[Congenital villous atrophy. Disease picture of congenital chronic diarrhea with poor prognosis].
We report on a patient with connatal chronic diarrhea. Electron microscopical examination revealed "microvillus inclusion disease", characterized by enterocyte brushborder abnormalities. Differential diagnoses are enteral infection and intolerance to formula protein, rarely disaccharidase deficiency. Therapeutic approaches with glucocorticosteroids, pentagastrin and somatostatin failed, just as using growth factor did. No differentiation of the enterocytes occurred with increasing age. Altogether the prognosis of this disease is very poor.
Cegla
M
M
Kinderklinik, Städtisches Krankenhaus Pforzheim.
Lohner
M
M
Schaefer
H E
HE
D001284:Atrophy; D003241:Consanguinity; D003968:Diarrhea, Infantile / Q000235:genetics* / Q000473:pathology; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D007223:Infant; D007231:Infant, Newborn; D007413:Intestinal Mucosa / Q000002:abnormalities* / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D008871:Microvilli / Q000473:pathology*; D011379:Prognosis
Kongenitale Zottenatrophie. Krankheitsbild einer konnatalen chronischen Diarrhö mit infauster Prognose.