INCREASED EXCRETION OF LOW MOLECULAR WEIGHT GLYCOSAMINOGLYCAN FRAGMENTS FOLLOWING PLASMA INFUSION IN A CASE OF HUNTER SYNDROME

摘要

A 3-year old boy with the severe form of Hunter syndrome (MPS IIA), was treated by the infusion of plasma from 6 pints of pooled blood over a period of 2 days. Total 24hr uronic acid excretion increased fourfold immediately following infusion. This increase was almost entirely due to glycosaminoglycan fragments not precipitated with 9-aminoacridine although there was also a small increase in the amount of precipitated (polymeric) material. There was an increase in the proportion of glycosaminoglycan fragments of lowest molecular wt. coupled with a decrease in intermediate sized fragments shown by Sephadex gel filtration. Degree of sulphation was stable before infusion, then fell and rose again in a cyclical pattern after treatment. Larged sized precipitable glycosaminoglycan fragments had a noticably higher content of glucosamine than the smaller supernatant fragments suggesting that the heparan sulphate glycosaminoglycans were less readily degradable than dermatan & chondroitin sulphates. This pattern of response differed qualitatively from those we have found in 2 types of (MPS III) (Dean et al, 1973.)