Age-Related Progression of Microvascular Dysfunction in Cystic Fibrosis: New Detection Ways and Clinical Outcomes.

There are concerns about altered vascular functions that could play an important role in the pathogenesis and influence the severity of chronic disease, however, increased cardiovascular risk in paediatric cystic fibrosis (CF) has not been yet fully understood. Aim was to analyse vascular disease risk and investigate changes over times in CF and controls. We prospectively enrolled 22 CF subjects (a median age of 16.07 years), and 22 healthy demographically matched controls (a median age of 17.28 years) and determined endothelial function. We utilised a combined diagnostic approach by measuring the plethysmographic Reactive Hyperemia Index (RHI) as the post-to preocclusive endothelium-dependent changes of vascular tone, and biomarkers that are known to be related to endothelial dysfunction (ED): asymmetric dimethyl arginine (ADMA), high-sensitive CRP (hsCRP), VCAM-1 and E-selectin. RHI values were significantly lower in CF young adults (p<0.005). HsCRP (p<0.005), E-selectin (p<0.001) and VCAM-1 (p<0.001) were significantly increased in CF patients since childhood. The findings have provided a detailed account of the ongoing process of microvascular dysfunction with gradual progression with the age of CF patients, making them further at risk of advanced vascular disease. Elevations of biomarkers in CF children with not yet demonstrated RHI changes but with significantly reduced RHI in adulthood and lipid profile changes indicate the possible occurrence of ED with CF-related specific risk factors over time and will enable us to provide the best possible support.

[Twenty years of care for cystic fibrosis adults in Czech Republic]. / Dvacet let péce o dospelé nemocné s cystickou fibrózou v Ceske republice.

INTRODUCTION: Most of cystic fibrosis (CF) patients survive now into adulthood and they are transferred to pulmonologist care. AIMS: An overview of progress in care for CF adults in Czech Republic and evaluation of relationship of pulmonary function, nutritional status and airway colonization. METHODS: All adult CF patients followed in pulmonary departments from December 1987 to December 2007 were included into study. Data about survival status, pulmonary function, nutritional status, airway colonization and other pulmonary and extrapulmonary manifestations of CF were collected from patients' records. RESULTS: Total of 206 patients (96 females) were followed. Pancreatic insufficiency was present in 175 (85.0%), liver disease in 61 (29.6%) and insulin treatment in 58 (28.2%) patients. Bone disease was found in 70 (46.7%) from 150 examined patients. Sixty-two patients (23 females) died at mean age 25.4 +/- 5.5 years (median 24.3 years). Worse survival was recorded in patients with Burkholderia cepacia complex (BCC) airway colonization (24.4 +/- 4.0 vs 28.5 +/- 7.0 years, p = 0.012). One hundred forty-four living patients were followed to date of the 31st December 2007 with mean age 27.5 +/- 6.5 years (median 26.5 years), FEV1 64.4 +/- 28.5% pred. and BMI 20.9 +/- 3.1 kg/m2. Worse pulmonary function was present in patients with BCC colonization (FEV1 58.8 +/- 21.9 vs 67.8 +/- 27.3% pred., p = 0.041) and in malnourished patients (FEv1 49.5 +/- 18.5 vs 69.7 +/- 25.9% pred., p < 0.0001). BCC colonization was found in 54 (37.5%), Pseudomonas aeruginosa (PA) colonization in 92 (63.9%) and colonization without BCC or PA in 40 (27.8%) patients, respectively. Malnutrition (BMI < 19.0 kg/m2) was recorded in 38 (26.4%) patients. CONCLUSION: This study confirms growing number of CF adults in Czech Republic, close relationship of pulmonary function and nutritional status and also unfavourable influence of BCC colonization.