Metabolic Bone Disease
A 6-year-old boy with MPS IV (Morquio syndrome) presents for preoperative assessment prior to genu valgum correction surgery. He has normal intelligence but marked short stature, thoracolumbar kyphosis, and bilateral genu valgum. His parents are concerned about anaesthetic risks. Regarding mucopolysaccharidoses and their orthopaedic manifestations:
Mark each as TRUE or FALSE
Mucopolysaccharidoses are lysosomal storage disorders caused by deficiency of enzymes required for g...
Dysostosis multiplex is the pathognomonic radiographic pattern in MPS comprising J-shaped sella turc...
MPS IV (Morquio syndrome) has severe cognitive impairment but mild skeletal disease; odontoid hypopl...
MPS IV (Morquio syndrome) is unique in having NORMAL intelligence but the most SEVERE skeletal manif...
All MPS patients require cervical spine assessment before any procedure due to odontoid hypoplasia a...
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Click T (True) or F (False) for each option