The Effects of Corticosteroids on Plasma and Leucocyte Glycosaminoglycans in the Mucopolysaccharidoses

Abstract

The effects of corticosteroid (CORT) administration to four patients with mucopolysaccharidoses (MPS), one each with MPS II, IIIA, V, and VI, on the glycosaminoglycan (GAG) content of plasma and leucocytes were examined. Plasma levels of GAG and GAG degradation products declined following initial increase in MPS II and IIIA and fluctuated throughout the investigation in MPS V and VI. The amount of polymeric GAG and degradation products in leucocytes from normal controls, as uronic acid, was about 3.5μg and 1.4μg per mg WBC protein respectively. The ratio of polymeric GAG to degradation products was lower in the patients as compared to the normal controls. Also, leucocytes from the patients contained, besides the normally present GAG, large amounts of heparan sulfate (HS) and/or dermatan sulfate (DS) depending on the type of MPS. During CORT administration, the amount of polymeric GAG in the leucocytes per mg of WBC protein, increased slightly in MPS II and VI and markedly in MPS IIIA and V; there was a large increase of degradation products in MPS II and a small decrease in MPS IIIA. It was concluded that during CORT administration the amount of GAG per WBC increased.