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2. The spectrum of neutralizing and non-neutralizing anti-FVIII antibodies in a nationwide cohort of 788 persons with hemophilia A

Author

Oomen, Ilja, primary, Verhagen, Marieke, additional, Miranda, Mariarosaria, additional, Allacher, Peter, additional, Beckers, Erik A. M., additional, Blijlevens, Nicole M. A., additional, Bom, Johanna G. van der, additional, Coppens, Michiel, additional, Driessens, Mariëtte, additional, Eikenboom, Jeroen C. J., additional, Fijnvandraat, Karin, additional, Hassan, Shermarke, additional, van Heerde, Waander L., additional, Hooimeijer, H. Louise, additional, Jansen, Joop H., additional, Kaijen, Paul, additional, Leebeek, Frank W. G., additional, Meijer, Daniëlle, additional, Paul, Helmut, additional, Rijpma, Sanna R., additional, Rosendaal, Frits R., additional, Smit, Cees, additional, van Vulpen, Lize F. D., additional, Voorberg, Jan, additional, Schols, Saskia E. M., additional, and Gouw, Samantha C., additional

Published
2024
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3. Patients with moderate hemophilia A and B with a severe bleeding phenotype have an increased burden of disease

Author

Poli Van Creveldkliniek Medisch, Verhagen, Marieke J A, van Balen, Erna C, Blijlevens, Nicole M A, Coppens, Michiel, van Heerde, Waander L, Leebeek, Frank W G, Rijpma, Sanna R, van Vulpen, Lize F D, Gouw, Samantha C, Schols, Saskia E M, Poli Van Creveldkliniek Medisch, Verhagen, Marieke J A, van Balen, Erna C, Blijlevens, Nicole M A, Coppens, Michiel, van Heerde, Waander L, Leebeek, Frank W G, Rijpma, Sanna R, van Vulpen, Lize F D, Gouw, Samantha C, and Schols, Saskia E M

Published
2024

4. Limited value of testing for factor XIII and α2‐antiplasmin deficiency in patients with a bleeding disorder of unknown cause.

Author

Ariëns, Sander, Huisman, Albert, Hovinga, Idske C. L. Kremer, Urbanus, Rolf T., van Galen, Karin P. M., van Vulpen, Lize F. D., Fischer, Kathelijn, and Schutgens, Roger E. G.

Subjects
BLOOD testing, VON Willebrand disease, BLOOD coagulation factors, HEMORRHAGE, ABSOLUTE value
Abstract

Introduction: In patients with an increased bleeding tendency, extensive diagnostic blood testing is often performed. When results of tier 1 assays of primary haemostasis are normal, protocols recommend additional testing to rule out rare disorders including coagulation factor XIII (FXIII) and α2‐antiplasmin (α2AP) deficiency. Aim: To evaluate the added diagnostic value of FXIII and α2AP levels in patients with a bleeding disorder of unknown cause (BDUC). Methods: A retrospective monocentre cohort study between August 2011 and August 2023 was conducted. In all patients with bleeding tendencies and normal diagnostic tests for von Willebrand disease and platelet function, FXIII and α2AP were measured. Results: We included 158 consecutive patients; mean ISTH‐BAT scores were 8.2 (SD ± 3.7) in children, 6.2 (SD ± 2.1) in men and 10.6 (SD ± 3.3) in women. Median age was 37 (range 5–79) years, 88.6% of patients were female. Patients displayed median FXIII activity of 111% (IQR = 97–131) and median α2AP activity of 112% (IQR = 103–119). Three (1.9%) patients had FXIII levels < 50%, respectively 43%, 45% and 46%. Corresponding ISTH‐BAT scores were 7, 12 and 14. No α2AP levels < 60% was observed. No significant association was found between FXIII levels and ISTH‐BAT scores. Conclusion: In our cohort of BDUC patients, no clinical relevant FXIII deficiencies were detected; absolute values were well above the 30% cutoff considered adequate for normal haemostasis. No α2AP deficiencies were detected. These data suggest that in BDUC patients, measuring FXIII or AP activity is of limited value. [ABSTRACT FROM AUTHOR]

Published
2024
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6. Towards Personalized Treatment in Haemophilia: The Role of Genetic Factors in Iron and Heme Control to Identify Patients at Risk for Haemophilic Arthropathy.

Author

van Vulpen, Lize F. D., Mastbergen, Simon C., Foppen, Wouter, Fischer, Kathelijn, Lafeber, Floris P. J. G., and Schutgens, Roger E. G.

Subjects
*BLOOD coagulation factor VIII, *HEMOPHILIA treatment, *JOINT diseases, *HEME, *HEMOPHILIACS, *IRON
Abstract

The treatment landscape for haemophilia is changing rapidly, creating opportunities for personalized treatment. As major morbidity is still caused by haemophilic arthropathy, understanding the factors affecting joint damage and joint damage progression might lead to more individualized treatment regimens. We investigated the association of HFE mutations or HMOX1 polymorphisms affecting iron/heme handling with radiographic joint damage in 252 haemophilia patients (severe and moderate). Although iron levels and transferrin saturation were significantly increased in the 95 patients with an HFE mutation, neither carrying this mutation nor the HMOX1 polymorphism was associated with radiographic joint damage, and the same was true after adjustment for well-known factors associated with arthropathy. In conclusion, this study does not support the hypothesis that HFE mutations or HMOX1 polymorphisms can be used to predict the development of haemophilic arthropathy. [ABSTRACT FROM AUTHOR]

