Sodium Status and Replacement in Children and Adults Living with Cystic Fibrosis: A Narrative Review


      Patients with cystic fibrosis (CF) have a two- to four-fold higher sodium chloride sweat content compared with healthy controls. This high sweat salt loss increases the risk for electrolyte disturbances, associated with subacute or chronic complications. Sodium status therefore needs to be adequately monitored and salt intake adjusted to individual needs. The lack of current evidence to formulate specific recommendations and assess sodium status is reflected in a variability of recommendations in international guidelines. This narrative review presents an overview of the current evidence. Infants with CF in particular are at risk for severe sodium deficiency, potentially leading to metabolic alkalosis due to low intake and high sweat losses. More research on the assessment of sodium status and efficacy of sodium chloride supplements in the population of patients with CF, especially given the changing era of CF transmembrane conductance regulator modulatory treatment, is warranted.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of the Academy of Nutrition and Dietetics
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Turck D.
        • Braegger C.P.
        • Colombo C.
        • et al.
        ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis.
        Clin Nutr. 2016; 35: 557-577
        • Scurati-Manzoni E.
        • Fossali E.F.
        • Agostoni C.
        • et al.
        Electrolyte abnormalities in cystic fibrosis: Systematic review of the literature.
        Pediatr Nephrol. 2014; 29: 1015-1023
        • Kessler W.R.
        • Andersen D.H.
        Heat prostration in fibrocystic disease of the pancreas and other conditions.
        Pediatrics. 1951; 8: 648-656
        • Di Sant'Agnese P.A.
        • Darling R.C.
        • Perera G.A.
        • Shea E.
        Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas; clinical significance and relationship to the disease.
        Pediatrics. 1953; 12: 549-563
        • Beckerman R.C.
        • Taussig L.M.
        Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis.
        Pediatrics. 1979; 63: 580-583
        • Borowitz D.
        • Robinson K.A.
        • Rosenfeld M.
        • et al.
        Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.
        J Pediatr. 2009; 155: S73-S93
        • Saxby N.
        • Painter C.
        • Kench A.
        • et al.
        Nutrition guidelines for cystic fibrosis in Australia and New Zealand.
        • Orenstein D.M.
        • Henke K.G.
        • Costill D.L.
        • Doershuk C.F.
        • Lemon P.J.
        • Stern R.C.
        Exercise and heat stress in cystic fibrosis patients.
        Pediatr Res. 1983; 17: 267-269
        • Orenstein D.M.
        • Henke K.G.
        • Green C.G.
        Heat acclimation in cystic fibrosis.
        J Appl Physiol Respir Environ Exerc Physiol. 1984; 57: 408-412
        • Brown M.B.
        • McCarty N.A.
        • Millard-Stafford M.
        High-sweat Na+ in cystic fibrosis and healthy individuals does not diminish thirst during exercise in the heat.
        Am J Physiol-Reg I. 2011; 301: R1177-R1185
        • Siegenthaler P.
        • Haller J.
        • Haller R.
        • Hampai A.
        • Muller A.F.
        Effect of experimental salt depletion and aldosterone load on sodium and chloride concentration in the sweat of patients with cystic fibrosis of the pancreas and of normal children.
        Arch Dis Child. 1964; 39: 61-65
        • Holland A.E.
        • Wilson J.W.
        • Kotsimbos T.C.
        • Naughton M.T.
        Metabolic alkalosis contributes to acute hypercapnic respiratory failure in adult cystic fibrosis.
        Chest. 2003; 124: 490-493
        • Guimaraes E.V.
        • Schettino G.C.M.
        • Camargos P.A.M.
        • Penna F.J.
        Prevalence of hyponatremia at diagnosis and factors associated with the longitudinal variation in serum sodium levels in infants with cystic fibrosis.
