Sulfated Mucopolysaccharides of Urine and Organs in Gargoylism (Hurler's Syndrome) II. Additional Studies.

Abstract
Summary 1) Further examples of quantity and types of urinary excretion of sulfated mucopolysaccharides in Hurler's syndrome have been studied in 9 cases. All excreted both ChS-B as the major component and heparitin sulfate as a minor constituent. 2) In one case, excretion of mucopolysaccharides dropped after prednisone administration. 3) From various organs of 5 autopsy cases, the 2 polysaccharides were isolated. 4) Livers of 4 cases contained between 1.2 and 6.7% of total mucopolysaccharides (on fat-free dry wt). In 3 of these, 90% was heparitin sulfate, but from all of these, also some ChS-B was obtained. One liver had approximately equal quantities of the 2 polysaccharides. 5) In one case, spleen, brain and kidney of the same individual also yielded both polysaccharides, but in proportions remarkably different from those of liver. Brain of 2 additional cases also yielded the 2 mucopolysaccharides. One of these contained 85% of ChS-B. The brain of a normal child did not yield any chondroitin sulfate, but hyaluronate and heparitin sulfate.