[Severe epithelio-exfoliative colitis in infants. Anatomical data].
Amongst the uncommon forms of congenital severe colitis, we wish to draw attention to a peculiar and probably previously never described condition that we propose calling provisionally, epithelio-exfoliative colitis. This condition appears to be characterized by the following features: its early beginning within the first weeks of life; the smooth, glossy appearance of the mucosa, without ulcerations visible to the naked eye; the prevalent degenerative changes of the epithelial cells which become vacuolated, break away prematurely from the basement membrane and finally exfoliate within the glandular lumens; the distension and rupture of the glands, the mucous contents of which intrude into the lamina propria and induce a localized, mild and non suppurative inflammatory reaction; accessory reactive traits: intense mucus production actively regenerating epithelium (high mitotic activity, syncytial cells) and increase of the cholinergic fibers within the lamina propria. Although patchily distributed, these lesions involve the colon exclusively. The cause of epithelio-exfoliative colitis is unknown. However, the ultrastructural studies and immunocytochemical investigations using anti-collagen IV, antilaminin, anti-fibronectin antibodies disclose in some glands localized thinning and rupture of the basement membrane. These data suggest a primary disorder within the molecular arrangement of either the basement membrane itself or the proteins which anchor the glandular cells to the basement membrane.
D003092:Colitis / Q000473:pathology*; D003638:Deafness / Q000150:complications*; D004848:Epithelium / Q000473:pathology; D005455:Fluorescent Antibody Technique; D006801:Humans; D007231:Infant, Newborn; D007413:Intestinal Mucosa / Q000473:pathology*; D008854:Microscopy, Electron
Colite grave épithélio-exfoliative du nourrisson. II. Données anatomiques.