Hypertrophic eosinophilic gastroenteropathy is associated with reduced enterocyte apoptosis.
OBJECTIVE
To investigate the cause of grossly elongated villi in four children presenting with obstruction due to a novel form of eosinophilic gastroenteropathy in which there was profound hyperplasia of the intestinal villi with grossly increased villous/crypt ratio and prominent mucosal eosinophilia. Increased eosinophils were also present in the muscularis propria and submucosa. All had intermittent diarrhoea and signs of a protein-losing enteropathy.


RESULTS
The cause of the grossly elongated villi was investigated by studying enterocyte proliferation (Ki67), survival factors (bcl-2) and apoptosis (TUNEL) in these patients (n = 4) and normal (jejunum n = 6, ileum n = 6) and disease (n = 6) controls. The most remarkable finding was that apoptotic enterocytes were undetectable in the elongated villi.


CONCLUSIONS
It seems likely that a defect in the regulation of apoptosis of the epithelium occurs which could explain the remarkable hyperplasia of the villi seen.
Cuperus
R
R
Gastroenterology, Great Ormond Street Hospital for Children NHS Trust, London, UK.
Schäppi
M G
MG
Shah
N
N
Lindley
K J
KJ
Milla
P J
PJ
Smith
V V
VV
D000293:Adolescent; D017209:Apoptosis; D016022:Case-Control Studies; D020895:Enterocytes / Q000378:metabolism / Q000473:pathology*; D004804:Eosinophils / Q000473:pathology*; D005260:Female; D006801:Humans; D006965:Hyperplasia; D006984:Hypertrophy; D007082:Ileum / Q000378:metabolism / Q000473:pathology; D007223:Infant; D007413:Intestinal Mucosa / Q000473:pathology; D007583:Jejunum / Q000378:metabolism / Q000473:pathology; D019394:Ki-67 Antigen / Q000378:metabolism; D008297:Male; D008871:Microvilli / Q000378:metabolism / Q000473:pathology; D011504:Protein-Losing Enteropathies / Q000503:physiopathology; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000378:metabolism
D019394:Ki-67 Antigen; D019253:Proto-Oncogene Proteins c-bcl-2