Structural and functional peculiarities of spine deformity development in neurofibromatosis NF-1

Abstract

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.

Keywords

• нейрофиброматоз
• пластинка роста
• склеротом
• деформация позвоночника
• экспрессия гена