Isabelle Ollivier-Hourmand1, Manon Allaire2, Nathalie Goutte3, Rémy Morello4, Carine Chagneau-Derrode5, Odile Goria6, Jerôme Dumortier7, Jean Paul Cervoni8, Sébastien Dharancy9, Nathalie Ganne-Carrié10, Christophe Bureau11, Nicolas Carbonell12, Armand Abergel13, Jean Baptiste Nousbaum14, Rodolphe Anty15, Hélène Barraud16, Marie Pierre Ripault17, Victor De Ledinghen18, Anne Minello19, Frédéric Oberti20, Sylvie Radenne21, Noelle Bendersky22, Olivier Farges23, Isabelle Archambeaud24, Anne Guillygomarc'h25, Marie Ecochard26, Violaine Ozenne27, Marie Noelle Hilleret28, Eric Nguyen-Khac29, Barbara Dauvois30, Jean Marc Perarnau31, Pascale Lefilliatre32, Jean Jacques Raabe33, Michel Doffoel34, Jean Philippe Becquart35, Eric Saillard36, Dominique Valla37, Thong Dao38, Aurélie Plessier37. 1. Hepato Gastroenterology Nutrition Department, University Hospital Center Côte de Nacre, Caen, France. Electronic address: ollivierhourmand-i@chu-caen.fr. 2. Hepato Gastroenterology Nutrition Department, University Hospital Center Côte de Nacre, Caen, France; Inserm Unit U1149, Research Center on Inflammation, Paris, France. 3. UMRS1193 and University Hospitals of Paris Sud, France. 4. Public Health Unit, University Hospital Center Côte de Nacre, Caen, France. 5. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire, Poitiers, France. 6. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Charles Nicolle, Rouen, France. 7. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Edouard Herriot, Lyon, France. 8. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Jean Minjoz, Besançon, France. 9. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Claude Huriez, Lille, France. 10. Service d'hépatologie, Centre Hospitalo-Universitaire Hôpital Jean Verdier, APHP, Bondy, France; Inserm UNR-1162, « Génomique Fonctionnelle des Tumeurs Solides », Paris, France. 11. Service d'Hépatologie, Centre Hospitalo-Universitaire Purpan, Toulouse, France. 12. Service d'Hépatologie, Centre Hospitalo-Universitaire Saint Antoine, APHP, Paris, France. 13. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Estaing, Clermont Ferrand, France. 14. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire La Cavale Blanche, Brest, France. 15. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire, Nice, France. 16. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire de Nancy, Vandœuvre-lès-Nancy, France. 17. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Saint Eloi, Montpellier, France. 18. Service d'Hépatologie, Hôpital Haut-Lévêque, Bordeaux, France. 19. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire de Dijon Bourgogne, Dijon, France. 20. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire, Angers, France; Laboratoire HIFIH, Université Bretagne Loire, Angers, France. 21. Service d'Hépato-gastroentérologie, Hôpital de la Croix Rousse, Lyon, France. 22. Service d'hépatologie, Hôpital Beaujon, APHP, Clichy-la-Garenne, France. 23. Service de Chirurgie Hépato-Pancréato-Biliaire, Hôpital Beaujon, APHP, Clichy-la-Garenne, France. 24. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Hôtel-Dieu, Nantes, France. 25. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Pontchaillou, Rennes, France. 26. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire, Saint-Etienne, France. 27. Service d'Hépatologie, Hôpital Lariboisière, APHP, Paris, France. 28. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Michallon, Grenoble, France. 29. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire, Amiens, France. 30. Service d'Hépato-gastroentérologie, Centre Hospitalier Régional, Orléans, France. 31. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire, Tours, France. 32. Service d'Hépato-gastroentérologie, Centre Hospitalier Général, Le Havre, France. 33. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Metz-Thionville, Ars-Laquenexy, France. 34. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire, Strasbourg, France. 35. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Saint Pierre, La Réunion, France. 36. Service d'Hépato-gastroentérologie, Centre Hospitalo-Universitaire Pointe à Pître, Guadeloupe, France. 37. Service d'hépatologie, Hôpital Beaujon, APHP, Clichy-la-Garenne, France; Centre de Recherche de l'Inflammation, Inserm UMR-1149, Université Paris Diderot, Paris, France. 38. Hepato Gastroenterology Nutrition Department, University Hospital Center Côte de Nacre, Caen, France.
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.
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.