Mobilization of heparan sulfate induced by immunostimulation in a patient with mucopolysaccharidosis IIIA.
Unspecific immunostimulation by bacterial vaccines of a patient with mucopolysaccharidosis IIIA (Sanfilippo A) syndrome induces a marked increase in the urinary excretion of heparan sulfate and of uronate-containing oligosaccharides. This event is presumably linked to an increased vascular permeability and exocytosis of storage material, elicited by mediators of inflammation, as well as to enhanced degradation of stored polymers in activated macrophages and surrounding tissue.
D001428:Bacterial Vaccines / Q000008:administration & dosage*; D002648:Child; D006025:Glycosaminoglycans / Q000652:urine*; D006497:Heparan Sulfate / Q000652:urine*; D006801:Humans; D007167:Immunotherapy; D017708:Leukocyte Transfusion; D008297:Male; D008970:Molecular Weight; D009083:Mucopolysaccharidoses / Q000652:urine*; D009084:Mucopolysaccharidosis III / Q000628:therapy / Q000652:urine*; D009844:Oligosaccharides / Q000652:urine
D001428:Bacterial Vaccines; D006025:Glycosaminoglycans; D006497:Heparan Sulfate; D009844:Oligosaccharides