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Intestinal Connective tissue abnormality may cause Intestinal Desmosis〔Holshneider AM &, Puri P.(2008."Hirschsprung's Disease and Allied Disorders". Ed.Springer〕〔Meier-Ruge W.A. and Bruder E. : “Pathology of Chronic Constipation in Pediatric and Adult Coloproctology“, Karger 2005〕 The absence of the tendinous plexus layer was first described in 1998 by Meier-Ruge.〔Meier-Ruge WA. (1998). "Desmosis of the colon: a working hypothesis of primary chronic constipation". Eur J Pediatr Surg.8; 299-303〕 Desmosis is implicated in disturbed gut motility. Normal peristalsis depends upon the interaction between muscles, nerve cells and tendinous connective tissue. A malfunction of any of these leads to intestinal motility disorders. Desmosis may be congenital (aplastic form) or acquired (atrophic form). The ''aplastic''〔Meier-Ruge WA, Bruder E. (2007). "The morphological characteristics of aplastic and atrophic desmosis of the intestine". Pathologe 28: 149-54〕 form is rare. Typical clinical findings are hypoperistalsis, and pseudo-obstruction. These are found in premature infants, associated with low birth weight. The ''atrophic''〔Meier-Ruge WA, Bruder E. (2005). "Atrophic desmosis as secondary connective tissue atrophy in muscularis propria". Pathobiology,72:78-81〕 form is more frequent. Inflammation of the muscularis propria releases enzymes including collagenases which destroy the connective tissue of the bowel wall. Primarily newborns and small children are affected, although this manifestation can also be found in adults. The most common location is the colon with a necrotizing enterocolitis as well as Crohn Disease and diverticulitis. If the taenia are also affected, the disease is defined as complete atrophic desmosis, all other forms without involvement of the taenia are referred to as incomplete. Clinically, patients demonstrate chronic constipation. As proposed by Giuseppe Martucciello,〔Martucciello G. (2008 Jun) "Hirschsprung's disease, one of the most difficult diagnoses in pediatric surgery: a review of the problems from clinical practice to the bench". Eur J Pediatr Surg. ;18(3):140-9. Review. PubMed PMID 18493886〕 microscopic diagnosis requires laparoscopic intestinal full-thickness biopsies from colon. Histological findings are absence of the tendinous plexus layer and connective tissue fibers in longitudinal and circular muscle layer.〔Meier-Ruge W.A. and Bruder E. : “Pathology of Chronic Constipation in Pediatric and Adult Coloproctology“, Karger 2005〕 ==References== 抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)』 ■ウィキペディアで「Desmosis」の詳細全文を読む スポンサード リンク
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