(1) The former include primary biliary cirrhosis (PBC), primary sclerosing cholangitis
(PSC), graft-versus-host-disease (GVHD), and drug-induced liver injury (DILI).
A 36-year-old man was admitted to hospital in November 2012 with recurrent cholangitis
. At the age of 5 years he had had an oesphageal variceal bleed secondary to an extrahepatic portal vein thrombosis following umbilical vein sepsis caused by a venous catheter used during a neonatal illness.
Estimates suggest that 25% to 50% of cases could be autosomal recessive, the balance being sporadic, although no clear genetic basis for the disease has been identified.[sup.5] Caroli's disease is characterized by segmental dilatation of the intrahepatic bile ducts without hepatic fibrosis as compared to Caroli's syndrome, which is defined as ectasia of the intrahepatic bile ducts with hepatic fibrosis.[sup.6,7] Clinically, patients with Caroli's disease often develop portal hypertension, fat-soluble vitamin deficiency (vitamin K in particular) and are at increased risk for intrahepatic stones and severe septic cholangitis
Data Source: A review of data from 54 patients seen at the Mayo Clinic, Rochester, Minn., for primary sclerosing cholangitis
and inflammatory bowel disease and later diagnosed with colonic neoplasms.
Over the next few months, the man was admitted for repeated cholangitis
Adults however present mainly with pain (2,3) and complications of longstanding cysts such as recurrent cholangitis
, pancreatitis and malignancies.
Only severe or worsening cholangitis
requires urgent intervention.
ChemomAb is a clinical stage biopharmaceutical company, specializing in the development of proprietary monoclonal antibodies, directed towards novel targets, for the treatment of fibrotic-inflammatory disorders including NASH, as well as the orphan indications Primary Sclerosing Cholangitis
and Systemic Sclerosis.
Announced FDA acceptance of an IND to initiate a Phase 2 clinical study of seladelpar in primary sclerosing cholangitis
Microbiome company BiomX Ltd said on Monday that it has entered into an agreement to license a new set of bacterial targets for the development and commercialisation of phage therapies for the treatment of orphan liver disease Primary Sclerosing Cholangitis
(PSC) from JSR Corporation, Japan.
Vestra said that Arquilita had been in the hospital since March 29 after he was diagnosed with anemia, cholangitis
or inflammation of the bile duct and viral Hepatitis B.