Published
2024
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7. Subclinical synovial proliferation in patients with severe haemophilia A: The value of ultrasound screening and biochemical markers

Author

van Bergen, Eline D. P., primary, van Leeuwen, Flora H. P., additional, Foppen, Wouter, additional, Timmer, Merel A., additional, Schutgens, Roger E. G., additional, Mastbergen, Simon C., additional, Lafeber, Floris P. J. G., additional, de Jong, Pim A., additional, Fischer, Kathelijn, additional, and van Vulpen, Lize F. D., additional

Published
2023
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8. Transition readiness among adolescents and young adults with haemophilia in the Netherlands: Nationwide questionnaire study

Author

Brands, Martijn R., primary, Janssen, Ebony A. M., additional, Cnossen, Marjon H., additional, Smit, Cees, additional, van Vulpen, Lize F. D., additional, van der Valk, Paul R., additional, Eikenboom, Jeroen, additional, Heubel‐Moenen, Floor C. J. I., additional, Hooimeijer, Louise, additional, Ypma, Paula, additional, Nieuwenhuizen, Laurens, additional, Coppens, Michiel, additional, Schols, Saskia E. M., additional, Laros‐van Gorkom, Britta A. P., additional, Leebeek, Frank W. G., additional, Driessens, Mariëtte H. E., additional, Rosendaal, Frits R., additional, van der Bom, Johanna G., additional, Fijnvandraat, Karin, additional, and Gouw, Samantha C., additional

Published
2023
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10. Unraveling facets of MECOM-associated syndrome: somatic genetic rescue, clonal hematopoiesis, and phenotype expansion

Author

Venugopal, Parvathy, Arts, Peer, Fox, Lucy C., Simons, Annet, Hiwase, Devendra K., Bardy, Peter G., Swift, Annette, Ross, David M., van Vulpen, Lize F. D., Buijs, Arjan, Bolton, Kelly L., Getta, Bartlomiej, Furlong, Eliska, Carter, Tina, Krapels, Ingrid, Hoeks, Marlijn, Al Kindy, Adila, Al Kindy, Farah, de Munnik, Sonja, Evans, Pamela, Frank, Mahalia S. B., Bournazos, Adam M., Cooper, Sandra T., Ha, Thuong Thi, Jackson, Matilda R., Arriola-Martinez, Luis, Phillips, Kerry, Brennan, Yvonne, Bakshi, Madhura, Ambler, Karen, Gao, Song, Kassahn, Karin S., Kenyon, Rosalie, Hung, Kevin, Babic, Milena, McGovern, Alan, Rawlings, Lesley, Vakulin, Cassandra, Dejong, Lucas, Fathi, Rema, McRae, Simon, Myles, Nicholas, Ladon, Dariusz, Jongmans, Marjolijn, Kuiper, Roland P., Poplawski, Nicola K., Barbaro, Pasquale, Blombery, Piers, Brown, Anna L., Hahn, Christopher N., and Scott, Hamish S.

Published
2024
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11. Clotting factor activity levels and bleeding risk in people with haemophilia playing sports

Author

Versloot, Olav, primary, Kemler, Ellen, additional, Blokzijl, Johan, additional, Timmer, Merel, additional, Schuuring, Marleen, additional, van Galen, Karin P. M., additional, Kremer Hovinga, Idske Cornelia L., additional, van der Valk, Paul R., additional, van Vulpen, Lize F. D., additional, Schutgens, Roger E. G., additional, van Koppenhagen, Casper F., additional, van der Net, Janjaap, additional, and Fischer, Kathelijn, additional

Published
2023
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13. Structural changes after ankle joint distraction in haemophilic arthropathy: an explorative study investigating biochemical markers and 3D joint space width

Author

van Bergen, Eline D. P., primary, Mastbergen, Simon C., additional, Lafeber, Floris P. J. G., additional, Bay‐Jensen, Anne‐Christine, additional, Madsen, Sofie F., additional, Port, Helena, additional, Foppen, Wouter, additional, Schutgens, Roger E. G., additional, Jansen, Mylène P., additional, and van Vulpen, Lize F. D., additional

Published
2023
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14. Pre-operative synovial hyperaemia in haemophilia patients undergoing total knee replacement and the effects of genicular artery embolization: A retrospective cohort study

Author

MS Radiologie, Brain, Circulatory Health, Researchgr. Systems Radiology, Cancer, Poli Van Creveldkliniek Medisch, MS Orthopaedie Algemeen, Infection & Immunity, Arts-assistenten Radiologie, Foppen, Wouter, van der Schaaf, Irene C, van Leeuwen, Flora H P, Verlind, David H, van Vulpen, Lize F D, Vogely, H Charles, Barentsz, Maarten W, MS Radiologie, Brain, Circulatory Health, Researchgr. Systems Radiology, Cancer, Poli Van Creveldkliniek Medisch, MS Orthopaedie Algemeen, Infection & Immunity, Arts-assistenten Radiologie, Foppen, Wouter, van der Schaaf, Irene C, van Leeuwen, Flora H P, Verlind, David H, van Vulpen, Lize F D, Vogely, H Charles, and Barentsz, Maarten W

