segmental neurofibromatosis: a report of 3 cases

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ID: 147063
2010
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Abstract
Neurofibromatosis is a genetic disorder of neural crest-derived cells that primarily affect growth of neural tissues. It is broadly divided into three categories: (a) von Recklinghausen′s neurofibromatosis or NF-1, (b) bilateral acoustic neuroma (NF-2), and (c) all other neurofibromatoses, including alternate or atypical forms of the disease. The patients with generalized form of NF1 are characterized by multiple café-au-lait spots and neurofibromas and diagnosed easily. But when an individual has small number of lesions in a limited region of the body it could be neglected by the patient or not be recognized by the clinicians as a segmental form of neurofibromatosis. We describe three cases of segmental neurofibromatosis (SNF). These cases have been classified as segmental NF according to Riccardi′s definition of SNF and classification of neurofibromatosis. Segmental form of NF may evolve into a complete form over time. Also, this disorder may be transmitted to the offspring′s of these individuals. Hence genetic counseling of these individuals must include these facts.
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sushma2010indiansegmental Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors ;Gabhane Sushma;Kotwal Mrunmayi;Bobhate Sudhakar
Journal heterocycles
Year 2010
DOI
DOI not found
URL
http://www.e-ijd.org/article.asp?issn=0019-5154;year=2010;volume=55;issue=1;spage=105;epage=108;aulast=Gabhane
Keywords
neurofibromatosis 1 café-au-lait spots

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