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Nutritional Status in Childhood as a Prognostic Factor in Patients with Cystic Fibrosis

Abstract

Introduction

There is a strong association between cystic fibrosis and malnutrition, mainly because of the higher energy needs combined with lower intake. There is also a well-established correlation between good nutritional status and better lung function. To date, however, there are no studies examining nutritional status in childhood and adult lung function. To respond to this need, this innovative study explored the long-term correlations between nutritional status in childhood and lung function in adulthood for the same patient population.

Methods

A retrospective patient file study was conducted to identify putative correlations between nutritional status in childhood and lung function in adulthood. The medical archives at Sheba Medical Center were examined for a period of 31 years between 1986 and 2017 for age, gender, mutations, pancreatic sufficiency or insufficiency (PI/PS), sputum cultures, cystic fibrosis related diabetes, body mass index (BMI) at the age of 10, and FEV1 at 20 and 30 in patients who underwent or did not undergo lung transplantation.

Results

The database was composed of the records of sixty-five patients, thirteen of whom underwent lung transplantation. The correlations (R²) between BMI at age of 10 years and FEV1 at the age of 20 and 30 years were 0.35 and 0.28, respectively, p < 0.001. A BMI of lower than − 0.75 at the age of 10 emerged as a risk factor for lung transplantation (OR 3.42 p = 0.023) and had a negative predictive value of 90%. Kaplan–Meier survival curve showed significant lower lung transplantation rate in the group of BMI z score higher than − 0.75 at the age of 10 years. Logistic regression found nutritional at the age of 10 years as a dominant risk factor for lung transplantation.

Conclusions

This study reports a clear, significant and important correlation for the first time between nutritional status in childhood and lung function for the same patients at adulthood. Hence, nutritional status sets a clear trajectory and should be treated aggressively. The findings emphasize the importance of new-born screening and early implementation of nutritional guidelines for cystic fibrosis patients.

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References

  1. Farrell PM (2008) The prevalence of cystic fibrosis in the European Union. J Cyst Fibros 7:450 e 3

    Article  PubMed  Google Scholar 

  2. Culhane S, George C, Pearo B, Spoede E (2013) Malnutrition in cystic fibrosis: a review. Nutr Clin Pract 28:676 e 83

    Article  PubMed  Google Scholar 

  3. Vaisman N, Pencharz PB, Corey M, Canny GJ, Hahn E (1987) Energy expenditure of patients with cystic fibrosis. J Pediatr 111:496 e 500

    Article  CAS  PubMed  Google Scholar 

  4. Perano S, Rayner CK, Couper J, Martin J, Horowitz M (2014) Cystic fibrosis related diabetes-a new perspective on the optimal management of postprandial glycemia. J Diabetes Complications 28:904 e 11

    Article  Google Scholar 

  5. Corey M, McLaughlin FJ, Williams M, Levison H (1988) A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol 41:583 e 91

    Article  CAS  PubMed  Google Scholar 

  6. Goss CH, Sykes J, Stanojevic S et al (2017) Comparison of nutrition and lung function outcomes in patients with cystic fibrosis living in Canada and the United States. Am J Respir Crit Care Med 197(6):768–775

    Article  Google Scholar 

  7. Konstan MW, Butler SM, Wohl ME et al (2003) Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. J Pediatr 142(6):624–630

    Article  PubMed  Google Scholar 

  8. Sanders DB, Fink A, Mayer-Hamblett N et al (2015) Early life growth trajectories in cystic fibrosis are associated with pulmonary function at age 6 years. J Pediatr 167(5):1081–1088.e1

    Article  PubMed  PubMed Central  Google Scholar 

  9. Connett GJ, Pike KC (2015) Nutritional outcomes in cystic fibrosis: are we doing enough? Paediatr Respir Rev 16(Suppl 1):31–34

    PubMed  Google Scholar 

  10. Peterson ML, Jacobs DR Jr, Milla CE (2003) Longitudinal changes in growth parameters are correlated with changes in pulmonary function in children with cystic fibrosis. Pediatrics 112(3 Pt 1):588–592

    Article  PubMed  Google Scholar 

  11. Turck D, Braegger CP, Colombo C, Declercq D, Morton A, Pancheva R, Robberecht E, Stern M, Strandvik B, Wolfe S, Schneider SM, Wilschanski M (2016) ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr 35(3):557–577

    Article  PubMed  Google Scholar 

  12. Yen EH, Quinton H, Borowitz D (2013) Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr 162(3):530–535.e1

    Article  PubMed  Google Scholar 

  13. Martin B, Schechter MS, Jaffe A, Cooper P, Bell SC, Ranganathan S (2012) Comparison of the US and Australian cystic fibrosis registries: the impact of newborn screening. Pediatrics 129(2):e348–e355

    Article  PubMed  Google Scholar 

  14. Efrati O, Mei-Zahav M, Rivlin J, Kerem E, Blau H, Barak A, Bujanover Y, Augarten A, Cochavi B, Yahav Y, Modan-Moses D (2006) Long term nutritional rehabilitation by gastrostomy in Israeli patients with cystic fibrosis: clinical outcome in advanced pulmonary disease. J Pediatr Gastroenterol Nutr 42(2):222–228

    Article  PubMed  Google Scholar 

  15. Efrati O, Kremer MR, Barak A, Augarten A, Reichart N, Vardi A, Modan-Moses (2007) D.Improved survival following lung transplantation with long-term use of bilevel positive pressure ventilation in cystic fibrosis. Lung 185(2):73–79

    Article  PubMed  Google Scholar 

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Correspondence to Moshe Ashkenazi.

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Ashkenazi, M., Nathan, N., Sarouk, I. et al. Nutritional Status in Childhood as a Prognostic Factor in Patients with Cystic Fibrosis. Lung 197, 371–376 (2019). https://doi.org/10.1007/s00408-019-00218-3

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  • DOI: https://doi.org/10.1007/s00408-019-00218-3

Keywords

  • Cystic fibrosis
  • Nutrition
  • Body mass index
  • Lung function
  • Lung transplantation