Published
2023

15. Desmopressin in nonsevere hemophilia A: patient perspectives on use and efficacy

Author

Poli Van Creveldkliniek Medisch, Romano, Lorenzo G R, van Vulpen, Lize F D, den Exter, Paul L, Heubel-Moenen, Floor C J I, Hooijmeijer, Helene L, Coppens, Michiel, Fijnvandraat, Karin, Schols, Saskia E M, Ypma, Paula F, Smit, Cees, Driessens, Mariëtte H E, Rosendaal, Frits R, van der Bom, Johanna G, Gouw, Samantha C, Kruip, Marieke J H A, Poli Van Creveldkliniek Medisch, Romano, Lorenzo G R, van Vulpen, Lize F D, den Exter, Paul L, Heubel-Moenen, Floor C J I, Hooijmeijer, Helene L, Coppens, Michiel, Fijnvandraat, Karin, Schols, Saskia E M, Ypma, Paula F, Smit, Cees, Driessens, Mariëtte H E, Rosendaal, Frits R, van der Bom, Johanna G, Gouw, Samantha C, and Kruip, Marieke J H A

Published
2023

16. Transition readiness among adolescents and young adults with haemophilia in the Netherlands: Nationwide questionnaire study

Author

Poli Van Creveldkliniek Medisch, Brands, Martijn R, Janssen, Ebony A M, Cnossen, Marjon H, Smit, Cees, van Vulpen, Lize F D, van der Valk, Paul R, Eikenboom, Jeroen, Heubel-Moenen, Floor C J I, Hooimeijer, Louise, Ypma, Paula, Nieuwenhuizen, Laurens, Coppens, Michiel, Schols, Saskia E M, Laros-van Gorkom, Britta A P, Leebeek, Frank W G, Driessens, Mariëtte H E, Rosendaal, Frits R, van der Bom, Johanna G, Fijnvandraat, Karin, Gouw, Samantha C, Poli Van Creveldkliniek Medisch, Brands, Martijn R, Janssen, Ebony A M, Cnossen, Marjon H, Smit, Cees, van Vulpen, Lize F D, van der Valk, Paul R, Eikenboom, Jeroen, Heubel-Moenen, Floor C J I, Hooimeijer, Louise, Ypma, Paula, Nieuwenhuizen, Laurens, Coppens, Michiel, Schols, Saskia E M, Laros-van Gorkom, Britta A P, Leebeek, Frank W G, Driessens, Mariëtte H E, Rosendaal, Frits R, van der Bom, Johanna G, Fijnvandraat, Karin, and Gouw, Samantha C

Published
2023

17. Structural changes after ankle joint distraction in haemophilic arthropathy: an explorative study investigating biochemical markers and 3D joint space width

Author

Poli Van Creveldkliniek Medisch, MS Reumatologie/Immunologie/Infectie, Infection & Immunity, Regenerative Medicine and Stem Cells, Lab Reumatologie/Klinische Immunologie, MS Radiologie, Child Health, Circulatory Health, Onderzoek Reumatologie, van Bergen, Eline D P, Mastbergen, Simon C, Lafeber, Floris P J G, Bay-Jensen, Anne-Christine, Madsen, Sofie F, Port, Helena, Foppen, Wouter, Schutgens, Roger E G, Jansen, Mylène P, van Vulpen, Lize F D, Poli Van Creveldkliniek Medisch, MS Reumatologie/Immunologie/Infectie, Infection & Immunity, Regenerative Medicine and Stem Cells, Lab Reumatologie/Klinische Immunologie, MS Radiologie, Child Health, Circulatory Health, Onderzoek Reumatologie, van Bergen, Eline D P, Mastbergen, Simon C, Lafeber, Floris P J G, Bay-Jensen, Anne-Christine, Madsen, Sofie F, Port, Helena, Foppen, Wouter, Schutgens, Roger E G, Jansen, Mylène P, and van Vulpen, Lize F D

Published
2023

18. Magnetic resonance imaging evidence for subclinical joint bleeding in a Dutch population of people with severe hemophilia on prophylaxis

Author

Cancer, Researchgr. Systems Radiology, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Infection & Immunity, Regenerative Medicine and Stem Cells, MS Radiologie, van Leeuwen, Flora H P, van Bergen, Eline D P, Timmer, Merel A, van Vulpen, Lize F D, Schutgens, Roger E G, de Jong, Pim A, Fischer, Kathelijn, Foppen, Wouter, Cancer, Researchgr. Systems Radiology, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Infection & Immunity, Regenerative Medicine and Stem Cells, MS Radiologie, van Leeuwen, Flora H P, van Bergen, Eline D P, Timmer, Merel A, van Vulpen, Lize F D, Schutgens, Roger E G, de Jong, Pim A, Fischer, Kathelijn, and Foppen, Wouter

Published
2023

19. Monitoring recovery of joints after bleeding: Physical examination and ultrasound are complementary

Author

Cancer, Researchgr. Systems Radiology, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, MS Radiologie, van Leeuwen, Flora H P, Fischer, Kathelijn, Foppen, Wouter, van Vulpen, Lize F D, Timmer, Merel A, Cancer, Researchgr. Systems Radiology, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, MS Radiologie, van Leeuwen, Flora H P, Fischer, Kathelijn, Foppen, Wouter, van Vulpen, Lize F D, and Timmer, Merel A

