UNLABELLED Defects in multidrug resistance 3 gene (MDR3), which encodes the canalicular phospholipid flippase, cause a wide spectrum of cholangiopathy phenotypes in humans. Mice deficient in Mdr2 (murine ortholog of MDR3) develop liver diseases that closely reproduce the biochemical, histological, and clinical features of human cholangiopathies such as progressive familial intrahepatic cholesta...

Cholangiopathies are chronic liver diseases in which damaged cholangiocytes trigger a proinflammatory and profibrotic reaction. The nuclear vitamin D receptor (VDR) is highly expressed exerts immune-regulatory functions these cells. In the present study, we examined protective function of VDR other signaling pathways cholangiopathy cholangiocytes. Vdr was invalidated Abcb4 knockout mice, widely...

Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown etiopathogenesis characterized by fibrous cholangiopathy of large and small bile ducts. Systemic administration of a murine TNF-related apoptosis-inducing ligand (TRAIL) receptor agonist induces a sclerosing cholangitis injury in C57BL/6 mice, suggesting endogenous TRAIL may contribute to sclerosing cholangitis syndr...

Hong Kong Med J ⎥ Volume 20 Number 1 ⎥ February 2014 ⎥ www.hkmj.org A 28-year-old woman presented to us in November 2010 because of deranged liver function test results; predominantly she had raised ductal enzyme levels (gamma-glutamyl transferase, 1088; reference range, 12-57 IU/mL); alkaline phosphatase (ALP) 579 (reference range, 46-127) IU/mL, alanine transaminase (ALT) 183 (reference range...

cholangiopathy and chronic diarrhea are relatively common manifestations of aids. cryptosporidium parvum infection is the most common cause of aids cholangiopathy globally. this is the first report of documented cryptosporidium parvum infection in a patient with aids in iran. the patient was a 39-year-old man from afghanistan with watery diarrhea, crampy periumbilical abdominal pain and 20 kg w...

OBJECTIVE X-linked hyper-immunoglobulin M (X-HIM) syndrome is a rare genetic immunodeficiency syndrome caused by mutations in the gene encoding CD40 ligand (CD40L, CD154). Allogeneic hematopoietic stem cell transplantation (HSCT) offers the prospect of immune reconstitution in X-HIM syndrome. Standard HSCT using high-dose chemoradiotherapy can be followed by serious hepatic problems, including ...

Cholangiopathy is a chronic immune-mediated disease of the liver, which is characterized by cholangitis, ductular reaction and biliary-type hepatic fibrosis. There is no proven medical therapy that changes the course of the disease. In previous studies, melittin was known for attenuation of hepatic injury, inflammation and hepatic fibrosis. This study investigated whether melittin provides inhi...