Iron status in children with cystic fibrosis.

Iron deficiency (ID) is common in children and adolescents with cystic fibrosis (CF). Proposed mechanisms for ID in CF may involve absolute ID and/or ID due to chronic inflammation. There are no data on the causes ID in children with CF.
Sputum from adult patients with CF contains increased amounts of total iron and ferritin and decreased amounts of transferrin compared with healthy controls. A significant relationship between the iron content of the CF lung microenvironment and the quantitative load of Pseudomonas Aeruginosa (PA) was demonstrated.

The study proposed here will investigate the prevalence and etiology of ID in children with CF. Furthermore we will investigate the sputum iron content in children with CF and the relationship with PA.

Our hypothesis is that ID in children with CF is common, and is caused by the combination of an absolute- and a functional-ID, which leads to relative anemia. In this study we will investigate the prevalence and etiology of ID in CF children.
Furthermore we expect to find increased sputum iron concentrations in children with CF compared with controls, due to increased hepcidin production.

We estimate iron content in serum and sputum during standard annual check-up.

According to our actual standard protocol, all children with CF undergo an annual check-up in the Juliana children’s hospital. During this check-up we perform a pulmonary function test in children >6 years and obtain sputum, blood and defecation samples to analyze pulmonary and gastrointestinal status. Parents/caretakers are asked to keep up a food diary for their child for a period of 3 days.
In addition to blood sampling as part of the regular check-up, extra blood will be taken to evaluate iron status. In sputum samples obtained during pulmonary function test we will assess iron, ferritin and total cell counts.