Cystic Fibrosis

CF: Assessment: Nutrition Parameters and Hard Outcomes (2017-19)

• Assessment
  In pediatric participants with CF, what is the longitudinal relationship (at least 3 months) between weight and growth parameters and FEV1?

  • Conclusion

    In pediatric participants with cystic fibrosis (CF), regaining birthweight z-score by age two and maintaining BMI and height z-score throughout childhood was associated with the highest FEV1% predicted later in life compared to those who did not regain and maintain z-scores in studies referencing CDC growth charts. Participants who maintained a weight, length, weight-for-length and BMI >50%ile from infancy and early childhood had better FEV1% predicted values, although there was no added improvement for those who maintained growth parameters >85 percentile compared with >50 percentile In general, normal growth parameters during childhood were associated with increased FEV1% predicted in long-term follow-up studies (4-16 yrs).

  • Grade: I
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In pediatric participants with CF, what is the longitudinal relationship (at least 3 months) between weight and growth parameters and FEV1?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • Darrah R, Nelson R, Damato E, Decker M, Matthews A, Hodges C. Growth Deficiency in Cystic Fibrosis Is Observable at Birth and Predictive of Early Pulmonary Function. Biological Research for Nursing 2016; 18:498-504
      • Emerson J, Rosenfeld M, McNamara S, Ramsey B, Gibson R. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatric Pulmonology 2002; 34:91-100
      • Goss C, Sykes J, Stanojevic S, Marshall B, Petren K, Ostrenga J, Fink A, Elbert A, Quon B, Stephenson A. Comparison of nutrition and lung function outcomes in [atients with cystic fibrosis living in Canada and the United States. American Journal of Respiratory and Critical Care Medicine 2018; 197:768-775
      • Keller B, Aebischer C, Kraemer R, Schöni M. Growth in prepubertal children with cystic fibrosis, homozygous for the Delta F508 mutation. Journal of Cystic Fibrosis 2003; 2:76-83
      • Konstan M, Morgan W, Butler S, Pasta D, Craib M, Silva S, Stokes D, Wohl M, Wagener J, Regelmann W, Johnson C. Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis. Journal of Pediatrics 2007; 151:134-9
      • Lai H, Shoff S, Farrell P. Recovery of birth weight z score within 2 years of diagnosis is positively associated with pulmonary status at 6 years of age in children with cystic fibrosis. Pediatrics 2009; 123:714-22
      • Machogu E, Cao Y, Miller T, Simpson P, Levy H, Quintero D, Goday P. Comparison of WHO and CDC growth charts in predicting pulmonary outcomes in cystic fibrosis. Journal of Pediatric Gastroenterology and Nutrition 2015; 60:378-83
      • McPhail G, Acton J, Fenchel M, Amin R, Seid M. Improvements in lung function outcomes in children with cystic fibrosis are associated with better nutrition, fewer chronic pseudomonas aeruginosa infections, and dornase alfa use. The Journal of Pediatrics 2008; 153:752-7
      • Sanders D, Li Z, Laxova A, Rock M, Levy H, Collins J, Ferec C, Farrell P. Risk factors for the progression of cystic fibrosis lung disease throughout childhood. Annals of the American Thoracic Society 2014; 11:63-72
      • Sanders D, Emerson J, Ren C, Schechter M, Gibson R, Morgan W, Rosenfeld M. Early childhood risk factors for decreased FEV1 at age six to seven years in young children with cystic fibrosis. Annals of the American Thoracic Society 2015; 12:1170-6
      • Sanders D, Fink A, Mayer-Hamblett N, Schechter M, Sawicki G, Rosenfeld M, Flume P, Morgan W. Early life growth trajectories in cystic fibrosis are associated with pulmonary function at age 6 years. Journal of Pediatrics 2015; 167:1081-8.e1
      • Sanders D, Zhang Z ,Farrell P, Lai H. Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis. Journal of Cystic Fibrosis 2018; 17:528-535
      • Usatin D, Yen E, McDonald C, Asfour F, Pohl J, Robson J. Differences between WHO AND CDC early growth measurements in the assessment of cystic fibrosis clinical outcomes. Journal of Cystic Fibrosis 2017; 16:503-509
      • Welsh L, Robertson C, Ranganathan S. Increased rate of lung function decline in Australian adolescents with cystic fibrosis. Pediatric Pulmonology 2014; 49:873-7
      • Woestenenk J, Stellato R, Terheggen-Lagro S, van der Ent C, Houwen R. The relationship between body growth and pulmonary function in children with cystic fibrosis. Acta Paediatrica 2014; 103:162-7
      • Yen E, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. The Journal of Pediatrics 2013; 162:530-535.e1
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In pediatric participants with CF, what is the longitudinal relationship (at least 3 months) between weight and growth parameters and mortality?

