Congenital hepatic fibrosis Congenital hepatic fibrosis Congenital hepatic fibrosis is an inherited fibrocystic liver disease associated with proliferation of interlobular bile ducts within the portal areas and fibrosis that do not alter hepatic lobular architecture. The fibrosis would affect resistance in portal veins leading to portal hypertension. The condition is usually congenital, but sporadic cases have also been reported. It may be associated with other congenital defects, commonly with autosomal recessive polycystic kidney disease, the most severe form of PKD. Some suggest that these two conditions are one disorder with different presentation. Embryogenically, congenital hepatic fibrosis is due to malformation of the duct
British Institute of Organ Studies British Institute of Organ Studies The British Institute of Organ Studies, more commonly known by its acronym BIOS, is a British organisation and registered charity which aims to promote study and appreciation of all aspects of the pipe organ. Further, it acts as a lobbying body to raise awareness of organ issues with appropriate statutory bodies. Membership is open to all. The aims of BIOS are BIOS publishes a quarterly Reporter newsletter and magazine and a yearly Journal which contains articles on organ history. BIOS is also responsible for the National Pipe Organ Register (NPOR), which aims to catalogue all