Cochrane · systematic review · CD012389

Interventions for preventing silent cerebral infarcts in people with sickle cell disease

Estcourt, LJ, Kimber, C, Hopewell, S, et al. · Cochrane Database of Systematic Reviews · 2020 · Cochrane Library

Cystic Fibrosis and Genetic Disorders2 versionsAMSTAR 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
38
current version
GRADE rated
30
No studies
8
Included studies
NR
No studies 8 · 21.1%Ungraded 0 · 0%Very low 15 · 39.5%Low 14 · 36.8%Moderate 1 · 2.6%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

Current version only

Long‐term RBC transfusion compared to standard care for prevention of SCI in people with SCD and normal TCD velocities

Population: prevention of SCI in people with SCD and normal TCD velocities
CD012389.PUB3 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Proportion of participants developing new or progressive SCI lesions
Long‐term RBC transfusion vs standard care
RR 0.7 [0.23, 2.13]1 study · 196 participantsLow
Downgraded: indirectness
All‐cause mortality
Long‐term RBC transfusion vs standard care
no estimate extracted1 study · 196 participantsLow
Downgraded: indirectness
SCD ‐ related SAEs ‐ ACS
Long‐term RBC transfusion vs standard care
RR 0.2 [0.08, 0.51]1 study · 196 participantsLow
Downgraded: indirectness
SCD‐related SAEs ‐ pain crisis
Long‐term RBC transfusion vs standard care
RR 0.56 [0.4, 0.78]1 study · 196 participantsLow
Downgraded: indirectness
Clinical stroke
Long‐term RBC transfusion vs standard care
RR 0.14 [0.02, 1.12]1 study · 196 participantsLow
Downgraded: indirectness
Cognitive function
Long‐term RBC transfusion vs standard care
no estimate extracted1 study · 196 participantsLow
Downgraded: indirectness
Quality of life
Long‐term RBC transfusion vs standard care
no estimate extracted1 study · 196 participantsLow
Downgraded: indirectness

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

Population: prevention of SCI in people with SCD and abnormal TCD velocities
CD012389.PUB3 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Proportion of participants developing new or progressive SCI lesions
Long‐term RBC transfusion vs standard care
RR 0.11 [0.02, 0.86]1 study · 124 participantsLow
Downgraded: risk of bias
All‐cause mortality
Long‐term RBC transfusion vs standard care
no estimate extracted1 study · 130 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
SCD ‐ related SAEs ‐ ACS
Long‐term RBC transfusion vs standard care
RR 0.3 [0.11, 0.87]1 study · 130 participantsLow
Downgraded: risk of bias, imprecision, indirectness
SCD‐related SAEs ‐ pain crisis
Long‐term RBC transfusion vs standard care
RR 0.9 [0.44, 1.86]1 study · 130 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Clinical stroke
Long‐term RBC transfusion vs standard care
RR 0.1 [0.01, 0.73]1 study · 130 participantsLow
Downgraded: risk of bias
Cognitive function ‐ not reported
Long‐term RBC transfusion vs standard care
no estimate extractedNRNo studies
Quality of life ‐ not reported
Long‐term RBC transfusion vs standard care
no estimate extractedNRNo studies

Long‐term RBC transfusions compared to standard care for SCD‐related SAEs and clinical stroke in people with SCD at risk of stroke and no previous long‐term RBC transfusions

Population: SCD‐related SAEs and clinical stroke in people with SCD at risk of stroke and no previous long‐term RBC transfusions
CD012389.PUB3 (current) · 3 questions
OutcomeEffectEvidenceStatus / GRADE
SCD ‐ related SAEs ‐ ACS
Long‐term RBC transfusions vs standard care
RR 0.24 [0.12, 0.49]2 studies · 326 participantsLow
Downgraded: risk of bias, indirectness
SCD‐related SAEs ‐ pain crisis
Long‐term RBC transfusions vs standard care
RR 0.63 [0.42, 0.95]2 studies · 326 participantsLow
Downgraded: risk of bias, indirectness
Clinical stroke
Long‐term RBC transfusions vs standard care
RR 0.12 [0.03, 0.49]2 studies · 326 participantsModerate
Downgraded: risk of bias, indirectness