Published
2023

20. Colorectal cancer screening in patients with inherited bleeding disorders: high cancer detection rate in hemophilia patients

Author

Poli Van Creveldkliniek Medisch, Kempers, Eva K, van Kwawegen, Calvin B, de Meris, Joke, Spaander, Manon C W, Schols, Saskia E M, Ypma, Paula F, Heubel-Moenen, Floor C J I, van Vulpen, Lize F D, Coppens, Michiel, van der Bom, Johanna G, Fijnvandraat, Karin, Meijer, Karina, Eikenboom, Jeroen, Gouw, Samantha C, Leebeek, Frank W G, Kruip, Marieke J H A, Poli Van Creveldkliniek Medisch, Kempers, Eva K, van Kwawegen, Calvin B, de Meris, Joke, Spaander, Manon C W, Schols, Saskia E M, Ypma, Paula F, Heubel-Moenen, Floor C J I, van Vulpen, Lize F D, Coppens, Michiel, van der Bom, Johanna G, Fijnvandraat, Karin, Meijer, Karina, Eikenboom, Jeroen, Gouw, Samantha C, Leebeek, Frank W G, and Kruip, Marieke J H A

Published
2023

21. The detrimental effects of iron to the joint; aggravation by inflammation

Author

Poli Van Creveldkliniek Medisch, MS Hematologie, Lab Reumatologie/Klinische Immunologie, Infection & Immunity, Regenerative Medicine and Stem Cells, van Vulpen, Lize F D, van Bergen, Eline D P, Pulles, Astrid E, Mastbergen, Simon C, Poli Van Creveldkliniek Medisch, MS Hematologie, Lab Reumatologie/Klinische Immunologie, Infection & Immunity, Regenerative Medicine and Stem Cells, van Vulpen, Lize F D, van Bergen, Eline D P, Pulles, Astrid E, and Mastbergen, Simon C

Published
2023

22. In patients with hemophilia, a decreased thrombin generation profile is associated with a severe bleeding phenotype

Author

Poli Van Creveldkliniek Medisch, Verhagen, Marieke J A, van Heerde, Waander L, van der Bom, Johanna G, Beckers, Erik A M, Blijlevens, Nicole M A, Coppens, Michiel, Gouw, Samantha C, Jansen, Joop H, Leebeek, Frank W G, van Vulpen, Lize F D, Meijer, Daniëlle, Schols, Saskia E M, Poli Van Creveldkliniek Medisch, Verhagen, Marieke J A, van Heerde, Waander L, van der Bom, Johanna G, Beckers, Erik A M, Blijlevens, Nicole M A, Coppens, Michiel, Gouw, Samantha C, Jansen, Joop H, Leebeek, Frank W G, van Vulpen, Lize F D, Meijer, Daniëlle, and Schols, Saskia E M

Published
2023

25. Ankle joint distraction is a promising alternative treatment for patients with severe haemophilic ankle arthropathy

Author

van Bergen, Eline D. P., primary, Mastbergen, Simon C., additional, Vogely, H. Charles, additional, Balani, Tanya N., additional, de Kleijn, Piet, additional, Foppen, Wouter, additional, van Roermund, Peter M., additional, Lafeber, Floris P. J. G., additional, Schutgens, Roger E. G., additional, and van Vulpen, Lize F. D., additional

Published
2022
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27. Socioeconomic participation of persons with hemophilia: Results from the sixth hemophilia in the Netherlands study

Author

Poli Van Creveldkliniek Medisch, van Balen, Erna C, Hassan, Shermarke, Smit, Cees, Driessens, Mariette H E, Beckers, Erik A M, Coppens, Michiel, Eikenboom, Jeroen C, Hooimeijer, Hélène L, Leebeek, Frank W G, Mauser-Bunschoten, Evelien P, van Vulpen, Lize F D, Schols, Saskia E M, Rosendaal, Frits R, van der Bom, Johanna G, Gouw, Samantha C, Poli Van Creveldkliniek Medisch, van Balen, Erna C, Hassan, Shermarke, Smit, Cees, Driessens, Mariette H E, Beckers, Erik A M, Coppens, Michiel, Eikenboom, Jeroen C, Hooimeijer, Hélène L, Leebeek, Frank W G, Mauser-Bunschoten, Evelien P, van Vulpen, Lize F D, Schols, Saskia E M, Rosendaal, Frits R, van der Bom, Johanna G, and Gouw, Samantha C

Published
2022

28. Validation of the pedHALshort and HALshort in Dutch children and adults with haemophilia

Author

Poli Van Creveldkliniek Medisch, KGW-algemeen, Child Health, Circulatory Health, Kuijlaars, Isolde A R, van der Net, Janjaap, van Vulpen, Lize F D, Driessens, Mariette H E, Schols, Saskia E M, Tan, Melanie, Gouw, Samantha C, Fischer, Kathelijn, Poli Van Creveldkliniek Medisch, KGW-algemeen, Child Health, Circulatory Health, Kuijlaars, Isolde A R, van der Net, Janjaap, van Vulpen, Lize F D, Driessens, Mariette H E, Schols, Saskia E M, Tan, Melanie, Gouw, Samantha C, and Fischer, Kathelijn

