Inherited coagulation disorders in Turkish children: A retrospective, single-center cohort study

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dc.contributor.authorOner, Nergiz
dc.contributor.authorGursel, Turkiz
dc.contributor.authorKaya, Zuhre
dc.contributor.authorKeskin, Ebru Yilmaz
dc.contributor.authorKocak, Ulker
dc.contributor.authorAlbayrak, Meryem
dc.contributor.authorYenicesu, Idil
dc.date.accessioned2021-01-14T18:10:36Z
dc.date.available2021-01-14T18:10:36Z
dc.date.issued2020
dc.departmentKKÜ
dc.descriptionAlbayrak, Meryem/0000-0003-2711-5150
dc.description.abstractObjective: This study aims to investigate the distribution, clinical characteristics and outcome of inherited coagulation disorders (ICD) in Turkish children. Subjects and methods: Data from all children (age < 18 years) with ICD examined in our center were retrospectively reviewed. Results: There were 403 children with ICD (233 males and 170 females) with a median age of four years at diagnosis. The percentages of von Willebrand disease (vWd), hemophilia and rare bleeding disorders (RBD) were 40 %, 34 % and 26 %, type-1, type-2 and type-3 vWd were 63 % 17 % and 20 %, hemophilia A and B were 84 % and 16 %, and severe, moderate and mild hemophilia were 48 %, 30 % and 22 %, respectively. Factor VII and FXI deficiencies were the most prevalent, comprising 56 % and 22 % of all children with RBD, respectively. Parental consanguinity rates were 72 % in type-3 vWd and 61 % in severe RBD. The overall prevalence of gastrointestinal bleedings was 4.5 % (18/403), intracranial bleeding (ICB) was 4.96 % (20/403), mortality from ICB was 30 % (6/20) and the overall mortality rate was 1.49 % (6/403). No life-threatening bleeding was seen during regular prophylaxis. Chronic arthropathy prevalence in severe hemophilia was 8 % with primary prophylaxis and 53 % with demand therap. Inhibitor prevalence was 14 % in hemophilia-A and 5 % in hemophiliaB. Conclusions: These data show that vWd is the most common ICD, type-3 vWd and RBD are prevalent due to frequent consanguineous marriages and diagnosis of ICD is substantially delayed in Turkish children. Prophylactic replacement therapy prevents occurrence of life-threatening bleedings and reduces the development of hemophilic arthropathy.en_US
dc.identifier.citationBu makale açık erişimli değildir.en_US
dc.identifier.doi10.1016/j.transci.2020.102728
dc.identifier.issn1473-0502
dc.identifier.issue3en_US
dc.identifier.pmid31980335
dc.identifier.scopus2-s2.0-85078638519
dc.identifier.scopusqualityQ3
dc.identifier.urihttps://doi.org/10.1016/j.transci.2020.102728
dc.identifier.urihttps://hdl.handle.net/20.500.12587/12689
dc.identifier.volume59en_US
dc.identifier.wosWOS:000538124200008
dc.identifier.wosqualityQ4
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherPERGAMON-ELSEVIER SCIENCE LTDen_US
dc.relation.ispartofTRANSFUSION AND APHERESIS SCIENCE
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectInherited coagulation disordersen_US
dc.subjectInherited bleeding disordersen_US
dc.subjectChildrenen_US
dc.subjectTurkeyen_US
dc.titleInherited coagulation disorders in Turkish children: A retrospective, single-center cohort studyen_US
dc.typeArticle

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