Findings

What do systematic reviews say?

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Evidence found and GRADE certainty

697 questions · 708 findings · 47 reviews · Cystic Fibrosis and Genetic Disorders

No studies found 263 · 37.1%GRADE not reported 12 · 1.7%Very low 146 · 20.6%Low 168 · 23.7%Moderate 98 · 13.8%High 21 · 3%

N‐acetylcysteine (1200 mg) vs placebo

QoL of participants living with SCD and their caregivers · sickle cell disease

It is very uncertain whether N‐acetylcysteine (1200 mg) changes qoL of participants living with SCD and their caregivers (measured… compared with placebo (very low certainty).

1 finding from 1 review

Vitamin C 1400 mg plus vitamin E 800 mg vs placebo

Severity of pain (use of opioid analgesics) · adults with sickle cell disease (adults with homozygous SCA or sickle beta 0 thalassaemia SCD)

It is very uncertain whether vitamin C 1400 mg plus vitamin E 800 mg changes severity of pain (use of opioid analgesics) compared with placebo (very low certainty).

1 finding from 1 review

L‐arginine vs placebo

Pain 0 to 100 visual analogue scale and 10‐point Likert scale (lower score = less pain) · sickle cell disease

L‐arginine showed no clear difference in severity of pain (assessed using a 10‐cm visual analogue scale from… compared with placebo (low certainty).

1 finding from 1 review

L‐arginine vs placebo

Frequency of hospitalization (number of days in hospital) · sickle cell disease

It is very uncertain whether L‐arginine changes frequency of hospitalization (number of days in hospital) compared with placebo (very low certainty).

1 finding from 1 review