Sökning: WFRF:(Baghaei M) >
Factor IX antibodie...
Factor IX antibodies and tolerance in hemophilia B in the Nordic countries - The impact of F9 variants and complications
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- Kihlberg, Kristina (författare)
- Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups,Skåne University Hospital
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- Baghaei, Fariba, 1964 (författare)
- Gothenburg University,Göteborgs universitet,Institutionen för medicin, avdelningen för invärtesmedicin och klinisk nutrition,Institute of Medicine, Department of Internal Medicine and Clinical Nutrition,Sahlgrenska Academy
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- Bruzelius, M. (författare)
- Karolinska Institutet,Karolinska University Hospital
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- Funding, Eva (författare)
- University of Copenhagen,Copenhagen University Hospital
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- Holme, P. A. (författare)
- Oslo university hospital
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- Lassila, R. (författare)
- University of Helsinki
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- Martin, Myriam (författare)
- Lund University,Lunds universitet,Proteinkemi, Malmö,Forskargrupper vid Lunds universitet,Protein Chemistry, Malmö,Lund University Research Groups,Regional Laboratories Region Skåne
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- Nummi, Vuokko (författare)
- University of Helsinki
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- Ranta, S. (författare)
- Karolinska Institutet,Karolinska University Hospital
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- Strandberg, Karin (författare)
- Lund University,Lunds universitet,Klinisk kemi, Malmö,Forskargrupper vid Lunds universitet,Clinical Chemistry, Malmö,Lund University Research Groups,Regional Laboratories Region Skåne
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- Andersson, Nadine Gretenkort (författare)
- Lund University,Lunds universitet,Pediatrisk hematologi,Forskargrupper vid Lunds universitet,Paediatric Haematology Research Unit,Lund University Research Groups,Skåne University Hospital
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- Berntorp, Erik (författare)
- Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups
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- Astermark, Jan (författare)
- Lund University,Lunds universitet,Institutionen för translationell medicin,Medicinska fakulteten,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Department of Translational Medicine,Faculty of Medicine,Clinical Coagulation, Malmö,Lund University Research Groups,Skåne University Hospital
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(creator_code:org_t)
- Elsevier BV, 2022
- 2022
- Engelska.
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Ingår i: Thrombosis Research. - : Elsevier BV. - 0049-3848 .- 1879-2472. ; 217, s. 22-32
- Relaterad länk:
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http://dx.doi.org/10... (free)
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https://gup.ub.gu.se...
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https://doi.org/10.1...
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http://kipublication...
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https://lup.lub.lu.s...
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Abstract
Ämnesord
Stäng
- Introduction: The development of inhibitory antibodies (inhibitors) in persons with hemophilia B (PwHB) causes significant morbidity. Data on the impact of the F9 variant and immune tolerance induction (ITI) outcome are limited.The aim of this study was to investigate the presence of neutralizing and non-neutralizing antibodies (NNA) in severe hemophilia B (HB) and to evaluate ITI outcome and complications in relation to the pathogenic F9 variant.Materials and methods: Persons with severe HB in the Nordic countries were enrolled and information on F9 variants, inhibitors, ITI and complications were collected. Analyses of anti-FIX antibodies with a fluorescence -immunoassay (xFLI) and an ELISA method were conducted.Results: Seventy-nine PwHB were enrolled. Null variants were seen in 33 (42 %) PwHB and 12 (15 %) had a current or former inhibitor. Eleven (92 %) of the inhibitor patients had experienced allergic manifestations and three (25 %) nephrotic syndrome. Of 10 PwHB with at least one ITI attempt, eight (80 %) were considered tolerant at enrolment. Immunosuppression was included in seven of eight successful or partially successful at-tempts. Five PwHB had at least one ITI failure before a successful or partially successful ITI. No NNA could be identified.Conclusion: A high proportion of severe F9 gene defects among persons with severe HB in the Nordic countries may explain the observed relatively high prevalence of inhibitors. ITI success was independent of the F9 variant and attained despite allergic manifestations and previous ITI failures. Inclusion of immunosuppression tenta-tively enhances the chances of ITI success. No NNA were observed.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Reumatologi och inflammation (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Rheumatology and Autoimmunity (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Nyckelord
- Hemophilia B
- Coagulation factor IX
- Inhibitors
- Immune tolerance
- induction
- Non -neutralizing antibodies
- F9 variant
- immune tolerance
- factor-viii
- inhibitors
- induction
- registry
- patient
- future
- Hematology
- Cardiovascular System & Cardiology
- Antibodies, Neutralizing
- Factor IX/genetics
- Factor VIII
- Hemophilia A
- Hemophilia B/genetics
- Humans
- Immune Tolerance/genetics
- Immunosuppression Therapy
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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Till lärosätets databas
- Av författaren/redakt...
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Kihlberg, Kristi ...
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Baghaei, Fariba, ...
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Bruzelius, M.
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Funding, Eva
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Holme, P. A.
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Lassila, R.
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visa fler...
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Martin, Myriam
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Nummi, Vuokko
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Ranta, S.
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Strandberg, Kari ...
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Andersson, Nadin ...
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Berntorp, Erik
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Astermark, Jan
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visa färre...
- Om ämnet
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- MEDICIN OCH HÄLSOVETENSKAP
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MEDICIN OCH HÄLS ...
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och Klinisk medicin
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och Reumatologi och ...
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- MEDICIN OCH HÄLSOVETENSKAP
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MEDICIN OCH HÄLS ...
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och Klinisk medicin
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och Hematologi
- Artiklar i publikationen
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Thrombosis Resea ...
- Av lärosätet
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Göteborgs universitet
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Karolinska Institutet
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Lunds universitet