Findings

What do systematic reviews say?

2,261 questions · 2,280 findings · 114 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 856 · 37.5%GRADE not reported 58 · 2.5%Very low 550 · 24.1%Low 502 · 22%Moderate 271 · 11.9%High 43 · 1.9%

Intervention not reported

Change in height velocity SDS (difference between baseline and final visit at one year) · people with thalassaemia (any age)

1 finding from 1 review

Growth hormone therapy for people with thalassaemia 2020 · Cochrane Headline outcomes only
no estimate extracted
1 study · 20 participants
Moderate

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

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
RR 0.7 [0.23, 2.13]
1 study · 196 participants
Low

Long‐term red cell transfusion vs usual care

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

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

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
RR 0.3 [0.11, 0.87]
1 study · 130 participants
Low

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

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
RR 0.9 [0.44, 1.86]
1 study · 130 participants
Very low

Long‐term red cell transfusion vs usual care

Stroke · prevention of SCI in people with SCD and abnormal TCD velocities

Long‐term RBC transfusion may leads to lower clinical stroke compared with standard care (low certainty).

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
RR 0.1 [0.01, 0.73]
1 study · 130 participants
Low

Long‐term red cell transfusion vs usual care

Cognitive function ‐ not reported · prevention of SCI in people with SCD and abnormal TCD velocities

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Long‐term red cell transfusion vs usual care

Quality of life ‐ not reported · prevention of SCI in people with SCD and abnormal TCD velocities

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

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

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
RR 0.24 [0.12, 0.49]
2 studies · 326 participants
Low

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

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
RR 0.63 [0.42, 0.95]
2 studies · 326 participants
Low

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

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
RR 0.12 [0.03, 0.49]
2 studies · 326 participants
Moderate

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

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
RR 0.29 [0.09, 0.97]
1 study · 77 participants
Low

Transfusions continued vs transfusions halted

All‐cause mortality · prevention of SCI in people with SCD and normalised TCD velocities

It is very uncertain whether transfusions continued changes all‐cause mortality compared with transfusions halted (very low certainty).

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
Peto OR 8 [0.16, 404.12]
1 study · 79 participants
Very low

Long‐term red cell transfusion vs usual care

All‐cause mortality · prevention of SCI in people with SCD and normal TCD velocities

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
1 study · 196 participants
Low

Transfusions continued vs transfusions halted

Stroke · prevention of SCI in people with SCD and normalised TCD velocities

It is very uncertain whether transfusions continued changes clinical stroke compared with transfusions halted (very low certainty).

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
RR 0.22 [0.01, 4.35]
1 study · 79 participants
Very low

Transfusions continued vs transfusions halted

SCD ‐ related SAEs ‐ ACS · prevention of SCI in people with SCD and normalised TCD velocities

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
1 study · 79 participants
Very low

Transfusions continued vs transfusions halted

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

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
1 study · 79 participants
Very low

Transfusions continued vs transfusions halted

Cognitive function ‐ not reported · prevention of SCI in people with SCD and normalised TCD velocities

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Transfusions continued vs transfusions halted

Quality of life ‐ not reported · prevention of SCI in people with SCD and normalised TCD velocities

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Hydroxyurea vs RBC transfusion

Proportion of participants developing new or progressive SCI lesions · prevention of SCI in people with SCD who have not had a stroke (primary prevention)

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
1 study · 121 participants
Very low

Hydroxyurea vs RBC transfusion

All‐cause mortality · prevention of SCI in people with SCD who have not had a stroke (primary prevention)

1 finding from 1 review

Interventions for preventing silent cerebral infarcts in people with sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
1 study · 1 participants
Very low