Bone ultrasonometry, bone density, and turnover markers in type 1 Gaucher disease

J Bone Miner Metab. 2002;20(1):34-8. doi: 10.1007/s774-002-8444-1.

Abstract

In 12 patients (mean age, 33 +/- 13 years) with type 1 Gaucher disease (GD), we evaluated bone mass by broadband ultrasound attenuation (BUA) of the calcaneus and dual X-ray absorptiometry (DXA) of the total body, lumbar spine, and hip. In all patients, we measured serum levels of osteocalcin (OC) and bone-specific alkaline phosphatase (BAP) and urinary excretion of pyridinoline (Pyr/Cr) and deoxypyridinoline (D-Pyr/Cr) cross-links. Compared to age- and sex-matched healthy controls, patients with GD showed marked osteopenia at all measuring sites as expected. Values of BUA (67.25 +/- 15.83 dB/MHz) were also significantly reduced. OC and BAP concentrations were within the normal range. Pyr/Cr and D-Pyr/Cr were significantly higher than in controls. Calculating T- and Z scores, we found a significant correlation between the Bone Severity Score Index (BSSI) and both BUA and BMD measurements. A significant correlation was also found between pyridinoline urinary excretion and both BSSI and BUA at the calcaneus. Our data suggest that type 1 GD in adulthood is associated with increased bone resorption and that BUA at the calcaneus may be a relevant tool in the assessment of bone status in these patients.

MeSH terms

  • Adult
  • Amino Acids / urine
  • Biomarkers*
  • Body Mass Index
  • Bone Density*
  • Bone Diseases, Metabolic / etiology
  • Bone and Bones / diagnostic imaging*
  • Female
  • Gaucher Disease / diagnostic imaging*
  • Gaucher Disease / pathology
  • Humans
  • Male
  • Middle Aged
  • Sicily
  • Ultrasonography

Substances

  • Amino Acids
  • Biomarkers
  • pyridinoline