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Evidence found and GRADE certainty

577 questions · 585 findings · 77 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 143 · 24.4%GRADE not reported 26 · 4.4%Very low 145 · 24.8%Low 156 · 26.7%Moderate 98 · 16.8%High 17 · 2.9%

Long‐term red cell transfusion vs no transfusion

All‐cause mortality · individuals with sickle cell disease who are at risk of a primary stroke who have had long‐term red cell transfusions to prevent a stroke

It is very uncertain whether long‐term red cell transfusion changes all‐cause mortality compared with no transfusion (very 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