Tags

Type your tag names separated by a space and hit enter

Structural basis of voiding dysfunction in megacystis microcolon intestinal hypoperistalsis syndrome.
J Pediatr Urol. 2006 Aug; 2(4):277-84.JP

Abstract

PURPOSE

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare, congenital and usually fatal condition of unknown etiology. It is characterized by abdominal distension caused by a distended, non-obstructed urinary bladder and intestinal hypoperistalsis with functional intestinal obstruction. Previous studies reported vacuolar degenerative changes in the smooth muscle cells of bowel and bladder suggesting that MMIHS may be due to a visceral myopathy. The aim of this study was to examine the expression of contractile, cytoskeletal and extracellular matrix proteins in the detrusor muscle of MMIHS patients.

MATERIAL AND METHODS

Bladder specimens were obtained from six MMIHS patients. Normal bladder specimens were obtained during partial cystectomy and served as controls. Single fluorescence immunohistochemistry for alpha-smooth muscle actin (SMA), desmin, dystrophin, vinculin and collagen types I and III was carried out. Specific connective tissue stains (trichrome Masson, van Gieson) and electron microscopical investigations were also performed.

RESULTS

Trichrome Masson and van Gieson staining demonstrated markedly increased dense connective tissue between the layers of the detrusor muscle in MMIHS compared to controls. Collagen type I immunoreactivity was markedly increased and SMA, desmin and dystrophin immunoreactivity was markedly reduced in the bladder muscle of MMIHS compared to controls. Electron microscopy revealed vacuolar degenerative changes in smooth muscle cells and an abundance of connective tissue between these cells.

CONCLUSION

These data suggest that the detrusor muscle in MMIHS is strikingly abnormal and is the likely cause of voiding dysfunction.

Authors+Show Affiliations

Department of Pediatric Surgery, University of Leipzig, Leipzig, Germany.No affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

18947621

Citation

Rolle, Udo, and Prem Puri. "Structural Basis of Voiding Dysfunction in Megacystis Microcolon Intestinal Hypoperistalsis Syndrome." Journal of Pediatric Urology, vol. 2, no. 4, 2006, pp. 277-84.
Rolle U, Puri P. Structural basis of voiding dysfunction in megacystis microcolon intestinal hypoperistalsis syndrome. J Pediatr Urol. 2006;2(4):277-84.
Rolle, U., & Puri, P. (2006). Structural basis of voiding dysfunction in megacystis microcolon intestinal hypoperistalsis syndrome. Journal of Pediatric Urology, 2(4), 277-84. https://doi.org/10.1016/j.jpurol.2006.01.017
Rolle U, Puri P. Structural Basis of Voiding Dysfunction in Megacystis Microcolon Intestinal Hypoperistalsis Syndrome. J Pediatr Urol. 2006;2(4):277-84. PubMed PMID: 18947621.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Structural basis of voiding dysfunction in megacystis microcolon intestinal hypoperistalsis syndrome. AU - Rolle,Udo, AU - Puri,Prem, Y1 - 2006/04/04/ PY - 2005/09/14/received PY - 2006/01/26/accepted PY - 2008/10/25/pubmed PY - 2008/10/25/medline PY - 2008/10/25/entrez SP - 277 EP - 84 JF - Journal of pediatric urology JO - J Pediatr Urol VL - 2 IS - 4 N2 - PURPOSE: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare, congenital and usually fatal condition of unknown etiology. It is characterized by abdominal distension caused by a distended, non-obstructed urinary bladder and intestinal hypoperistalsis with functional intestinal obstruction. Previous studies reported vacuolar degenerative changes in the smooth muscle cells of bowel and bladder suggesting that MMIHS may be due to a visceral myopathy. The aim of this study was to examine the expression of contractile, cytoskeletal and extracellular matrix proteins in the detrusor muscle of MMIHS patients. MATERIAL AND METHODS: Bladder specimens were obtained from six MMIHS patients. Normal bladder specimens were obtained during partial cystectomy and served as controls. Single fluorescence immunohistochemistry for alpha-smooth muscle actin (SMA), desmin, dystrophin, vinculin and collagen types I and III was carried out. Specific connective tissue stains (trichrome Masson, van Gieson) and electron microscopical investigations were also performed. RESULTS: Trichrome Masson and van Gieson staining demonstrated markedly increased dense connective tissue between the layers of the detrusor muscle in MMIHS compared to controls. Collagen type I immunoreactivity was markedly increased and SMA, desmin and dystrophin immunoreactivity was markedly reduced in the bladder muscle of MMIHS compared to controls. Electron microscopy revealed vacuolar degenerative changes in smooth muscle cells and an abundance of connective tissue between these cells. CONCLUSION: These data suggest that the detrusor muscle in MMIHS is strikingly abnormal and is the likely cause of voiding dysfunction. SN - 1873-4898 UR - https://www.unboundmedicine.com/medline/citation/18947621/Structural_basis_of_voiding_dysfunction_in_megacystis_microcolon_intestinal_hypoperistalsis_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1477-5131(06)00026-X DB - PRIME DP - Unbound Medicine ER -
Try the Free App:
Prime PubMed app for iOS iPhone iPad
Prime PubMed app for Android
Prime PubMed is provided
free to individuals by:
Unbound Medicine.