title: Sarcoglycan immunoreactivity is lacking in infantile hypertrophic pyloric stenosis. A confocal laser scanning microscopic study.
abstract: OBJECTIVE
  The Dystrophin-Glycoprotein Complex (DGC) is a large multisubunit complex that plays a crucial role in maintaining the structural integrity and physiology of muscle fibers. Dystrophin has been reported to be absent in the pyloric muscle of infantile hypertrophic pyloric stenosis (IHPS) patients. The present study was designed to investigate the other two patterns of DGC (dystroglycan and sarcoglycan complexes) in normal pyloric muscle and their possible modifications in IHPS patients.
  
  
  METHODS
  Ten pyloric muscle biopsies were obtained from babies operated for IHPS and five control pylorus biopsy taken at autopsy from cases without gastrointestinal disease. The DGC sub-complexes (beta-dystroglican and beta, delta- sarcoglycans) were localized immunohistochemically using specific monoclonal antibodies. The results were evaluated using a confocal laser scanning microscope.
  
  
  RESULTS
  Positive immunolocalization of the two DGC sub complexes was demonstrated in the smooth muscle cells (SMCs) of the pyloric region of control patients. Similarly, a positive immune expression of beta-dystroglican was observed in the pyloric SMCs of IHPS patients. On the other hand a negative immunoreaction for sarcoglycans was recorded within the full thickness of the pyloric SMCs of these patients.
  
  
  CONCLUSIONS
  The absence of sarcoglycans within the hypertrophied pyloric muscle may be a predisposing factor in the pathogenesis of IHPS since it could alter the normal physiology of SMCs through the modifications of structural integrity of sarcolemma and signaling between the extracellular and intracellular compartment.
authors.affiliation: Dipartimento di Scienze Pediatriche Mediche e Chirurgiche, U.O. di Chirurgia Pediatrica, Messina. romeoc@unime.it
authors.forename: C
authors.initials: C
authors.lastname: Romeo
authors.forename: G
authors.initials: G
authors.lastname: Santoro
authors.forename: P
authors.initials: P
authors.lastname: Impellizzeri
authors.forename: A
authors.initials: A
authors.lastname: Manganaro
authors.forename: G
authors.initials: G
authors.lastname: Cutroneo
authors.forename: E
authors.initials: E
authors.lastname: Trimarchi
authors.forename: P
authors.initials: P
authors.lastname: Antonuccio
authors.forename: G
authors.initials: G
authors.lastname: Anastasi
authors.forename: B
authors.initials: B
authors.lastname: Zuccarello
mesh_terms: D001706:Biopsy; D049030:Dystroglycans / Q000276:immunology / Q000378:metabolism; D005455:Fluorescent Antibody Technique; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D018613:Microscopy, Confocal; D018485:Muscle Fibers, Skeletal / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D046248:Pyloric Stenosis, Hypertrophic / Q000276:immunology* / Q000378:metabolism / Q000473:pathology*; D016024:Receptors, Cytoadhesin / Q000276:immunology / Q000378:metabolism; D049031:Sarcoglycans / Q000276:immunology*
chemical_list: D016024:Receptors, Cytoadhesin; D049031:Sarcoglycans; D049030:Dystroglycans