The Journal of Bone and Joint Surgery (American). 2009;91:438-442.
doi:10.2106/JBJS.H.00148
© 2009 The Journal of Bone and Joint Surgery, Inc.
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Cervical Stenosis and Spastic Quadriparesis in Morquio Disease (MPS IV)

A Case Report with Twenty-six-Year Follow-up

Klane K. White, MD, MSc1, Suzanne Steinman, MD2 and Scott J. Mubarak, MD2

1 Department of Orthopedic Surgery, Seattle Children's Hospital, 4800 Sand Point Way, W-7706, Seattle, WA 98105. E-mail address: klane.white@seattlechildrens.org
2 Department of Orthopaedic Surgery, Rady Children's Hospital, University of California at San Diego, 3030 Children's Way, Suite 410, San Diego, CA 92123

Investigation performed at the Department of Orthopaedic Surgery, Rady Children's Hospital of San Diego, San Diego, California

The first 150 words of the full text of this article appear below.


   Introduction
 
The mucopolysaccharidoses (MPS) are a family of lysosomal storage diseases that result in the abnormal accumulation of glycosaminoglycans throughout the body1. There is a broad spectrum in severity of expression of the musculoskeletal and neurological manifestations of these diseases, ranging from an early and obvious presentation, with rapid degeneration and death, to a nearly asymptomatic phenotype and normal life expectancy. Morquio disease (MPS IV) is an autosomal-recessive defect in the activity of one of two enzymes, N-acetylgalactosamine-6-sulfatase (MPS IVA) or β-galactosidase (MPS IVB), resulting in the accumulation of keratan sulfate and chondroitin-6 sulfate2. The estimated incidence of this disease ranges from 1 in 76,000 to 1 in 450,000 live births, with affected individuals typically living into their third decade, and frequently longer2-4. MPS IV is the prototypical MPS disorder with regard to the expression of dysostosis multiplex. This pervasive skeletal dysplasia consists of a short-trunked dwarfism, thoracolumbar . . . [Full Text of this Article]


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