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Aetiological factors of Budd-Chiari syndrome in Algeria

World Journal of hepatology 2015年第6期7卷 903-909页

作者： Nawel Afredj Nawal Guessab Abdelbasset Nani Sid Ahmed Faraoun Ibtissem Ouled Cheikh Rafik Kerbouche Djouhar hannoun Zine Charef Amir hayet Ait Kaci Kamel Bentabak Aurélie Plessier Dominique-Charles Valla valerie cazals-hatem.m.rie-hélène denninger Tadjeddine Boucekkine Nabil Debzi hepatology Unit Department of Medicine Mustapha Hospital Radiology Unit Centre Pierre and Marie Curie National Institute of Public health (INSP) Pathology Unit CHU Mustapha Pathology Unit Centre Pierre and Marie Curie Surgery Unit Centre Pierre and Marie Curie hepatology Unit Beaujon Hospital Pathology Unit Beaujon Hospital Immunohematology laboratory Beaujon Hospital

syndrome （BCS） and identify the aetiologies of thisdisease in ***： Patients with BCS, hospitalised in our unitfrom January 2004 until June 2010 were included and theaetiological factors were assessed. Patients presentinga BCS in the setting of advanced-stage cirrhosis or aliver transplantation were excluded from the study. Thediagnosis was established when an obstruction of hepaticvenous outflow （thrombosis, stenosis or compression）was demonstrated. We diagnosed myeloproliferativedisease （mPD） by bone marrow biopsy and V617FJAK2 mutation. Anti-phospholipid syndrome （APlS） wasdetected by the presence of anticardiolipin antibodies,anti-β2 glycoprotein antibodies and lupus *** also detected paroxysmal nocturnal haemoglobinuria（PNh） by flow cytometry. Celiac disease and Beh？etdisease were systematically investigated in our *** anticoagulant protein deficiencies were alsoassessed. We tested our patients for the G20210Amutation at Beaujon hospital. Imaging procedures wereperformed to determine a local cause of BCS, such as ahydatid cyst or a liver ***： One hundred and fifteen patients wereincluded. mean follow up： 32.12 mo. mean age： 34.41years, m/F = 0.64. Chronic presentation was frequent：63.5%. The revealing symptoms for the BCS wereascites （74.8%） and abdominal pain （42.6%）. Themost common site of thrombosis was the hepatic veins（72.2%）. Involvement of the inferior vena cava alonewas observed in 3 patients. According to the radiologicalinvestigations, BCS was primary in 94.7% of the cases（n = 109） and secondary in 5.2% （n = 6）. An aetiologywas identified in 77.4% of the patients （n = 89）; itwas multifactorial in 27% （n = 31）. The predominantaetiology of BCS in our patients was a myeloproliferativedisease, observed in 34.6% of cases. APlS was foundin 21.7% and celiac disease in 11.4%. Other acquiredconditions were： PNh （n = 4）, systemic disease （n = 6）and inflammatory bowel disease （n = 5）. Anticoagulantprotein defici

关键词： Algeria Etiologie Celiac disease Budd-Chiari Thrombosis

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特发性肝窦扩张与抗磷脂综合征免疫学特征的相关

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世界核心医学期刊文摘（胃肠病学分册） 2005年第2期1卷 38-38页

作者： Saadoun D. cazals-hatem D. denninger m.-h. D. Valla 翟惠虹 Service d'hép atalogie Hpital Beaujon 92118 Clichy France

Background: Isolated sinusoidal dilatation is an uncommon hepatic lesion and t he cause is largely unknown. Objective: To investigate whether prothrombotic dis orders or perisinusoidal cell changes could be involved in pure idiopathic hepat ic sinusoidal dilatation (hSD). methods: Evaluation for associated conditions, p rothrombotic disorders, and studies of hepatic perisinusoidal cell activation in consecutive patients, seen between 1993 and 2002, with isolated sinusoidal dila tation unrelated to outflow block, sinusoidal infiltration, or hepatic granuloma s. Results: Among 11 patients, associated conditions were prothrombotic disorder s (n = 5) and oral contraceptive use (n = 3). Prothrombotic disorders were polyc ythemia vera (n = 1) and anticardiolipin antibodies combined with lupus anticoag ulant (n = 4). No genetic thrombophilia factor was found. Of four patients with lupus anticoagulant, three had antinuclear factors and high serum levels of anti cardiolipin antibodies at repeated testing. There was no evidence of intrahepati c or extrahepatic thrombosis in any of the patients. Sinusoidal dilatation was m arked in six of 11 patients (54%), including two patients with antiphospholipid antibodies. Activated perisinusoidal cells were only found around markedly dila ted sinusoids. Conclusion: Idiopathic pure hSD is frequently associated with the immunological features of the antiphospholipid syndrome. Therefore, finding pur e hSD in a liver biopsy specimen should prompt the search for antiphospholipid a ntibodies.

关键词：抗磷脂综合征免疫学特征肝窦扩张凝血酶原肝肉芽肿心肌磷脂窦状隙肝活检标本流出道梗阻狼疮抗凝因子

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胰腺破骨巨细胞瘤

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世界核心医学期刊文摘（胃肠病学分册） 2005年第8期1卷 19-19页

作者： Beaufour A cazals-hatem D Regimbeau J.-m A. Sauvanet 雷小英 Service de Chirurgie Digestive Hpital BeaujonAP-HP 100 bd du Gal Leclerc 92118 Clich y CedexFrance

Osteoclast giant cell tumours are bone tumours that occur in adults, and that are considered benign by WhO but locally aggressive. Strictly identical tumours are described in the pancreas,without simultaneous bone localization. We report the case of a 62-year woman with an osteoclast giant cell tumour of the distal pancreas, without any epithelial component, which was diagnosed after pancreatic resection and with no signs of recurrence after a 24-month follow-up. These p ancreatic tumours are rare, with a very poor prognosis, an unclear histogenesis; they are often confused with pleomorphic or undifferentiated pancreatic carcinom as including a component of osteoclast giant cell. These osteoclast giant cell t umours of the pancreas usually present as large cystic tumours. In certain cases , completeresection can result in long-term survival.

关键词：骨巨细胞瘤骨肿瘤侵蚀性骨组织上皮细胞组织发生

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