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Prof. dr. C.J. Fijnvandraat

Position
Professor
Main activities
Patient care, Research, Teaching
Specialisation
Pediatric Hematology
Focus of research

Hemophilia

Sickle Cell Disease

 

Key publications
  • Eckhardt CL, van Velzen AS, Peters M, Astermark J, Brons PP, Castaman G, Cnossen MH, Dors N , Escuriola-Ettingshausen C, Hamulyak K, Hart DP, Hay CRM, Haya S, van Heerde WL, Hermans C, Holmström M, Jimenez-Yuste V, Keenan RD, Klamroth R, Laros-van Gorkom BAP, Leebeek FWG, Liesner R, Mäkipernaa A, Male C, Mauser-Bunschoten E, Mazzucconi MG, McRae S, Meijer K, Mitchell M, Morfini M, Nijziel M, Oldenburg J, Peerlinck K, Petrini P, Platokouki H, Reitter-Pfoertner SE, Santagostino E, Schinco P, Smiers FJ, Siegmund B, Tagliaferri A, Yee TT, Kamphuisen PW, van der Bom JG, Fijnvandraat K, Factor VIII gene (F8) mutation and risk of inhibitor development in nonsevere hemophilia A. BLOOD 2013;122 (11):1954-1962 [PubMed]
  • Castaman G, Fijnvandraat K, Molecular and clinical predictors of inhibitor risk and its prevention and treatment in mild hemophilia A. BLOOD 2014;124 (15):2333-2336 [PubMed]
  • Fijnvandraat K, Cnossen MH, Leebeek FWG, Peters M, Diagnosis and management of haemophilia. BMJ-BRIT MED J 2012;344:e2707 [PubMed]
  • van Velzen AS, Eckhardt CL, Streefkerk N, Peters M, Hart DP, Hamulyak K, Klamroth R, Meijer K, Nijziel M, Schinco P, Yee TT, van der Bom JG, Fijnvandraat K [Contributors: Fijnvandraat K, Peters M, Kamphuisen PW, van der Bom JG, Peerlinck K, Oldenburg J, Hay CRM, Santagostino E, Astermark J, Eckhardt CL, van Velzen AS, Streefkerk N, Loomans JL, van Eijkelenburg A, Jansen AJ, Kruijt CC, van Tienoven B, van Baar ACG, Corten IW, Bultje AC, Visser GR, Meijer K, Nijziel M, Dors N, Hamulyak K, Brons PP, Laros-van Gorkom BAP, van Heerde WL, Leebeek FWG, Kruip M, Cnossen MH, Mauser-Bunschoten EP, Fischer K, Smiers FJ, Hermans C, Schwaab R, Siegmund B, Klamroth R, Escuriola-Ettingshausen C, Königs C, Petrini P, Holmström M, Mäkipernaa A, Male C, Pabinger I, Reitter- Pfoertner SE, Keenan RD, Liesner R, Khair K, Yee TT, Griffioen A, Hart DP, Rangarajan S, Mitchell M, Thompson G, Haya S, Moret A, Cid AR, Casaña P, Jimenez-Yuste V, Mancuso ME, Mazzuconni MG, Santoro C, Morfini M, Castaman G, Schinco P, Valeri F, Tagliaferri A, Di Perna C, Platokouki H, McRae S]] , The incidence and treatment of bleeding episodes in non-severe haemophilia A patients with inhibitors. THROMB HAEMOSTASIS 2016;115 (3):543-550 [PubMed]
  • Eckhardt CL, Loomans JI, van Velzen AS, Peters M, Mauser-Bunschoten EP, Schwaab R, Mazzucconi MG, Tagliaferri A, Siegmund B, Reitter-Pfoertner SE, van der Bom JG, Fijnvandraat K [Contributors: Fijnvandraat K, Peters M, Kamphuisen PW, van der Bom JG, Peerlinck K, Oldenburg J, Santagostino E, Astermark J, Eckhardt CL, van Velzen AS, Streefkerk N, Loomans JL, van Eijkelenburg A, Jansen AJ, Kruijt CC, van Tienoven B, van Baar ACG, Corten IW, Meijer K, Nijziel MR, Dors N, Hamulyak K, Beckers E, Brons PP, Laros-van Gorkom BAP, van Heerde WL, Leebeek F, Kruip M, Cnossen MH, Mauser-Bunschoten E, Fischer K, Smiers FJ, Hermans C, Schwaab R, Siegmund B, Klamroth R, Escuriola-Ettingshausen C, Königs C, Petrini P, Holmström M, Mäkipernaa A, Male C, Pabinger I, Reitter-Pfoertner SE, Keenan RD, Liesner R, Khair K, Yee TT, Hart DP, Rangarajan S, Mitchell M, Thompson G, Haya S, Moret A, Cid AR, Jimenez-Yuste V, Mancuso ME, Mazzuconni MG, Santoro C, Morfini M, Castaman G, Schinco P, Tagliaferri A, Rivolta GF, Platokouki H, McRae S]] , Inhibitor development and mortality in non-severe hemophilia A. J THROMB HAEMOST 2015;13 (7):1217-1225 [PubMed]
All Publications
Research programmes

Etiology of inhibitor development in hemophilia & Alloimunisation in Sickle Cell Disease

Etiology of inhibitor development in hemophilia

Hemophilia A (Factor VIII deficiency) is a rare bleeding disorder that is treated by intravenous administration of clotting factor concentrates. A major challenge in the treatment is the development of inhibiting antibodies (inhibitors), directed towards F VIII. Inhibitors block the action of F VIII and thereby increase morbidity and mortality.

