Abstract
We investigated plasma YKL-40 levels and chitotriosidase (CHIT1) activity in patients with cystic fibrosis (CF) lung disease and evaluated clinically relevant factors that may affect their levels. Plasma samples were obtained from pediatric (n = 19) and adult patients (n = 15) during exacerbation, discharge, and stable period of the disease. YKL-40 levels and chitotriosidase activity were measured by enzyme-linked immunosorbent assay and fluorometric assay, respectively. Data were compared with healthy children and adults of similar age. YKL-40 levels of pediatric and adult CF patients at all periods were significantly higher than controls (p < 0.001 and p < 0.05). CHIT1 activities of adult patients at all periods were significantly higher compared to controls (p < 0.05). On the other hand, CHIT1 activities of pediatric CF patients were similar with controls. YKL-40 levels of exacerbation period of adult CF patients were negatively correlated with forced vital capacity (FVC) (r = - 0.800, p = 0.014) and forced expiratory volume in 1s (FEV1) (r = - 0.735, p = 0.008). YKL-40 levels in the exacerbation period of pediatric CF patients were negatively correlated with FVC (r = - 0.697, p = 0.0082) and FEV1 (r = - 0.720, p = 0.006). CHIT1 activity may be a valuable marker of chronic inflammation in adult CF patients who suffer from CF for a longer period compared to pediatric patients. Increased YKL-40 levels in both pediatric and adult patients compared to controls may point to a role in between CF pathology.
Highlights
Cystic fibrosis (CF) is an inherited autosomal recessive disorder that is characterized by disrupted epithelial transport of chloride due to a mutation in cystic fibrosis transmembrane regulatory, CFTR, gene
CHIT1 activity may be a valuable marker of chronic inflammation in adult CF patients who suffer from CF for a longer period of time compared to pediatric patients
Increased YKL-40 levels in both pediatric and adult patients compared to controls may point to a role in between CF pathology
Summary
Cystic fibrosis (CF) is an inherited autosomal recessive disorder that is characterized by disrupted epithelial transport of chloride due to a mutation in cystic fibrosis transmembrane regulatory, CFTR, gene. This results with dehydration, increased viscosity of the mucus and obstruction of the ductal tract in various organs including lung, pancreas, liver, intestine, sweat glands and epididymis. Investigating the blood during pulmonary inflammation and remission in CF may give clues on the pathology of the disease and help to find novel biomarkers for monitoring CF. We investigated plasma YKL-40 levels and chitotriosidase (CHIT1) activity in patients with cystic fibrosis (CF) lung disease and evaluated clinically relevant factors that may affect their levels
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