Open AccessStructural and functional peculiarities of spine deformity development in neurofibromatosis NF-1
Author(s) -
А. М. Зайдман,
Mikhail Mikhailovsky,
Ye. L. Zaviyalova,
Vasily Suzdalov,
M. A. Sadovoy
Publication year - 2010
Publication title -
bûlletenʹ sibirskoj mediciny
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.135
H-Index - 3
eISSN - 1819-3684
pISSN - 1682-0363
DOI - 10.20538/1682-0363-2010-6-34-40
Subject(s) - neurofibromatosis , scoliosis , deformity , anatomy , spinal deformity , medicine , intervertebral disc , pathology , surgery
To study pathogenetic mechanisms of the development of spinal deformity in neurofibromatosis. Structural components of the spine were presented as specimens obtained after surgical correction of spinal deformity performed in 10 children with III—IV grade scoliosis associated with neurofibromatosis. Etiologic factor of the development of spinal deformity in neurofibromatosis is a mutation of the NF-1 gene in cells of ganglious lamella. Migration of cells carrying mutant gene into one of the sclerotome zones results in oncogene activation and intensive proliferation of chondro-, osteo-, and fibroblasts in the growth plate, intervertebral disc, and vertebral body. Progressive development of the spinal deformity after surgical intervention is accounted both for proliferation of chondro- and fibroblasts in the vertebral body and intervertebral disc and for disturbance of the NF-1 and lumican genes expression.