Evidence map›Paper›PMID 42780071›Full record

ArticleResearch and practice in thrombosis and haemostasis2026

Genetic and hemostatic characterization of PAI-1 deficiency and isolated hyperfibrinolysis: data from the RBiN study.

Bauke Haisma, Thomas Nieuwenstein, Sanna R Rijpma, Annet Simons, Nicole M A Blijlevens, Waander L van Heerde, Saskia E M Schols

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Article in Research and practice in thrombosis and haemostasis, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Bauke HaismaDepartment of Hematology, Radboud University Medical Center, Nijmegen, the Netherlands.
Thomas NieuwensteinDepartment of Hematologie, Radboud University Medical Center, Hemophilia Treatment Center Nijmegen-Eindhoven-Maastricht, Nijmegen, the Netherlands.
Sanna R RijpmaDepartment of Hematologie, Radboud University Medical Center, Hemophilia Treatment Center Nijmegen-Eindhoven-Maastricht, Nijmegen, the Netherlands.
Annet SimonsDepartment of Hematologie, Radboud University Medical Center, Hemophilia Treatment Center Nijmegen-Eindhoven-Maastricht, Nijmegen, the Netherlands.
Nicole M A BlijlevensDepartment of Hematology, Radboud University Medical Center, Nijmegen, the Netherlands.
Waander L van HeerdeDepartment of Hematologie, Radboud University Medical Center, Hemophilia Treatment Center Nijmegen-Eindhoven-Maastricht, Nijmegen, the Netherlands.
Saskia E M ScholsDepartment of Hematology, Radboud University Medical Center, Nijmegen, the Netherlands.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Plasminogen activator inhibitor 1 (PAI-1) is a key regulator of fibrinolysis, and its deficiency causes bleeding symptoms. PAI-1 deficiency is rare, and pathogenic Objectives: To assess Methods: This substudy of the nationwide, cross-sectional RBiN cohort included patients with PAI-1 deficiency, isolated hyperfibrinolysis, and RCFDs. Results: Fourteen patients with PAI-1 deficiency, 15 with isolated hyperfibrinolysis, and 214 with RCFDs were included. Conclusion: PAI-1 deficiency and isolated hyperfibrinolysis showed enhanced plasmin generation, but were not linked to

Indexed as

Coagulation Protein DisordersFibrinolysisHemostasisPlasminogen Activator Inhibitor 1AdolescentAdultChildCross-Sectional StudiesFemaleFibrinolysinGenetic Predisposition to DiseaseHemorrhagic DisordersHumansMaleMiddle AgedPolymorphism, GeneticFibrinolysinPlasminogen Activator Inhibitor 1SERPINE1 protein, humanexomeexome sequencinggenetic polymorphismgenetic promoter regiongenotypeinherited blood coagulation disordersmutationplasminogen activator inhibitor-1 deficiency

Identifiers

PMID42780071
PMCPMC13597289

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.