| pubmed-article:17409308 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:17409308 | lifeskim:mentions | umls-concept:C0030705 | lld:lifeskim |
| pubmed-article:17409308 | lifeskim:mentions | umls-concept:C0232804 | lld:lifeskim |
| pubmed-article:17409308 | lifeskim:mentions | umls-concept:C0332174 | lld:lifeskim |
| pubmed-article:17409308 | lifeskim:mentions | umls-concept:C0002986 | lld:lifeskim |
| pubmed-article:17409308 | lifeskim:mentions | umls-concept:C0598391 | lld:lifeskim |
| pubmed-article:17409308 | lifeskim:mentions | umls-concept:C0439834 | lld:lifeskim |
| pubmed-article:17409308 | lifeskim:mentions | umls-concept:C0585332 | lld:lifeskim |
| pubmed-article:17409308 | lifeskim:mentions | umls-concept:C0443252 | lld:lifeskim |
| pubmed-article:17409308 | pubmed:issue | 5 | lld:pubmed |
| pubmed-article:17409308 | pubmed:dateCreated | 2007-4-26 | lld:pubmed |
| pubmed-article:17409308 | pubmed:abstractText | This study was performed to determine whether adult male patients with Fabry disease who demonstrate a continuing decline in renal function despite 2 to 4 yr of conventionally dosed agalsidase alfa therapy (0.2 mg/kg every other week [EOW]) show an improved slope of decline with weekly administration using the same dosage. Eleven (27%) of 41 adult male patients with Fabry disease who participated in long-term agalsidase alfa clinical trials and who had demonstrated a slope of decline in estimated GFR (eGFR) of > or =5 ml/min per 1.73 m(2)/yr while receiving long-term treatment with agalsidase alfa at the currently recommended dosage of 0.2 mg/kg, infused EOW, were enrolled in this open-label, prospective study. Patients were switched from EOW to weekly infusions and followed for an additional 24 mo. Before switching to weekly dosing, eGFR was 53.7 +/- 6.3 ml/min per 1.73 m(2) (mean +/- SEM), and mean rate of change in eGFR was -8.0 +/- 0.8 ml/min per 1.73 m(2)/yr. During the 24-mo follow-up period after switching to weekly dosing, the mean rate of change in eGFR was observed to slow to -3.3 +/- 1.4 ml/min/1.73 m(2)/yr (P = 0.01 versus EOW). After switching to weekly dosing, three patients demonstrated an improvement in eGFR and six patients demonstrated a slowing in the rate of eGFR decline; only two patients failed to improve their eGFR slope. A multiple regression model confirmed that the weekly infusion regimen was the strongest explanatory variable for the change in eGFR (P = 0.0008), with a weaker contribution from the concomitant use of angiotensin converting enzyme inhibitors/angiotensin receptor blockers (P = 0.02). These results suggest that weekly infusions of agalsidase alfa at a dosage of 0.2 mg/kg may be beneficial in the subgroup of patients who have Fabry disease and whose kidney function continues to decline after 2 to 4 yr or more of standard EOW dosing. | lld:pubmed |
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| pubmed-article:17409308 | pubmed:language | eng | lld:pubmed |
| pubmed-article:17409308 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:17409308 | pubmed:citationSubset | IM | lld:pubmed |
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| pubmed-article:17409308 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:17409308 | pubmed:month | May | lld:pubmed |
| pubmed-article:17409308 | pubmed:issn | 1046-6673 | lld:pubmed |
| pubmed-article:17409308 | pubmed:author | pubmed-author:SchiffmannRap... | lld:pubmed |
| pubmed-article:17409308 | pubmed:author | pubmed-author:BradyRoscoe... | lld:pubmed |
| pubmed-article:17409308 | pubmed:author | pubmed-author:RiesMarkusM | lld:pubmed |
| pubmed-article:17409308 | pubmed:author | pubmed-author:AskariHasanH | lld:pubmed |
| pubmed-article:17409308 | pubmed:author | pubmed-author:RobinsonCheva... | lld:pubmed |
| pubmed-article:17409308 | pubmed:author | pubmed-author:TimmonsMargar... | lld:pubmed |
| pubmed-article:17409308 | pubmed:author | pubmed-author:BenkoWilliamW | lld:pubmed |
| pubmed-article:17409308 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:17409308 | pubmed:volume | 18 | lld:pubmed |
| pubmed-article:17409308 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:17409308 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:17409308 | pubmed:pagination | 1576-83 | lld:pubmed |
| pubmed-article:17409308 | pubmed:dateRevised | 2009-11-18 | lld:pubmed |
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| pubmed-article:17409308 | pubmed:meshHeading | pubmed-meshheading:17409308... | lld:pubmed |
| pubmed-article:17409308 | pubmed:year | 2007 | lld:pubmed |
| pubmed-article:17409308 | pubmed:articleTitle | Weekly enzyme replacement therapy may slow decline of renal function in patients with Fabry disease who are on long-term biweekly dosing. | lld:pubmed |
| pubmed-article:17409308 | pubmed:affiliation | Developmental and Metabolic Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10, Room 3D03, 9000 Rockville Pike, Bethesda, MD 20892-1260, USA. rs4e@nih.gov | lld:pubmed |
| pubmed-article:17409308 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:17409308 | pubmed:publicationType | Clinical Trial | lld:pubmed |
| pubmed-article:17409308 | pubmed:publicationType | Research Support, N.I.H., Intramural | lld:pubmed |
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