Imaging features of Fabry disease

AJR Am J Roentgenol. 2006 Apr;186(4):1184-91. doi: 10.2214/AJR.05.0019.

Abstract

Objective: Our objective was to describe the various imaging patterns of Fabry disease, including cerebrovascular, renal, cardiac, and other organ involvement. Fabry disease, an X-linked inborn error of glycosphingolipid catabolism resulting from a deficient activity of the hydrolase alpha-galactosidase A, displays more complications in men than in heterozygous women.

Conclusion: It is up to radiologists to evoke the diagnosis, help practitioners in treating patients early with enzyme replacement therapy, and monitor its efficacy.

MeSH terms

  • Adult
  • Fabry Disease / diagnosis*
  • Fabry Disease / diagnostic imaging
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Tomography, X-Ray Computed
  • Ultrasonography