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

J Acad Nutr Diet. 2020 Sep;120(9):1517-1529. doi: 10.1016/j.jand.2020.05.011. Epub 2020 Jul 14.


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.

Keywords: Cystic fibrosis; Nutritional status; Pseudo-Bartter syndrome; Salt; Sodium.

Publication types

  • Review

MeSH terms

  • Adult
  • Child
  • Child, Preschool
  • Cystic Fibrosis / blood*
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / blood
  • Dietary Supplements / statistics & numerical data*
  • Electrolytes / blood
  • Female
  • Humans
  • Hyponatremia / etiology
  • Infant
  • Male
  • Nutritional Status*
  • Sodium / blood*
  • Sodium / deficiency
  • Sodium Chloride / administration & dosage*
  • Sodium Chloride / analysis
  • Sweat / chemistry
  • Water-Electrolyte Balance


  • CFTR protein, human
  • Electrolytes
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Sodium Chloride
  • Sodium