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mucopolysaccharidosis vii/asthenia
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3 results
Altered lumbar spine structure, biochemistry, and biomechanical properties in a canine model of mucopolysaccharidosis type VII.
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Mucopolysaccharidosis VII (MPS VII) is a lysosomal storage disorder characterized by a deficiency in beta-glucuronidase activity, leading to systemic accumulation of poorly degraded glycosaminoglycans (GAG). Along with other morbidities, MPS VII is associated with pediatric spinal deformity. The
Craniovertebral instability with spinal cord compression in a 17-month-old boy with Sly syndrome (mucopolysaccharidosis type VII): a surgical dilemma.
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METHODS
A case study with review of surgical technique in craniovertebral stabilization for young children with mucopolysaccharidosis.
OBJECTIVE
To describe an interesting patient with a rare metabolic disorder and review surgical technique for craniovertebral instability in this rare patient
Beta-glucuronidase deficiency in a dog: a model of human mucopolysaccharidosis VII.
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This report describes a third mucopolysaccharidosis in animals: canine mucopolysaccharidosis VII. The affected dog was the offspring of a father-daughter mating. Weakness in the rear legs was evident at 8 weeks of age and became progressively worse. He had a large head, a shortened maxilla, and
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