Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

Amarapurkar, Deepak; Punamiya, Sundeep; Patel, Nikhil D

doi:10.3748/wjg.14.278

Cited by 72 publications

(50 citation statements)

References 76 publications

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“…This same pattern is generally reported in European series [11,16] , but not in Asian series, particularly those from Japan, where IVC obstruction is frequent [5,17] . Splanchnic thrombosis, which worsens the prognosis of patients with BCS, was observed in 23% in our series, which contrast with the literature, where this association is noted in less than 14% [8,9,11] . The number of aetiological factors has been significantly related to the extension of the thrombosis into the splanchnic territory [18] .…”

Section: Discussioncontrasting

confidence: 56%

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Aetiological factors of Budd-Chiari syndrome in Algeria

Afredj¹

2015

WJH

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AIM: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: Aetiological factors of Budd-Chiari syndrome in AlgeriaProspective Study ORIGINAL ARTICLE63.5%. 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). CONCLUSION: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.

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Section: Discussioncontrasting

confidence: 56%

“…Except for a few series in Asia, where the mean age was less than 30 years [6,7] and the M/F sex-ratio > 1 [8] , BCS has generally been observed in young female patients [9][10][11] . This was the case in our series where the M/F ratio was 0.6, and the mean age was 34 years.…”

Section: Discussionmentioning

confidence: 99%

Aetiological factors of Budd-Chiari syndrome in Algeria

Afredj¹

2015

WJH

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“…Considerable differences have been reported between Asian and the western HVOTO patients, particularly on its aetiology, level and extent of venous block, clinical presentation and frequency of HCC occurrence. [20][21][22] Therefore, the present study was conducted to ascertain the incidence, prevalence of HCC and the possible risk factors for development of HCC in Indian patients with HVOTO. To our knowledge, this is the first study of its kind to address these issues in depth.…”

Section: Discussionmentioning

confidence: 99%

Incidence and risk factors of hepatocellular carcinoma in patients with hepatic venous outflow tract obstruction

Paul

Shalimar

Sreenivas

et al. 2015

Aliment Pharmacol Ther

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“…Membranous occlusion of IVC and HV, presenting as the chronic form of the disease, is more common in Indians (Figure 1) [8][9][10][11][12] . However, there are a few studies documenting prevalence of hypercoagulable states in Indian patients; thus there is a change of spectrum of BCS in India [25,26] . BCS may also be seen in patients with metastatic involvement of the IVC and HV secondary to kidney, liver, or suprarenal gland tumors (Figure 1).…”

Section: Etio-pathologymentioning

confidence: 99%

“…A possible radiological intervention in these patients consists of creating a shunt across the portal vein and IVC. With a relatively good midterm outcome, currently TIPS is the preferred intervention for patients having chronic BCS [23][24][25][35][36][37][38][39][40][41] . Creating TIPS in patients having chronic BCS with no available hepatic vein may be technically challenging and difficult and in some cases there may not even be a small notch in the IVC to suggest the site of confluence of hepatic vein and IVC.…”

Section: Transjugular Intrahepatic Portosystemic Shuntmentioning

confidence: 99%

Imaging and interventions in Budd-Chiari syndrome

Mukund

Gamanagatti²

2011

WJR

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Budd-Chiari syndrome (BCS) consists of a group of disorders with obstruction of hepatic venous outflow leading to increased hepatic sinusoidal pressure and portal hypertension. Clinically, two forms of disease (acute and chronic) are recognized. Mostly the patients present with ascites, hepatomegaly, and portal hypertension. In acute disease the liver is enlarged with thrombosed hepatic veins (HV) and ascites, whereas in the chronic form of the disease there may be membranous occlusion of HV and/or the inferior vena cava (IVC), or there may be short or long segment fibrotic constriction of HV or the suprahepatic IVC. Due to advances in radiological interventional techniques and hardware, there have been changes in the management protocol of BCS with surgery being offered to patients not suitable for radiological interventions or having acute liver failure requiring liver transplantation. The present article gives an insight into various imaging findings and interventional techniques employed in the management of BCS.

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Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

Cited by 72 publications

References 76 publications

Aetiological factors of Budd-Chiari syndrome in Algeria

Aetiological factors of Budd-Chiari syndrome in Algeria

Incidence and risk factors of hepatocellular carcinoma in patients with hepatic venous outflow tract obstruction

Imaging and interventions in Budd-Chiari syndrome

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