Published
2022

29. Reduced cardiovascular morbidity in patients with hemophilia: results of a 5-year multinational prospective study

Author

Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Infection & Immunity, Van Der Valk, Paul, Makris, Michael, Fischer, Kathelijn, Tait, Robert C, Chowdary, Pratima, Collins, Peter W, Meijer, Karina, van Vulpen, Lize F D, Mauser-Bunschoten, Eveline, Schutgens, Roger E G, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Infection & Immunity, Van Der Valk, Paul, Makris, Michael, Fischer, Kathelijn, Tait, Robert C, Chowdary, Pratima, Collins, Peter W, Meijer, Karina, van Vulpen, Lize F D, Mauser-Bunschoten, Eveline, and Schutgens, Roger E G

Published
2022

31. Liver‐related complications before and after successful treatment of chronic hepatitis C virus infection in people with inherited bleeding disorders.

Author

Isfordink, Cas J., van Erpecum, Karel J., Fischer, Kathelijn, van der Valk, Paul R., van Vulpen, Lize F. D., Schutgens, Roger E. G., Arends, Joop E., and Mauser‐Bunschoten, Evelien P.

Subjects
HEPATITIS C, CHRONIC hepatitis C, TREATMENT effectiveness, HEPATITIS C virus
Abstract

Introduction: With availability of direct‐acting antivirals (DAA), most persons with inherited bleeding disorders are currently cured of hepatitis C virus (HCV) infection. The risk of liver‐related complications following HCV cure has not been reported for this population. Aim: Reporting liver‐related complications during long‐term chronic HCV infection and following sustained virological response (SVR) in this population. Methods: Retrospective follow‐up of a prospective single‐centre cohort of HCV antibody‐positive persons with inherited bleeding disorders. Primary endpoint was liver‐related complications [hepatocellular carcinoma (HCC), decompensated cirrhosis, bleeding gastroesophageal varices]. Liver‐related complications were reported separately during chronic HCV and following SVR, stratified for interferon‐based and DAA‐based SVR. Results: In total 309/381 (81%) HCV antibody‐positive individuals developed chronic HCV infection. Median follow‐up was 44 years [interquartile range (IQR): 34–50]. Liver‐related complications occurred in 36/309 (12%) of individuals with chronic HCV infection after median 31 years of chronic infection. Of 199 individuals with SVR, 97 were cured with interferon‐based regimens and 102 with DAA after median infection durations of 29 and 45 years, respectively. At end of follow‐up, respectively, 21% and 42% had advanced fibrosis or cirrhosis. Post‐SVR, seven (4%) individuals had a liver‐related complication, mainly HCC (n = 4). Incidence of liver‐related complications per 100 patient‐years post‐SVR follow‐up was.2 for interferon‐cured and 1.0 for DAA‐cured individuals (p =.01). Conclusion: Successful HCV treatment does not eliminate the risk of liver‐related complications in persons with inherited bleeding disorders. Due to higher baseline risk, incidence was higher after DAA than interferon‐based SVR. We advise continuing HCC surveillance post‐SVR in all with advanced fibrosis or cirrhosis. [ABSTRACT FROM AUTHOR]

Published
2023
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32. Validation of the pedHALshort and HALshort in Dutch children and adults with haemophilia.

Author

Kuijlaars, Isolde A. R., van der Net, Janjaap, van Vulpen, Lize F. D., Driessens, Mariette H. E., Schols, Saskia E. M., Tan, Melanie, Gouw, Samantha C., and Fischer, Kathelijn

Subjects
DUTCH people, HEMOPHILIA, HEMOPHILIACS, TEST validity, SECONDARY analysis
Abstract

Introduction: The Haemophilia Activities List (HAL) and paediatric HAL assess self‐reported limitations in various daily activities. To reduce patient burden, shorter versions of the pedHAL (22 items) and HAL (18 items) have been developed. Aim: This study aimed to determine the agreement between the pedHAL/HALfull and pedHAL/HALshort and construct validity and internal consistency of the pedHAL/HALshort in persons with haemophilia (PWH). Methods: A cross‐sectional secondary analysis of the Hemophilia in the Netherlands‐6 national survey was performed. Adult and paediatric PWH completed the original pedHAL/HALfull, from which pedHAL/HALshort were derived. Score differences between the original and short versions were calculated. Construct validity was studied by testing hypotheses regarding the relationship of the pedHAL/HALshort with the pedHAL/HALfull, Haemophilia & Exercise Project Test‐Questionnaire (HEP‐Test‐Q), Canadian Haemophilia Outcomes‐Kids' Life Assessment Tool (CHO‐KLAT) and RAND 36‐item Health Survey (RAND‐36) (convergent/discriminant validity) as well as its ability to discriminate between subgroups (known‐group validity). Internal consistency was assessed with Cronbach's α. Results: We included 113 children (median 10y [range 4–17], 53% severe haemophilia) and 691 adults (median 51y [range 18–88], 35% severe). Scores of the pedHAL/HALfull and pedHAL/HALshort were similar with high correlations (>0.9). Construct validity was confirmed for the pedHAL/HALshort. The HALshort was able to discriminate between different disease severities and ages. Cronbach's α of the pedHAL/HALshort was 0.95–0.97. Conclusion: This study confirmed the agreement between the pedHAL/HALfull and the pedHAL/HALshort and the construct validity of the pedHAL/HALshort. The next step is to study construct validity of the pedHAL/HALshort when administered as short forms. [ABSTRACT FROM AUTHOR]

