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image/svg+xml Jakob Voss, based on art designer at PLoS, modified by Wikipedia users Nina and Beao Closed Access logo, derived from PLoS Open Access logo. This version with transparent background. http://commons.wikimedia.org/wiki/File:Closed_Access_logo_transparent.svg Jakob Voss, based on art designer at PLoS, modified by Wikipedia users Nina and Beao Digestive and Liver ...arrow_drop_down
image/svg+xml Jakob Voss, based on art designer at PLoS, modified by Wikipedia users Nina and Beao Closed Access logo, derived from PLoS Open Access logo. This version with transparent background. http://commons.wikimedia.org/wiki/File:Closed_Access_logo_transparent.svg Jakob Voss, based on art designer at PLoS, modified by Wikipedia users Nina and Beao
Digestive and Liver Disease
Article . 2008 . Peer-reviewed
License: Elsevier TDM
Data sources: Crossref
image/svg+xml Jakob Voss, based on art designer at PLoS, modified by Wikipedia users Nina and Beao Closed Access logo, derived from PLoS Open Access logo. This version with transparent background. http://commons.wikimedia.org/wiki/File:Closed_Access_logo_transparent.svg Jakob Voss, based on art designer at PLoS, modified by Wikipedia users Nina and Beao
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Intestinal plexiform neurofibromas

Authors: CALLIGARIS L; MARCHETTI F; VENTURA, ALESSANDRO;

Intestinal plexiform neurofibromas

Abstract

a m A 6-year-old girl affected by type-1 neurofibromatosis NF1) presented with 1-month history of isolated abdominal ain. Physical examination revealed a palpable mass in the loer right abdominal section. Laboratory data were all normal. An abdominal ultrasound scan revealed a thickened wall f the last ileal loop and, under the liver, an image suggestive f a chronic intestinal intussusception. The CT-scan with contrast showed a middle-density mass 5 cm× 10 cm) enclosing dilated and congested mesenteric essels and a thickened wall of the last ileal loop with ileoecal valve intussusception in to the cecum (Fig. 1). Hystopathologic study revealed enlarged, myxoid appearng nerve trunks with proliferations of neurites and Schwann ells situated in a pale-staining stroma, allowing the diagnois of plexiform neurofibromatosis involving the mesentery nd bowel. In our case complete surgical excision was impossible and treatment with thalidomide was started. NF1 is a genetic condition with a minimum prevalence f one in 4–5000 [1]. The most distinctive clinical features re multiple peripheral neurofibromas and coetaneous pigentation. Involvement of the gastrointestinal tract affect pproximately 25% of patients but lesions are symptomatic in ess than 5% [2]. Plexiform neurofibromas, which are uncomon but are pathognomonic of NF1, diffusely infiltrate the ayers of the gut wall and mesentery [2]. Intestinal plexiform neurofibromas may present with lceration, bleeding, intestinal obstruction, and rarely, intususception, volvulus, and perforation. Intestinal vascular and ymphatic obstruction may result in bowel oedema, loss of rotein and hypoproteinemia [3].

Country
Italy
Related Organizations
Keywords

Neurofibroma, Plexiform, Angiogenesis Inhibitors, Sensitivity and Specificity, Thalidomide, Diagnosis, Differential, Ileal Neoplasms, Neoplasms, Multiple Primary, Treatment Outcome, Humans, Female, Mesentery, Child, Peritoneal Neoplasms

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selected citations
These citations are derived from selected sources.
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
3
Average
Average
Average
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