Article

Altered distribution of interstitial cells of Cajal in Hirschsprung disease.

Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin, Ireland.
Archives of pathology & laboratory medicine (Impact Factor: 2.88). 09/2002; 126(8):928-33. DOI: 10.1043/0003-9985(2002)126<0928:ADOICO>2.0.CO;2
Source: PubMed

ABSTRACT Constipation or recurrent intestinal dysmotility problems are common after definitive surgical treatment in Hirschsprung disease (HD). c-Kit-positive interstitial cells of Cajal (ICCs) play a key role in the motility function and development of the gastrointestinal tract. Interstitial cells of Cajal that carry the tyrosine kinase receptor (c-Kit) develop as either myenteric ICCs or muscular ICCs under the influence of the kit ligand, which can be provided by neuronal and nonneuronal cells, for example, smooth muscle cells.
To investigate the distribution of myenteric and muscular ICCs in different parts of the colon in HD.
Resected bowel specimens from 8 patients with rectosigmoid HD were investigated using combined staining with c-Kit enzyme and fluorescence immunohistochemistry and acetylcholinesterase and nicotinamide adenine dinucleotide phosphate (NADPH) histochemistry in whole-mount preparations and conventional frozen sections.
In the normal bowel, ICCs formed a dense network surrounding the myenteric plexus and at the innermost part of the circular muscle. Myenteric ICCs were absent or sparse in the aganglionic bowel and sparse in the transitional zone. The expression of myenteric ICCs in the ganglionic bowel in HD was reduced compared to that in the normal bowel, and they formed only sparse networks. Muscular ICCs were found in the aganglionic bowel, transitional zone, and normoganglionic bowel of HD in a reduced density compared to the normal bowel.
This study demonstrates altered distribution of ICCs in the entire resected bowel of HD patients. This finding suggests that persistent dysmotility problems after pull-through operation in HD may be due to altered distribution and impaired function of ICCs.

Full-text

Available from: Udo Rolle, Sep 25, 2014
1 Follower
 · 
72 Views
  • Source
    World Journal of Gastroenterology 01/2010; 16(26):3239. DOI:10.3748/wjg.v16.i26.3239 · 2.43 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Interstitial cells of Cajal (ICCs) were discovered in the gastrointestinal tract over 100 years ago and since then numerous digestive tract pathologies involving ICCs have been described. Many researchers explored ICCs presence and function in the upper urinary tract. Currently, we know that ICCs have potential plasticity, their own spontaneous activity and that they are responsible for Ca2+ waves generation and neuromuscular transmission. ICCs are also involved in the conjugation, propagation and modulation of peristaltic waves in the upper urinary tract. Despite everything we know about ICCs, their role in the pathogenesis of the upper urinary tract abnormalities remains still unclear and results of published studies are confusing. The authors' intention was to review the scientific literature regarding ICCs and to summarise the current knowledge about their nature in the upper urinary tract.
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Hirschsprung's disease (HD) is a disorder that involves several medical specialties such as paediatric gastroenterology, paediatric surgery, and pathology. Hirschsprung's disease is a congenital bowel innervation disorder characterised by the absence of ganglion cells in myenteric (Auerbach) and submucosal (Meissner) plexus in the distal colon in its classical form. Rapid and accurate diagnosis of HD is a key element in further treatment patterns. The efficiency of different diagnostic methods used in HD patients may vary. Using one limited diagnostic procedure can lead to as much as a few per cent of overlooked cases. In recent years, rectal biopsy was recognised as an important diagnostic tool that allows for a definitive HD diagnosis with an accuracy of 95% of cases. A correct diagnosis depends on the localisation of the biopsied sample, its representativeness, the number of specimens, and proper interpretation of microscopic studies supported by histochemical and immunohistochemical methods. When several methods are used and all diagnostic criteria are used, the diagnostic sensitivity can almost eliminate cases of undiagnosed patients.
    Przegląd Gastroenterologiczny 01/2014; 9(5):264-9. DOI:10.5114/pg.2014.46160 · 0.38 Impact Factor