What do systematic reviews say?

62 questions · 62 findings · 17 reviews · Cystic Fibrosis and Genetic Disorders

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No studies found 26 · 41.9%GRADE not reported 4 · 6.5%Very low 15 · 24.2%Low 14 · 22.6%Moderate 2 · 3.2%High 1 · 1.6%

Hypertonic saline 3% to 7% vs Normal saline

All‐cause mortality · adults and children with cystic fibrosis (during acute exacerbations of lung disease)

1 finding from 1 review

Nebulised hypertonic saline for cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 142 participants
Low

Hypertonic saline vs rhDNase with

All‐cause mortality · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Nebulised hypertonic saline for cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Hypertonic saline vs amiloride

All‐cause mortality · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Nebulised hypertonic saline for cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Hypertonic saline vs sodium‐2‐mercaptoethane sulphonate (Mistabron®)

All‐cause mortality · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Nebulised hypertonic saline for cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Hypertonic saline vs mannitol

All‐cause mortality · adults and children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Nebulised hypertonic saline for cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Hypertonic saline 3% to 7% vs Normal saline

All‐cause mortality · adults and children with cystic fibrosis (stable lung disease)

The review found no eligible studies for this question.

1 finding from 1 review

Nebulised hypertonic saline for cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Hypertonic saline 7% vs xylitol

All‐cause mortality · adults with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Nebulised hypertonic saline for cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Hypertonic saline 7% vs hypertonic saline

All‐cause mortality · children with cystic fibrosis

The review found no eligible studies for this question.

1 finding from 1 review

Nebulised hypertonic saline for cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Medication management vs usual care

All‐cause mortality

The review found no eligible studies for this question.

1 finding from 1 review

Interventions for improving adherence to iron chelation therapy in people with sickle cell disease or thalassaemia 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Summary of findings for nasal irrigation with topical steroids plus sinus surgery vs nasal irrigation with topical steroids alone

All‐cause mortality · children and adults diagnosed with cystic fibrosis and chronic rhinosinusitis with nasal polyposis

The review found no eligible studies for this question.

1 finding from 1 review

Surgery for nasal polyposis in cystic fibrosis 2023 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Dietary advice vs no advice

All‐cause mortality · adults with disease‐related malnutrition

Dietary advice showed no clear difference in mortality compared with no advice (low certainty).

1 finding from 1 review

Dietary advice with or without oral nutritional supplements for disease‐related malnutrition in adults 2021 · Cochrane Headline outcomes only
RR 0.87 [0.26, 2.96]
7 studies · 574 participants
Low

Dietary advice vs dietary advice plus oral nutritional supplements

All‐cause mortality · adults with disease‐related malnutrition

Dietary advice showed no clear difference in mortality compared with dietary advice plus oral nutritional supplements (low certainty).

1 finding from 1 review

Dietary advice with or without oral nutritional supplements for disease‐related malnutrition in adults 2021 · Cochrane Headline outcomes only
RR 0.92 [0.47, 1.8]
10 studies · 777 participants
Low

Dietary advice plus supplements vs no advice

All‐cause mortality · adults with disease‐related malnutrition

Dietary advice plus supplements if required showed no clear difference in mortality compared with no advice (low certainty).

1 finding from 1 review

Dietary advice with or without oral nutritional supplements for disease‐related malnutrition in adults 2021 · Cochrane Headline outcomes only
RR 0.82 [0.58, 1.16]
15 studies · 1261 participants
Low

Dietary advice plus supplements vs no dietary advice plus no supplements

All‐cause mortality · adults with disease‐related malnutrition

Dietary advice plus supplements showed no clear difference in mortality compared with no dietary advice plus no supplements (low certainty).

1 finding from 1 review

Dietary advice with or without oral nutritional supplements for disease‐related malnutrition in adults 2021 · Cochrane Headline outcomes only
RR 0.91 [0.55, 1.52]
7 studies · 797 participants
Low

Dietary advice vs oral nutritional supplements

All‐cause mortality · adults with disease‐related malnutrition

Dietary advice showed no clear difference in mortality compared with oral nutritional supplements (low certainty).

1 finding from 1 review

Dietary advice with or without oral nutritional supplements for disease‐related malnutrition in adults 2021 · Cochrane Headline outcomes only
RR 0.66 [0.34, 1.26]
8 studies · 576 participants
Low

Summary of findings ‐ AZLI vs placebo

All‐cause mortality · adults and children with CF

Summary of findings ‐ AZLI showed no clear difference in mortality compared with placebo (moderate certainty).

1 finding from 1 review

Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis 2021 · Cochrane Headline outcomes only
RR 5.41 [0.27, 109.87]
1 study · 100 participants
Moderate

Aggressive vs conservative blood transfusion

All‐cause mortality · people with SCD

1 finding from 1 review

Preoperative blood transfusions for sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
1 study · 230 participants
GRADE not reported

Preoperative blood transfusion vs no transfusion

All‐cause mortality · people with SCD

1 finding from 1 review

Preoperative blood transfusions for sickle cell disease 2020 · Cochrane Headline outcomes only
no estimate extracted
2 studies · 434 participants
GRADE not reported

Summary of findings for combination antimicrobial susceptibility testing vs conventional treatment (separate testing)

All‐cause mortality · adults and children with pulmonary exacerbation due to Pseudomonas aeruginosa

The review found no eligible studies for this question.

1 finding from 1 review

Combination antimicrobial susceptibility testing for acute exacerbations in chronic infection of Pseudomonas… 2020 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found

Intervention not reported

Mortality · children and adults with CF

The review found no eligible studies for this question.

1 finding from 1 review

Vitamin A and beta (β)‐carotene supplementation for cystic fibrosis 2018 · Cochrane Headline outcomes only
no estimate extracted
Studies not reported
No studies found