Abstract
Dental examinations on nine patients with mucopolysaccharidosis type IV A (MPS IV A, Morquio's disease type A) were carried out. Detailed medical, radiologic, and biochemical studies of each case were also performed independently. Dental changes were present in all cases, although the severity varied. The severity of the dental changes did not correlate with the clinical or biochemical findings in all cases. These dental changes are seen only in MPS IV A (N-acetylgalactosamine-6-sulphate sulphatase deficiency) and are not found in MPS IV B (β-galactosidase deficiency) or the recently delineated MPS IV C (enzyme defect unknown). The dental changes can aid in the diagnosis of patients affected by MPS IV A and are especially useful in mild atypical cases.
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