دورية أكاديمية
أعرض في EDS

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

التفاصيل البيبلوغرافية
العنوان: Burden of Poor Health Conditions and Quality of Life in 656 Children with Primary Immunodeficiency.
المؤلفون: Barlogis V; 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, Aix-Marseille University, Provence, France. Electronic address: vincent.barlogis@ap-hm.fr., Mahlaoui N; 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, Sorbonne Paris Cité, University Paris Descartes, Paris, France., Auquier P; Department of Public Health-EA 3279 Research Unit, Aix-Marseille University, Provence, France., Fouyssac F; 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., Pellier I; 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., Vercasson C; Department of Public Health-EA 3279 Research Unit, Aix-Marseille University, Provence, France., Allouche M; Department of Pediatric Hematology-Oncology, Assistance Publique-Hôpitaux de Marseille, Marseille, France., De Azevedo CB; French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France., Moshous D; 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, Sorbonne Paris Cité, University Paris Descartes, Paris, France., Neven B; 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, Sorbonne Paris Cité, University Paris Descartes, Paris, France., Pasquet M; 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, University Hospital of Toulouse, IUCT-Oncopole, INSERM, Toulouse, France., Jeziorski E; 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 Arnaud de Villeneuve, Montpellier University Hospital, Montpellier, France., Aladjidi N; 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, CIC 0005, INSERM CICP, University Hospital of Bordeaux, Bordeaux, France., Thomas C; 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, University Hospital of Nantes, Nantes, France., Gandemer V; 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., Mazingue F; 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., Picard C; 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, Sorbonne Paris Cité, University Paris Descartes, Paris, France., Blanche S; 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, Sorbonne Paris Cité, University Paris Descartes, Paris, France., Michel G; 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, Aix-Marseille University, Provence, France., Fischer A; 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, Sorbonne Paris Cité, University Paris Descartes, Paris, France; Division of Médecine Expérimentale, Collège de France, Paris, France.
المصدر: The Journal of pediatrics [J Pediatr] 2018 Mar; Vol. 194, pp. 211-217.e5. Date of Electronic Publication: 2017 Dec 06.
نوع المنشور: Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: Mosby Country of Publication: United States NLM ID: 0375410 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1097-6833 (Electronic) Linking ISSN: 00223476 NLM ISO Abbreviation: J Pediatr Subsets: MEDLINE
أسماء مطبوعة: Original Publication: St. Louis, MO : Mosby
مواضيع طبية MeSH: Cost of Illness* , Health Status* , Quality of Life*, Immunologic Deficiency Syndromes/*complications, Adolescent ; Child ; Child, Preschool ; Cohort Studies ; Female ; Follow-Up Studies ; France ; Humans ; Male ; Prospective Studies ; Registries ; Surveys and Questionnaires
مستخلص: Objective: To gain insight into how primary immunodeficiencies (PIDs) affect children's health status and quality of life.
Study Design: 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.
Results: 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.
Conclusions: 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.
Trial Registration: ClinicalTrials.gov: NCT02868333 and EudraCT 2012-A0033-35.
(Copyright © 2017 Elsevier Inc. All rights reserved.)
فهرسة مساهمة: Keywords: health status; primary immunodeficiency; transition to adulthood
سلسلة جزيئية: ClinicalTrials.gov NCT02868333
تواريخ الأحداث: Date Created: 20171205 Date Completed: 20190219 Latest Revision: 20190320
رمز التحديث: 20221213
DOI: 10.1016/j.jpeds.2017.10.029
PMID: 29198545
قاعدة البيانات: MEDLINE
الوصف
تدمد:1097-6833
DOI:10.1016/j.jpeds.2017.10.029