Acute Budd-Chiari syndrome with hepatic failure and obstruction of the inferior vena cava as presenting manifestations of hereditary protein C deficiency.

Bourlière, Marc; Treut, Yves Patrice Le; Arnoux, D.; Castellani, Paul; Bordigoni, L; Maillot, Adrien; Antoni, M.; Botta, D.; Pol, Bernard; Gauthier, A.

doi:10.1136/gut.31.8.949

Cited by 36 publications

(15 citation statements)

References 40 publications

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“…Enhanced intravenous coagulation due to Protein C ,Protein S and Antithrombin III deficiency are well recognized to cause BCS 13,14 .In this case patient had severe deficiency of these factors (measured before starting anticoagulant ) which were responsible for clot formation inside hepatic veins and IVC .Genetic testing for mutated gene may identify whether these factor deficiency are hereditary or not but blood level less than 50% normal suggest hereditary deficiencies. Anti Phospholipid antibody syndrome, paroxysmal nocturnal haemoglobinuria, DIC were excluded from history ,physical exam and laboratory investigations.Other common causes of BCS like membranous webs ,myeloproliferative syndrome(e.g.…”

Section: Discussionmentioning

confidence: 93%

Acute Budd Chiari Syndrome

Uddin¹,

Wareshuzzaman²,

Banik³

et al. 2014

J. Bangladesh Coll. Phys.

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-Chiari syndrome (BCS) is a rare disease in population characterized by hepatic venous outflow obstruction at a site that may vary from the level of the small hepatic veins up to the junction of the inferior vena cava with the right atrium . Here we presented a case of 35 year old male who presented with abdominal pain, abdominal distension and leg oedema . Subsequent physical examination, laboratory data, abdominal ultrasonography and duplex study all confirmed the diagnosis of BCS with Protein C , protein S , and antithrombin III deficiency . But we were unable to detect whether these factor deficiency were primary or secondary. Genetic study may be used to elucidate the cause. Whether primary or secondary, standard protocol should be used to treat Budd Chiari syndrome with these factor deficiency.(J Banagladesh Coll Phys Surg 2013; 31: 223-226)

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Section: Discussionmentioning

confidence: 93%

Acute Budd Chiari Syndrome

Uddin¹,

Wareshuzzaman²,

Banik³

et al. 2014

J. Bangladesh Coll. Phys.

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“…Budd-Chiari syndrome caused by protein C deficiency has been described [29,30]. Although protein C levels in inflammatory bowel disease are generally normal [7,31], there are reports of protein C deficiency secondary to ulcerative colitis causing thromboembolic complications [13,32].…”

Section: Discussionmentioning

confidence: 99%

Acute Budd-Chiari syndrome, portal and splenic vein thrombosis in a patient with ulcerative colitis associated with antiphospholipid antibodies and protein C deficiency

Junge

Wienke²,

A³

2001

Z Gastroenterol

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We report the case of a female patient who had severe thrombotic complications in peripheral (V. jugularis, subclavia, brachialis, poplitea) and visceral (portal and splenic) veins 4 years after the first diagnosis of severe ulcerative pancolitis. A thrombolysis therapy for subclavian and jugular vein thrombosis was performed without complication, but she soon developed acute thrombosis of the hepatic veins (acute Budd-Chiari syndrome). She quickly recovered after liver transplantation and now - 6 years later - she lives a normal life with continuous anticoagulation and medical therapy of the colitis.3 possible causes for the severe coagulation defect in this patient can be supposed: Thrombocytosis, protein C deficiency and an antiphospholipid antibody syndrome.

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“…In the United States and Western countries, thrombosis with apparent pathogenesis is the most common cause of HVOO. Previously reported conditions that predispose to thrombosis are myeloproliferative disorders such as polycythemia vera and essential thrombocytosis, including preclinical stage, 10 and abnormal clotting tendencies due to deficiencies of antithrombin III, 11 protein C, 12,13 or protein S, 14 blood constituents that protect against thrombosis. Oral contraceptive use, 15 the presence of lupus anticoagulant 16 and anticardiolipin antibodies, 17 paroxysmal nocturnal hemoglobinuria (PNH), 18 and factor V Leiden mutation 19 also predispose to thrombosis.…”

Section: Discussionmentioning

confidence: 99%

Aged Budd-Chiari syndrome attributed to chronic deep venous thrombosis with alcoholic liver cirrhosis

Hoshida

Yamakado

Shinoki

et al. 1999

Journal of Gastroenterology

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Budd-Chiari syndrome is a rare disease, but there are many known causes. Recent studies showed that it can be an acquired lesion resulting from thrombosis in some elderly patients. We report a 74-year-old man with Budd-Chiari syndrome attributed to chronic deep venous thrombosis and alcoholic liver cirrhosis. When he was aged 45 years, stasis ulcers of the lower extremities appeared. Cerebral infarction and left hemiparesis occurred at age 71. Ultrasonography, venacavography, and three-dimensional-magnetic resonance imaging on admission demonstrated total obstruction of the inferior vena cava with several massive thrombi and developed collateral vessels. Although the etiology of the thrombosis remained obscure, we made some speculative assumptions that chronic disseminated intravascular coagulation (which is frequently observed in cirrhosis) or hereditary coagulopathy could be involved, from his familial history of thrombotic phenomena and a severe deficiency of clotting inhibitors. Despite the high mortality of untreated Budd-Chiari syndrome reported in previous studies, this patient had been alive for about 30 years from the suspected onset.

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Acute Budd-Chiari syndrome with hepatic failure and obstruction of the inferior vena cava as presenting manifestations of hereditary protein C deficiency.

Cited by 36 publications

References 40 publications

Acute Budd Chiari Syndrome

Acute Budd Chiari Syndrome

Acute Budd-Chiari syndrome, portal and splenic vein thrombosis in a patient with ulcerative colitis associated with antiphospholipid antibodies and protein C deficiency

Aged Budd-Chiari syndrome attributed to chronic deep venous thrombosis with alcoholic liver cirrhosis

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