{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T14:45:31Z","timestamp":1787496331709,"version":"build-2736575974"},"reference-count":35,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2018,8,23]],"date-time":"2018-08-23T00:00:00Z","timestamp":1534982400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Shire Endowment Fund for Training in Pediatric Hemostasis"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2018,10]]},"abstract":"Summary<\/jats:title>\n \n Acquired von Willebrand syndrome (\n AVWS<\/jats:styled-content>\n ) is reported in high\u2010flow high\u2010shear congenital cardiac disorders. We hypothesized that the narrowed pulmonary vasculature in idiopathic pulmonary arterial hypertension (\n IPAH<\/jats:styled-content>\n ) may induce\n AVWS<\/jats:styled-content>\n . We conducted a cross\u2010sectional evaluation of children with\n IPAH<\/jats:styled-content>\n . Patients with bleeding symptoms and\/or laboratory abnormalities (thrombocytopenia, anomalies in coagulation screening tests) were tested in\u2010depth for haemostatic defects. Fourteen children were followed with\n IPAH<\/jats:styled-content>\n of which 8 were eligible. Four children exhibited abnormal bleeding scores (International Society on Thrombosis and Haemostasis Bleeding Assessment Tool: 3\u20135). All 8 patients showed very prolonged platelet function analyser (\n PFA<\/jats:styled-content>\n )\u2010100 closure times. Six children demonstrated either mild thrombocytopenia or low\u2010normal von Willebrand factor (\n VWF<\/jats:styled-content>\n ) antigen (\n VWF<\/jats:styled-content>\n :Ag) or\n VWF<\/jats:styled-content>\n activity [mean (range), in iu\/dl:\n VWF<\/jats:styled-content>\n :Ag: 70 (61\u201391);\n VWF<\/jats:styled-content>\n activity: 57 (34\u201370)]. Average\n VWF<\/jats:styled-content>\n collagen binding capacity (\n VWF<\/jats:styled-content>\n :\n CB<\/jats:styled-content>\n ) was 64 iu\/dl (range: 53\u2013123 iu\/dl), with low\u2010normal\n VWF<\/jats:styled-content>\n activity\/\n VWF<\/jats:styled-content>\n :Ag or\n VWF<\/jats:styled-content>\n :\n CB<\/jats:styled-content>\n \/\n VWF<\/jats:styled-content>\n :Ag ratios occurring in five patients. All children had normal multimers distribution patterns. One patient underwent a lung transplantation, with normalization of haemostatic abnormalities post\u2010surgery. Overall, 8 out of 14 children with\n IPAH<\/jats:styled-content>\n had mild to moderate bleeding symptoms and\/or laboratory abnormalities in keeping with\n AVWS<\/jats:styled-content>\n . Normalization of the haemostatic defects following lung transplantation and lack of family history of bleeding attests to the acquired nature of their defects.\n <\/jats:p>","DOI":"10.1111\/bjh.15530","type":"journal-article","created":{"date-parts":[[2018,8,23]],"date-time":"2018-08-23T20:55:31Z","timestamp":1535057731000},"page":"267-275","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Idiopathic pulmonary arterial hypertension \u2013 a unrecognized cause of high\u2010shear high\u2010flow haemostatic defects (otherwise referred to as acquired von Willebrand syndrome) in children"],"prefix":"10.1111","volume":"183","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2364-7205","authenticated-orcid":false,"given":"Marie\u2010Claude","family":"Pelland\u2010Marcotte","sequence":"first","affiliation":[{"name":"Division of Haematology\/Oncology Department of Paediatrics The Hospital for Sick Children and University of 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