Mucopolysaccaharidoses (MPSs) are a group of inherited storage diseases caused by a deficiency of lysosomal enzymes that degrade glycosaminoglycans (GAGs). The MPSs are a heterogenous group characterised by intra-lysosomal accumulation of GAGs, excessive urinary excretion of GAGs, and variable degrees of progressive mental and physical deterioration. Mucopolysaccharidosis type IV (Morquio syndrome) is autosomal recessively inherited. The primary pathology is defective degradation of keratan sulphate due to one of two enzyme deficiencies; n-acetylgalactoseamine-6-sulfate (chromosome 16q) or β-galactosidase (chromosome 3p) .
Ceylon Medical Journal Vol. 53, No. 1, March 2008 pp. 24-25