Catalog

Portal hypertension in atypical cystic fibrosis

No MMS code

Link to WHO Definition

Portal hypertension in atypical cystic fibrosis – http://id.who.int/icd/entity/306537006

This is hypertension (high blood pressure) in the portal vein system, which is composed by the portal vein, and its branches and tributaries, 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.