Liver cirrhosis in atypical cystic fibrosis (Q100276)

This is a consequence of chronic liver disease characterised by replacement of liver tissue by fibrosis, scar tissue and regenerative nodules (lumps that occur as a result of a process in which damaged tissue is regenerated), leading to loss of liver function, in an atypical autosomal recessive genetic disorder that affects most critically the lungs, and also the pancreas, liver, and intestine. It is characterised by abnormal transport of chloride and sodium across an epithelium, leading to thick, viscous secretions.