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The epidemiology of Budd–Chiari syndrome in France

Isabelle Ollivier-Hourmand 1, * Manon Allaire 1, 2 Nathalie Goutte Rémy Morello Carine Chagneau-Derrode 3 Odile Goria 4 Jérôme Dumortier 5 Jean Paul Cervoni 6 Sébastien Dharancy 7 Nathalie Ganne-Carrié 8 Christophe Bureau Nicolas Carbonell 9 Armand Abergel 10 Jean Baptiste Nousbaum 11 Rodolphe Anty Helene Barraud 12 Marie Pierre Ripault Victor de Ledinghen 13 Anne Minello 14 Frédéric Oberti 15 Sylvie Radenne 16 Noelle Bendersky 17 Olivier Farges 18 Isabelle Archambeaud 19 Anne Guillygomarc'H Marie Ecochard 20 Violaine Ozenne 21 Marie Noëlle Hilleret 17 Noelle Hilleret 17 Eric Nguyen-Khac 22 Barbara Dauvois 23 Jean Marc Perarnau 24 Pascale Lefilliatre Jean Jacques Raabe 25 Michel Doffoel 26 Jean Philippe Becquart Eric Saillard 27 Dominique Valla Thong Dao 1 Aurélie Plessier 18 - French Network For Vascular Disorders of The Liver 
* Auteur correspondant
Abstract : Introduction Epidemiological data is lacking on primary Budd–Chiari syndrome (BCS) in France. Methods Two approaches were used: (1) A nationwide survey in specialized liver units for French adults. (2) A query of the French database of discharge diagnoses screening to identify incident cases in adults. BCS associated with cancer, alcoholic/viral cirrhosis, or occurring after liver transplantation were classified as secondary. Results Approach (1) 178 primary BCS were identified (prevalence 4.04 per million inhabitants (pmi)), of which 30 were incident (incidence 0.68 pmi). Mean age was 40 ± 14 yrs. Risk factors included myeloproliferative neoplasms (MPN) (48%), oral contraceptives (35%) and factor V Leiden (16%). None were identified in 21% of patients, ≥2 risk factors in 25%. BMI was higher in the group without any risk factor (25.7 kg/m2 vs 23.7 kg/m2, p < 0.001). Approach (2) 110 incident primary BCS were admitted to French hospitals (incidence 2.17 pmi). MPN was less common (30%) and inflammatory local factors predominated (39%). Conclusion The entity of primary BCS as recorded in French liver units is 3 times less common than the entity recorded as nonmalignant hepatic vein obstruction in the hospital discharge database. The former entity is mostly related to MPN whereas the latter with abdominal inflammatory diseases.
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Soumis le : mardi 5 mars 2019 - 09:56:34
Dernière modification le : vendredi 8 avril 2022 - 16:18:10



Isabelle Ollivier-Hourmand, Manon Allaire, Nathalie Goutte, Rémy Morello, Carine Chagneau-Derrode, et al.. The epidemiology of Budd–Chiari syndrome in France. Digestive and Liver Disease, WB Saunders, 2018, 50 (9), pp.931-937. ⟨10.1016/j.dld.2018.04.004⟩. ⟨hal-02057088⟩



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