Large Renal Arteriovenous Malformation in a Patient With von Willebrand Disease

Schlaepfer, Charles; Weese, Jonathan; Picus, Daniel; Venkatesh, Ramakrishna

doi:10.1016/j.urology.2019.04.024

Cited by 1 publication

(1 citation statement)

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“…Our search identified 10,019 unique studies. After screening by title and abstract, and full‐text review, 61 studies were included in the review of which 54 had patient‐level data comprising 146 patients 8,10,15–66 and nine reported data usable at the population or survey level 9,11,19,51,67–71 (Figure 1). There were no new studies for inclusion identified by review of reference lists.…”

Section: Resultsmentioning

confidence: 99%

Vascular abnormalities in patients with von Willebrand disease: A scoping review

Chornenki

Shanjer

James

2021

Journal of Thrombosis and Haemostasis

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Background: Qualitative or quantitative defects of von Willebrand factor (VWF) such as in von Willebrand disease (VWD) are associated with vascular abnormalities, especially in the gastrointestinal (GI) tract. However, the locations, extent, and natural history of vascular abnormalities in patients with VWD is not well understood. To summarize the existing literature on the topic, we conducted a scoping review of vascular abnormalities in patients with VWD. Methods:We searched MEDLINE and EMBASE from inception to September 1, 2020, for studies clinically describing vascular abnormalities in VWD patients. Screening and data extraction was completed independently and in duplicate and each abnormality was documented individually.Results: After screening, 54 studies that reported patient level data comprising 146 patients were included. Type 2A (39%) and type 3 (14.4%) were the most common VWD subtypes. The most common site of vascular malformation was the GI tract, occurring in 124 patients (84.9%), whereas 18 (12.3%) had non-GI vascular abnormalities and 4 (2.7%) had both GI and non-GI vascular abnormalities. With respect to outcomes, the clinical course was not specified in the majority (55.5%) of patients.Survey and population level data were reported in nine studies, demonstrating vascular abnormalities occurred at higher rates in VWD and that VWD patients are overrepresented among those with those abnormalities. Conclusion:Vascular malformations in patients with VWD occur primarily in the gastrointestinal tract. Type 2A and type 3 VWD are the most common subtypes affected.The clinical treatment and natural history of these abnormalities remains understudied and further research is needed.

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Section: Resultsmentioning

confidence: 99%