Transfusions continued compared to transfusions halted for prevention of SCI in people with SCD and normalised TCD velocities

Population: prevention of SCI in people with SCD and normalised TCD velocities
CD012389.PUB3 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Proportion of participants developing new or progressive SCI lesions
Transfusions continued vs transfusions halted
RR 0.29 [0.09, 0.97]1 study · 77 participantsLow
Downgraded: risk of bias
All‐cause mortality
Transfusions continued vs transfusions halted
Peto OR 8 [0.16, 404.12]1 study · 79 participantsVery low
Downgraded: risk of bias
Clinical stroke
Transfusions continued vs transfusions halted
RR 0.22 [0.01, 4.35]1 study · 79 participantsVery low
Downgraded: risk of bias
SCD‐related SAEs ‐ ACS
Transfusions continued vs transfusions halted
no estimate extracted1 study · 79 participantsVery low
Downgraded: risk of bias
SCD‐related SAEs ‐ pain crisis
Transfusions continued vs transfusions halted
no estimate extracted1 study · 79 participantsVery low
Downgraded: risk of bias
Cognitive function ‐ not reported
Transfusions continued vs transfusions halted
no estimate extractedNRNo studies
Quality of life ‐ not reported
Transfusions continued vs transfusions halted
no estimate extractedNRNo studies

Hydroxyurea compared to RBC transfusions for prevention of SCI in people with SCD who have not had a stroke (primary prevention)

Population: prevention of SCI in people with SCD who have not had a stroke (primary prevention)
CD012389.PUB3 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Proportion of participants developing new or progressive SCI lesions
Hydroxyurea vs RBC transfusions
no estimate extracted1 study · 121 participantsVery low
Downgraded: indirectness
All‐cause mortality
Hydroxyurea vs RBC transfusions
no estimate extracted1 study · 1 participantsVery low
Downgraded: indirectness
SCD‐related SAEs ‐ ACS
Hydroxyurea vs RBC transfusions
RR 2.03 [0.39, 10.69]1 study · 121 participantsVery low
Downgraded: indirectness
SCD‐related SAEs ‐ pain crisis
Hydroxyurea vs RBC transfusions
RR 5.08 [0.61, 42.23]1 study · 121 participantsVery low
Downgraded: indirectness
Clinical stroke
Hydroxyurea vs RBC transfusions
no estimate extracted1 study · 121 participantsVery low
Downgraded: indirectness
Cognitive function ‐ not reported
Hydroxyurea vs RBC transfusions
no estimate extractedNRNo studies
Quality of life ‐ not reported
Hydroxyurea vs RBC transfusions
no estimate extractedNRNo studies

Hydroxyurea compared to RBC transfusion for prevention of SCI in people with SCD who had a stroke (secondary prevention)

Population: prevention of SCI in people with SCD who had a stroke (secondary prevention)
CD012389.PUB3 (current) · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Proportion of participants developing new or progressive SCI lesions
Hydroxyurea vs RBC transfusion
Peto OR 7.28 [0.14, 366.91]1 study · 133 participantsVery low
Downgraded: risk of bias
All‐cause mortality
Hydroxyurea vs RBC transfusion
OR 1.02 [0.06, 16.41]1 study · 133 participantsVery low
Downgraded: risk of bias
SCD‐related SAEs ‐ ACS
Hydroxyurea vs RBC transfusion
RR 0.33 [0.04, 3.08]1 study · 133 participantsVery low
Downgraded: risk of bias
SCD‐related SAEs ‐ pain crisis
Hydroxyurea vs RBC transfusion
RR 3.15 [1.23, 8.11]1 study · 133 participantsLow
Downgraded: risk of bias
Clinical stroke
Hydroxyurea vs RBC transfusion
RR 14.78 [0.86, 253.66]1 study · 133 participantsVery low
Downgraded: risk of bias
Cognitive function ‐ not reported
Hydroxyurea vs RBC transfusion
no estimate extractedNRNo studies
Quality of life ‐ not reported
Hydroxyurea vs RBC transfusion
no estimate extractedNRNo studies

