Findings

What do systematic reviews say?

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Evidence found and GRADE certainty

280 questions · 280 findings · 13 reviews · Cystic Fibrosis and Genetic Disorders

No studies found 127 · 45.4%GRADE not reported 3 · 1.1%Very low 85 · 30.4%Low 46 · 16.4%Moderate 19 · 6.8%High 0 · 0%

Summary of findings ‐ DVD plus information booklet vs information booklet alone

Compliance or adherence to the trial intervention (including satisfaction): use of intervention at least once · adults and children with hemophilia

Summary of findings ‐ DVD plus information booklet showed no clear difference in compliance or adherence to the trial intervention (including… compared with information booklet alone (low certainty).

1 finding from 1 review

Long‐term red cell transfusion vs usual care

Proportion of participants developing new or progressive SCI lesions · prevention of SCI in people with SCD and normal TCD velocities

Long‐term RBC transfusion showed no clear difference in proportion of participants developing new or progressive SCI lesions compared with standard care (low certainty).

1 finding from 1 review

Long‐term red cell transfusion vs usual care

SCD‐related SAEs ‐ pain crisis · prevention of SCI in people with SCD and abnormal TCD velocities

It is very uncertain whether long‐term RBC transfusion changes SCD‐related SAEs ‐ pain crisis compared with standard care (very low certainty).

1 finding from 1 review

Long‐term red cell transfusion vs usual care

SCD ‐ related SAEs ‐ ACS · SCD‐related SAEs and clinical stroke in people with SCD at risk of stroke and no previous long‐term RBC transfusions

Long‐term RBC transfusions may leads to lower SCD ‐ related SAEs ‐ ACS compared with standard care (low certainty).

1 finding from 1 review

Long‐term red cell transfusion vs usual care

SCD‐related SAEs ‐ pain crisis · SCD‐related SAEs and clinical stroke in people with SCD at risk of stroke and no previous long‐term RBC transfusions

Long‐term RBC transfusions may leads to lower SCD‐related SAEs ‐ pain crisis compared with standard care (low certainty).

1 finding from 1 review

Long‐term red cell transfusion vs usual care

Stroke · SCD‐related SAEs and clinical stroke in people with SCD at risk of stroke and no previous long‐term RBC transfusions

Long‐term RBC transfusions probably leads to lower clinical stroke compared with standard care (moderate certainty).

1 finding from 1 review

Transfusions continued vs transfusions halted

Proportion of participants developing new or progressive SCI lesions · prevention of SCI in people with SCD and normalised TCD velocities

Transfusions continued may leads to lower proportion of participants developing new or progressive SCI lesions compared with transfusions halted (low certainty).

1 finding from 1 review