Abstract | BACKGROUND: METHODS: Four male patients with hemizygous classical Fabry disease received recombinant alpha-galactsidase A biweekly, and urinary sediments were assessed at 3-month intervals. RESULTS: The morphologic and immunohistochemical changes in urinary sediment at 6 and 18 months suggested that accumulations of glycosphingolipids in renal tissues were cleared by enzyme replacement. CONCLUSION: Examination of urinary sediments could serve as noninvasive monitoring of the effect of therapy in patients with Fabry disease.
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Authors | Kouichi Utsumi, Futoshi Mitsuhashi, Koichi Asahi, Makoto Sakurazawa, Kazumasa Arii, Yuichi Komaba, Toshiya Katsumata, Ken-Ichiro Katsura, Ryoichi Kase, Yasuo Katayama |
Journal | Clinica chimica acta; international journal of clinical chemistry
(Clin Chim Acta)
Vol. 360
Issue 1-2
Pg. 103-7
(Oct 2005)
ISSN: 0009-8981 [Print] Netherlands |
PMID | 16112661
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Glycosphingolipids
- alpha-Galactosidase
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Topics |
- Adult
- Drug Monitoring
(methods)
- Enzyme Therapy
- Fabry Disease
(drug therapy, urine)
- Glycosphingolipids
(urine)
- Humans
- Immunohistochemistry
- Male
- Urine
(chemistry, cytology)
- alpha-Galactosidase
(administration & dosage)
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