  • Conclusion

    In pediatric participants with CF, one prospective cohort study demonstrated no difference in risk of mortality by 18 years of age according to WFA percentile at 3-5 years of age when results were adjusted for FEV1% at 6-8 years of age.

  • Grade: III
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In pediatric participants with CF, what is the longitudinal relationship (at least 3 months) between weight and growth parameters and mortality?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • McColley S, Schechter M, Morgan W, Pasta D, Craib M, Konstan M. Risk factors for mortality before age 18 years in cystic fibrosis. Pediatric Pulmonology 2017; 52:909-915
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In pediatric and adult participants with CF combined, what is the longitudinal relationship (at least 3 months) between weight parameters and mortality?

  • Conclusion

    In pediatric and adult participants combined, underweight (BMI <10-12^th percentile for pediatric and <18.5 k/m² for adult) was associated with increased risk of mortality (HR: 2.12) after a median of 12 years. There was a linear relationship between each unit increase in BMI z-score at baseline and mortality after a median of 13.4 years (HR: 1.2). Stunting (defined as height <5^th percentile) was associated with increased odds of mortality (OR: 2.2).

  • Grade: II
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In pediatric and adult participants with CF combined, what is the longitudinal relationship (at least 3 months) between weight parameters and mortality?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • Chamnan P, Shine B, Haworth C, Bilton D, Adler A. Diabetes as a determinant of mortality in cystic fibrosis. Diabetes Care 2010; 33:311-6
      • Stephenson A, Tom M, Berthiaume Y, Singer L, Aaron S, Whitmore G, Stanojevic S. A contemporary survival analysis of individuals with cystic fibrosis: a cohort study. The European Respiratory Journal 2015; 45:670-9
      • Vieni G, Faraci S, Collura M, Lombardo M, Traverso G, Cristadoro S, Termini L, Lucanto M, Furnari M, Trimarchi G, Triglia M, Costa S, Pellegrino S, Magazzù G. Stunting is an independent predictor of mortality in patients with cystic fibrosis. Clinical Nutrition (Edinburgh, Scotland) 2013; 32:382-5
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In pediatric participants with CF, what is the longitudinal relationship (at least 3 months) between vitamin D levels and FEV1?

  • Conclusion

    In pediatric participants with cystic fibrosis (CF), one cohort study found no longitudinal relationship between 25-hydroxyvitamin D levels in 6-18 year olds with PI and FEV1% predicted four years later. 

  • Grade: III
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In pediatric participants with CF, what is the longitudinal relationship (at least 3 months) between vitamin D levels and FEV1?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • McCauley L, Thomas W, Laguna T, Regelmann W, Moran A, Polgreen L. Vitamin D deficiency is associated with pulmonary exacerbations in children with cystic fibrosis. Annals of the American Thoracic Society 2014; 11:198-204
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In pediatric participants with CF, what is the longitudinal relationship (at least 3 months) between cystic fibrosis-related diabetes (CFRD) and FEV1?

  • Conclusion

    Pediatric participants with cystic fibrosis (CF) who were 5-20 years old with cystic fibrosis-related diabetes (CFRD) had significantly greater decline in FEV1% predicted after at least ten years of follow-up, compared to those without CFRD.

