title: Acute pulmonary exacerbation and lung function decline in patients with cystic fibrosis: high-mobility group box 1 (HMGB1) between inflammation and infection.
abstract: Airway inflammation plays a central role in cystic fibrosis (CF) lung disease, and biomarkers of inflammation, such as high-mobility group box 1 (HMGB1) could be used to monitor disease activity. The main aim of this study was to confirm the role of HMGB1 in CF patients, correlating its serum and sputum levels with pulmonary function and inflammation. Serum and sputum HMGB1 were evaluated in a cohort of 31 CF patients and 30 non-smoking healthy subjects (HS group). Acute pulmonary exacerbation events and lung function decline have been also evaluated during a 3-year follow-up period. Serum HMGB1 levels were significantly higher than those measured in HS, such as sputum HMGB1. Kaplan-Meier survival curves revealed that patients with high HMGB1 values experienced a significantly faster evolution to decline of lung function. A multiple Cox regression analysis assessed that an increase of serum HMGB1 was associated with 5% increased risk of pulmonary disease progression, whereas elevated sputum HMGB1 was related to a 10% increased risk of lung function decline. In CF patients, HMGB1 closely reflects the entity of pulmonary impairment and represents a strong and independent risk marker for progression of lung function decline.
authors: Department of Pediatric Sciences, Genetics and Immunology Paediatrics Unit, University of Messina, Messina, Italy. Electronic address: valeriachirico@hotmail.it.
authors: V
authors: V
authors: Chirico
authors: Department of Internal Medicine, Nephrology and Dialysis Unit, University of Messina, Messina, Italy.
authors: A
authors: A
authors: Lacquaniti
authors: Pediatric Bronchopneumology and Cystic Fibrosis Unit, University of Catania, Catania, Italy.
authors: S
authors: S
authors: Leonardi
authors: Department of Pediatric Sciences, Genetics and Immunology Paediatrics Unit, University of Messina, Messina, Italy.
authors: L
authors: L
authors: Grasso
authors: Pediatric Bronchopneumology and Cystic Fibrosis Unit, University of Catania, Catania, Italy.
authors: N
authors: N
authors: Rotolo
authors: Department of Pediatric Sciences, Genetics and Immunology Paediatrics Unit, University of Messina, Messina, Italy.
authors: C
authors: C
authors: Romano
authors: Pediatric Bronchopneumology and Cystic Fibrosis Unit, University of Catania, Catania, Italy.
authors: G
authors: G
authors: Di Dio
authors: Pediatric Bronchopneumology and Cystic Fibrosis Unit, University of Catania, Catania, Italy.
authors: E
authors: E
authors: Lionetti
authors: Department of Neuroscience and Anesthesiology, University of Messina, Italy.
authors: A
authors: A
authors: David
authors: Department of Pediatric Sciences, Genetics and Immunology Paediatrics Unit, University of Messina, Messina, Italy.
authors: T
authors: T
authors: Arrigo
authors: Department of Pediatric Sciences, Genetics and Immunology Paediatrics Unit, University of Messina, Messina, Italy.
authors: C
authors: C
authors: Salpietro
authors: Pediatric Bronchopneumology and Cystic Fibrosis Unit, University of Catania, Catania, Italy.
authors: M
authors: M
authors: La Rosa
mesh_terms: D000293:Adolescent; D000328:Adult; D001996:Bronchopneumonia / Q000175:diagnosis / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications*; D005260:Female; D024243:HMGB1 Protein / Q000032:analysis* / Q000097:blood*; D006801:Humans; D007249:Inflammation / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D011446:Prospective Studies; D012129:Respiratory Function Tests; D044967:Serum / Q000737:chemistry; D013183:Sputum / Q000737:chemistry; D055815:Young Adult
chemical_list: D024243:HMGB1 Protein; C585491:HMGB1 protein, human
keywords: Acute pulmonary exacerbation; HMGB1; cystic fibrosis; lung function decline; pulmonary Pseudomonas infection