These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Congenital visceral myopathy with a predominantly hypertrophic pattern treated by multivisceral transplantation.
    Author: Koh S, Bradley RF, French SW, Farmer DG, Cortina G.
    Journal: Hum Pathol; 2008 Jun; 39(6):970-4. PubMed ID: 18329691.
    Abstract:
    Chronic intestinal pseudoobstruction due to visceral myopathy may cause intestinal failure. The anatomical pathology of visceral myopathy is dominated by an atrophic muscularis propria. We present a case that is overwhelmingly dominated by a hypertrophic muscularis propria. The hypertrophic pattern was documented from ages 2 to 28 years. Most descriptions of visceral myopathy focus on the atrophic pattern of injury to the muscularis propria, typified by vacuolar degeneration of myocytes, loss of muscle fibers, and frequently with a honeycomb pattern of fibrosis. Hypertrophic changes can be observed in visceral myopathy, but the hypertrophy typically represents only a minor feature, typically limited to the internal layer of the muscularis propria. In our patient, the observed muscular hypertrophy differed from most descriptions because it (a) was the dominant pattern of injury and (b) involved both layers of muscularis propria rather than just the inner circular layer. The patient had intestinal failure and the complications induced cirrhosis, eventually treated by multivisceral transplantation to correct the intestinal failure and cirrhosis.
    [Abstract] [Full Text] [Related] [New Search]