  • Grade: II
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In pediatric participants with CF, what is the longitudinal relationship (at least 3 months) between cystic fibrosis-related diabetes (CFRD) and FEV1?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • Goss C, Sykes J, Stanojevic S, Marshall B, Petren K, Ostrenga J, Fink A, Elbert A, Quon B, Stephenson A. Comparison of nutrition and lung function outcomes in [atients with cystic fibrosis living in Canada and the United States. American Journal of Respiratory and Critical Care Medicine 2018; 197:768-775
      • Welsh L, Robertson C, Ranganathan S. Increased rate of lung function decline in Australian adolescents with cystic fibrosis. Pediatric Pulmonology 2014; 49:873-7
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In pediatric participants with CF, what is the longitudinal relationship (at least 3 months) between cystic fibrosis-related diabetes (CFRD) and Quality of Life or Mortality?

  • Conclusion

    There were no included studies examining the longitudinal relationship (at least 3 months) between cystic fibrosis-related diabetes (CFRD) and QoL or mortality in pediatric participants with cystic fibrosis.

  • Grade: V
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In adults with CF, what is the longitudinal relationship (at least 3 months) between weight parameters and FEV1?

  • Conclusion

    In adults with cystic fibrosis (CF), data was mixed, but one large retrospective cohort study suggested that body mass index (BMI) ≥ 25 kg/m² is associated with decreased decline in FEV1% predicted and BMI <18.5 kg/m² is associated with increased decline in FEV1% predicted after a follow-up of up to 13 years. Baseline FEV1% predicted was also associated with change in BMI over time.  

  • Grade: II
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In adults with CF, what is the longitudinal relationship (at least 3 months) between weight parameters and FEV1?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • Amadori A, Antonelli A, Balteri I, Schreiber A, Bugiani M, De Rose V. Recurrent exacerbations affect FEV(1) decline in adult patients with cystic fibrosis. Respiratory Medicine 2009; 103:407-13
      • Goss C, Sykes J, Stanojevic S, Marshall B, Petren K, Ostrenga J, Fink A, Elbert A, Quon B, Stephenson A. Comparison of nutrition and lung function outcomes in [atients with cystic fibrosis living in Canada and the United States. American Journal of Respiratory and Critical Care Medicine 2018; 197:768-775
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In adults with CF, what is the longitudinal relationship (at least 3 months) between weight parameters and mortality?

  • Conclusion

    In adults with cystic fibrosis (CF), baseline BMI of ≤18-19 kg/m² was associated with higher risk of mortality after up to 24 years of follow-up [HR ranged from 1.52-1.57].

  • Grade: II
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In adults with CF, what is the longitudinal relationship (at least 3 months) between weight parameters and mortality?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • George P, Banya W, Pareek N, Bilton D, Cullinan P, Hodson M, Simmonds N. Improved survival at low lung function in cystic fibrosis: cohort study from 1990 to 2007. BMJ (Clinical research ed.) 2011; 342:d1008
      • Ramos K, Quon B, Heltshe S, Mayer-Hamblett N, Lease E, Aitken M, Weiss N, Goss C. Heterogeneity in survival in adult patients with cystic fibrosis with FEV < 30% of predicted in the United States. Chest 2017; 151:1320-1328
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In adults with CF, what is the longitudinal relationship (at least 3 months) between cystic fibrosis-related diabetes (CFRD) and FEV1 or mortality?

  • Conclusion

    In adults with cystic fibrosis (CF), presence of cystic fibrosis-related diabetes (CFRD) was associated with increased decline in FEV1% predicted after a follow up of 5 to up to 13 years. In adults with CF, one large cohort study determined that presence of CFRD was associated with higher risk of mortality after a follow-up of up to 24 years. However, this increased risk was not significant in another smaller study.

