BONE-DENSITY IN TYPE-1 GAUCHER DISEASE

Skeletal involvement is a major source of complications in patients wi th Type 1 Gaucher disease. To investigate the bone density and potenti al usefulness of bone densitometry in Gaucher disease? dual-energy X-r ay absorptiometry was used to measure the density of the lumbar spine, femoral neck, trochanter, and distal radius in 61 adult patients rang ing in age from 22 to 77 years, The mean bone density at each site was significantly lower than expected for age and sex, The severity of th e osteopenia correlated significantly with other clinical indicators o f disease severity, including the N370S/84GG genotype, prior splenecto my, and hepatomegaly, The bone density measurements also correlated si gnificantly,vith the severity of skeletal disease as assessed by skele tal radiography, Vertebral density remained an independent predictor o f the severity of bone involvement even after controlling for age, sex weight, genotype, splenectomy, and hepatomegaly, These findings sugge st that bone density measurements provide a quantitative assessment of bone involvement in Type 1 Gaucher disease, which may permit serial, noninvasive monitoring of bone changes in this progressive disorder.