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Sökning: WFRF:(Stichel D.) > (2017) > Anti-factor VIII an...

  • Kahle, J.University Hospital Frankfurt (författare)

Anti-factor VIII antibodies in brothers with haemophilia A share similar characteristics

  • Artikel/kapitelEngelska2017

Förlag, utgivningsår, omfång ...

  • 2016-11-08
  • Wiley,2017

Nummerbeteckningar

  • LIBRIS-ID:oai:lup.lub.lu.se:da4c2432-4f11-4792-9e2b-b89165aadc90
  • https://lup.lub.lu.se/record/da4c2432-4f11-4792-9e2b-b89165aadc90URI
  • https://doi.org/10.1111/hae.13105DOI

Kompletterande språkuppgifter

  • Språk:engelska
  • Sammanfattning på:engelska

Ingår i deldatabas

Klassifikation

  • Ämneskategori:art swepub-publicationtype
  • Ämneskategori:ref swepub-contenttype

Anmärkningar

  • Introduction: The development of neutralizing antibodies (inhibitors) against coagulation factor VIII (FVIII) is currently the most serious complication for patients with haemophilia A undergoing FVIII replacement therapy. Several genetic factors have been acknowledged as risk factors for inhibitor development. Aim: To analyze the influence of genetic factors on the nature of the humoral immune response to FVIII in eight brother pairs with inhibitors. Methods: The domain specificity of FVIII-specific IgG was analysed by antibody binding to FVIII fragments and homologue-scanning mutagenesis (HSM). The FVIII-specific IgG subclasses were measured by direct ELISA. Results: Of the 16 patient analysed with both methods, 12 had A2- and 13 had C2-specific IgG. The presence of A1-, A3- or C1-specific IgG was identified in nine of 14 patients analysed by HSM. IgG1, IgG2 and IgG4 subclasses contributed to the anti-FVIII IgG response, and the amount of FVIII-specific IgG1 (r = 0.66) and IgG4 (r = 0.69) correlated significantly with inhibitor titres. Patients with high concentrations of total anti-FVIII IgG (r = 0.69) or high inhibitor titres (r = 0.52) had a high proportion of FVIII-specific IgG4. Statistical analysis revealed trends/evidence that the subclass distribution (P = 0.0847) and domain specificity to HC/LC (P = 0.0883) and A2/C2 (P = 0.0011) of anti-FVIII IgG were more similar in brothers compared to unrelated subjects. Conclusion: Overall, our data provide a first hint that anti-FVIII IgG characteristics are comparable among haemophilic brothers with inhibitors. Whether genetic factors also influence the nature of patients' antibodies needs to be confirmed in a larger study population.

Ämnesord och genrebeteckningar

Biuppslag (personer, institutioner, konferenser, titlar ...)

  • Orlowski, A.University Hospital Frankfurt (författare)
  • Stichel, D.University Hospital Frankfurt (författare)
  • Healey, J. F.Children's Healthcare of Atlanta (författare)
  • Parker, E. T.Children's Healthcare of Atlanta (författare)
  • Donfield, S. M.Rho, Inc. (författare)
  • Astermark, J.Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups,Skåne University Hospital(Swepub:lu)medf-jas (författare)
  • Berntorp, E.Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups,Skåne University Hospital(Swepub:lu)medf-ebe (författare)
  • Lollar, P.Children's Healthcare of Atlanta (författare)
  • Schwabe, D.University Hospital Frankfurt (författare)
  • Königs, C.University Hospital Frankfurt (författare)
  • University Hospital FrankfurtChildren's Healthcare of Atlanta (creator_code:org_t)

Sammanhörande titlar

  • Ingår i:Haemophilia: Wiley23:2, s. 292-2991351-8216

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