| Title | Renal histology before and after effective enzyme replacement therapy in a patient with classical Fabry's disease. | | Author(s) | Hirashio S, Taguchi T, Naito T, Maki K, Ogata S, Taniyama K, Taniguchi Y, Yorioka N | | Institution | 1Department of Nephrology, Hiroshima University Hospital, 2Department of Nephrology, National Hospital Organization Kure Medical Center and Chugoku Cancer Center, 3Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, 4Department of Internal Medicine, Sanyo Hospital, 5Institute for Clinical Research, National Hospital Organization Kure Medical Center and Chugoku Cancer Center, 6Division of Clinical Pharmacotherapeutics, Department of Pharmaceutical Science, Hiroshima International University, 7Department of Advanced Nephrology, and Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan. | | Source | Clin Nephrol 2009 May; Volume 71(May):550-556. | | Abstract | A 38-year-old man underwent renal biopsy because of proteinuria. It revealed swelling and vacuolation of glomerular epithelial cells, as well as myelin-like structures characteristic of Fabry's disease. Detection of decreased plasma activity of alpha-galactosidase A confirmed the diagnosis. Enzyme replacement therapy was provided with recombinant agalsidase-beta, resulting in improvement of his symptoms. When renal biopsy was repeated, specific staining for globotriaosylceramide showed that renal deposits were decreased by enzyme therapy. | | Language | ENG | | Pub Type(s) | JOURNAL ARTICLE
| | PubMed ID | 19473616 |
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