD), hemophilia, other congenital clotting factor deficiencies, inherited platelet disorders, defects of fibrinolysis, and connective tissue disorders, have both maternal and fetal implications. Although mild platelet defects may actually be more prevalent, the most common diagnosed bleeding disorder among women is VWD. Other bleeding disorders, including hemophilia carriership, are much less common, but hemophilia carriers are unique in that they are at risk of giving birth to a severely affected male neonate. General guidance for maternal management of inherited bleeding disorders includes obtaining clotting factor levels in the third trimester, planning for delivery at a center with hemostasis expertise if factor levels do not meet the minimum threshold (eg, less than 0.50 international units/1 mL [50%] for von Willebrand factor, factor VIII, or factor IX), and using hemostatic agents such as factor concentrates, desmopressin, or tranexamic acid. General guidance for fetal management includes prepregnancy counseling, the option of preimplantation genetic testing for hemophilia, and consideration of delivery of potentially affected male neonates with hemophilia by cesarean delivery to reduce the risk of neonatal intracranial hemorrhage. In addition, delivery of possibly affected neonates should occur in a facility where there is newborn intensive care and pediatric hemostasis expertise. For patients with other inherited bleeding disorders, unless a severely affected neonate is anticipated, mode of delivery should be dictated by obstetric indications. Nonetheless, invasive procedures such as fetal scalp clip or operative vaginal delivery should be avoided, if possible, in any fetus potentially affected with a bleeding disorder.

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“…In the article by Laursen et al, 1 time at risk is expressed in 100,000 person‐years instead of 1,000,000 person‐years throughout the article. Hence, all estimated incidence rates are stated per 100,000 person‐years instead of 1,000,000 person‐years.…”

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confidence: 99%

2022

Haemophilia

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Incidence of von Willebrand disease in Denmark, 1995‐2016: A cohort study

Cited by 4 publications

References 19 publications

Reported prevalence of von Willebrand disease worldwide in relation to income classification

Reported prevalence of von Willebrand disease worldwide in relation to income classification

Von Willebrand Disease, Hemophilia, and Other Inherited Bleeding Disorders in Pregnancy

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