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  • 1
    ISSN: 1437-9813
    Keywords: Hirschsprung's disease ; Aganglionic segment ; Alpha-naphthylesterase ; Histochemistry ; Pseudo-Hirschsprung's disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The authors present their experience with the histotopochemical alpha-naphthylesterase reaction for intraoperative evaluation of the length of the aganglionic segment in Hischsprung's disease (HD). The activity of nonspecific esterases (Davis and Ornstein technique [1]) was used to demonstrate the presence or absence of intramural ganglion cells in seromuscular intestinal biopsies taken at various levels in 50 patients with the preoperative diagnosis of HD; in 44 cases the biopsies were taken during a radical HD operation (endorectal pull-through, Soave technique). In the remaining 6 cases, which presented with intestinal obstruction, the biopsies were taken during laparotomy. The authors consider this histochemical technique a reliable, quick, simple, and economical method for intraoperative evaluation of the length of the aganglionic segment. It is also useful for confirming the diagnosis of HD and is of great help in the study of so-called pseudo-HD.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1437-9813
    Keywords: Total colonic aganglionosis ; Hirschsprung's disease ; Neurocristopathies ; Pathogenesis ; Chromosome 10
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We present the case of a patient with total colonic aganglionosis and small-bowel involvement (TCSA) associated with a 46, XX, del 10 (q11.21 q21.2) karyotype. Seromuscular biopsies were taken from the rectum, colon, cecum, and terminal ileum. The alpha-naphthylesterase technique was applied to demonstrate intestinal ganglia of the myenteric plexus. The patient did not have associated anomalies. The association of TCSA with 10 long-arm interstitial deletion has not been reported in the literature. Molecular studies in cases of TCSA might reveal whether chromosome 10 plays a role in the pathogenesis of the disease.
    Type of Medium: Electronic Resource
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