Cochrane · systematic review · CD013521
Statins for Smith‐Lemli‐Opitz syndrome
Ballout, RA, Livinski, A, Fu, Y-P, et al. · Cochrane Database of Systematic Reviews · 2022 · 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
6
current version
GRADE rated
4
No studies
2
Included studies
NR
No studies 2 · 33.3%Ungraded 0 · 0%Very low 3 · 50%Low 1 · 16.7%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
Statins with cholesterol supplementation versus cholesterol supplementation only
Population: children and adults with Smith‐Lemli‐Opitz syndrome
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Overall survival Statins with cholesterol supplementation vs cholesterol supplementation only | no estimate extracted | NR | No studies |
Change in neurobehavioral manifestations Statins with cholesterol supplementation vs cholesterol supplementation only | no estimate extracted | 1 study · 14 participants | Very low Downgraded: risk of bias, inconsistency |
Statin‐related adverse events Statins with cholesterol supplementation vs cholesterol supplementation only | RR 0.33 [0.01, 7.68] | 1 study · 18 participants | Very low Downgraded: risk of bias, imprecision |
Changes in growth (number of children showing change) Statins with cholesterol supplementation vs cholesterol supplementation only | no estimate extracted | 1 study · 18 participants | Very low Downgraded: risk of bias, inconsistency |
Changes in biochemical markers plasma dehydrocholesterol levels (mM); plasma cholesterol levels (mM); plasma 7DHC levels (mM); plasma CoQ levels (uM); CSF cholesterol levels (ug/mL); CSF dehydrocholesterol levels (ug/mL) Statins with cholesterol supplementation vs cholesterol supplementation only | no estimate extracted | 1 study · 18 participants | Low Downgraded: risk of bias, inconsistency |
QoL Statins with cholesterol supplementation vs cholesterol supplementation only | no estimate extracted | NR | No studies |