Table 2.
Search strategy
| Medline (Ovid SP) | PubMed | Embase | |
|---|---|---|---|
| Search by disease (limit to human and English) | 2800 | 980 | 3766 |
| Limit to therapy | 557 | 195 | 1106 |
| Limit to clinical trial | 72 | 62 | 273 |
| Exclusions of case reports, studies not on ERT effect, not on FD, and general reviews on FD | 30 | 20 | 231 |
| Final included | 41 |
Notes: We formulated a comprehensive search strategy in an attempt to identify all relevant studies published in English language. An Ovid search was conducted using the Ovid databases: MEDLINE® 1950 to present with daily update and Embase (1980 to date). The following search terms were used: Fabry* disease, enzyme replacement, agalsidase, Replagal, and Fabrazyme. These terms were entered as MESH subject heading terms as well. ADJ2 = words closely adjacent within two words of each other; $ = truncation, any number of characters; and Boolean operator Or/And were used to combine search terms. Filters for randomized-controlled trials were used. In addition, reference lists of relevant published articles were searched to make the search as complete as possible.
Abbreviations: FD, Fabry disease; MESH, Medical Subject Headings; ERT, enzyme replacement therapy.