Hepatopulmonary Syndrome in a Patient with Primary Biliary Cirrhosis

Sorafenib treatment improves hepatopulmonary syndrome in rats with biliary cirrhosis.

HPS (hepatopulmonary syndrome) is characterized by oxygen desaturation in patients with chronic liver disease. The initiation of HPS comes from abnormal pulmonary vasodilatation and/or angiogenesis. In the present study, we evaluated anti-angiogenesis therapy using sorafenib in experimental HPS animals. HPS was induced by CBDL (common bile duct ligation) in rats. A 2-week 10 mg·(kg of body weig...

a patient with primary biliary cirrhosis accompanied by wilson’s disease

conclusions co-occurrence of pbc with wd is rare, which can cause diffusely intrahepatic copper deposition. early liver biopsy and genetic testing are necessary for the diagnosis. the combination of ursodeoxycholic acid with zinc and sodium dimercaptopropane sulfonate is effective. introduction both primary biliary cirrhosis (pbc) and wilson’s disease (wd) can cause copper retention in the live...

Goodpasture's syndrome associated with primary biliary cirrhosis.

A 73-year-old woman who had a history of primary biliary cirrhosis developed rapidly progressive glomerulonephritis and pulmonary-renal syndrome. She was found to have anti-mitochondrial antibody (AMA) and myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA). She also had weak anti-glomerular basement membrane (GBM) antibody. She was treated with methylprednisolone pulse therapy, b...

Lymphocytic bronchitis/bronchiolitis in a patient with primary biliary cirrhosis.

A 39 year old woman with severe primary biliary cirrhosis progressively developed exercise dyspnoea due to airflow obstruction. Sjögren's syndrome was not present. Bronchial and pulmonary biopsies demonstrated constrictive lymphocytic bronchitis/bronchiolitis, possibly a component of a generalized autoimmune process in this patient.

Primary biliary cirrhosis in a patient with interstitial lung fibrosis.