URINE GLYCOSAMINOGLYCAN CONCENTRATIONS IN MUCOPOLYSACCHARIDOSIS VI-AFFECTED CATS FOLLOWING BONE-MARROW TRANSPLANTATION OR LEUKOCYTE INFUSION

Urinary glycosaminoglycan (GAG) concentrations were determined in nine teen normal cats (eleven kittens and eight adult cats), eighteen mucop olysaccharidosis VI (MPS VI)-affected untreated cats (ten kittens and eight adult cats), thirteen MPS VI-affected cats following bone marrow transplantation (BMT), and two MPS VI-affected cats following intrave nous infusion of leukocytes from normal cats. Mucopolysaccharidosis VI -affected cats treated with BMT had a precipitous decrease in urinary GAG by day 7 post-BMT, then a transient increase just prior to engraft ment, followed by a sustained decrease to within, or near, the range o f urinary GAG concentration established for normal cats. The pre-engra ftment changes in urinary GAG excretion were reproduced by leukocyte i nfusion. After infusion of comparable numbers of normal peripheral blo od leukocytes, a significant decrease in urinary GAG concentrations, s pecifically dermatan sulfate (DS), was seen with a nadir at day 5 post -infusion, followed by a return by day 9 to pre-infusion values. Post- engraftment, a continued low urinary GAG concentration with a specific decrease in DS can be utilized to document successful autologous engr aftment in MPS VI-affected cats. (C) 1997 Elsevier Science B.V.