STRUCTURAL AND FUNCTIONAL PECULIARITIES OF SPINE DEFORMITY DEVELOPMENT IN NEUROFIBROMATOSIS NF-1
https://doi.org/10.14531/ss2008.3.73-80
Abstract
Objective. To study pathogenetic mechanisms of the development of spinal deformity in neurofibromatosis. Material and Methods. 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. Tissues were investigated by conventional histochemical and ultrastructural methods. The levels of aggrecan and lumnican gene expression were studied using PCR method. Results. 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. Since the mutation involves the earliest stages of embriogenesis, the formation of definitive structural components in pathologically altered zones is disturbed. Conclusion. 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 lumnican gene expression.
About the Authors
Alla Mikhailovna Zaidman
Novosibirsk Research Institute of Traumatology and Orthopaedics
Russian Federation
Russian Federation
Mikhail Vitalyevich Mikhailovsky
Novosibirsk Research Institute of Traumatology and Orthopaedics
Russian Federation
Russian Federation
Tatyana Nikiforovna Sadovaya
Novosibirsk Research Institute of Traumatology and Orthopaedics
Russian Federation
Russian Federation
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Review
For citations:
Zaidman A.M., Mikhailovsky M.V., Sadovaya T.N. STRUCTURAL AND FUNCTIONAL PECULIARITIES OF SPINE DEFORMITY DEVELOPMENT IN NEUROFIBROMATOSIS NF-1. Russian Journal of Spine Surgery (Khirurgiya Pozvonochnika). 2008;(3):073-080. https://doi.org/10.14531/ss2008.3.73-80
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