Modification of urinary mucopolysaccharide excretion in patients with Hurler's syndrome.

Abstract

Several variants of Hurler's syndrome can be distinguished both genetically and biochemically. All these patients excrete excess acid mucopolysaccharides in their urine, but the ratio of chondroitin sulfate B to heparitin sulfate varies in different families. When the patients are in a basic condition and are maintained on a constant diet, the amount of 24 hour urinary acid mucopolysaccharides and the ratio of chondroitin sulfate B to heparitin sulfate show little daily fluctuation. Seven children with Hurler's syndrome were studied in their basic state and consecutively while receiving corticosteroids, thyroid, human growth hormone, hydroxychloroquine, and salicylates. It was demonstrated that administration of hydroxychloroquine increases urinary excretion of acid mucopolysaccharides, while prednisone tends to decrease the excretion of urinary acid mucopolysaccharides. Thyroid and corticosteroids produced marked changes in the ratio of chondroitin sulfate B to heparitin sulfate, especially in patients who excreted predominantly heparitin sulfate in their basic state. Salicylates and human growth hormone did not induce significant changes in the pattern of urinary excretion of acid mucopolysaccharides.