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Bone density changes with enzyme therapy for Gaucher disease.

Ehud Lebel1, Altoon Dweck, A Joseph Foldes

  • 1Department of Orthopedic Surgery, Shaare Zedek Medical Center, Jerusalem, Israel.

Journal of Bone and Mineral Metabolism
|October 19, 2004
PubMed
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Enzyme replacement therapy for Gaucher disease showed limited bone density improvement, especially in younger patients. Older adults experienced some bone density gains, suggesting potential benefits later in life.

Area of Science:

  • Biochemistry
  • Genetics
  • Metabolic Disorders

Background:

  • Gaucher disease is a prevalent lysosomal storage disorder.
  • Enzyme replacement therapy (ERT) improves hematological and visceral parameters.
  • The effect of ERT on bone density (BMD) in Gaucher disease remains unclear.

Purpose of the Study:

  • To evaluate long-term bone density changes in Gaucher disease patients undergoing ERT.
  • To assess the impact of ERT on BMD in different age groups.

Main Methods:

  • Follow-up of BMD in ten Israeli patients treated with low-dose recombinant enzyme for up to 108 months.
  • Dual-energy X-ray absorptiometry (DEXA) used to measure BMD at the femoral neck and lumbar spine.
  • BMD measurements taken at baseline, 3-6 months, 18-24 months, and at most recent follow-up.

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Main Results:

  • All patients presented with very low BMD at onset, which did not normalize.
  • A decrease in BMD was observed in all patients at 3-6 months.
  • Older patients (>30 years) showed some BMD improvement during treatment, while younger patients (18-23 years) did not achieve statistically significant improvement.

Conclusions:

  • ERT may not restore peak bone density in Gaucher disease patients.
  • Older patients demonstrated better z-scores, suggesting a potential catch-up period.
  • Combination therapy with anti-osteoporosis agents for young adults and ERT for older adults may be beneficial.