📇 Rhumatologue · BEAUCE LA ROMAINE · (41) · Salarié
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2 raisons identifiées
Référence presse grand public
Cité 1 fois dans les médias — pédagogie reconnue
Délais de RDV courts dans la région
86 rhumatos / 100 000 hab. — département bien doté
✨ Génération du profil synthétique IA en cours…
CENTRE DE SANTÉ TERRES DU VAL DE LOIRE
5 PL DU 8 MAI, 41160 BEAUCE LA ROMAINE
Secteur de conventionnement non disponible (médecin hospitalier ou non présent dans l'Annuaire santé CNAM des libéraux conventionnés).
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Lien Doctolib = recherche Google site:doctolib.fr (le 1er résultat est presque toujours le profil correct s'il existe).
Source : Google News (recherche par nom complet — homonymes possibles, vérifier le contenu).
📰 La République du Centre · 06/07/2024
<a href="https://news.google.com/rss/articles/CBMimAFBVV95cUxPTTFrX1RJbEZHcWM0Z0xGam5jQ3V0RGRuaFZyZEg4ZWhFWXJsa3dYb2ZkZU5EczRmVWwxMG9LVW5hY1NKNWZYN2JEX0I4NWpGR2NnekljYXRrRktfT1Brc2NGRzRIanRneTFZWHh1M0tBZjBFX3FPdXY1U0JFSHdXTXVaTUl1Ny14VHpLVnhNZ1JjajBnRGRwRA?oc=5" target="_blank">Le centre de santé in
Blood advances · 2026
Abstract Congenital thrombotic thrombocytopenic purpura (cTTP) is caused by a severe inherited ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13) deficiency. Although acute episodes are life-threatening, long-term burden of ischemic complications, and effectiveness of prophylactic strategies remain underexplored. We conducted a 25-year national, multicenter study of 88 patients with cTTP enrolled in the French Thrombotic Microangiopathy registry. Patients were stratified by age and clinical context at disease onset: pediatric (n = 42), pregnancy (n = 33), and adult onset (n = 13). Clinical features, genotypes, treatment regimens, and long-term ischemic outcomes were analyzed. Pediatric patients exhibited early-onset disease (median age at diagnosis, 2 years [interquartile range, 0-13]) with recurrent episodes and a high burden of neurological complications. In patients with pregnancy-onset disease, no relapses were observed outside pregnancy. Adult-onset patients, typically diagnosed aged &gt;50 years, showed prevalent cardiovascular disease, stroke, and kidney injury. Mental health disorders were common across all groups. Despite long-term plasma prophylaxis, organ dysfunction persisted, particularly in pediatric- and adult-onset groups. In pregnancy-onset cTTP, tailored midterm prophylaxis during pregnancy reduced maternal and fetal complications. Thirty-nine patients received recombinant human ADAMTS13 (rhADAMTS13) prophylaxis (median follow-up, 5 months [interquartile range, 6-17]). Prophylactic treatment significantly improved relapse-free survival, with a comparable outcome using intensive plasma-derived products and rhADAMTS13 in pediatric-onset cases, although adverse events were more prevalent using plasma therapy. Our findings highlight the clinical and genetic heterogeneity of cTTP and the burden of organ dysfunction. Intensive prophylaxis improves relapse-free survival. rhADAMTS13 represents a safe and promising first-line option that should help reduce long-term organ damage and improve quality of life.
Source PubMed · Recherche par auteur (homonymes possibles, vérifier l'affiliation).
Blood advances · 2026 · Journal Article
Joly BS, Joseph A, Bouzid R, Boisseau P, et al.