Association of Budd-Chiari syndrome and celiac disease

Afredj, Nawel; Metatla, S.; Faraoun, S.A.; Nani, Abdelbasset; Guessab, N.; Benhalima, M.; Bendib, S.E.; Debzi, N.; Layaida, K.; Gamar, L.; Baiod, N.; Balamane, Maya; Kaddache, N.; Bounab, N.; Kécili, L.; Boucekkine, T.

doi:10.1016/j.gcb.2010.07.007

Cited by 24 publications

(20 citation statements)

References 16 publications

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“…There are also reported cases of hepatic vein obstruction in celiac patients, particularly from North Africa or Southern Europe [71, 72] and isolation in other locations [73, 74]. This disorder has been related to deficiencies in protein C and antithrombin III, malabsorption of vitamin K, decreased synthesis of coagulation factors dependent on this vitamin such as protein C or protein S, and even with dietary or environmental agents.…”

Section: Autoimmune Disorders In Celiac Diseasementioning

confidence: 99%

Celiac Disease and Autoimmune-Associated Conditions

Lauret

Rodrigo

2013

BioMed Research International

151

111

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Celiac disease (CD) is frequently accompanied by a variety of extradigestive manifestations, thus making it a systemic disease rather than a disease limited to the gastrointestinal tract. This is primarily explained by the fact that CD belongs to the group of autoimmune diseases. The only one with a known etiology is related to a permanent intolerance to gluten. Remarkable breakthroughs have been achieved in the last decades, due to a greater interest in the diagnosis of atypical and asymptomatic patients, which are more frequent in adults. The known presence of several associated diseases provides guidance in the search of oligosymptomatic cases as well as studies performed in relatives of patients with CD. The causes for the onset and manifestation of associated diseases are diverse; some share a similar genetic base, like type 1 diabetes mellitus (T1D); others share pathogenic mechanisms, and yet, others are of unknown nature. General practitioners and other specialists must remember that CD may debut with extraintestinal manifestations, and associated illnesses may appear both at the time of diagnosis and throughout the evolution of the disease. The implementation of a gluten-free diet (GFD) improves the overall clinical course and influences the evolution of the associated diseases. In some cases, such as iron deficiency anemia, the GFD contributes to its disappearance. In other disorders, like T1D, this allows a better control of the disease. In several other complications and/or associated diseases, an adequate adherence to a GFD may slow down their evolution, especially if implemented during an early stage.

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Section: Autoimmune Disorders In Celiac Diseasementioning

confidence: 99%

Celiac Disease and Autoimmune-Associated Conditions

Lauret

Rodrigo

2013

BioMed Research International

151

111

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“…These factors were excluded in our patients. The immunogenetic study that was performed did not show any association with a specific major histocompatibility complex antigen in these patients [36] . Further information from a broader genetic study might be useful.…”

Section: Discussionmentioning

confidence: 63%

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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“…The disease is categorized into primary BCS and secondary BCS. The etiology of primary BCS remains unclear, but the involvement of thrombus, abnormal angioplasty, abnormal blood coagulation, and myeloproliferative disorder has been suggested . The causes of secondary BCS include liver tumors .…”

Section: Guidelines For Diagnosis Of Aberrant Portal Hemodynamicsmentioning

confidence: 99%

Diagnosis and treatment guidelines for aberrant portal hemodynamics

Furuichi

Tanaka

Takikawa

et al. 2017

Hepatology Research

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Idiopathic portal hypertension (IPH), causing aberrant portal hemodynamics, is a disease with an as yet unidentified cause and no established treatment protocol. The Japanese research group on IPH in Japan was set up in 1975 by the Ministry of Health, Labor and Welfare. Extrahepatic portal obstruction and Budd-Chiari syndrome (BCS) have since been added to the group's research subjects. The aims of the research group are to accurately evaluate the current status of the three diseases in Japan, elucidate their etiology and pathogenesis, and develop new treatments. Due to the long-term efforts of the Japanese research group, aberrant portal hemodynamics has been investigated in a variety of aspects, from epidemiological and pathological studies to molecular biology analyses. As a result, it has been shown that there are abnormal genes in the liver, specific for IPH. In addition, pathological findings of BCS were internationally compared and the difference in findings between Japan and Europe (or North America) has been clarified. Furthermore, it was found that complication rates of hepatocellular carcinoma in BCS were higher in Japan. Based on the research, "Diagnosis and treatment of aberrant portal hemodynamics (2001)", including diagnostic criteria for aberrant portal hemodynamics, was published in 2001. In 2013, it was revised to "Diagnosis and treatment guidelines for aberrant portal hemodynamics (2013)" after the incorporation of diagnosis and treatment in accordance with its current status.

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Association of Budd-Chiari syndrome and celiac disease

Cited by 24 publications

References 16 publications

Celiac Disease and Autoimmune-Associated Conditions

Celiac Disease and Autoimmune-Associated Conditions

Aetiological factors of Budd-Chiari syndrome in Algeria

Diagnosis and treatment guidelines for aberrant portal hemodynamics

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