BACKGROUND Kyphoscoliosis is a skeletal condition involving the hyperflexion of the thoracic spine. It is characterized by reduced chest wall compliance and impaired respiratory mechanisms leading to progressive hypo-ventilation. We evaluated the effectiveness and the safety of non-invasive ventilation (NIV) in patients after an episode of acute respiratory failure (ARF). METHODS Eighteen pat...

Background. Halo gravity traction (HGT) is one of the most commonly used perioperative techniques for the treatment of severe kyphoscoliosis. This study was to explore the influence of HGT on the BMD of these patients. Methods. Patients with severe kyphoscoliosis treated by preoperative HGT for at least 2 months were included. Patients' BMD were assessed by dual-energy X-ray absorptiometry at l...

Vanishing bone disease is an extremely rare disorder of unknown etiology characterized by idiopathic osteolysis of bone. We describe a case of vanishing bone disease of chest wall and spine with kyphoscoliosis and neurological deficit. A 17-year-old male presented with gradually progressive deformity of back and dorsal compressive myelopathy with nonambulatory power in lower limbs. Radiographs ...

Study Design The study is a case report. Objective The authors aim to report an unusual injury pattern in a patient previously treated for thoracic kyphoscoliosis. Methods A postoperative (computed tomography) CT of a healthy 24-year-old man who underwent posterior instrumentation and fusion for a kyphoscoliosis deformity was compared with a CT performed after a motor vehicle accident (MVA) 1 y...

Pax1 and Pax9 play redundant, synergistic functions in the patterning and differentiation of the sclerotomal cells that give rise to the vertebral bodies and intervertebral discs (IVD) of the axial skeleton. They are conserved in mice and humans, whereby mutation/deficiency of human PAX1/PAX9 has been associated with kyphoscoliosis. By combining cell-type-specific transcriptome and ChIP-sequenc...

BACKGROUND The Ehlers-Danlos syndrome type VI (EDSVI) is an autosomal recessive connective tissue disease which is characterized by severe hypotonia at birth, progressive kyphoscoliosis, skin hyperelasticity and fragility, joint hypermobility and (sub-)luxations, microcornea, rupture of arteries and the eye globe, and osteopenia. The enzyme collagen lysyl hydroxylase (LH1) is deficient in these...