1. [Health status and quality of life in β-thalassemia adults in Marseille, France].
- Author
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Soubrier C, Jean E, De Sainte Marie B, Agouti I, Seguier J, Lavoipierre V, Clapasson C, Iline N, Gonin J, Giorgi R, Schleinitz N, Thuret I, Badens C, and Bernit E
- Subjects
- Humans, France epidemiology, Male, Female, Adult, Retrospective Studies, Young Adult, Middle Aged, Blood Transfusion statistics & numerical data, Iron Overload epidemiology, Iron Overload etiology, Surveys and Questionnaires, Adolescent, Quality of Life, beta-Thalassemia therapy, beta-Thalassemia epidemiology, beta-Thalassemia complications, beta-Thalassemia psychology, Health Status
- Abstract
Introduction: The life expectancy of β-thalassemia patients has increased over the last 20 years. In this study, we evaluated the current health status and quality of life of these patients managed in a reference center in Marseille., Methods: This is a single-center, descriptive study conducted between June and August 2019 in patients over 18 years of age with β-thalassemia major or intermedia. Clinical and paraclinical data were collected retrospectively and the SF-36 health survey questionnaire was proposed to each patient., Results: 43 of 64 selected patients were included and divided into 2 groups: 35 patients with transfusion-dependent β-thalassemia and 8 patients with non-transfusion-dependent β-thalassemia. Liver iron overload is the most frequent complication, present in 80% of transfusion-dependent and 62.5% of non-transfusion-dependent patients. Cardiac iron overload is present only in the transfusion dependent β-thalassemia group (20%). Hypogonadotropic hypogonadism remains the most common endocrine disorder (41.9%) followed by osteoporosis (37.2%). Among the 31 patients who completed the SF-36 questionnaire, physical and mental quality of life scores were lowered in transfusion dependent (respectively 42.7 and 46.8) as in non-transfusion-dependent patients (respectively 43.8 and 28.9)., Conclusion: Despite an improvement in medical care, our patients with β-thalassemia show an alteration in their quality of life that will need to be characterized in the entire French cohort., (Copyright © 2024 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)
- Published
- 2024
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