  • Grade: II
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In adults with CF, what is the longitudinal relationship (at least 3 months) between cystic fibrosis-related diabetes (CFRD) and FEV1 or mortality?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • Amadori A, Antonelli A, Balteri I, Schreiber A, Bugiani M, De Rose V. Recurrent exacerbations affect FEV(1) decline in adult patients with cystic fibrosis. Respiratory Medicine 2009; 103:407-13
      • George P, Banya W, Pareek N, Bilton D, Cullinan P, Hodson M, Simmonds N. Improved survival at low lung function in cystic fibrosis: cohort study from 1990 to 2007. BMJ (Clinical research ed.) 2011; 342:d1008
      • Goss C, Sykes J, Stanojevic S, Marshall B, Petren K, Ostrenga J, Fink A, Elbert A, Quon B, Stephenson A. Comparison of nutrition and lung function outcomes in [atients with cystic fibrosis living in Canada and the United States. American Journal of Respiratory and Critical Care Medicine 2018; 197:768-775
      • Ramos K, Quon B, Heltshe S, Mayer-Hamblett N, Lease E, Aitken M, Weiss N, Goss C. Heterogeneity in survival in adult patients with cystic fibrosis with FEV < 30% of predicted in the United States. Chest 2017; 151:1320-1328
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In adults with CF, what is the longitudinal relationship (at least 3 months) between cystic fibrosis-related diabetes (CFRD) and QoL?

  • Conclusion

    In adults with cystic fibrosis, there were no included studies examining the longitudinal relationship (at least 3 months) between cystic fibrosis-related diabetes (CFRD) and quality of life (QoL).

  • Grade: V
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In participants with CF, what is the longitudinal relationship (at least 3 months) between composite nutrition assessment/screening scores and hard outcomes (FEV1, Quality of Life or mortality)?

  • Conclusion

    There were no studies identified that examined the longitudinal relationship (at least 3 months) between composite nutrition assessment/screening scores and hard outcomes (FEV1, Quality of Life or mortality) in participants with cystic fibrosis (CF).

  • Grade: V
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In participants with CF, what is the longitudinal relationship (at least 3 months) between Nutrition Focused Physical Exam and hard outcomes (FEV1, Quality of Life or mortality)?

  • Conclusion

    There were no studies included that examined the longitudinal relationship (at least 3 months) between Nutrition Focused Physical Exam and hard outcomes (FEV1, Quality of Life or mortality) in participants with cystic fibrosis (CF).

  • Grade: V
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In participants with CF, what is the longitudinal relationship (at least 3 months) between weight parameters and QoL?

  • Conclusion

    No included studies investigated the relationship between weight/growth parameters and Quality of Life. 

  • Grade: V
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In participants with CF, what is the longitudinal relationship (at least 3 months) between body composition and hard outcomes (FEV1, Quality of Life or mortality)?

  • Conclusion

    There were no studies examining the longitudinal relationship (at least 3 months) between body composition and hard outcomes (FEV1, Quality of Life or mortality) in participants with cystic fibrosis.

  • Grade: V
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In participants with CF, what is the longitudinal relationship (at least 3 months) between handgrip strength and hard outcomes (FEV1, Quality of Life or mortality)?

  • Conclusion

    There were no included studies examining the longitudinal relationship (at least 3 months) between handgrip strength and hard outcomes (FEV1, Quality of Life or mortality).

  • Grade: V
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In participants with CF, what is the longitudinal relationship (at least 3 months) between essential fatty acid (EFA) deficiencies and FEV1?

  • Conclusion

    In pediatric participants with cystic fibrosis (CF), one cohort study suggests that linoleic acid levels ≥21mol% may be associated with higher FEV1% predicted after one year, but there was no relationship with essential fatty acids (EFA) deficiency measured by Triene:Tetraene ratio and FEV1% predicted after one year.

    In participants with CF ranging from 1-41 years and homozygous for F508del mutation, one cohort study demonstrated that Docosahexaenoic acid levels were positively associated with FEV1% predicted three years later.

     

  • Grade: III
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In participants with CF, what is the longitudinal relationship (at least 3 months) between essential fatty acid (EFA) deficiencies and FEV1?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • Maqbool A, Schall J, Garcia-Espana J, Zemel B, Strandvik B, Stallings V. Serum linoleic acid status as a clinical indicator of essential fatty acid status in children with cystic fibrosis. Journal of Pediatric Gastroenterology and Nutrition 2008; 47:635-44
      • Ollero M, Astarita G, Guerrera I, Sermet-Gaudelus I, Trudel S, Piomelli D, Edelman A. Plasma lipidomics reveals potential prognostic signatures within a cohort of cystic fibrosis patients. Journal of Lipid Research 2011; 52:1011-22
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In participants with CF, what is the longitudinal relationship (at least 3 months) between micronutrient deficiencies (vitamins A, D, E and zinc) and hard outcomes (FEV1, Quality of Life or mortality)?

