| Title |
Safety and efficacy of enzyme replacement therapy in the nephropathy of Fabry disease
|
|---|---|
| Published in |
Biologics: Targets & Therapy, November 2008
|
| DOI | 10.2147/btt.s3770 |
| Pubmed ID | |
| Authors |
David Warnock, Fernando Fervenza, Roser Torra, G E Linthorst |
| Abstract |
Kidney involvement with progressive loss of kidney function (Fabry nephropathy) is an important complication of Fabry disease, an X-linked lysosomal storage disorder arising from deficiency of alpha-galactosidase activity. Clinical trials have shown that enzyme replacement therapy (ERT) with recombinant human alpha-galactosidase clears globotriaosylceramide from kidney cells, and can stabilize kidney function in patients with mild to moderate Fabry nephropathy. Recent trials show that patients with more advanced Fabry nephropathy and overt proteinuria do not respond as well to ERT alone, but can benefit from anti-proteinuric therapy given in conjunction with ERT. This review focuses on the use of enzyme replacement therapy with agalsidase-alfa and agalsidase-beta in adults with Fabry nephropathy. The current results are reviewed and evaluated. The issues of dosing of enzyme replacement therapy, the use of adjunctive agents to control urinary protein excretion, and the individual factors that affect disease severity are reviewed. |
Mendeley readers
The data shown below were compiled from readership statistics for 41 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Spain | 1 | 2% |
| Brazil | 1 | 2% |
| Unknown | 39 | 95% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Researcher | 9 | 22% |
| Student > Bachelor | 7 | 17% |
| Other | 6 | 15% |
| Student > Master | 5 | 12% |
| Student > Postgraduate | 3 | 7% |
| Other | 7 | 17% |
| Unknown | 4 | 10% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 12 | 29% |
| Agricultural and Biological Sciences | 7 | 17% |
| Biochemistry, Genetics and Molecular Biology | 6 | 15% |
| Chemistry | 3 | 7% |
| Pharmacology, Toxicology and Pharmaceutical Science | 2 | 5% |
| Other | 5 | 12% |
| Unknown | 6 | 15% |
Attention Score in Context
This research output has an Altmetric Attention Score of 3. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 03 September 2010.
All research outputs
#8,595,692
of 25,522,520 outputs
Outputs from Biologics: Targets & Therapy
#110
of 285 outputs
Outputs of similar age
#37,493
of 105,516 outputs
Outputs of similar age from Biologics: Targets & Therapy
#1
of 8 outputs
Altmetric has tracked 25,522,520 research outputs across all sources so far. This one is in the 43rd percentile – i.e., 43% of other outputs scored the same or lower than it.
So far Altmetric has tracked 285 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 6.2. This one is in the 46th percentile – i.e., 46% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 105,516 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 17th percentile – i.e., 17% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 8 others from the same source and published within six weeks on either side of this one. This one has scored higher than all of them