MORQUIO'S DISEASE

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Abstract
The radiologic and morphologic changes seen in a patient with Morquio's disease who came to autopsy are described and discussed. Pathologic changes were limited to cartilage and consisted of cytochemically definable lesions of the matrix characterized by the presence of amorphous and fibrillar lesions and the accumulation of foam cells. An abnormal accumulation of mucopolysaccharides in foam cells and cartilage matrix was present. No evidence of visceral storage of mucopolysaccharides, such as is seen in Hurler's disease, was present. In addition to Morquio's disease, this patient had a patent ductus arteriosus. Clinical features and radiologic changes in an older living sibling, who has had Morquio's disease and is now apparently developing the Morquio-Ullrich variant, are described.

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