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      Hirschsprung's Disease and Allied Disorders 

      Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome

      Springer Berlin Heidelberg

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          Most cited references 63

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          Interstitial cells of Cajal in the human normal urinary bladder and in the bladder of patients with megacystis-microcolon intestinal hypoperistalsis syndrome.

          To investigate the distribution of c-kit-positive interstitial cells of Cajal (ICCs) in normal bladder and bladders from patients with megacystis-microcolon-intestinal peristalsis syndrome (MMIHS, a rare congenital and generally fatal cause of functional intestinal obstruction in the newborn), the most characteristic feature of which is abdominal distension caused by a distended unobstructed urinary bladder. Full-thickness bladder specimens were obtained from four infants with MMIHS and four controls, and processed as paraffin-wax and frozen sections. Sections were assessed using single immunohistochemistry with monoclonal and polyclonal anti-c-kit antibodies. Anti-alpha-smooth muscle actin (SMA) antibody was used to investigate the contractile apparatus in smooth muscle cells of the urinary bladder. Specimens were examined using light and confocal scanning microscopy. There were many c-kit positive ICCs in the normal urinary bladder, appearing as small, long, bipolar cells with only two long and several short processes. In contrast, ICCs were absent in the MMIHS bladder. alpha-SMA immunoreactivity was lower in MMIHS urinary bladder than in control sections. This study shows for the first time the presence of c-kit-positive ICCs in the normal human urinary bladder. The lack of ICCs in the MMIHS bladder may contribute to the voiding dysfunction in this disease.
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            Megacystis microcolon intestinal hypoperistalsis syndrome.

             Prem Puri,  M Shinkai (2005)
            Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare and the most severe form of functional intestinal obstruction in the newborn. The major features of this congenital and usually lethal anomaly are abdominal distension, bile-stained vomiting, and absent or decreased bowel peristalsis. Abdominal distension is a consequence of the distended, unobstructed urinary bladder with or without upper urinary tract dilation. Most patients with MMIHS are not able to void spontaneously. This article reviews the pathogenesis of MMIHS as well as the clinical, radiological, surgical and histological findings in all reported cases of this syndrome.
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              Histological phenotypes of enteric smooth muscle disease causing functional intestinal obstruction in childhood.

               V V Smith,  P Milla (1997)
              Functional intestinal obstruction or chronic idiopathic intestinal pseudo-obstruction is due to defects either in the enteric innervation or in intestinal smooth muscle. We have studied full-thickness intestinal biopsies from 27 patients with functional intestinal obstruction due to enteric smooth muscle disease by routine histology and electron microscopy together with histochemical and immunohistochemical techniques to detect changes in the intestinal smooth muscle.
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                Author and book information

                Book
                978-3-540-33934-2
                978-3-540-33935-9
                2008
                10.1007/978-3-540-33935-9
                Book Chapter
                : 267-273
                10.1007/978-3-540-33935-9_19

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