syndrome （BCS） and identify the aetiologies of thisdisease in Algeria.METHODS： Patients with BCS, hospitalised in our unitfrom January 2004 until June 2010 were included and theaetiological factors were assessed. P...syndrome （BCS） and identify the aetiologies of thisdisease in Algeria.METHODS： 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 anticoagulant.We also detected paroxysmal nocturnal haemoglobinuria（PNH） by flow cytometry. Celiac disease and Beh？etdisease were systematically investigated in our patients.Hereditary 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 tumour.RESULTS： 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 deficiency was diagnosed in 28% of the patients（n = 18）, dominated by protein C deficiency （n = 13）.Secondary BCS was caused by a compressing hydaticcyst （n = 5） and hepatocellular carcinoma （n = 1）.CONCLUSION： The main aetiologic factor of BCS inAlgeria is MPD. The frequency of celiac disease justifiesits consideration when BCS is diagnosed in our region.展开更多
文摘syndrome （BCS） and identify the aetiologies of thisdisease in Algeria.METHODS： 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 anticoagulant.We also detected paroxysmal nocturnal haemoglobinuria（PNH） by flow cytometry. Celiac disease and Beh？etdisease were systematically investigated in our patients.Hereditary 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 tumour.RESULTS： 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 deficiency was diagnosed in 28% of the patients（n = 18）, dominated by protein C deficiency （n = 13）.Secondary BCS was caused by a compressing hydaticcyst （n = 5） and hepatocellular carcinoma （n = 1）.CONCLUSION： The main aetiologic factor of BCS inAlgeria is MPD. The frequency of celiac disease justifiesits consideration when BCS is diagnosed in our region.
