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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Iron deficiency occurs frequently in children with cystic fibrosis

Lieke Uijterschout1, Marianne Nuijsink, Daniëlle Hendriks

  • 1Department of Pediatrics, Juliana Children's Hospital/HAGA Hospital, The Hague, The Netherlands.

Pediatric Pulmonology
|September 4, 2013
PubMed

Insights

Iron deficiency (ID) is common in children with cystic fibrosis (CF), affecting over 60% of patients. Functional ID, linked to inflammation, is prevalent even in well-nourished children, with its exact causes in CF needing further research.

Area of Science:

  • Pediatric Hematology
  • Pediatric Pulmonology
  • Nutritional Science

Background:

  • Iron deficiency (ID) is common in adult cystic fibrosis (CF) patients, primarily functional due to chronic inflammation.
  • Recent data on the causes of ID and iron deficiency anemia (IDA) in pediatric CF populations are lacking.
  • Improved nutrition has delayed inflammation and pulmonary disease onset in children with CF.

Purpose of the Study:

  • To investigate the iron status in children with CF.
  • To determine if ID and IDA are associated with dietary iron intake, lung disease severity, and Pseudomonas aeruginosa (PA) infection.
  • To compare the prevalence of ID in children with CF to that in adult CF patients.

Main Methods:

  • Retrospective review of clinical charts for 53 children with CF (aged 0-16).
  • Analysis of follow-up data ranging from 1 to 14 years, totaling 343 annual observations.
  • Assessment of iron status indicators, including ferritin (Fer), and correlation with age, diet, lung disease, and PA infection.

Main Results:

  • Iron deficiency (ID) was present in 60.4% of children with CF in at least one year, observed in 24.5% of all annual observations.
  • Iron deficiency anemia (IDA) was present in 11.3% of children with CF in at least one year.
  • Higher ferritin (Fer) values correlated positively with age, suggesting increased inflammation rather than improved iron status in older children.

Conclusions:

  • Iron deficiency (ID) is common in children with CF, even those who are relatively healthy and well-nourished.
  • The precise mechanisms causing ID in pediatric CF patients remain unknown.
  • Further prospective studies utilizing soluble transferrin receptor and ferritin are recommended to elucidate the incidence and causes of ID in children with CF.

Keywords:
Pseudomonas aerigunosaanemiachildrenferritin

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