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1. Choice of factor VIII/IX regimen in adolescents and young adults with severe or moderately severe haemophilia. A French national observational study (ORTHem 15-25)

Author

Sandrine Meunier, Roseline d'oiron, Hervé Chambost, Edita Dolimier, Benoît Guillet, Annie Borel-Derlon, Jeanne-Yvonne Borg, Ségolène Claeyssens, Roseline d'Oiron, Valérie Gay, Stéphane Girault, Jenny Goudemand, Yves Gruel, Benoit Guillet, Abel Hassoun, Philippe Nguyen, Brigitte Pautard, Jocelyne Peynet, Chantal Rothschild, Jean-François Schved, Marc Trossaert, Alain Marques-Verdier, Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Nutrition, obésité et risque thrombotique (NORT), Institut National de la Recherche Agronomique (INRA)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire d'hématologie biologique [Hôpital de la Timone - Hôpital Nord - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE), Shire France, Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Baxalta, Université d'Angers (UA)-Université de Rennes 1 (UR1), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Adolescent, Haemophilia A, Hemorrhage, 030204 cardiovascular system & hematology, Hemophilia A, Haemophilia, Factor IX, Young Adult, 03 medical and health sciences, 0302 clinical medicine, On demand, Hemarthrosis, Humans, Medicine, Young adult, Retrospective Studies, Factor VIII, Coagulants, business.industry, Prophylaxis, musculoskeletal, neural, and ocular physiology, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Hematology, Bleed, medicine.disease, 3. Good health, Regimen, Treatment Outcome, nervous system, Haemorrhage, Female, Severe haemophilia A, Observational study, On-demand treatment, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, France, Age groups, business, 030215 immunology
Abstract

International audience; Introduction: The value and challenges of long-term prophylaxis (LTP) in adolescents and young adults need further characterisation.Aim: To determine the proportions of adolescents and young adults with severe or moderately severe haemophilia in France under LTP and treatment on demand (OD).Methods: Patients 15 to 25 years old with haemophilia A or B, factor VIII/IX ≤ 2% and no current inhibitor could be included if they had been under factor VIII/IX treatment at least 12 months and kept a treatment and bleeding diary.Results: LTP was administered to 169/212 patients (79.7%) and OD treatment to 40/212 patients (18.9%). The most frequent reasons for initiating LTP were joint bleeding, target joints and frequent bleeds; whereas OD treatment was most often selected on the basis of mild bleeding phenotype or because of constraints on LTP. The mean annual bleed rate (ABR) in the OD group (6.33) was higher than in the LTP group (3.07, p 18–21 and > 21–25 years), but was significantly higher for patients with severe haemophilia (4.02) as compared to those with moderate haemophilia (1.97, p = 0.002). No significant difference was observed in mean ABR for joint bleeds between the LTP and OD groups. Physician reported LTP compliance was good or excellent in 97.0% of patients.Conclusion: LTP is the predominant factor VIII/IX treatment among adolescents and young adults with severe or moderately severe haemophilia in France. LTP was associated with low ABR and high compliance. © 2017 The Authors

Published
2017
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2. Familial history of cancer and childhood acute leukemia: a French population-based case-control study

Author

Lionel de Lumley, Corinne Armari-Alla, Martine Munzer, Jacqueline Clavel, Alain Robert, Jean-Pierre Lamagnere, Christian Berthou, Patrick Boutard, Florence Menegaux, Virginie Gandemer, Patrick Lutz, Francoise Mechinaud, Emmanuel Plouvier, Brigitte Pautard, Frédéric Millot, Jean-Pierre Vannier, M. Ripert, Geneviève Margueritte, Xavier Rialland, Yves Perel, Epidémiologie environnementale des cancers, Université Paris-Sud - Paris 11 (UP11)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Pellegrin, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Hôpital mère et enfant, Hôpital Saint-Jacques, Hôpital Sud, hôpital Sud, Hôpital Civil, Hopital Civil, CHU Rouen, Normandie Université (NU), CHRU Clocheville, SFO Enseignement, SFO, Hôpital Côte de Nacre [CHU Caen], CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Hôpital d'enfants, Hôpital de la Tronche, American Memorial Hospital, American Memory Hospital, Hôpital Jean Bernard, CHU Limoges, Hôpital Morvan, Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Centre Robert Debré, Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), CHU Amiens-Picardie, Pôle Enfants [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), and Secretariat, U754

Subjects
Male, Oncology, Cancer Research, Pediatrics, Epidemiology, 0302 clinical medicine, Neoplasms, MESH: Child, Medicine, MESH: Neoplasms, 030212 general & internal medicine, Child, Acute leukemia, education.field_of_study, MESH: Infant, Newborn, leukemia, familial history, Myeloid leukemia, MESH: Case-Control Studies, MESH: Infant, 3. Good health, Leukemia, Child, Preschool, 030220 oncology & carcinogenesis, Acute Disease, MESH: Acute Disease, Female, medicine.medical_specialty, Adolescent, case-control study, Population, Article, 03 medical and health sciences, Internal medicine, MESH: Leukemia, Humans, Family, education, MESH: Family, childhood, MESH: Adolescent, MESH: Humans, business.industry, MESH: Child, Preschool, Infant, Newborn, Public Health, Environmental and Occupational Health, Case-control study, Infant, Cancer, Odds ratio, medicine.disease, MESH: Male, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, Case-Control Studies, Etiology, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, business, MESH: Female
Abstract

International audience; A case-control study was conducted to investigate the role of a familial history of cancer in the etiology of childhood acute leukemia. The history of cancer in the relatives of 472 cases was compared with that of 567 population-based controls. Recruitment was frequency matched on age, sex and region. The familial history of cancer in each child's relatives was reported by the mother in response to a standardized self-administered questionnaire. A familial history of solid tumor in first or second-degree relatives was associated with an increased risk of acute lymphoblastic leukemia (odds ratio (OR)=1.6 [95% confidence interval, 1.2-2.1]), while a familial history of hematopoietic malignancies in first or second-degree relatives was associated with an increased risk of acute myeloid leukemia (OR=4.3 [1.4-13]). The ORs for the histories of cancer increased with the number of relatives with cancer (OR=1.5 [1.1-2.0] for one relative and OR=2.3 [1.3-3.8] for two relatives or more; Ptrend

Published
2007
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