Premium
The relationship of clinical and inflammatory markers to outcome in stable patients with cystic fibrosis
Author(s) -
Downey Damian G.,
Martin S. Lorraine,
Dempster Martin,
Moore John E.,
Keogan Mary T.,
Starcher Barry,
Edgar Julia,
Bilton Diana,
Elborn J. Stuart
Publication year - 2007
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.20553
Subject(s) - medicine , gastroenterology , sputum , cystic fibrosis , neutrophil elastase , white blood cell , absolute neutrophil count , inflammation , pathology , chemotherapy , neutropenia , tuberculosis
Decreased survival in patients with cystic fibrosis has been related to FEV 1 , BMI, and infection with Burkholderia cepacia complex (BCC). We have assessed the relationship of blood, sputum, and urine inflammatory markers to lung function, BMI, colonization with B cenocepacia (Bc), and patient survival. Thirty‐nine stable cystic fibrosis (CF) patients (10 with Bc) were enrolled in a study to determine the effect of alpha‐1‐antitrypsin on airways inflammation. Pre‐treatment measurements were used in this study. Demographics, sputum microbiology, heart rate, oxygen saturation, lung function were recorded. Blood samples were obtained for white blood count (WBC), C‐Reactive Protein (CRP), and plasma neutrophil elastase/AAT complexes (pNEC). Neutrophil elastase (NE), neutrophil elastase/AAT complexes (sNEC), interleukin‐8 (IL‐8), TNF‐receptor 1 (sTNFr), and myeloperoxidase (MPO) were measured in sputum and urinary desmosine concentration determined. Patients with Bc had significantly higher levels of pNEC, 332 ± 91.4 ng/ml (mean ± SEM) versus 106 ± 18.2 ng/ml ( P = 0.0005) and sNEC, 369 ± 76.6 ng/ml versus 197 ± 36.0 ng/ml compared to those who were not. Five deaths were reported at the end of 1 year, (four with Bc) ( P = 0.011). Patients who subsequently died had significantly lower lung function FEV 1 , 1.2 ± 0.2 L versus 2.0 ± 0.1 L ( P = 0.03) and FVC, 2 ± 0.3 L versus 3.1 ± 0.2 L ( P = 0.01), compared to those that survived. There was significantly higher NE activity, 3.6 ± 1.6 U/ml versus 1.5 ± 0.6 U/ml ( P = 0.03), pNEC, 274 ± 99 ng/ml versus 142 ± 30 ng/ml ( P = 0.05), MPO, 163 ± 62 mcg/ml versus 54 ± 6.9 mcg/ml ( P = 0.03), and urinary desmosines 108 ± 19.9 pM/mg creatinine versus 51.1 ± 3.3 pM/mg creatinine ( P = 0.001), in those patients who subsequently died compared to those that survived. These data suggest there is increased neutrophil degranulation in patients infected with Bc and these patients have a poor outcome. Pediatr Pulmonol. 2007; 42:216–220. © 2007 Wiley‐Liss, Inc.