[Variability of clinical symptoms in neuronal intestinal dysplasia].
Neuronal intestinal dysplasia is defined as a structural disorder of the innervation of the gut which clinically resembles Hirschsprung's disease. Between 1977 and 1988 12 patients were diagnosed by enzyme histochemistry. In 3 of these patients Hirschsprung's disease was associated. Constipation was the main symptom in 6 patients with neuronal intestinal disease and in all three patients with associated Hirschsprung's disease. The other patients firstly presented with an enterocolitis, a congenital atresia of the jejunum and a chronic enteritis with malabsorption. The wide clinical variability and the lack of a clear therapeutic management valid for all patients is conspicuous.
Kinderklinik, Universität Heidelberg.
H
H
Bussmann
H
H
Roth
O
O
von Deimling
W
W
Nützenadel
D000110:Acetylcholinesterase / Q000378:metabolism*; D002675:Child, Preschool; D003248:Constipation / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D006627:Hirschsprung Disease / Q000473:pathology*; D006801:Humans; D006965:Hyperplasia; D007124:Immunoenzyme Techniques; D007223:Infant; D007413:Intestinal Mucosa / Q000294:innervation*; D008297:Male; D009197:Myenteric Plexus / Q000473:pathology*; D013368:Submucous Plexus / Q000473:pathology*
D000110:Acetylcholinesterase
Variabilität klinischer Symptome bei neuronaler intestinaler Dysplasie.