The effect of phlebotomy as a treatment of Fabry disease.

Abstract

Senescent erythrocytes are considered a major source of the ceramide trihexoside which accumulates in Fabry disease patients. We have evaluated weekly phlebotomy as a method to reduce the catabolic load imposed by the senescence of erythrocytes and thereby ceramide trihexoside in one Fabry patient; no change was observed in either plasma or urinary level of ceramide trihexoside. The implications of this observation are discussed with regard to glycolipid metabolism in man.

• Authors: E Beutler, B Westwood, G L Dale
• Journal: Biochemical medicine (Biochem Med) Vol. 30 Issue 3 Pg. 363-8 (Dec 1983) ISSN: 0006-2944 [Print] United States
• PMID: 6418150 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Trihexosylceramides

Topics

• Bloodletting
• Erythrocyte Aging
• Erythrocytes (metabolism)
• Fabry Disease (blood, therapy)
• Humans
• Male
• Trihexosylceramides (blood)