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  • Hirschsprung's disease  (3)
  • Key words Esophageal atresia  (1)
  • 1
    Digitale Medien
    Digitale Medien
    Hirschsprung's disease: alpha-naphthylesterase activity for enzyme-histochemical evaluation of the extent of the aganglionic segment during surgery (1988)
    Springer
    Pediatric surgery international 3 (1988), S. 269-274 
    Details
    ISSN: 1437-9813
    Schlagwort(e): Hirschsprung's disease ; Aganglionic segment ; Alpha-naphthylesterase ; Histochemistry ; Pseudo-Hirschsprung's disease
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00179255
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  • 2
    Digitale Medien
    Digitale Medien
    Total colonic aganglionosis associated with interstitial deletion of the long arm of chromosome 10 (1992)
    Springer
    Pediatric surgery international 7 (1992), S. 308-310 
    Details
    ISSN: 1437-9813
    Schlagwort(e): Total colonic aganglionosis ; Hirschsprung's disease ; Neurocristopathies ; Pathogenesis ; Chromosome 10
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00183991
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  • 3
    Digitale Medien
    Digitale Medien
    Hirschsprung's disease as a neurochristopathy (1996)
    Springer
    Pediatric surgery international 12 (1996), S. 2-10 
    Details
    ISSN: 1437-9813
    Schlagwort(e): Hirschsprung's disease ; Neurocristopathies ; RET proto-oncogene
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Recent molecular-genetic and histochemical studies of intestinal aganglionosis have confirmed the initial classification established by Bolande, who considered Hirschsprung's disease (HD) a neurocristopathy. This paper is a critical review of the results of molecular-genetic studies carried out from 1992 to date. In particular, the author focuses on the possible clinical impact of the identification ofRET as a causative gene for HD.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF01194793
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  • 4
    Digitale Medien
    Digitale Medien
    Primary anastomosis in esophageal atresia type I without a gap (1999)
    Springer
    Pediatric surgery international 15 (1999), S. 376-377 
    Details
    ISSN: 1437-9813
    Schlagwort(e): Key words Esophageal atresia ; Intestinal atresia ; Neonatal surgery ; Duodenal atresia
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract This paper reports the case of an infant born with type I esophageal atresia (EA) associated with duodenal atresia (DA). The critical condition of the patient necessitated an exploratory laparotomy, which revealed severe dilatation of the stomach and duodenum. The routine procedure for repairing type I EA is a delayed primary anastomosis after 10 weeks of age because of the long gap between the two esophageal segments. In our case, due to the concomitant DA, the lower pouch was long enough to allow primary neonatal anastomosis. A radiograph taken with a Hegar dilator in the lower segment via the gastrostomy confirmed this suspicion, and the baby underwent a thoracotomy and primary anastomosis between the esophageal pouches. The authors propose the possibility of primary esophageal anastomosis in similar cases.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/s003830050602
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