Long‐term RBC transfusion compared to standard care for prevention of SCI in people with SCD and normal TCD velocities

Population: prevention of SCI in people with SCD and normal TCD velocities
CD012389.PUB2 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Proportion of participants developing new or progressive SCI lesions
Long‐term RBC transfusion vs standard care
RR 0.7 [0.23, 2.13]1 study · 196 participantsLow
Downgraded: indirectness
All‐cause mortality
Long‐term RBC transfusion vs standard care
no estimate extracted1 study · 196 participantsLow
Downgraded: indirectness
SCD ‐ related SAEs ‐ ACS
Long‐term RBC transfusion vs standard care
RR 0.2 [0.08, 0.51]1 study · 196 participantsLow
Downgraded: indirectness
SCD‐related SAEs ‐ pain crisis
Long‐term RBC transfusion vs standard care
RR 0.56 [0.4, 0.78]1 study · 196 participantsLow
Downgraded: indirectness
Clinical stroke
Long‐term RBC transfusion vs standard care
RR 0.14 [0.02, 1.12]1 study · 196 participantsLow
Downgraded: indirectness
Cognitive function
Long‐term RBC transfusion vs standard care
no estimate extracted1 study · 196 participantsLow
Downgraded: indirectness
Quality of life
Long‐term RBC transfusion vs standard care
no estimate extracted1 study · 196 participantsLow
Downgraded: indirectness

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

Population: prevention of SCI in people with SCD and abnormal TCD velocities
CD012389.PUB2 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Proportion of participants developing new or progressive SCI lesions
Long‐term RBC transfusion vs standard care
RR 0.11 [0.02, 0.86]1 study · 124 participantsLow
Downgraded: risk of bias
All‐cause mortality
Long‐term RBC transfusion vs standard care
no estimate extracted1 study · 130 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
SCD ‐ related SAEs ‐ ACS
Long‐term RBC transfusion vs standard care
RR 0.3 [0.11, 0.87]1 study · 130 participantsLow
Downgraded: risk of bias, imprecision, indirectness
SCD‐related SAEs ‐ pain crisis
Long‐term RBC transfusion vs standard care
RR 0.9 [0.44, 1.86]1 study · 130 participantsVery low
Downgraded: risk of bias, imprecision, indirectness
Clinical stroke
Long‐term RBC transfusion vs standard care
RR 0.1 [0.01, 0.73]1 study · 130 participantsLow
Downgraded: risk of bias
Cognitive function ‐ not reported
Long‐term RBC transfusion vs standard care
no estimate extractedNRNo studies
Quality of life ‐ not reported
Long‐term RBC transfusion vs standard care
no estimate extractedNRNo studies

Long‐term RBC transfusions compared to standard care for SCD‐related SAEs and clinical stroke in people with SCD at risk of stroke and no previous long‐term RBC transfusions

Population: SCD‐related SAEs and clinical stroke in people with SCD at risk of stroke and no previous long‐term RBC transfusions
CD012389.PUB2 · 3 questions
OutcomeEffectEvidenceStatus / GRADE
SCD ‐ related SAEs ‐ ACS
Long‐term RBC transfusions vs standard care
RR 0.24 [0.12, 0.49]2 studies · 326 participantsLow
Downgraded: risk of bias, indirectness
SCD‐related SAEs ‐ pain crisis
Long‐term RBC transfusions vs standard care
RR 0.63 [0.42, 0.95]2 studies · 326 participantsLow
Downgraded: risk of bias, indirectness
Clinical stroke
Long‐term RBC transfusions vs standard care
RR 0.12 [0.03, 0.49]2 studies · 326 participantsModerate
Downgraded: risk of bias, indirectness

Transfusions continued compared to transfusions halted for prevention of SCI in people with SCD and normalised TCD velocities

