I have speculated on such an association in a young man from armenia with atypical, reversible HUS and skin lesion similar to those in Fabry`s disease. Than I found the paper :Fabry Disease in Mice Protects against Lethal Disease Caused by Shiga Toxin–Expressing Enterohemorrhagic Escherichia coli Salvatore A. Cilmi, et al in the J Infectious Dis 2006. So I just wonder :-)
the association of Fabry disease and TMA in humans has never been reported to date to my knowledge. CD77 (Gb3) expression is increased on human peripheral blood leukocytes in FD; this has not been looked at in gut mucosal cells. What effect this might have on shiga toxin binding in man is unknown.
In Würzburg we did not observe such an association. Was the man from armenia tested for Fabry disease?
Meinen Sie M. Fabry und thrombotic microangiopathy (TMG)? Diese Kombination ist mir nicht bekannt. Ein großer Fabry-Spezialist ist z.B. R.J. Desnick [email protected]
Thanks for your answers and remarks.Thrombotic microangiopathy, abbreviated TMA, is a pathology that results in thrombosis in capillaries and arterioles, due to an endothelial injury. We are waiting for the results of testing for Fabry, unfortunately the skin lesions are not this typical. I just found the association discussed in the cited publication and described by Michael West very challenging....
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