{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T13:59:11Z","timestamp":1790949551938,"version":"4.1.0"},"reference-count":23,"publisher":"Georg Thieme Verlag KG","issue":"01","funder":[{"DOI":"10.13039\/501100004350","name":"Studienstiftung des Deutschen Volkes","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100004350","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thromb Haemost"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:p>\n            Background\u2003Discrepancy in factor IX activity (FIX:C) between one-stage assay (OSA) and chromogenic substrate assay (CSA) in patients with hemophilia B (PwHB) introduces challenges for clinical management.<\/jats:p><jats:p>\n            Aim\u2003To study the differences in FIX:C using OSA and CSA in moderate and mild hemophilia B (HB), their impact on classification of severity, and correlation with genotype.<\/jats:p><jats:p>\n            Methods\u2003Single-center study including 21 genotyped and clinically characterized PwHB. FIX:C by OSA was measured using ActinFSL (Siemens) and CSA by Biophen (Hyphen). In addition, in vitro experiments with wild-type FIX were performed. Reproducibility of CSA was assessed between three European coagulation laboratories.<\/jats:p><jats:p>\n            Results\u2003FIX:C by CSA was consistently lower than by OSA, with 10\/17 PwHB having a more severe hemophilia type by CSA. OSA displayed a more accurate description of the clinical bleeding severity, compared with CSA. A twofold difference between OSA:CSA FIX:C was present in 12\/17 PwHB; all patients had genetic missense variants in the FIX serine protease domain. Discrepancy was also observed with diluted normal plasma, most significant for values below 0.10\u2009IU\/mL. Assessment of samples with low FIX:C showed excellent reproducibility of the CSA results between the laboratories.<\/jats:p><jats:p>\n            Conclusion\u2003FIX:C was consistently higher by OSA compared with the CSA. Assessing FIX:C by CSA alone would have led to diagnosis of a more severe hemophilia type in a significant proportion of patients. Our study suggests using both OSA and CSA FIX:C together with genotyping to classify HB severity and provide essential information for clinical management.<\/jats:p>","DOI":"10.1055\/a-2142-0262","type":"journal-article","created":{"date-parts":[[2023,7,26]],"date-time":"2023-07-26T23:10:32Z","timestamp":1690413032000},"page":"032-039","source":"Crossref","is-referenced-by-count":5,"title":["Clinical Implications of Discrepancy between One-Stage Clotting and Chromogenic Factor IX Activity in Hemophilia B"],"prefix":"10.1055","volume":"124","author":[{"given":"David E.","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden"},{"name":"Paediatric Coagulation, Astrid Lindgren Children\u2019s Hospital, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u00c5sa","family":"Truedsson","sequence":"additional","affiliation":[{"name":"Clinical Chemistry, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annelie","family":"Str\u00e5lfors","sequence":"additional","affiliation":[{"name":"Clinical Chemistry, Karolinska University Hospital, Stockholm, Sweden"},{"name":"Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johanne Andersen","family":"Hojbjerg","sequence":"additional","affiliation":[{"name":"Department of Clinical Biochemistry, Aarhus University Hospital, Aarhus, Denmark"},{"name":"Department of Clinical Medicine, Aarhus University, Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nida","family":"Soutari","sequence":"additional","affiliation":[{"name":"Clinical Chemistry, Karolinska University Hospital, Stockholm, Sweden"},{"name":"Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margareta","family":"Holmstr\u00f6m","sequence":"additional","affiliation":[{"name":"Coagulation Unit, Department of Hematology, Karolinska University Hospital, Stockholm, Sweden"},{"name":"Department of Health, Medicine and Caring Sciences, Link\u00f6ping University, Link\u00f6ping, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanna","family":"Ranta","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden"},{"name":"Paediatric Coagulation, Astrid Lindgren Children\u2019s Hospital, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Letelier","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences, Lund University, Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette","family":"Bowyer","sequence":"additional","affiliation":[{"name":"Department of Coagulation, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf","family":"Ljung","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences, Lund University, Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jovan","family":"Antovic","sequence":"additional","affiliation":[{"name":"Clinical Chemistry, Karolinska University Hospital, Stockholm, Sweden"},{"name":"Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Bruzelius","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden"},{"name":"Coagulation Unit, Department of Hematology, Karolinska University Hospital, Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2023,9,18]]},"reference":[{"issue":"01","key":"ref1","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.blre.2017.08.007","article-title":"Haemophilia B: where are we now and what does the future hold?","volume":"32","author":"G Dolan","year":"2018","journal-title":"Blood Rev"},{"issue":"04","key":"ref2","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1111\/hae.13804","article-title":"Phenotypic characterization of haemophilia B - 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