Clinically relevant therapeutic endpoints in type I Gaucher disease

J Inherit Metab Dis. 2001:24 Suppl 2:97-105; discussion 87-8. doi: 10.1023/a:1012492429191.

Abstract

The introduction of enzyme supplementation therapy for Gaucher disease has had a great impact on the lives of many patients. Organomegaly, cytopenia and bone disease have been shown to improve in response to treatment, resulting in an improvement in quality of life. However, the assessment of organ system involvement is not always done in such a way that the relationship with clinically relevant endpoints is clear. The lack of adequately validated methods of assessment, especially for bone disease, has hindered the establishment of treatment goals and guidelines for treatment optimization.

Publication types

  • Review

MeSH terms

  • Biomarkers
  • Bone Diseases / etiology
  • Endpoint Determination
  • Enzyme Therapy*
  • Gaucher Disease / blood
  • Gaucher Disease / pathology
  • Gaucher Disease / therapy*
  • Glucosylceramidase / therapeutic use*
  • Hepatomegaly / drug therapy
  • Hepatomegaly / pathology
  • Humans
  • Quality of Life
  • Splenomegaly / drug therapy
  • Splenomegaly / pathology
  • Treatment Outcome

Substances

  • Biomarkers
  • Glucosylceramidase