| Diagnosis of megacystis-microcolon intestinal hypoperistalsis syndrome with aplastic desmosis in adulthood: a case report. | |
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MedLine Citation:
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PMID: 18334881 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Megacystis-microcolon intestinal hypoperistalsis syndrome (MMHIS or Berdon syndrome) is an autosomal-recessive disorder characterized by chronic intestinal obstruction. Although the disease is often diagnosed in female infants we describe a man with late diagnosis in adulthood. Our patient presented soon after birth with intestinal obstruction and developed short bowel syndrome after multiple intestinal resections. Of note, the connective tissue net within the muscle layers of the intestinal wall was absent ('aplastic desmosis'). This case illustrates the variable clinical features of MMHIS and aplastic desmosis, which might delay the correct diagnosis of a severe disorder. |
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Authors:
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Jonel Trebicka; Erwin Biecker; Frank Gruenhage; Manfred Stolte; William A Meier-Ruge; Tilman Sauerbruch; Frank Lammert |
Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: European journal of gastroenterology & hepatology Volume: 20 ISSN: 0954-691X ISO Abbreviation: Eur J Gastroenterol Hepatol Publication Date: 2008 Apr |
Date Detail:
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Created Date: 2008-03-12 Completed Date: 2008-07-24 Revised Date: 2009-10-16 |
Medline Journal Info:
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Nlm Unique ID: 9000874 Medline TA: Eur J Gastroenterol Hepatol Country: England |
Other Details:
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Languages: eng Pagination: 353-5 Citation Subset: IM |
Affiliation:
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Department of Internal Medicine I, University Hospital Bonn, University of Bonn, Bonn, Germany. jonel.trebicka@ukb.uni-bonn.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple*
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surgery,
therapy,
ultrasonography Adult Colonic Diseases / etiology*, surgery, ultrasonography Connective Tissue / abnormalities* Enteral Nutrition Humans Intestinal Obstruction / etiology*, surgery, ultrasonography Male Peristalsis* Short Bowel Syndrome / etiology* Syndrome Treatment Outcome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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