Preferred Label : Intestinal epithelial dysplasia;
ICD-11 definition : Intestinal epithelial dysplasia, also known as tufting enteropathy, is a congenital
enteropathy presenting with early-onset severe intractable diarrhea 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 disorganization of surface enterocytes with focal crowding, resembling
tufts. Several associated specific features were reported, including abnormal deposition
of laminin and heparan sulfate proteoglycan (HSPG) in the basement membrane, increased
expression of desmoglein and ultrastructural changes in the desmosomes, and abnormal
distribution of alpha2beta1 integrin adhesion molecules.;
ICD-11 synonym : IED - [Intestinal epithelial dysplasia];
ICD-11 acronym : IED;
ICD-11 inclusion : Tufting enteropathy;
Origin ID : 1536004957;
Automatic exact mappings (from CISMeF team)
Intestinal epithelial dysplasia, also known as tufting enteropathy, is a congenital
enteropathy presenting with early-onset severe intractable diarrhea 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 disorganization of surface enterocytes with focal crowding, resembling
tufts. Several associated specific features were reported, including abnormal deposition
of laminin and heparan sulfate proteoglycan (HSPG) in the basement membrane, increased
expression of desmoglein and ultrastructural changes in the desmosomes, and abnormal
distribution of alpha2beta1 integrin adhesion molecules.
