Congenital microvillous atrophy: report of two cases.
2 infants with chronic severe diarrhoea from birth and with lethal outcome have been studied. Small intestinal biopsies were examined by light and electron microscopy. Severe villous atrophy, complete loss of microvilli or rudimentary forms, intracytoplasmic vesicles, and microvillous inclusions are the characteristic features of the disease. The etiology is unknown. A disturbance in the transport of brush-border proteins to the cell surface is assumed to be the reason for congenital microvillous atrophy.
Steininger
H
H
Institute of Pathology, Erlangen-Nuremberg, Germany.
Behrens
R
R
Faller
G
G
Schindler
C
C
Kirchner
T
T
D001284:Atrophy; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007410:Intestinal Diseases / Q000151:congenital* / Q000473:pathology*; D007413:Intestinal Mucosa / Q000473:pathology* / Q000648:ultrastructure*; D007421:Intestine, Small / Q000473:pathology / Q000648:ultrastructure; D008297:Male; D008871:Microvilli / Q000473:pathology / Q000648:ultrastructure