Matches in SemOpenAlex for { <https://semopenalex.org/work/W3120400276> ?p ?o ?g. }
Showing items 1 to 96 of
96
with 100 items per page.
- W3120400276 endingPage "211" @default.
- W3120400276 startingPage "211" @default.
- W3120400276 abstract "Background: Congenital factor (F) VII deficiency is a rare coagulation factor deficiency with an estimated incidence of 1 per 500,000 individuals. Patients with severe FVII deficiency present a broad range of clinical presentations. Alloimmunization against exogenous FVII, as the main challenge of replacement therapy, is an extremely rare phenomenon that is accompanied by a high rate of life-threatening bleeding, that renders replacement therapy less effective. Due to the importance of the issue, we performed a systematic literature review in order to assess incidence, molecular basis, clinical presentations, and therapeutic challenge and management of inhibitor in congenital FVII deficiency. Strategy of search: This systematic review was performed in accordance with PRISMA guidelines. We performed an English-language literature review in the PubMed, EMBASE, Scopus, and Google Scholar databases, using the following keywords: “factor VII inhibitor”, “factor VII inhibitors”, “FVII inhibitors”, “congenital FVII deficiency”, “recombinant factor VII”, “anti rFVIIa”, “replacement therapy”, and “alloantibody”. Results: Out of 380 patients in the 13 studies, 27 had inhibitor against FVII; 18 were male, 7 were female, while the sex of 2 was not stated. The majority (92%) developed a high-titer inhibitor (Bethesda Unit > 5). All patients had severe FVII deficiency (FVII:C < 10%), and the majority received recombinant FVII prior to inhibitor development (N: 24, 89%). Among ten patients with a detected mutation, three subjects had a common non-sense (30%), and two had a deletion (20%). Conclusions: Inhibitor development is a relatively rare phenomenon seen only in severe FVII deficiency, where it is associated with severe and life-threatening presentations, treatment challenge, and economic burden on the patients and their families." @default.
- W3120400276 created "2021-01-18" @default.
- W3120400276 creator A5065243230 @default.
- W3120400276 creator A5083088838 @default.
- W3120400276 creator A5086037026 @default.
- W3120400276 creator A5089891465 @default.
- W3120400276 date "2021-01-08" @default.
- W3120400276 modified "2023-10-10" @default.
- W3120400276 title "Inhibitor in Congenital Factor VII Deficiency; a Rare but Serious Therapeutic Challenge—A Systematic Literature Review" @default.
- W3120400276 cites W1608427014 @default.
- W3120400276 cites W1608715949 @default.
- W3120400276 cites W1980377877 @default.
- W3120400276 cites W1987485262 @default.
- W3120400276 cites W2008256582 @default.
- W3120400276 cites W2017506858 @default.
- W3120400276 cites W2021075966 @default.
- W3120400276 cites W2023769918 @default.
- W3120400276 cites W2046072910 @default.
- W3120400276 cites W2070212236 @default.
- W3120400276 cites W2090413445 @default.
- W3120400276 cites W2097530615 @default.
- W3120400276 cites W2098837289 @default.
- W3120400276 cites W2118222502 @default.
- W3120400276 cites W2128487729 @default.
- W3120400276 cites W2134999939 @default.
- W3120400276 cites W2145248188 @default.
- W3120400276 cites W2147887043 @default.
- W3120400276 cites W2412454978 @default.
- W3120400276 cites W2510048745 @default.
- W3120400276 cites W2770475456 @default.
- W3120400276 cites W2905937465 @default.
- W3120400276 cites W2969431747 @default.
- W3120400276 cites W3043667682 @default.
- W3120400276 doi "https://doi.org/10.3390/jcm10020211" @default.
- W3120400276 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/7827513" @default.
- W3120400276 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/33435610" @default.
- W3120400276 hasPublicationYear "2021" @default.
- W3120400276 type Work @default.
- W3120400276 sameAs 3120400276 @default.
- W3120400276 citedByCount "7" @default.
- W3120400276 countsByYear W31204002762021 @default.
- W3120400276 countsByYear W31204002762022 @default.
- W3120400276 countsByYear W31204002762023 @default.
- W3120400276 crossrefType "journal-article" @default.
- W3120400276 hasAuthorship W3120400276A5065243230 @default.
- W3120400276 hasAuthorship W3120400276A5083088838 @default.
- W3120400276 hasAuthorship W3120400276A5086037026 @default.
- W3120400276 hasAuthorship W3120400276A5089891465 @default.
- W3120400276 hasBestOaLocation W31204002761 @default.
- W3120400276 hasConcept C120665830 @default.
- W3120400276 hasConcept C121332964 @default.
- W3120400276 hasConcept C126322002 @default.
- W3120400276 hasConcept C187212893 @default.
- W3120400276 hasConcept C2778382381 @default.
- W3120400276 hasConcept C2778961111 @default.
- W3120400276 hasConcept C2780930249 @default.
- W3120400276 hasConcept C61511704 @default.
- W3120400276 hasConcept C66112548 @default.
- W3120400276 hasConcept C71924100 @default.
- W3120400276 hasConcept C89560881 @default.
- W3120400276 hasConcept C90924648 @default.
- W3120400276 hasConceptScore W3120400276C120665830 @default.
- W3120400276 hasConceptScore W3120400276C121332964 @default.
- W3120400276 hasConceptScore W3120400276C126322002 @default.
- W3120400276 hasConceptScore W3120400276C187212893 @default.
- W3120400276 hasConceptScore W3120400276C2778382381 @default.
- W3120400276 hasConceptScore W3120400276C2778961111 @default.
- W3120400276 hasConceptScore W3120400276C2780930249 @default.
- W3120400276 hasConceptScore W3120400276C61511704 @default.
- W3120400276 hasConceptScore W3120400276C66112548 @default.
- W3120400276 hasConceptScore W3120400276C71924100 @default.
- W3120400276 hasConceptScore W3120400276C89560881 @default.
- W3120400276 hasConceptScore W3120400276C90924648 @default.
- W3120400276 hasIssue "2" @default.
- W3120400276 hasLocation W31204002761 @default.
- W3120400276 hasLocation W31204002762 @default.
- W3120400276 hasLocation W31204002763 @default.
- W3120400276 hasOpenAccess W3120400276 @default.
- W3120400276 hasPrimaryLocation W31204002761 @default.
- W3120400276 hasRelatedWork W1969903104 @default.
- W3120400276 hasRelatedWork W2016184368 @default.
- W3120400276 hasRelatedWork W2087571456 @default.
- W3120400276 hasRelatedWork W2091908750 @default.
- W3120400276 hasRelatedWork W2100367826 @default.
- W3120400276 hasRelatedWork W2118814044 @default.
- W3120400276 hasRelatedWork W2184660501 @default.
- W3120400276 hasRelatedWork W2411441380 @default.
- W3120400276 hasRelatedWork W2515988201 @default.
- W3120400276 hasRelatedWork W4320726907 @default.
- W3120400276 hasVolume "10" @default.
- W3120400276 isParatext "false" @default.
- W3120400276 isRetracted "false" @default.
- W3120400276 magId "3120400276" @default.
- W3120400276 workType "article" @default.