Published
2022
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33. Additional file 1 of Detecting low blood concentrations in joints using T1 and T2 mapping at 1.5, 3, and 7 T: an in vitro study

Author

van Leeuwen, Flora H. P., Lena, Beatrice, Zwanenburg, Jaco J. M., van Vulpen, Lize F. D., Bartels, Lambertus W., Fischer, Kathelijn, Nap, Frank J., de Jong, Pim A., Bos, Clemens, and Foppen, Wouter

Abstract

Additional file 1: Figure S1. Detailed description of the position of the samples in the phatom in the different batches scanned. Percentages correspond to the blood percentage in the sample. a) First batch scanned at all field strenghts. b) Second batch scanned at all field strengths. c) Third batch scanned at 3T. Figure S2. (A) Signal intensity in the ROI vs IR delays for the 0% blood tube at 3T: the experimental data are indicated with blue ��� and the fitting with a red line. (B) Analysis of the residuals: each ��� represents the residual of each experimental data point from the one predicted with the fitting. The mean of the residual is also reported as dotted line. Figure S3. T2 mapping at 1.5T with different echo spacing on dependence on the blood concentration. Figure S4. T2 mapping at 1.5T (A) and 3 T (B) with a multi-slice accelerated scans and single slice non accelerated scans.

Published
2021
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35. Hepatitis C virus in hemophilia: Health-related quality of life after successful treatment in the sixth Hemophilia in the Netherlands study

Author

MS MDL 1, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Isfordink, Cas J, Gouw, Samantha C, van Balen, Erna C, Hassan, Shermarke, Beckers, Erik A M, van der Bom, Johanna G, Coppens, Michiel, Eikenboom, Jeroen, Fischer, Kathelijn, Hooimeijer, Louise, Leebeek, Frank W G, Rosendaal, Frits R, Schols, Saskia E M, Smit, Cees, van Vulpen, Lize F D, Mauser-Bunschoten, Eveline P, MS MDL 1, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Isfordink, Cas J, Gouw, Samantha C, van Balen, Erna C, Hassan, Shermarke, Beckers, Erik A M, van der Bom, Johanna G, Coppens, Michiel, Eikenboom, Jeroen, Fischer, Kathelijn, Hooimeijer, Louise, Leebeek, Frank W G, Rosendaal, Frits R, Schols, Saskia E M, Smit, Cees, van Vulpen, Lize F D, and Mauser-Bunschoten, Eveline P

Published
2021

36. Generic PROMIS item banks in adults with hemophilia for patient-reported outcome assessment: Feasibility, measurement properties, and relevance

Author

Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Kuijlaars, Isolde A R, Teela, Lorynn, van Vulpen, Lize F D, Timmer, Merel A, Coppens, Michiel, Gouw, Samantha C, Peters, Marjolein, Kruip, Marieke J H A, Cnossen, Marjon H, Muis, Jelmer J, van Hoorn, Evelien S, Haverman, Lotte, Fischer, Kathelijn, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Kuijlaars, Isolde A R, Teela, Lorynn, van Vulpen, Lize F D, Timmer, Merel A, Coppens, Michiel, Gouw, Samantha C, Peters, Marjolein, Kruip, Marieke J H A, Cnossen, Marjon H, Muis, Jelmer J, van Hoorn, Evelien S, Haverman, Lotte, and Fischer, Kathelijn

Published
2021

37. Detecting low blood concentrations in joints using T1 and T2 mapping at 1.5, 3, and 7 T: an in vitro study

Author

Cancer, Researchgr. Systems Radiology, Beeldverwerking ISI, Highfield Research Group, Circulatory Health, Regenerative Medicine and Stem Cells, Poli Van Creveldkliniek Medisch, Child Health, MS Radiologie, Infection & Immunity, Researchgr. Beeldg. Moleculaire Interv., van Leeuwen, Flora H P, Lena, Beatrice, Zwanenburg, Jaco J M, van Vulpen, Lize F D, Bartels, Lambertus W, Fischer, Kathelijn, Nap, Frank J, de Jong, Pim A, Bos, Clemens, Foppen, Wouter, Cancer, Researchgr. Systems Radiology, Beeldverwerking ISI, Highfield Research Group, Circulatory Health, Regenerative Medicine and Stem Cells, Poli Van Creveldkliniek Medisch, Child Health, MS Radiologie, Infection & Immunity, Researchgr. Beeldg. Moleculaire Interv., van Leeuwen, Flora H P, Lena, Beatrice, Zwanenburg, Jaco J M, van Vulpen, Lize F D, Bartels, Lambertus W, Fischer, Kathelijn, Nap, Frank J, de Jong, Pim A, Bos, Clemens, and Foppen, Wouter

