| Title
of the article |
Renal histology before and after effective enzyme replacement therapy in a patient with classical Fabry's disease. |
| Authors |
Hirashio S, Taguchi T, Naito T, Maki K, Ogata S, Taniyama K, Taniguchi Y, Yorioka N |
| Journal
|
Clin Nephrol. 2009 May;71(5):550-6. |
| Subject
Consultant |
V. Nickeleit |
| Score
(1 to 3 stars) |
** |
| Comments |
Interesting case study. The presented data suggest that in the patient under discussion with Fabry's disease enzyme therapy resulted in the improvement of symptoms but not in the improvement of histologic lesions. Additional immuohistochemical analyses demonstrated under enzyme therapy a reduced intracellular load of globotriaosylceramide in renal cells- a finding that may explain some of the therapeutic effects seen with enzyme treatment in Fabry's disease. |
