Body composition and bone metabolism in young Gaucher disease type I patients treated with imiglucerase.

Abstract

Although the patients had been receiving ERT, they presented a significant diminution in all body composition parameters, the decrease was more evident in those receiving the lower dose. The reduction in bone mass was associated with an imbalance in bone turnover (increased bone resorption). The correlation between LM and bone mass, suggests that metabolic disturbance occurring in GDI patients may be indirectly responsible for bone mass reduction in GDI patients, by altering body composition.