Published
2021

38. Validation of PROMIS Profile-29 in adults with hemophilia in the Netherlands

Author

Poli Van Creveldkliniek Medisch, van Balen, Erna C, Haverman, Lotte, Hassan, Shermarke, Taal, Elisabeth M, Smit, Cees, Driessens, Mariëtte H, Beckers, Erik A M, Coppens, Michiel, Eikenboom, Jeroen, Hooimeijer, Hélène L, Leebeek, Frank W G, van Vulpen, Lize F D, Schols, Saskia E M, Terwee, Caroline B, Rosendaal, Frits R, van der Bom, Johanna G, Gouw, Samantha C, Poli Van Creveldkliniek Medisch, van Balen, Erna C, Haverman, Lotte, Hassan, Shermarke, Taal, Elisabeth M, Smit, Cees, Driessens, Mariëtte H, Beckers, Erik A M, Coppens, Michiel, Eikenboom, Jeroen, Hooimeijer, Hélène L, Leebeek, Frank W G, van Vulpen, Lize F D, Schols, Saskia E M, Terwee, Caroline B, Rosendaal, Frits R, van der Bom, Johanna G, and Gouw, Samantha C

Published
2021

39. On-demand treatment with the iron chelator deferasirox is ineffective in preventing blood-induced joint damage in haemophilic mice

Author

Unit Opleiding Aios, Poli Van Creveldkliniek Medisch, Lab Reumatologie/Klinische Immunologie, Infection & Immunity, Regenerative Medicine and Stem Cells, Circulatory Health, Pulles, Astrid E, van Vulpen, Lize F D, Coeleveld, Katja, Mastbergen, Simon C, Schutgens, Roger E G, Lafeber, Floris P J G, Unit Opleiding Aios, Poli Van Creveldkliniek Medisch, Lab Reumatologie/Klinische Immunologie, Infection & Immunity, Regenerative Medicine and Stem Cells, Circulatory Health, Pulles, Astrid E, van Vulpen, Lize F D, Coeleveld, Katja, Mastbergen, Simon C, Schutgens, Roger E G, and Lafeber, Floris P J G

Published
2021

40. Health and treatment outcomes of patients with hemophilia in the Netherlands, 1972-2019

Author

Poli Van Creveldkliniek Medisch, Hassan, Shermarke, van Balen, Erna C, Smit, Cees, Mauser-Bunschoten, Evelien P, van Vulpen, Lize F D, Eikenboom, Jeroen, Beckers, Erik A M, Hooimeijer, Louise, Ypma, Paula F, Nieuwenhuizen, Laurens, Coppens, Michiel, Schols, Saskia E M, Leebeek, Frank W G, Driessens, Mariëtte H, Rosendaal, Frits R, van der Bom, Johanna G, Gouw, Samantha C, Poli Van Creveldkliniek Medisch, Hassan, Shermarke, van Balen, Erna C, Smit, Cees, Mauser-Bunschoten, Evelien P, van Vulpen, Lize F D, Eikenboom, Jeroen, Beckers, Erik A M, Hooimeijer, Louise, Ypma, Paula F, Nieuwenhuizen, Laurens, Coppens, Michiel, Schols, Saskia E M, Leebeek, Frank W G, Driessens, Mariëtte H, Rosendaal, Frits R, van der Bom, Johanna G, and Gouw, Samantha C

Published
2021

41. Mortality, life expectancy and causes of death of persons with hemophilia in the Netherlands 2001-2018

Author

Poli Van Creveldkliniek Medisch, Hassan, Shermarke, Monahan, Rory C, Mauser-Bunschoten, Evelien P, van Vulpen, Lize F D, Eikenboom, Jeroen, Beckers, Erik A M, Hooimeijer, Louise, Ypma, Paula F, Nieuwenhuizen, Laurens, Coppens, Michiel, Schols, Saskia E M, Leebeek, Frank W G, Smit, Cees, Driessens, Mariëtte H, le Cessie, Saskia, van Balen, Erna C, Rosendaal, Frits R, van der Bom, Johanna G, Gouw, Samantha C, Poli Van Creveldkliniek Medisch, Hassan, Shermarke, Monahan, Rory C, Mauser-Bunschoten, Evelien P, van Vulpen, Lize F D, Eikenboom, Jeroen, Beckers, Erik A M, Hooimeijer, Louise, Ypma, Paula F, Nieuwenhuizen, Laurens, Coppens, Michiel, Schols, Saskia E M, Leebeek, Frank W G, Smit, Cees, Driessens, Mariëtte H, le Cessie, Saskia, van Balen, Erna C, Rosendaal, Frits R, van der Bom, Johanna G, and Gouw, Samantha C

Published
2021

42. Treatment of acquired hemophilia A, a balancing act: results from a 27-year Dutch cohort study

Author

Afdeling van Creveldkliniek - Zorg, CTI Boes, Unit Opleiding Aios, Infection & Immunity, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Schep, Sarah J, van Dijk, Wobke E M, Beckers, Erik A M, Meijer, Karina, Coppens, Michiel, Eikenboom, Jeroen, Leebeek, Frank W G, van Vulpen, Lize F D, Fischer, Kathelijn F, Schutgens, Roger E G, Dutch Society of Haemophilia Treaters, The Netherlands, Afdeling van Creveldkliniek - Zorg, CTI Boes, Unit Opleiding Aios, Infection & Immunity, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Schep, Sarah J, van Dijk, Wobke E M, Beckers, Erik A M, Meijer, Karina, Coppens, Michiel, Eikenboom, Jeroen, Leebeek, Frank W G, van Vulpen, Lize F D, Fischer, Kathelijn F, Schutgens, Roger E G, and Dutch Society of Haemophilia Treaters, The Netherlands