  • Conclusion

    There were no studies included that examined the longitudinal relationship (at least 3 months) between micronutrient deficiencies (vitamins A, E and zinc) and hard outcomes (FEV1, Quality of Life or mortality) in participants with cystic fibrosis (CF). There were no studies included that examined the longitudinal relationship (at least 3 months) between vitamin D levels and hard outcomes in adults with CF.

  • Grade: V
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In participants with CF, what is the longitudinal relationship (at least 3 months) between essential fatty acid (EFA) deficiencies and QoL or mortality?

  • Conclusion

    In participants with cystic fibrosis (CF), there were no included studies that examined the longitudinal relationship (at least 3 months) between essential fatty acid (EFA) deficiencies and qualilty of life or mortality.

  • Grade: V
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In participants with CF, what is the longitudinal relationship (at least 3 months) between lipid profile and FEV1?

  • Conclusion

    In participants with cystic fibrosis (CF) ranging from 1-41 years and homozygous for F508del mutation, one cohort study detected no longitudinal relationship between triacylglyceride levels and later FEV1% predicted.

  • Grade: III
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In participants with CF, what is the longitudinal relationship (at least 3 months) between lipid profile and FEV1?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • Ollero M, Astarita G, Guerrera I, Sermet-Gaudelus I, Trudel S, Piomelli D, Edelman A. Plasma lipidomics reveals potential prognostic signatures within a cohort of cystic fibrosis patients. Journal of Lipid Research 2011; 52:1011-22
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In participants with CF, what is the longitudinal relationship (at least 3 months) between lipid profile and QoL or mortality?

  • Conclusion

    There were no studies included that examined the longitudinal relationship (at least 3 months) between lipid profile and quality of life (QoL) or mortality in participants with cystic fibrosis.

  • Grade: V
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018
     
  In pediatric and adults with CF combined, what is the longitudinal relationship (at least 3 months) between CFRD and mortality?

  • Conclusion

    In a combined group of adult and pediatric participants with cystic fibrosis (CF), the longitudinal relationship between cystic fibrosis-related diabetes (CFRD) and association with later mortality was mixed, with the largest study suggesting an association with increased risk of mortality for those with CFRD at baseline (HR:1.31) after 2.9 years, but other studies reporting no difference in mortality according to CFRD.

  • Grade: III
    • Grade I means there is Good/Strong evidence supporting the statement;
    • Grade II is Fair;
    • Grade III is Limited/Weak;
    • Grade IV is Expert Opinion Only;
    • Grade V is Not Assignable.
    • High (A) means we are very confident that the true effect lies close to that of the estimate of the effect;
    • Moderate (B) means we are moderately confident in the effect estimate;
    • Low (C) means our confidence in the effect estimate is limited;
    • Very Low (D) means we have very little confidence in the effect estimate.
    • Ungraded means a grade is not assignable.
  • Evidence Summary: In pediatric and adults with CF combined, what is the longitudinal relationship (at least 3 months) between CFRD and mortality?
    • Detail
    • Quality Rating Summary
      For a summary of the Quality Rating results, click here.
    • Worksheets
      • Chamnan P, Shine B, Haworth C, Bilton D, Adler A. Diabetes as a determinant of mortality in cystic fibrosis. Diabetes Care 2010; 33:311-6
      • Hofer M, Schmid C, Benden C, Speich R, Inci I, Weder W, Boehler A. Diabetes mellitus and survival in cystic fibrosis patients after lung transplantation. Journal of Cystic Fibrosis 2012; 11:131-6
      • Vieni G, Faraci S, Collura M, Lombardo M, Traverso G, Cristadoro S, Termini L, Lucanto M, Furnari M, Trimarchi G, Triglia M, Costa S, Pellegrino S, Magazzù G. Stunting is an independent predictor of mortality in patients with cystic fibrosis. Clinical Nutrition (Edinburgh, Scotland) 2013; 32:382-5
  • Search Plan and Results: CF: Nutrition Assessment Predictors of Hard Outcomes 2018