Population: prevention of SCI in people with SCD and normalised TCD velocities
CD012389.PUB2 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Proportion of participants developing new or progressive SCI lesions
Transfusions continued vs transfusions halted
RR 0.29 [0.09, 0.97]1 study · 77 participantsLow
Downgraded: risk of bias
All‐cause mortality
Transfusions continued vs transfusions halted
Peto OR 8 [0.16, 404.12]1 study · 79 participantsVery low
Downgraded: risk of bias
Clinical stroke
Transfusions continued vs transfusions halted
RR 0.22 [0.01, 4.35]1 study · 79 participantsVery low
Downgraded: risk of bias
SCD‐related SAEs ‐ ACS
Transfusions continued vs transfusions halted
no estimate extracted1 study · 79 participantsVery low
Downgraded: risk of bias
SCD‐related SAEs ‐ pain crisis
Transfusions continued vs transfusions halted
no estimate extracted1 study · 79 participantsVery low
Downgraded: risk of bias
Cognitive function ‐ not reported
Transfusions continued vs transfusions halted
no estimate extractedNRNo studies
Quality of life ‐ not reported
Transfusions continued vs transfusions halted
no estimate extractedNRNo studies

Hydroxyurea compared to RBC transfusions for prevention of SCI in people with SCD who have not had a stroke (primary prevention)

Population: prevention of SCI in people with SCD who have not had a stroke (primary prevention)
CD012389.PUB2 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Proportion of participants developing new or progressive SCI lesions
Hydroxyurea vs RBC transfusions
no estimate extracted1 study · 121 participantsVery low
Downgraded: indirectness
All‐cause mortality
Hydroxyurea vs RBC transfusions
no estimate extracted1 study · 1 participantsVery low
Downgraded: indirectness
SCD‐related SAEs ‐ ACS
Hydroxyurea vs RBC transfusions
RR 2.03 [0.39, 10.69]1 study · 121 participantsVery low
Downgraded: indirectness
SCD‐related SAEs ‐ Pain crisis
Hydroxyurea vs RBC transfusions
RR 5.08 [0.61, 42.23]1 study · 121 participantsVery low
Downgraded: indirectness
Clinical stroke
Hydroxyurea vs RBC transfusions
no estimate extracted1 study · 121 participantsVery low
Downgraded: indirectness
Cognitive function ‐ not reported
Hydroxyurea vs RBC transfusions
no estimate extractedNRNo studies
Quality of life ‐ not reported
Hydroxyurea vs RBC transfusions
no estimate extractedNRNo studies

Hydroxyurea compared to RBC transfusion for prevention of SCI in people with SCD who had a stroke (secondary prevention)

Population: prevention of SCI in people with SCD who had a stroke (secondary prevention)
CD012389.PUB2 · 7 questions
OutcomeEffectEvidenceStatus / GRADE
Proportion of participants developing new or progressive SCI lesions
Hydroxyurea vs RBC transfusion
Peto OR 7.28 [0.14, 366.91]1 study · 133 participantsVery low
Downgraded: risk of bias
All‐cause mortality
Hydroxyurea vs RBC transfusion
OR 1.02 [0.06, 16.41]1 study · 133 participantsVery low
Downgraded: risk of bias
SCD‐related SAEs ‐ ACS
Hydroxyurea vs RBC transfusion
RR 0.33 [0.04, 3.08]1 study · 133 participantsVery low
Downgraded: risk of bias
SCD‐related SAEs ‐ Pain crisis
Hydroxyurea vs RBC transfusion
RR 3.15 [1.23, 8.11]1 study · 133 participantsLow
Downgraded: risk of bias
Clinical stroke
Hydroxyurea vs RBC transfusion
RR 14.78 [0.86, 253.66]1 study · 133 participantsVery low
Downgraded: risk of bias
Cognitive function ‐ not reported
Hydroxyurea vs RBC transfusion
no estimate extractedNRNo studies
Quality of life ‐ not reported
Hydroxyurea vs RBC transfusion
no estimate extractedNRNo studies