Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study

Vincent Barlogis; Nizar Mahlaoui; Pascal Auquier; Isabelle Pellier; Fanny Fouyssac; Camille Vercasson; Maya Allouche; Carolina Brito De Azevedo; Felipe Suarez; Despina Moshous; Bénédicte Neven; Marlène Pasquet; Eric Jeziorski; Nathalie Aladjidi; Nicolas Schleinitz; Caroline Thomas; Virginie Gandemer; Françoise Mazingue; Patrick Lutz; Olivier Hermine; Capucine Picard; Stéphane Blanche; Gérard Michel; Alain Fischer

doi:10.1016/j.jaci.2016.08.027

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Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study

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Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study

Vincent Barlogis, Nizar Mahlaoui, Pascal Auquier, Isabelle Pellier, Fanny Fouyssac, Camille Vercasson, Maya Allouche, Carolina Brito De Azevedo, Felipe Suarez, Despina Moshous, …

Journal of allergy and clinical immunology, Vol.139(4), pp.1275-1281.e7

01/04/2017

DOI: https://doi.org/10.1016/j.jaci.2016.08.027

PMID: 27697497

Résumé

childhood

health status

Primary immunodeficiency

quality of life

young adults

Most children with primary immunodeficiencies (PIDs) now reach adulthood. However, few studies have evaluated their health status and health-related quality of life (HRQoL). To investigate long-term morbidity, the French Reference Center for PIDs initiated a prospective multicenter cohort: the French Childhood Immune Deficiency Long-term Cohort. The data collected were used to assess the physical health condition of patients who reached adulthood and the effect on their quality of life. Patients were asked to complete health status questionnaires. A severity score (grade 1 [mild] to grade 4 [life-threatening]) was assigned to each health condition. The HRQoL of patients was compared with age- and sex-matched French normal values by using the 36-item Short-Form Survey (SF-36) HRQoL questionnaire. Among 329 participants, the mean age at evaluation was 27.6 years, with a 21-year mean follow-up after diagnosis; 43% reported at least 1 grade 4 health condition, and 86% reported at least 1 grade 3 (severe) or 4 health condition. Twenty-five (7.6%) patients had been treated for cancer. Compared with the French normal values, adults with PIDs scored significantly lower for all HRQoL domains. HRQoL was strongly associated with the burden of health conditions. The association with grade 4 or grade 3-4 health conditions was highly significant for all physical and mental domains. Adults with PIDs diagnosed during childhood experienced a heavy burden of health conditions, which affected their HRQoL. Our results emphasize the need to closely monitor this vulnerable population.

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Titre: Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study
Créateurs - sans rôle: Vincent Barlogis - Department of Pediatric Hematology-Oncology, Assistance Publique-Hôpitaux de Marseille, Marseille, France
Nizar Mahlaoui - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Pascal Auquier - Department of Public Health–EA 3279 Research Unit, University Hospital Marseille, Aix-Marseille University, Marseille, France
Isabelle Pellier - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Fanny Fouyssac - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Camille Vercasson - Department of Public Health–EA 3279 Research Unit, University Hospital Marseille, Aix-Marseille University, Marseille, France
Maya Allouche - Department of Pediatric Hematology-Oncology, Assistance Publique-Hôpitaux de Marseille, Marseille, France
Carolina Brito De Azevedo - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Felipe Suarez - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Despina Moshous - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Bénédicte Neven - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Marlène Pasquet - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Eric Jeziorski - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Nathalie Aladjidi - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Nicolas Schleinitz - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Caroline Thomas - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Virginie Gandemer - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Françoise Mazingue - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Patrick Lutz - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Olivier Hermine - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Capucine Picard - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Stéphane Blanche - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Gérard Michel - Assistance Publique Hôpitaux de Marseille
Alain Fischer - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Détails de publication: Journal of allergy and clinical immunology, Vol.139(4), pp.1275-1281.e7
Éditeur: Elsevier Inc
Identifiants: 99148749609311
Unité académique: Pathogenesis and Control of Chronic and Emerging Infections - PCCEI
Langue: English
Type de ressource: Journal article