Intestinal Connective tissue abnormality may cause Intestinal Desmosis The absence of the tendinous plexus layer was first described in 1998 by Meier-Ruge. 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).
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- Desmosi intestinale (it)
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| - Intestinal Connective tissue abnormality may cause Intestinal Desmosis The absence of the tendinous plexus layer was first described in 1998 by Meier-Ruge. 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). (en)
- La desmosi intestinale è una patologia infantile che consiste nell'alterazione del contenuto connettivale della parete intestinale, che altera la normale composizione della tonaca muscolare (muscolatura liscia intestinale) determinando gravi alterazioni della motilità dell'intestino. La desmosi è stata descritta per la prima volta da Meier-Ruge WA nel 1998, sulla base di osservazioni svolte in casi di pseudo-Hirschsprung o pseudo-ostruzione intestinale studiati con tecniche istologiche tricromiche (Picrus Sinus Red, tecnica di Masson, Azan-Mallory). (it)
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| - Intestinal Connective tissue abnormality may cause Intestinal Desmosis The absence of the tendinous plexus layer was first described in 1998 by Meier-Ruge. 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 form is rare. Typical clinical findings are hypoperistalsis, and pseudo-obstruction. These are found in premature infants, associated with low birth weight. The atrophic 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 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, 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. (en)
- La desmosi intestinale è una patologia infantile che consiste nell'alterazione del contenuto connettivale della parete intestinale, che altera la normale composizione della tonaca muscolare (muscolatura liscia intestinale) determinando gravi alterazioni della motilità dell'intestino. La desmosi è stata descritta per la prima volta da Meier-Ruge WA nel 1998, sulla base di osservazioni svolte in casi di pseudo-Hirschsprung o pseudo-ostruzione intestinale studiati con tecniche istologiche tricromiche (Picrus Sinus Red, tecnica di Masson, Azan-Mallory). Esiste una forma descritta aplastica, caratterizzata da disturbi della rete connettivale intestinale nei neonati pre-maturi, con quadro clinico di ipoperistalsi e pseudo-ostruzione.Esiste una seconda forma descritta atrofica: caratterizzata da infiammazione della tonaca muscolare con rilascio di enzimi (collagenasi) e conseguente distruzione secondaria del tessuto connettivo intestinale. Questa seconda forma oltre che neonati e bambini, interessa anche l'età adulta. Clinicamente la desmosi atrofica si manifesta con stitichezza. (it)
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