Glycosaminoglycan excretion in osteogenesis imperfecta.

This study was carried out in order to determine whether osteogenesis imperfecta affected individuals showed a different pattern of glycosaminoglycan excretion in urine than do normals. Quantitative excretion was compared by three different methods, and the qualitative composition of the excreted glycosaminoglycan was compared by electrophoresis. No difference was noted in the amount of glycosaminoglycan excreted by normal or affected individuals as measured by macromolecular uronic acid, Alcian Blue bound by urinary constituents or amount of glycosaminoglycan actually isolated by partition. However, the affected individuals showed a statistically significant increase in Alcian Blue binding over macromolecular uronic acid not seen in the normal group, which may possibly indicate the excretion of a substance capable of binding Alcian Blue, but which is not glycosaminoglycan, by osteogenesis imperfecta affected individuals. No qualitative differences in glycosaminoglycan excretion between the two groups were detectable by electrophoresis.