[Health status and quality of life in β-thalassemia adults in Marseille, France].
Journal: La Revue de medecine interne
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Affiliated Institutions:
Service de médecine interne et médecine polyvalente, centre hospitalier d'Ajaccio Notre-Dame de la Miséricorde, site du Stilettu, , route A.-Madunuccia, Ajaccio, France. Electronic address: caroline.soubrier@ch-ajaccio.fr.
Département de médecine interne, AP-HM, hôpital Timone, , rue Saint-Pierre, Marseille cedex, France.
Centre de référence des syndromes drépanocytaires majeurs, thalassémies et autres pathologies rares du globule rouge et de l'érythropoïèse, hôpital de la Timone, AP-PH, , rue Saint-Pierre, Marseille cedex, France.
Service de médecine interne, centre hospitalier de Martigues, , boulevard des Rayettes, BP , Martigues cedex, France.
Établissement français du sang, région PACA, , boulevard Baille, Marseille, France.
AP-HM, Hop Timone, BioSTIC, biostatistiques et technologies de l'information et de la communication, Marseille, France.
AP-HM, Hop Timone, BioSTIC, biostatistiques et technologies de l'information et de la communication, Marseille, France; Aix Marseille université, AP-HM, Inserm, IRD, SESSTIM, sciences économiques & sociales de la santé & traitement de l'information médicale, ISSPAM, Marseille, France.
Département de médecine interne, AP-HM, hôpital Timone, , rue Saint-Pierre, Marseille cedex, France; Aix-Marseille université, Marseille, France.
Laboratoire de biochimie, AP-HM, hôpital Timone, , rue Saint-Pierre, Marseille cedex, France.
Unité transversale de la drépanocytose, centre de référence Antilles-Guyane pour la drépanocytose, les thalassémies et les maladies constitutives du globule rouge et de l'érythropoïèse, CHU Guadeloupe, pôle parents-enfants, hôpital Ricou, BP, Pointe-à-Pitre cedex, France.
Abstract summary
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.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.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).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.
Authors & Co-authors:
Soubrier
Jean
De Sainte Marie
Agouti
Seguier
Lavoipierre
Clapasson
Iline
Gonin
Giorgi
Schleinitz
Thuret
Badens
Bernit
Study Outcome
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