Cochrane · systematic review · CD006663
Enzyme replacement therapy for Anderson‐Fabry disease
El Dib, R, Gomaa, H, Carvalho, RP, et al. · Cochrane Database of Systematic Reviews · 2016 · Cochrane Library
Cystic Fibrosis and Genetic DisordersAMSTAR 2: not assessed
SoF tableOnly the outcomes this review put in its Summary of Findings table are listed (the Cochrane Handbook caps them at about seven per comparison). Its other analyses are not shown here.
Questions
18
current version
GRADE rated
10
No studies
8
Included studies
NR
No studies 8 · 44.4%Ungraded 0 · 0%Very low 9 · 50%Low 1 · 5.6%Moderate 0 · 0%High 0 · 0%
Ungraded means an effect was reported without a GRADE rating; it is not a gap. No studies means the review found nothing to answer the question. The six categories are a display order, not a score.
Questions and results
Comparison
Population: Anderson‐Fabry disease
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Changes in globotriaosylceramide (Gb3) concentration in plasma | no estimate extracted | 2 studies · 39 participants | Very low Downgraded: risk of bias, imprecision, inconsistency |
Death | no estimate extracted | NR | No studies |
Pain The Brief Pain Inventory severity | no estimate extracted | 1 study · 26 participants | Low Downgraded: risk of bias, imprecision, inconsistency |
Renal function by creatinine clearance | no estimate extracted | 1 study · 24 participants | Very low Downgraded: risk of bias, imprecision, inconsistency |
Symptoms and complications of disease:cardiac events | no estimate extracted | NR | No studies |
Symptoms and complications of disease: cerebrovascular events | no estimate extracted | NR | No studies |
Comparison
Population: Anderson‐Fabry disease
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Changes in globotriaosylceramide (Gb3) concentration in plasma | no estimate extracted | 1 study · 58 participants | Very low Downgraded: risk of bias, imprecision |
Death | no estimate extracted | 1 study · 82 participants | Very low Downgraded: risk of bias, imprecision |
Pain | no estimate extracted | NR | No studies |
Renal function events (ITT population) | no estimate extracted | 1 study · 82 participants | Very low Downgraded: risk of bias, imprecision |
Symptoms and complications of disease: cardiac events | no estimate extracted | 1 study · 82 participants | Very low Downgraded: risk of bias, imprecision |
Symptoms and complications of disease: cerebrovascular events (ITT population) | no estimate extracted | 1 study · 82 participants | Very low Downgraded: risk of bias, imprecision |
Comparison
Population: Anderson‐Fabry disease
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Changes in globotriaosylceramide (Gb3) concentration in plasma and tissue | no estimate extracted | NR | No studies |
Death | no estimate extracted | 1 study · 36 participants | Very low Downgraded: risk of bias, imprecision |
Pain Acroparaesthesia and Fabry crises | no estimate extracted | NR | No studies |
Renal function | no estimate extracted | NR | No studies |
Symptoms and complications of disease: cardiac events | no estimate extracted | 1 study · 29 participants | Very low Downgraded: risk of bias, imprecision |
Symptoms and complications of disease: cerebrovascular events | no estimate extracted | NR | No studies |