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  • Title: [Variability of clinical symptoms in neuronal intestinal dysplasia].
    Author: Bussmann H, Roth H, von Deimling O, Nützenadel W.
    Journal: Monatsschr Kinderheilkd; 1990 May; 138(5):284-7. PubMed ID: 2198464.
    Abstract:
    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.
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