Published
2021

43. Synovitis and synovectomy in haemophilia

Author

Poli Van Creveldkliniek Medisch, van Vulpen, Lize F D, Thomas, Sylvia, Keny, Swapnil A, Mohanty, Shubhranshu S, Poli Van Creveldkliniek Medisch, van Vulpen, Lize F D, Thomas, Sylvia, Keny, Swapnil A, and Mohanty, Shubhranshu S

Published
2021

44. Real-Life Pharmacokinetics of rFVIII-Fc and rFIX-Fc

Author

CDL Rode cel, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Poli VCK, Infection & Immunity, Traets, Marissa J M, Fischer, Kathelijn, Uitslager, Nanda, van der Valk, Paul R, Kremer Hovinga, Idske C L, van Vulpen, Lize F D, Schutgens, Roger E G, CDL Rode cel, Poli Van Creveldkliniek Medisch, Child Health, Circulatory Health, Poli VCK, Infection & Immunity, Traets, Marissa J M, Fischer, Kathelijn, Uitslager, Nanda, van der Valk, Paul R, Kremer Hovinga, Idske C L, van Vulpen, Lize F D, and Schutgens, Roger E G

Published
2020

47. Models of arthropathy: what can we learn from them to improve patient care?

Author

Unit Opleiding Aios, Lab Reumatologie/Klinische Immunologie, Infection & Immunity, Regenerative Medicine and Stem Cells, Poli Van Creveldkliniek Medisch, Pulles, Astrid E, Lafeber, Floris P J G, van Vulpen, Lize F D, Unit Opleiding Aios, Lab Reumatologie/Klinische Immunologie, Infection & Immunity, Regenerative Medicine and Stem Cells, Poli Van Creveldkliniek Medisch, Pulles, Astrid E, Lafeber, Floris P J G, and van Vulpen, Lize F D

Published
2019

48. An update on the ‘danger theory’ in inhibitor development in hemophilia A

Author

CTI Boes, Immuno/reuma onderzoek 2 (Boes), Infection & Immunity, Child Health, Poli Van Creveldkliniek Medisch, Circulatory Health, Regenerative Medicine and Stem Cells, Schep, Sarah J, Boes, Marianne L, Schutgens, Roger E G, van Vulpen, Lize F D, CTI Boes, Immuno/reuma onderzoek 2 (Boes), Infection & Immunity, Child Health, Poli Van Creveldkliniek Medisch, Circulatory Health, Regenerative Medicine and Stem Cells, Schep, Sarah J, Boes, Marianne L, Schutgens, Roger E G, and van Vulpen, Lize F D

Published
2019

49. Severe Wound Healing Impairment in a Patient with Dysfibrinogenaemia

Author

van Vulpen, Lize F D, Amin, Shwan N, and Makris, Michael

Subjects
Hematology
Abstract

Fibrinogen has a principal role in blood clot formation. It is a soluble protein that is converted to fibrin and polymerizes into insoluble fibrin fibres forming a clot-stabilizing network. This is further strengthened by activated factor XIII (FXIIIa) via cross-linking and incorporation of α2-antiplasmin and thrombin activatable fibrinolysis inhibitor (TAFI). Fibrinogen is synthesized in hepatocytes and composed of three pairs of different polypeptide chains (Aα, Bβ, γ), interconnected via disulphide bridges. Each chain is encoded by a single gene (FGA, FGB and FGG). In congenital dysfibrinogenaemia, a qualitative defect is caused by mutations within any of these genes. According to the online database of dysfibrinogenaemias, 1,215 cases are reported at present: 626 with a defect in the Aα chain, 154 in the Bβ chain, and 435 in the γ chain.[1] The clinical spectrum of this disorder is heterogeneous: 26% of the patients have a bleeding tendency, 21% of the patients have a thrombotic tendency, and 53% of the patients are asymptomatic.[2] [3] [4] Dysfibrinogenaemia can thus lead to both bleeding and thrombosis, illustrating the versatile functions of fibrinogen in coagulation. Furthermore, fibrinogen is important for wound healing. The fibrin network serves as a provisional scaffold at the site of injury and supports cell proliferation and migration.[5] This role of fibri(noge)n in tissue repair is illustrated by the use of fibrin sealants to promote wound healing,[6] although this fibrin concentration is much higher than in physiological conditions. Delayed wound healing and/or wound dehiscence is a clinical feature in FXIII deficiency due to decreased cross-linking of fibrin and decreased linking to other proteins.[7] Surprisingly, impaired wound healing is not a well-known feature in congenital dysfibrinogenaemia. We here report a case of severely impaired wound healing in a man with dysfibrinogenemia due to fibrinogen Longmont, a heterozygous dysfibrinogenaemia causing impaired polymerization.

Published
2018

50. Severe Wound Healing Impairment in a Patient with Dysfibrinogenaemia

Author

Poli Van Creveldkliniek Medisch, Regenerative Medicine and Stem Cells, van Vulpen, Lize F D, Amin, Shwan N, Makris, Michael, Poli Van Creveldkliniek Medisch, Regenerative Medicine and Stem Cells, van Vulpen, Lize F D, Amin, Shwan N, and Makris, Michael

Published
2018

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