title: Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study.
abstract: BACKGROUND
  Most children with primary immunodeficiencies (PIDs) now reach adulthood. However, few studies have evaluated their health status and health-related quality of life (HRQoL).
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatric Hematology-Oncology, Assistance Publique-Hôpitaux de Marseille, Marseille, France; French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Public Health-EA 3279 Research Unit, University Hospital Marseille, Aix-Marseille University, Marseille, France. Electronic address: vincent.barlogis@ap-hm.fr.
authors.forename: Vincent
authors.initials: V
authors.lastname: Barlogis
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France.
authors.forename: Nizar
authors.initials: N
authors.lastname: Mahlaoui
authors.affiliation: Department of Public Health-EA 3279 Research Unit, University Hospital Marseille, Aix-Marseille University, Marseille, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Auquier
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatric Oncohematology, University Hospital of Angers, Angers, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Pellier
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatric Hematology-Oncology, University Hospital of Nancy, Nancy, France.
authors.forename: Fanny
authors.initials: F
authors.lastname: Fouyssac
authors.affiliation: Department of Public Health-EA 3279 Research Unit, University Hospital Marseille, Aix-Marseille University, Marseille, France.
authors.forename: Camille
authors.initials: C
authors.lastname: Vercasson
authors.affiliation: Department of Pediatric Hematology-Oncology, Assistance Publique-Hôpitaux de Marseille, Marseille, France.
authors.forename: Maya
authors.initials: M
authors.lastname: Allouche
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors.forename: Carolina Brito
authors.initials: CB
authors.lastname: De Azevedo
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France; Adult Hematology Unit, Necker University Hospital, APHP, University Paris Descartes, Paris, France; CNRS ERL8254, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France.
authors.forename: Felipe
authors.initials: F
authors.lastname: Suarez
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France.
authors.forename: Despina
authors.initials: D
authors.lastname: Moshous
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France.
authors.forename: Bénédicte
authors.initials: B
authors.lastname: Neven
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Département d'Hématologie, Centre Hospitalier Universitaire Toulouse Purpan and INSERM, CRCT, IUCT-Oncopole, Toulouse, France.
authors.forename: Marlène
authors.initials: M
authors.lastname: Pasquet
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Service de Pédiatrie Générale et Infectiologie et Immunologie Clinique, Hôpital Arnaud de Villeneuve, Montpellier University Hospital, Montpellier, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Jeziorski
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatric Hematology and CIC 0005, INSERM CICP, University Hospital of Bordeaux, Bordeaux, France.
authors.forename: Nathalie
authors.initials: N
authors.lastname: Aladjidi
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Internal Medicine, CHU Timone, Assistance Publique-Hôpitaux de Marseille, Marseille, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Hematology-Oncology Unit, Hôpital Mère Enfant, University Hospital of Nantes, Nantes, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Thomas
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatric Hematology/Oncology, University Hospital of Rennes, Rennes, France.
authors.forename: Virginie
authors.initials: V
authors.lastname: Gandemer
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatrics, Hôpital Jeanne de Flandre, University Hospital of Lille, Lille, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Mazingue
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Hematology-Oncology, EA3430, Strasbourg, France.
authors.forename: Patrick
authors.initials: P
authors.lastname: Lutz
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France; Adult Hematology Unit, Necker University Hospital, APHP, University Paris Descartes, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France.
authors.forename: Capucine
authors.initials: C
authors.lastname: Picard
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Blanche
authors.affiliation: Department of Pediatric Hematology-Oncology, Assistance Publique-Hôpitaux de Marseille, Marseille, France; French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Public Health-EA 3279 Research Unit, University Hospital Marseille, Aix-Marseille University, Marseille, France.
authors.forename: Gérard
authors.initials: G
authors.lastname: Michel
authors.affiliation: French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France; Collège de France, Paris, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Fischer
mesh_terms: D000328:Adult; D017668:Age of Onset; D002648:Child; D015331:Cohort Studies; D005260:Female; D005602:France; D006304:Health Status; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D008297:Male; D011788:Quality of Life; D011795:Surveys and Questionnaires; D013995:Time
keywords: Primary immunodeficiency; childhood; health status; quality of life; young adults