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Megacystis-microcolon-intestinal hypoperistalsis syndrome: evidence of intestinal myopathy.
Pediatr Surg Int. 2002 Jan; 18(1):2-5.PS

Abstract

We investigated small- and large-bowel specimens of three newborn infants presenting with the clinical and radiological symptoms of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). Conventional histological staining revealed marked thinning of the longitudinal muscle layer. Electron-microscopic investigations showed typical "central core" vacuolic degeneration of smooth-muscle-cells combined with proliferation of col lagen fibres. The expression of alpha-smooth-muscle actin was absent or markedly reduced in the circular and longitudinal muscle layers and muscularis mucosae compared to the normal controls. These findings suggest that the intestinal obstruction in MMIHS is due to an abnormality of the smooth-muscle cells.

Authors+Show Affiliations

Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, Dublin 12, Ireland.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

11793054

Citation

Rolle, Udo, et al. "Megacystis-microcolon-intestinal Hypoperistalsis Syndrome: Evidence of Intestinal Myopathy." Pediatric Surgery International, vol. 18, no. 1, 2002, pp. 2-5.
Rolle U, O'Briain S, Pearl RH, et al. Megacystis-microcolon-intestinal hypoperistalsis syndrome: evidence of intestinal myopathy. Pediatr Surg Int. 2002;18(1):2-5.
Rolle, U., O'Briain, S., Pearl, R. H., & Puri, P. (2002). Megacystis-microcolon-intestinal hypoperistalsis syndrome: evidence of intestinal myopathy. Pediatric Surgery International, 18(1), 2-5.
Rolle U, et al. Megacystis-microcolon-intestinal Hypoperistalsis Syndrome: Evidence of Intestinal Myopathy. Pediatr Surg Int. 2002;18(1):2-5. PubMed PMID: 11793054.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Megacystis-microcolon-intestinal hypoperistalsis syndrome: evidence of intestinal myopathy. AU - Rolle,Udo, AU - O'Briain,Sean, AU - Pearl,Richard H, AU - Puri,Prem, PY - 2002/1/17/pubmed PY - 2002/3/27/medline PY - 2002/1/17/entrez SP - 2 EP - 5 JF - Pediatric surgery international JO - Pediatr Surg Int VL - 18 IS - 1 N2 - We investigated small- and large-bowel specimens of three newborn infants presenting with the clinical and radiological symptoms of megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). Conventional histological staining revealed marked thinning of the longitudinal muscle layer. Electron-microscopic investigations showed typical "central core" vacuolic degeneration of smooth-muscle-cells combined with proliferation of col lagen fibres. The expression of alpha-smooth-muscle actin was absent or markedly reduced in the circular and longitudinal muscle layers and muscularis mucosae compared to the normal controls. These findings suggest that the intestinal obstruction in MMIHS is due to an abnormality of the smooth-muscle cells. SN - 0179-0358 UR - https://www.unboundmedicine.com/medline/citation/11793054/Megacystis_microcolon_intestinal_hypoperistalsis_syndrome:_evidence_of_intestinal_myopathy_ L2 - https://doi.org/10.1007/s003830200001 DB - PRIME DP - Unbound Medicine ER -