Aetiological factors of Budd-Chiari syndrome in Algeria.
OBJECTIVE
To study the clinical presentation of Budd-Chiari syndrome (BCS) and identify the aetiologies of this disease in Algeria.


METHODS
Patients with BCS, hospitalised in our unit from January 2004 until June 2010 were included and the aetiological factors were assessed. Patients presenting a BCS in the setting of advanced-stage cirrhosis or a liver transplantation were excluded from the study. The diagnosis was established when an obstruction of hepatic venous outflow (thrombosis, stenosis or compression) was demonstrated. We diagnosed myeloproliferative disease (MPD) by bone marrow biopsy and V617F JAK2 mutation. Anti-phospholipid syndrome (APLS) was detected 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 Behçet disease were systematically investigated in our patients. Hereditary anticoagulant protein deficiencies were also assessed. We tested our patients for the G20210A mutation at Beaujon Hospital. Imaging procedures were performed to determine a local cause of BCS, such as a hydatid cyst or a liver tumour.


RESULTS
One hundred and fifteen patients were included. Mean follow up: 32.12 mo. Mean age: 34.41 years, M/F = 0.64. Chronic presentation was frequent: 63.5%. The revealing symptoms for the BCS were ascites (74.8%) and abdominal pain (42.6%). The most common site of thrombosis was the hepatic veins (72.2%). Involvement of the inferior vena cava alone was observed in 3 patients. According to the radiological investigations, BCS was primary in 94.7% of the cases (n = 109) and secondary in 5.2% (n = 6). An aetiology was identified in 77.4% of the patients (n = 89); it was multifactorial in 27% (n = 31). The predominant aetiology of BCS in our patients was a myeloproliferative disease, observed in 34.6% of cases. APLS was found in 21.7% and celiac disease in 11.4%. Other acquired conditions were: PNH (n = 4), systemic disease (n = 6) and inflammatory bowel disease (n = 5). Anticoagulant protein deficiency was diagnosed in 28% of the patients (n = 18), dominated by protein C deficiency (n = 13). Secondary BCS was caused by a compressing hydatic cyst (n = 5) and hepatocellular carcinoma (n = 1).


CONCLUSIONS
The main aetiologic factor of BCS in Algeria is MPD. The frequency of celiac disease justifies its consideration when BCS is diagnosed in our region.
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Nawel
N
Afredj
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Nawal
N
Guessab
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Abdelbasset
A
Nani
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Sid Ahmed
SA
Faraoun
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Ibtissem
I
Ouled Cheikh
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Rafik
R
Kerbouche
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Djouhar
D
Hannoun
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Zine Charef
ZC
Amir
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Hayet
H
Ait Kaci
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Kamel
K
Bentabak
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Aurélie
A
Plessier
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Dominique-Charles
DC
Valla
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Valerie
V
Cazals-Hatem
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Marie-Hélène
MH
Denninger
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Tadjeddine
T
Boucekkine
Nawel Afredj, Nawal Guessab, Abdelbasset Nani, Ibtissem Ouled Chikh, Rafik Kerbouche, Tadjeddine Boucekkine, Nabil Debzi, Hepatology Unit, Department of Medicine Mustapha Hospital, Algiers 16010, Algeria.
Nabil
N
Debzi
Algeria; Budd-Chiari; Celiac disease; Etiologie; Thrombosis