Burden of Poor Health Conditions and Quality of Life in 656 Children with Primary Immunodeficiency

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

doi:10.1016/j.jpeds.2017.10.029

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Burden of Poor Health Conditions and Quality of Life in 656 Children with Primary Immunodeficiency

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Burden of Poor Health Conditions and Quality of Life in 656 Children with Primary Immunodeficiency

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

The Journal of pediatrics, Vol.194, pp.211-217.e5

01/03/2018

DOI: https://doi.org/10.1016/j.jpeds.2017.10.029

PMID: 29198545

Résumé

health status

primary immunodeficiency

transition to adulthood

To gain insight into how primary immunodeficiencies (PIDs) affect children's health status and quality of life. The French Reference Center for PIDs conducted a prospective multicenter cohort that enrolled participants who met all criteria: patients included in the French Reference Center for PIDs registry, children younger than18 years, and living in France. Participants were asked to complete both a health questionnaire and a health-related quality of life (HR-QoL) questionnaire. A severity score was assigned to each health condition: grade 1 (mild) to grade 4 (life-threatening). HR-QoL in children was compared with age- and sex-matched French norms. Among 1047 eligible children, 656 were included in the study, and 117 had undergone hematopoietic stem cell transplantation; 40% experienced at least one grade 4 condition, and 83% experienced at least one grade 3 or 4 condition. Compared with the French norms, children with PID scored significantly lower for most HR-QoL domains. Low HR-QoL scores were associated strongly with burden of poor conditions. Our results quantify the magnitude of conditions in children with PID and demonstrate that the deleterious health effects borne by patients already are evident in childhood. These results emphasize the need to closely monitor this vulnerable population and establish multidisciplinary healthcare teams from childhood. ClinicalTrials.gov: NCT02868333 and EudraCT 2012-A0033-35.

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Titre: Burden of Poor Health Conditions and Quality of Life in 656 Children with Primary Immunodeficiency
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, Aix-Marseille University, Provence, France
Fanny Fouyssac - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Isabelle Pellier - French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
Camille Vercasson - Aix-Marseille Université
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
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
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
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: The Journal of pediatrics, Vol.194, pp.211-217.e5
Éditeur: Elsevier Inc
Note de subvention: French National Program for Clinical Research
Identifiants: 99148043509311
Unité académique: Pathogenesis and Control of Chronic and Emerging Infections - PCCEI
Langue: English
Type de ressource: Journal article