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  • 1
    ISSN: 1437-9813
    Keywords: Neuronal intestinal dysplasia ; Hirschsprung's disease ; Colon sonography ; Colon motility ; Colon biopsy, diagnostic
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Twenty-four cases of concomitant Hirschsprung's disease (HD) and neuronal intestinal dysplasia (NID) are presented. The clinical picture is characterized by the early and acute onset of HD symptoms. The diagnosis is established by means of rectal and colonic biopsies. Open biopsies during laparotomy should be taken without injury to the mucosa. Early surgical therapy consists of extended resection of the aganglionic segment and the colon affected by NID up to the splenic flexure. Complications are imminent if the aganglionosis masks the symptoms of NID and, accordingly, only the aganglionic segment is resected. As an alternative, postponed resection of the aganglionic segment alone is proposed once the NID-affected bowel develops functional maturation. In children who do not show an improvement of colon dysmotility, however, extended resection is recommended at the age of 4 years. In follow-up studies of colon motility, functional colon sonography is used.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric surgery international 2 (1987), S. 81-85 
    ISSN: 1437-9813
    Keywords: Sphincter ani internus ; Aganglionosis ; Neuronal intestinal dysplasia (type B) ; Sphincter achalasia ; Biopsy diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Enzyme-histotopochemical studies of parasympathetic innervation in the internal anal sphincter muscle of 73 children with anal sphincter achalasia indicated that in Hirschsprung's disease and type B neuronal intestinal dysplasia (NID) the internal sphincter displays a pattern of changes analogous to that seen in the wall of the rectum. In Hirschsprung's disease the internal sphincter is also aganglionic, and its parasympathetic fibres exhibit increased acetylcholinesterase (ACE) activity in NID there is moderately increased ACE activity, with ganglionic neurons embedded singly or in groups in the thick afferent parasympathetic fibers. The innervation defect may be confined to the sphincter, or aganglionosis of the sphincter may be associated with proximal NID.
    Type of Medium: Electronic Resource
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