This research line aims to identify clinical and genetic risk factors for inhibitor development, especially in mild/moderate hemophilia.

  • The INSIGHT study is an international cohort study performed in 34 Hemophilia Treatment Centers in 11 countries that has included 2700 moderate and mild hemophilia A patients. Genetic and clinical risk factors for inhibitor development will be identified in this cohort.
  • Treatment of inhibitors in moderate and mild hemophilia is investigated in the TRIM study.
  • DDAVP can be used as a treatment for bleedings circumventing the use of clotting factor concentrates, thereby reducing the risk of inhibitor development. The RISE study identifies predictors of DDAVP response in mild hemophilia A patients.
  • Small changes in immunological responses may identify patients that will develop an inhibitor in an early stage. The PUPS study aims to analyze the immunological response in young children after their first exposures to F VIII concentrate.

 

Cerebral infarction and vaso-occlusion in Sickle Cell Disease


Sickle Cell Disease is a hereditary anemia caused by a mutation in the beta-globulin gene, that occurs predominantly in persons with African ancestry. Hallmarks of the disease are chronic haemolytic anemia and vascular occlusion, that may cause irreversible damage to all vital organs.


This research line aims to identify risk factors for “silent” cerebral infarction and to identify and treat its neuropsychological sequelae. We also study the pathophysiological processes of (painfull) vaso-occlusive crises and interventions to prevent these.
• FIND study: Finding Indicators of Neurological Damage is a multicenter observational cohort study that aims to identify genetic and clinical risk factors for “silent” cerebral infarction
• KLIK study: Quality of Life in Clinical Practice identifies (neuropsychological) problems in children with Sickle Cell Disease. By early identification therapeutic measures can be initiated as early as possible
• STAR is a multicenter observational study aiming to unravel allo-antibody formation in patients with Sickle Cell Disease
 

Faculty
Prof. dr. C.J. Fijnvandraat
Dr. M. Peters

Postdocs
C.L. Eckhardt MD PhD
S.C. Gouw MD PhD

PhD Students
A. Abdi MD
J.J. Gerritsma
Drs. S.F. Gunnink MD (Sanquin)
M.R. Heesterbeek
F.R. Kloosterman
A. Zwagemaker

Prof. dr. R.A.A. Mathôt (Population pharmacokinetic-pharmacodynamic (PK-PD) research)

Prof. dr. T.W. Kuijpers MD PhD (Infection and inflammation: on the crossroad of hematology and immunology)

Other research related activities
  • Membership of medical or scientific committee, ZonMw (Netherlands Organisation for Health Research and Development), VIDI
  • Membership of medical or scientific committee, Willebrand in Nederland, National multicenter study of von Willebrands Disease in the Netherlands
  • Membership of medical or scientific committee, European Hemophilia Treatment and Standardisation Board, International collaboration among 15 european countries to develop evidence based treatment guisdelines for hemophilia
  • Contribution to guidelines and protocols, Diagnosis and Treatment of Hemophilia
  • Membership of editorial board / Editorship, TIJDSCHRIFT VOOR KINDERGENEESKUNDE
  • Board of directors / Trustees / Supervisory board, Nederlands Signalerings Centrum Kindergeneeskunde
  • Membership of medical or scientific committee, LWHB, Landelijke Werkgroep Hemoglobinopathie Behandelaren
  • Contribution to training of professionals, Annul National Dutch course for MD specialists in Pedatrics and Internal Medicine on Hemophilia and Allies Disorders
Current research funding
  • AMC
  • Baxalta Innovations GmbH
  • Baxalta US Inc., a Delaware corporation
  • BAYER B.V.
  • Bristol Myers Squibb
  • CSL Behring BV
  • Erasmus MC
  • Global Blood Therapeutics
  • IQVIA RDS Netherlands B.V.
  • NIEUWE DEB IND
  • Novo Nordisk B.V.
  • Pfizer B.V. (4726)
  • Pfizer Inc. GFSS - Americas
  • Roche Nederland B.V.
  • Sanquin Blood Supply Foundation
  • Stichting tot Steun EKZ-AMC
  • Swedish Orphan Biovitrum BVBA/SPRL
  • The Hospital for Sick Children