High prevalence of bleeders of unknown cause among patients with inherited mucocutaneous bleeding. A prospective study of 280 patients and 299 controls

Simple item page
dc.contributor.authorQuiroga, Teresa
dc.contributor.authorGoycoolea, Manuela
dc.contributor.authorPanes, Olga
dc.contributor.authorAranda, Eduardo
dc.contributor.authorMartinez, Carlos
dc.contributor.authorBelmont, Sabine
dc.contributor.authorMunoz, Blanca
dc.contributor.authorZuniga, Pamela
dc.contributor.authorPereira, Jaime
dc.contributor.authorMezzano, Diego
dc.date.accessioned2024-01-10T12:05:49Z
dc.date.available2024-01-10T12:05:49Z
dc.date.issued2007
dc.description.abstractBackground and Objectives
dc.description.abstractMucocutaneous bleeding (MCB) is the main expression of inherited disorders of primary hemostasis. However, the relative prevalence of these disorders, their clinical differential diagnosis, and the proportion of patients with MCB of unknown cause (BUC) after an initial comprehensive laboratory testing are unknown.
dc.description.abstractDesign and Methods
dc.description.abstractWe studied prospectively 280 consecutive patients with MCB and 299 matched controls, using strict inclusion and exclusion criteria. A single physician recorded the clinical data in a bleeding score and estimated the severity of bleeding in clinical categories. Laboratory criteria for the diagnosis of von Willebrand's disease (VWD) and platelet function defects were established from reference values derived from controls.
dc.description.abstractResults
dc.description.abstractFifty patients (17.9%) had VWD (type 1VWD=45, type 2=5). Platelet function defects and mild clotting factor deficiencies were found in 65 (23.2%) and 11 (3.9%) patients, respectively. Thirteen (11.5%) patients had combined defects. The remaining 167(59.6%) patients had BUC, with prolonged bleeding time in 18.6% as their only abnormality. All these disorders, including BUC, were clinically undistinguishable. Moreover, no relationship was found between the severity of bleeding and VWF/platelet function variables.
dc.description.abstractInterpretation and Conclusions
dc.description.abstractThe diagnostic efficacy of a first laboratory testing in patients with hereditary MCB is 40.4%. Most patients have a disease(s) of high prevalence but unknown pathogenesis. Concurrent bleeding disorders in the same patient are frequent. Our results support the proposal that low plasma VWF levels, but also platelet function defects, should be considered risk factors rather than unequivocal causes of hemorrhages.
dc.description.funderFondecyt, Chile
dc.fechaingreso.objetodigital2024-05-23
dc.format.extent9 páginas
dc.fuente.origenWOS
dc.identifier.doi10.3324/haematol.10816
dc.identifier.issn0390-6078
dc.identifier.pubmedidMEDLINE:17339185
dc.identifier.urihttps://doi.org/10.3324/haematol.10816
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/76074
dc.identifier.wosidWOS:000506914200017
dc.information.autorucMedicina;Mezzano D;S/I;99455
dc.information.autorucMedicina;Pereira J;S/I;99371
dc.issue.numero3
dc.language.isoen
dc.nota.accesocontenido parcial
dc.pagina.final365
dc.pagina.inicio357
dc.publisherFERRATA STORTI FOUNDATION
dc.revistaHAEMATOLOGICA
dc.rightsacceso restringido
dc.subjectbleeding of unknown cause
dc.subjectdiagnosis of inherited disorders of primary hemostasis
dc.subjectmucous and skin bleeding
dc.subjectmild bleeding disorders
dc.subjectplatelet function study
dc.subjectrelative frequency of disorders of primary hemostasis
dc.subjectVON-WILLEBRAND-DISEASE
dc.subjectSTORAGE POOL DEFICIENCY
dc.subjectABO BLOOD-GROUP
dc.subjectPLATELET-FUNCTION
dc.subjectFACTOR-XI
dc.subjectDIAGNOSIS
dc.subjectTIME
dc.subjectTYPE-1
dc.subjectPROSTACYCLIN
dc.subjectPFA-100(R)
dc.subject.ods03 Good Health and Well-being
dc.subject.odspa03 Salud y bienestar
dc.titleHigh prevalence of bleeders of unknown cause among patients with inherited mucocutaneous bleeding. A prospective study of 280 patients and 299 controls
dc.typeartículo
dc.volumen92
sipa.codpersvinculados99455
sipa.codpersvinculados99371
sipa.indexWOS
sipa.indexScopus
sipa.trazabilidadCarga SIPA;09-01-2024
Files
Original bundle
Now showing 1 - 1 of 1
Thumbnail Image
Name:
2024-05-23. High prevalence of bleeders of unknown cause among patients with inherited mucocutaneous bleeding. A prospective study of 280 patients and 299 controls.pdf
Size:
5.02 KB
Format:
Adobe Portable Document Format
Description:
Download
Collections
Artículos de revistas