        J Pediatr. 2012; 161: 285-289
        • Kriemler S.
        • Wilk B.
        • Schurer W.
        • Wilson W.M.
        • Bar-Or O.
        Preventing dehydration in children with cystic fibrosis who exercise in the heat.
        Med Sci Sports Exerc. 1999; 31: 774-779
        • Knepper C.
        • Ellemunter H.
        • Eder J.
        • et al.
        Low sodium status in cystic fibrosis—as assessed by calculating fractional Na(+) excretion-is associated with decreased growth parameters.
        J Cyst Fibros. 2016; 15: 400-405
        • Coates A.J.
        • Crofton P.M.
        • Marshall T.
        Evaluation of salt supplementation in CF infants.
        J Cyst Fibros. 2009; 8: 382-385
        • Al-Ghimlas F.
        • Faughnan M.E.
        • Tullis E.
        Metabolic alkalosis in adults with stable cystic fibrosis.
        Open Respir Med J. 2012; 6: 59-62
        • Del Ciampo I.R.L.
        • Martins N.
        • Sawamura R.
        • Facincani I.
        • Del Ciampo L.A.
        • Fernandes M.I.M.
        Prevalence and nutritional status of infants with cystic fibrosis and pseudo-Bartter syndrome during the first year of life.
        Child Obes Nutr. 2014; 6: 313-319
        • Bower T.R.
        • Pringle K.C.
        • Soper R.T.
        Sodium deficit causing decreased weight gain and metabolic acidosis in infants with ileostomy.
        J Pediatr Surg. 1988; 23: 567-572
        • Lommatzsch S.T.
        • Taylor-Cousar J.L.
        The combination of tezacaftor and ivacaftor in the treatment of patients with cystic fibrosis: Clinical evidence and future prospects in cystic fibrosis therapy.
        Ther Adv Respir Dis. 2019; 13 (1753466619844424)
        • Davis P.B.
        • Schluchter M.D.
        • Konstan M.W.
        Relation of sweat chloride concentration to severity of lung disease in cystic fibrosis.
        Pediatr Pulmonol. 2004; 38: 204-209
        • Wainwright C.E.
        • Elborn J.S.
        • Ramsey B.W.
        • et al.
        Lumacaftor-Ivacaftor in patients with cystic fibrosis homozygous for Phe508del CFTR.
        N Engl J Med. 2015; 373: 220-231
        • Taylor-Cousar J.L.
        • Munck A.
        • McKone E.F.
        • et al.
        Tezacaftor-Ivacaftor in patients with cystic fibrosis homozygous for Phe508del.
        N Engl J Med. 2017; 377: 2013-2023
        • Southern K.W.
        • Patel S.
        • Sinha I.P.
        • Nevitt S.J.
        A systematic Cochrane Review of correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.
        Paediatr Respir Rev. 2019; 30: 25-26
        • Faraji-Goodarzi M.
        Pseudo-Bartter syndrome in children with cystic fibrosis.
        Clin Case Rep. 2019; 7: 1123-1126
        • Varasteh Kia M.
        • Barone S.
        • McDonough A.A.
        • Zahedi K.
        • Xu J.
        • Soleimani M.
        Downregulation of the Cl-/HCO3-exchanger pendrin in kidneys of mice with cystic fibrosis: Role in the pathogenesis of metabolic alkalosis.
        Cell Physiol Biochem. 2018; 45: 1551-1565
        • van der Haak N.
        • King S.J.
        • Crowder T.
        • et al.
        Highlights from the nutrition guidelines for cystic fibrosis in Australia and New Zealand.
        J Cyst Fibros. 2020; 19: 16-25
        • Nutricia
        Nutrition composition table.
        • McLean R.M.
        Measuring population sodium intake: A review of methods.
        Nutrients. 2014; 6: 4651-4662


      D. Declercq is a dietitian, Cystic Fibrosis Reference Center, Ghent University Hospital, Gent, Belgium.


      S. Van daele is a professor, Cystic Fibrosis Reference Center, Ghent University Hospital, Gent, Belgium.


      S. Van Biervliet is a professor, Cystic Fibrosis Reference Center, Ghent University Hospital, Gent, Belgium.


      E. Van Braeckel, is a professor, Department of Respiratory Medicine, Ghent University Hospital, Gent, Belgium.


      S. Marchand is a coordinator with the Center for Nutrition and Dietetics, Ghent University Hospital, Gent, Belgium.