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

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 P...

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Bibliographic Details
Published in:The Journal of pediatrics 2018-03, Vol.194, p.211-217.e5
Main Authors: Barlogis, Vincent, Mahlaoui, Nizar, Auquier, Pascal, Fouyssac, Fanny, Pellier, Isabelle, Vercasson, Camille, Allouche, Maya, De Azevedo, Carolina Brito, Moshous, Despina, Neven, Bénédicte, Pasquet, Marlène, Jeziorski, Eric, Aladjidi, Nathalie, Thomas, Caroline, Gandemer, Virginie, Mazingue, Françoise, Picard, Capucine, Blanche, Stéphane, Michel, Gérard, Fischer, Alain
Format: Article
Language:English
Subjects:
health status
Life Sciences
primary immunodeficiency
transition to adulthood
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Summary: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.
ISSN:0022-3476
1097-6833
DOI:10.1016/j.jpeds.2017.10.029