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Cervicothoracic Myelopathy in Children With Morquio Syndrome A: A Report of 4 Cases
Journal of Pediatric Orthopaedics, 2014Craniovertebral junction anomalies and C1-C2 instability resulting in myelopathy have been well described in the literature on mucopolysaccharidosis IV (MPS-IV). Spinal involvement in MPS-IV patients, with neurological impairment, other than atlanto-axial instability and thoracolumbar kyphosis, has been scarcely mentioned in the literature ...
W. Baratela +6 more
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A&A practice
Morquio syndrome, a lysosomal storage disease, is associated with an increased risk of spinal cord ischemia with positioning changes.1 Though uncommon in non-spine surgeries, intraoperative neuromonitoring (IONM) can facilitate prompt detection of ...
Archana D Bharadwaj +3 more
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Morquio syndrome, a lysosomal storage disease, is associated with an increased risk of spinal cord ischemia with positioning changes.1 Though uncommon in non-spine surgeries, intraoperative neuromonitoring (IONM) can facilitate prompt detection of ...
Archana D Bharadwaj +3 more
semanticscholar +1 more source
Management of Difficult Airway in a Child with Morquio Syndrome: A Case Report
International Journal of Science and Research (IJSR): We are reporting a case of Morquio syndromic child with a challenging airway, a 14‑years‑old boy known case of morquio syndrome with a fixed cervical spine planned for extension of fusion and decompression of sub axial spine under IONM, successfully ...
Vidhya Deshmukh, Reshma Sathish
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A Complete Clinical Spectrum of Morquio Syndrome.
Neurology IndiaTungish Bansal, Arpit Sahu, S. Kale
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A Case of Morquio-Brailford Syndrome
British Journal of Psychiatry, 1964The principal clinical features of this rare, familial disease are the triad of skeletal deformities, structural anomalies of the cardiovascular system, and mental subnormality.The syndrome is attributed to a recessive gene, and Morquio (1929) held the view that the underlying pathology consisted of mucoid changes occurring in the cartilages in place ...
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A rare case of Morquio syndrome Type A: Its diagnostic and management hurdles
IJEM Case Reports, 2023Morquio syndrome Type A is a rare autosomal recessive lysosomal storage disorder caused by a deficiency in N-acetylgalactosamine-6-sulfate sulfatase. Multisystem involvement is a usual presentation but early onset of the disease can only have isolated ...
K. B. Devi +3 more
semanticscholar +1 more source
Cervical kyphotic myelopathy in a child with Morquio syndrome
Child's Nervous System, 1996A child with Morquio syndrome developed severe cervical kyphosis causing myelopathy. Decompression of the spinal cord was accomplished by an anterior approach using multilevel corpectomies and stabilization with calvarial bone grafts and halo immobilization.
C B, Piccirilli, W M, Chadduck
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Inspiratory muscle training in Morquio's syndrome: A case study
Pediatric Pulmonology, 2006We reported a case of MPS IV A presented with dyspnea on exertion and respiratory muscle weakness. The patient underwent inspiratory muscle training (IMT) using threshold loading for 18 weeks. After 6 weeks of initial IMT, aerobic exercise training consisting of walking was added to the treatment program.
Sema, Savci +5 more
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Morquio B syndrome: A primary defect in β‐galactosidase
American Journal of Medical Genetics, 1983AbstractFibroblasts from patients with Morquio B syndrome contain normal numbers of β‐galactosidase molecules with normal turnover but strongly reduced activity per enzyme molecule. Various substrate affinities are abnormal: the KM for methylum belliferyl (MU)‐β‐galactoside is 4–10‐fold elevated and affinity for keratan sulphate and oligosaccharides ...
G T, van der Horst +5 more
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American journal of orthopedics (Belle Mead, N.J.), 1997
Morquio syndrome is one of the mucopolysaccharidoses. Glycosaminoglycans accumulate within the cells, leading to many systemic alterations. Alterations in connective tissue and cartilage ground substance result in abnormal formation and growth of the skeletal system.
M, Mikles, R P, Stanton
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Morquio syndrome is one of the mucopolysaccharidoses. Glycosaminoglycans accumulate within the cells, leading to many systemic alterations. Alterations in connective tissue and cartilage ground substance result in abnormal formation and growth of the skeletal system.
M, Mikles, R P, Stanton
openaire +1 more source