Celiac disease with Budd–Chiari syndrome: A rare association

Budd–Chiari syndrome is characterized by hepatic venous outflow tract obstruction. We describe an 18-year-old female of known celiac disease presented with progressive abdomen distention and shortness of breath for the last 1 month. Computed tomography of abdomen revealed hepatic vein obstruction. The patient was diagnosed with Budd–Chiari syndrome. Coagulation profile showed an increased homocysteine level. Serum folate level was also decreased. The patient was put on oral anticoagulant with a gluten-free diet. After 4 weeks, the patient showed significant improvement with decreased ascites. The association of Budd–Chiari syndrome with Celiac disease has not yet been fully understood. There have been few reports that described this rare association. Budd–Chiari syndrome should be considered as an important differential in a patient with unexplained ascites and celiac disease.

Renal Cell Carcinoma Embolization for the Treatment of Budd–Chiari Syndrome

Renal cell carcinoma (RCC) is the eighth most common malignancy, accounting for 3% of all newly diagnosed neoplasms. RCC venous invasion occurs in 4 to 10% of cases. We report a case of RCC with extensive vascular invasion and new-onset liver dysfunction. The etiology of the liver dysfunction was attributed to hepatic vein obstruction; herein we describe the first reported case of transcatheter embolization of RCC for Budd–Chiari syndrome.

Hepatic amyloidosis - primary AL type, sub nephrotic proteinuria and budd chairi syndrome – A nugget

We describe a case of 62-year-old gentleman presenting with abdominal pain associated with loss of weight, nocturia, oedema of feet, constipation, altered sleep rhythm and dyspepsia. On evaluation he had hepatomegaly with raised alkaline phosphatase and raised GGT levels with normal transaminases and bilirubin. On imaging he had diffuse enlargement of liver with heterogeneous contrast uptake in liver. His viral marker and autoimmune markers were negative. Liver biopsy depicted deposition of amorphous eosinophilic substance within the sinusoids which revealed apple green birefringence on polarizing microscopy after Congo red staining; Congophilia persisted even after treating with KMnO4. Abdominal fat pad was negative for amyloid deposit. Cardiac evaluation was unremarkable and renal evaluation showed Subnephrotic proteinuria and microhematuria. Serum and urine immunofixation electrophoresis showed positive kappa, lambda and gamma globulin. Immunoperoxidase staining for serum amyloid associated protein for secondary amyloidosis was negative from liver biopsy. Here we report a case of primary hepatic amyloidosis that presented with features of hepatic vein obstruction. The association of Budd-Chiari's syndrome, with amyloidosis may be related to the increased risk of thrombosis observed in the latter disease also due to loss of anticoagulants due to significant proteinuria. Patient could not be treated either with chemotherapy or with surgery. We lost him for progressive liver failure.Asian Journal of Medical Sciences Vol.8(2) 2017 97-100