Mucosal nerve deficiency in chronic childhood constipation: a postmigration defect?

Wendelschafer‐Crabb, Gwen; Neppalli, Vishala T.; Jessurun, José; Hodges, James S.; Vance, Karl K.; Saltzman, Daniel A.; Acton, Robert D.; Kennedy, William R.

doi:10.1016/j.jpedsurg.2008.08.005

Cited by 6 publications

(3 citation statements)

References 25 publications

(27 reference statements)

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“…The diameter of myenteric nerve fibers/trunks or the nerve area/volume has been analyzed in five reports 13,19,41,55,56 . However, they differ in the methods used and regions or layers of the gut that were studied and such results are hard to compare.…”

Section: Resultsmentioning

confidence: 99%

Quantitation of cellular components of the enteric nervous system in the normal human gastrointestinal tract - report on behalf of the Gastro 2009 International Working Group

Knowles

Veress

Kapur

et al. 2010

Neurogastroenterology &Amp; Motility

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The aim of this report was to summarize all relevant available published quantitative data for elements of the human enteric nervous system (neuronal cell bodies, glial cells, and nerve fibers) from the perspective of the practicing pathologist. Forty studies meeting inclusion criteria were systematically reviewed with data tabulated in detail and discussed in the context of methodological variations and limitations. The results reveal a lack of concordance between observations of different investigators resulting in data insufficient to produce robust normal ranges. This diversity highlights the need to standardize the way pathologists collect, process, and quantitate neuronal and glial elements in enteric neuropathologic samples, as suggested by recent international guidelines on gastrointestinal neuromuscular pathology.

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Section: Resultsmentioning

confidence: 99%

Quantitation of cellular components of the enteric nervous system in the normal human gastrointestinal tract - report on behalf of the Gastro 2009 International Working Group

Knowles

Veress

Kapur

et al. 2010

Neurogastroenterology &Amp; Motility

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“…These patients had decreased innervation of the rectal mucosa. 37 Thus, our study may demonstrate maternal chimeric cells clustered in areas of absent enteric neurons in two conditions.…”

Section: Discussionmentioning

confidence: 64%

Maternal Microchimerism in Hirschsprung’s Disease

Kiefer¹,

Lang²,

Hein³

et al. 2011

Amer J Perinatol

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Hirschsprung's disease (HD) presents with severe constipation due to absent ganglion cells in the distal rectum. We sought to determine whether maternal chimeric cells are present in aganglionic bowel. We hypothesize that chimeric cells are part of the unfavorable microenvironment that leads to the destruction of enteric neurons in HD. Intestinal biopsies and resections from seven male patients with HD were compared with four male patients with chronic constipation and six with bowel atresia. Fluorescence in situ hybridization was used to identify chimeric cells based on male/female (XX/XY) differences. The location and immunophenotype of chimeric cells were also studied. Chimeric cells were present more often in the small intestine and rectum, compared with the appendix and colon. Patients with HD had a greater number of chimeric cells per 10× magnification field than patients with chronic constipation or congenital atresia. Chimeric cells were predominantly in the submucosa and outer longitudinal muscle layer in HD. Immunophenotyping identified over 40% of chimeric cells as inflammatory. Chimeric cells are present in greater numbers in aganglionic bowel than in other disorders. Clustering of chimeric cells in areas of absent ganglia lends support to the proposed role of maternal microchimerism in allo-autoimmune responses.

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“…A problem with any of these components may disturb coordinated functioning and cause constipation. A recent study found decreased nerve density in the bowel lining of children with chronic constipation and postulated that this is the pathologic basis [10].…”

Section: Pathophysiologymentioning

confidence: 99%

Recent advances in chronic constipation

Walia

Mahajan

Steffen

2009

Current Opinion in Pediatrics

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The recent literature on chronic constipation has provided some new knowledge providing a more evidence-based approach to treating these patients. We review the scope of this problem and the estimated cost of treating constipation. The availability of long-term outcome data enhances our understanding of the natural history of this problem. Newer pharmacological approaches provide optimism for treatment for this common problem.

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Mucosal nerve deficiency in chronic childhood constipation: a postmigration defect?

Cited by 6 publications

References 25 publications

Quantitation of cellular components of the enteric nervous system in the normal human gastrointestinal tract - report on behalf of the Gastro 2009 International Working Group

Quantitation of cellular components of the enteric nervous system in the normal human gastrointestinal tract - report on behalf of the Gastro 2009 International Working Group

Maternal Microchimerism in Hirschsprung’s Disease

Recent advances in chronic constipation

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