Matches in SemOpenAlex for { <https://semopenalex.org/work/W2982716574> ?p ?o ?g. }
Showing items 1 to 88 of
88
with 100 items per page.
- W2982716574 abstract "Hereditary angioedema with normal C1 inhibitor (HAE-nC1 INH) is a rare, underappreciated condition characterized by recurrent subcutaneous angioedema. The underlying pathophysiology and diagnostic criteria continues to evolve. There is a significant overlap between HAE-nC1 INH and idiopathic nonhistaminergic angioedema, ultimately this may be found to be the same condition. Characterization of cohorts suspected to have either of these conditions is warranted to help refine diagnosis, pathophysiology, and treatment response.A retrospective chart review of 418 patients diagnosed with angioedema was conducted. The following inclusion criteria was used: lack of response to antihistamines, steroids, and epinephrine; normal C4, C1 inhibitor (C1 INH) level and function; lack of urticaria or pruritus; occurrence without offending drugs; and positive family history. Enzyme immunoassays for C1 INH function were performed at the Mayo Clinic. Charts meeting these criteria were reviewed for frequency and type of episodes as well as use and response to therapies.6 patients met the above criteria. 3 of these completed genetic testing, none were found to have factor XII abnormalities. None had angiopoietin 1 or plasminogen gene sequencing. 5 of 6 patients were successfully treated with C1 INH or tranexamic acid for acute treatment of attacks (4 with C1 INH and 1 with tranexamic acid). 4 patients have used Icatibant with good response (typically under 40 min for near full recovery); of these, 3 required Icatibant as acute treatment after other therapies (C1 inhibitor and tranexamic acid) were ineffective. There were 9 patients who otherwise met criteria, but due to a lack of family history were classified as having idiopathic non-histaminergic angioedema.This retrospective chart review found 6 HAE-nC1 INH patients in Manitoba. 1 responded to tranexamic acid and not C1 INH, 4 typically responded to C1 INH, and 1 responded exclusively to Icatibant. All patients-4 total-who used Icatibant responded; of these 4 patients, 3 required Icatibant after other therapies had failed." @default.
- W2982716574 created "2019-11-22" @default.
- W2982716574 creator A5034040285 @default.
- W2982716574 creator A5034593049 @default.
- W2982716574 creator A5050435955 @default.
- W2982716574 creator A5065328602 @default.
- W2982716574 date "2019-11-12" @default.
- W2982716574 modified "2023-10-14" @default.
- W2982716574 title "Review of the Manitoba cohort of patients with hereditary angioedema with normal C1 inhibitor" @default.
- W2982716574 cites W1919376524 @default.
- W2982716574 cites W2067723030 @default.
- W2982716574 cites W2125142944 @default.
- W2982716574 cites W2787815450 @default.
- W2982716574 doi "https://doi.org/10.1186/s13223-019-0381-y" @default.
- W2982716574 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6852747" @default.
- W2982716574 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/31749860" @default.
- W2982716574 hasPublicationYear "2019" @default.
- W2982716574 type Work @default.
- W2982716574 sameAs 2982716574 @default.
- W2982716574 citedByCount "8" @default.
- W2982716574 countsByYear W29827165742020 @default.
- W2982716574 countsByYear W29827165742021 @default.
- W2982716574 countsByYear W29827165742022 @default.
- W2982716574 countsByYear W29827165742023 @default.
- W2982716574 crossrefType "journal-article" @default.
- W2982716574 hasAuthorship W2982716574A5034040285 @default.
- W2982716574 hasAuthorship W2982716574A5034593049 @default.
- W2982716574 hasAuthorship W2982716574A5050435955 @default.
- W2982716574 hasAuthorship W2982716574A5065328602 @default.
- W2982716574 hasBestOaLocation W29827165741 @default.
- W2982716574 hasConcept C10162356 @default.
- W2982716574 hasConcept C126322002 @default.
- W2982716574 hasConcept C141071460 @default.
- W2982716574 hasConcept C16005928 @default.
- W2982716574 hasConcept C170493617 @default.
- W2982716574 hasConcept C2777416354 @default.
- W2982716574 hasConcept C2777788586 @default.
- W2982716574 hasConcept C2777995640 @default.
- W2982716574 hasConcept C2778211319 @default.
- W2982716574 hasConcept C2779522080 @default.
- W2982716574 hasConcept C2779591629 @default.
- W2982716574 hasConcept C2779637338 @default.
- W2982716574 hasConcept C2779931649 @default.
- W2982716574 hasConcept C2781179581 @default.
- W2982716574 hasConcept C2991743468 @default.
- W2982716574 hasConcept C71924100 @default.
- W2982716574 hasConcept C90924648 @default.
- W2982716574 hasConceptScore W2982716574C10162356 @default.
- W2982716574 hasConceptScore W2982716574C126322002 @default.
- W2982716574 hasConceptScore W2982716574C141071460 @default.
- W2982716574 hasConceptScore W2982716574C16005928 @default.
- W2982716574 hasConceptScore W2982716574C170493617 @default.
- W2982716574 hasConceptScore W2982716574C2777416354 @default.
- W2982716574 hasConceptScore W2982716574C2777788586 @default.
- W2982716574 hasConceptScore W2982716574C2777995640 @default.
- W2982716574 hasConceptScore W2982716574C2778211319 @default.
- W2982716574 hasConceptScore W2982716574C2779522080 @default.
- W2982716574 hasConceptScore W2982716574C2779591629 @default.
- W2982716574 hasConceptScore W2982716574C2779637338 @default.
- W2982716574 hasConceptScore W2982716574C2779931649 @default.
- W2982716574 hasConceptScore W2982716574C2781179581 @default.
- W2982716574 hasConceptScore W2982716574C2991743468 @default.
- W2982716574 hasConceptScore W2982716574C71924100 @default.
- W2982716574 hasConceptScore W2982716574C90924648 @default.
- W2982716574 hasIssue "1" @default.
- W2982716574 hasLocation W29827165741 @default.
- W2982716574 hasLocation W29827165742 @default.
- W2982716574 hasLocation W29827165743 @default.
- W2982716574 hasLocation W29827165744 @default.
- W2982716574 hasLocation W29827165745 @default.
- W2982716574 hasLocation W29827165746 @default.
- W2982716574 hasOpenAccess W2982716574 @default.
- W2982716574 hasPrimaryLocation W29827165741 @default.
- W2982716574 hasRelatedWork W1495135135 @default.
- W2982716574 hasRelatedWork W2038179462 @default.
- W2982716574 hasRelatedWork W2067408146 @default.
- W2982716574 hasRelatedWork W2330139527 @default.
- W2982716574 hasRelatedWork W2897256132 @default.
- W2982716574 hasRelatedWork W2982716574 @default.
- W2982716574 hasRelatedWork W3125263654 @default.
- W2982716574 hasRelatedWork W3131722049 @default.
- W2982716574 hasRelatedWork W4245068413 @default.
- W2982716574 hasRelatedWork W4323041592 @default.
- W2982716574 hasVolume "15" @default.
- W2982716574 isParatext "false" @default.
- W2982716574 isRetracted "false" @default.
- W2982716574 magId "2982716574" @default.
- W2982716574 workType "article" @default.