Catalog

Intestinal epithelial dysplasia

No MMS code

Link to WHO Definition

Intestinal epithelial dysplasia – http://id.who.int/icd/entity/1536004957

Intestinal epithelial dysplasia, also known as tufting enteropathy, is a congenital enteropathy presenting with early-onset severe intractable diarrhoea sometimes causing irreversible intestinal failure. IED is thought to be related to abnormal enterocytes development and/or differentiation, with specific histological abnormalities involving the epithelium with disorganisation of surface enterocytes with focal crowding, resembling tufts. Several associated specific features were reported, including abnormal deposition of laminin and heparan sulphate proteoglycan (HSPG) in the basement membrane, increased expression of desmoglein and ultrastructural changes in the desmosomes, and abnormal distribution of alpha2beta1 integrin adhesion molecules.