title: Iron deficiency in cystic fibrosis: relationship to lung disease severity and chronic Pseudomonas aeruginosa infection.
abstract: BACKGROUND
  Iron deficiency (ID) is common in patients with cystic fibrosis (CF) and may be related to GI factors and chronic inflammation. Pseudomonas aeruginosa (PA) infection is predominantly responsible for chronic lung suppuration in patients with CF, but its survival is critically dependent on the availability of extracellular iron, which it obtains via highly efficient mechanisms.
  
  
  OBJECTIVE
  To determine whether ID in CF patients is directly related to the severity of suppurative lung disease.
  
  
  METHODS
  We determined the iron status of 30 randomly selected adult CF patients (13 women) and assessed the relationship to lung disease severity and GI factors by determining their daily sputum volume, FEV(1) percent predicted, C-reactive protein (CRP) level, erythrocyte sedimentation rate, and degree of pancreatic supplementation. Additionally, we measured the sputum concentrations of iron and ferritin in a randomly selected subgroup of 13 of the 30 subjects.
  
  
  METHODS
  Adult CF Service in a tertiary-care center.
  
  
  RESULTS
  Seventy-four percent of subjects experienced ID (ie, serum iron levels < or = 12 micromol/L and/or transferrin saturation levels < or = 16%). There was no relationship found with the degree of pancreatic supplementation. The daily sputum volume was strongly associated with low serum iron levels, transferrin saturation, ferritin/CRP ratio, and FEV(1) percent predicted (p < 0.05). Serum iron levels and transferrin saturation were negatively related to CRP (r = -0.8 and r = -0.7, respectively; p < 0.01) and erythrocyte sedimentation rate (r = -0.5 and r = -0.4, respectively; p < 0.05). FEV(1) percent predicted was positively related to serum iron level (r = 0.5; p < 0.01), transferrin saturation (r = 0.4; p < 0.05), and ferritin/CRP ratio (r = 0.7; p < 0.05). Sputum iron concentration (median, 63 micromol/L; range, 17 to 134 micromol/L) and ferritin concentration (median, 5,038 microg/L; range, 894 to 6,982 microg/L) exceeded plasma levels and negatively correlated with FEV(1) percent predicted (r = -0.6 and r = -0.5, respectively; p < or = 0.05).
  
  
  CONCLUSIONS
  In our CF patients, ID was directly related to the increased severity of suppurative lung disease but not to the degree of pancreatic insufficiency. Iron loss into the airway may contribute to ID and may facilitate PA infection.
authors.affiliation: Cystic Fibrosis Service, Department of Respiratory Medicine, Monash University Medical School, Alfred Hospital, Prahran, Melbourne, VIC, Australia. d.reid@alfred.org.au
authors.forename: David W
authors.initials: DW
authors.lastname: Reid
authors.forename: Nicholas J
authors.initials: NJ
authors.lastname: Withers
authors.forename: Libby
authors.initials: L
authors.lastname: Francis
authors.forename: John W
authors.initials: JW
authors.lastname: Wilson
authors.forename: Thomas C
authors.initials: TC
authors.lastname: Kotsimbos
mesh_terms: D000328:Adult; D018798:Anemia, Iron-Deficiency / Q000175:diagnosis*; D002908:Chronic Disease; D003550:Cystic Fibrosis / Q000175:diagnosis*; D010188:Exocrine Pancreatic Insufficiency / Q000175:diagnosis; D005260:Female; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D008297:Male; D018410:Pneumonia, Bacterial / Q000175:diagnosis*; D011552:Pseudomonas Infections / Q000175:diagnosis*; D011550:Pseudomonas aeruginosa; D012307:Risk Factors