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. 2020 Apr 6;2020(4):CD012389. doi: 10.1002/14651858.CD012389.pub3

Summary of findings 2. Long‐term RBC transfusion compared to standard care for prevention of SCI in people with SCD and abnormal TCD velocities.

Long‐term RBC transfusion compared to standard care for prevention of SCI in people with SCD and abnormal TCD velocities
Patient or population: prevention of SCI in people with SCD and abnormal TCD velocities
 Setting: outpatients
 Intervention: long‐term RBC transfusion
 Comparison: standard care
Outcomes Anticipated absolute effects* (95% CI) Relative effect
 (95% CI) № of participants
 (studies) Quality of the evidence
 (GRADE) Comments
Risk with standard care Risk with long‐term RBC transfusion
Proportion of participants developing new or progressive SCI lesions Trial population RR 0.11
 (0.02 to 0.86) 124
 (1 RCT) ⊕⊕⊝⊝
 LOW 1 2  
159 per 1000 18 per 1000
 (3 to 137)
All‐cause mortality No deaths occurred in either trial arm 130
 (1 RCT) ⊕⊝⊝⊝
 VERY LOW 2 3 4  
SCD ‐ related SAEs ‐ ACS Trial population RR 0.30
 (0.11 to 0.87) 130
 (1 RCT) ⊕⊕⊝⊝
 LOW 2 3  
209 per 1000 63 per 1000
 (23 to 182)
SCD‐related SAEs ‐ pain crisis Trial population RR 0.90
 (0.44 to 1.86) 130
 (1 RCT) ⊕⊝⊝⊝
 VERY LOW 2 3 5  
197 per 1000 177 per 1000
 (87 to 366)
Clinical stroke Trial population RR 0.10
 (0.01 to 0.73) 130
 (1 RCT) ⊕⊕⊝⊝
 LOW 1 2  
164 per 1000 16 per 1000
 (2 to 120)
Cognitive function ‐ not reported  
Quality of life ‐ not reported  
*The risk in the intervention group (and its 95% CI) is based on the assumed risk in the comparison group and the relative effect of the intervention (and its 95% CI).
 
 ACS: acute chest syndrome; CI: confidence interval; RR: risk ratio; RCT: randomised controlled trial; SAEs: serious adverse events; SCD: sickle cell disease; SCI: silent cerebral infarcts; TCD: transcranial doppler
GRADE Working Group grades of evidenceHigh quality: we are very confident that the true effect lies close to that of the estimate of the effect
 Moderate quality: we are moderately confident in the effect estimate: The true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different
 Low quality: our confidence in the effect estimate is limited: The true effect may be substantially different from the estimate of the effect
 Very low quality: we have very little confidence in the effect estimate: The true effect is likely to be substantially different from the estimate of effect

1 We downgraded the quality of evidence by 1 due to risk of bias. The analysis was not intention‐to‐treat

2 We downgraded the quality of evidence by 1 due to indirectness. Only children with HbSS or HbSβº thalassaemia included. If this review was only considering the quality of evidence for children with HbSS the quality of evidence would not have been downgraded for indirectness.

3 We downgraded the quality of evidence by 1 due to risk of bias. Some outcome assessment not blinded and the trial was stopped early

4 We downgraded the quality of evidence by 1 due to imprecision. Rare event. No deaths occurred

5 We downgraded the quality of evidence by 1 due to imprecision. The estimate has wide CIs that include clinically relevant benefit and harm