[Fabry disease and cystinosis, two lysosomal diseases: similarities and differences]

Rev Med Interne. 2010 Dec:31 Suppl 2:S226-8. doi: 10.1016/S0248-8663(10)70017-5.
[Article in French]

Abstract

Fabry disease and cystinosis are both lysosomal diseases. Some clinical features (such as renal and corneal involvement) are shared by both diseases whereas many other features are different (mode of inheritance, rate of progression, mechanism of lysosomal storage, therapeutic modalities etc.). Intermediary mechanisms that lead from lysosomal overload to lesions and disease are still incompletely understood.

Publication types

  • English Abstract

MeSH terms

  • Biomarkers / blood
  • Cystine / blood
  • Cystinosis / complications*
  • Cystinosis / genetics
  • Cystinosis / metabolism
  • Cystinosis / pathology*
  • Diagnosis, Differential
  • Disease Progression
  • Fabry Disease / complications*
  • Fabry Disease / genetics
  • Fabry Disease / metabolism
  • Fabry Disease / pathology*
  • Humans
  • Kidney / pathology
  • Kidney Diseases / etiology
  • Lysosomes / pathology
  • alpha-Galactosidase / blood

Substances

  • Biomarkers
  • Cystine
  • alpha-Galactosidase