ÖZGE ATAY

and 7 more

Background: Cystic fibrosis (CF) is an inherited autosomal recessive disorder that causes chronic airway disease. In addition to genetic factors, environmental factors may affects the clinical phenotype of CF. In our study, the presence of aeroallergen sensitivity in our patients with CF and its effects on clinical findings were evaluated. Materials and Methods: Demographic characteristics, clinical and laboratory findings, skin prick test (SPT) results, and Modified Shwachman-Kulczycki (MSK) scores of patients diagnosed with CF followed in the Pediatric Respiratory and Allergy Clinic of Dokuz Eylul University Faculty of Medicine were evaluated. Results: We evaluated 51 patients with CF with a median age of 10 (6-18) years. The mean MSK score of the patients was 72.54±11.50, and the mean predictive value of forced expiratory volume (FEV1) in the first second of 41 patients was 80.43±19.50. According to SPT, aeroallergen sensitivity was detected in 17 (33.3%). The frequency of bacterial colonization and bronchiectasis was higher, MSK scores were lower in AF-sensitive patients (p≤0.01). However, there was no similar difference in other allergen sensitivities. MSK scores and predictive FEV1 values of 25 (49%) patients with bacterial colonization were significantly lower than those without colonization (p=0.001, p=0.005, respectively). Conclusion: Aeroallergen sensitivity was detected in approximately 1/3 of CF patients. Although it has been emphasized in studies that environmental factors may have an impact on lung functions and clinical conditions in CF, the effect of allergens other than AF sensitivity may be less important compared to other environmental factors such as the presence of bacterial colonization. Keywords: Cystic fibrosis, atopy, children, asthma, Aspergillus fumigatus, Pseudomonas aeruginosa

Ozgur Kizilca

and 6 more

Abstract Background: Cystic fibrosis may lead to left ventricular dysfunction. This dysfunction can be documented by methods such as tissue doppler echocardiographic imaging and two-dimensional speckle tracking echocardiography in early stage. Patients and Methods: A total of 34 patients diagnosed with cystic fibrosis (mean age and SD 9.9±4.9 years) and 37 healthy control subjects with a comparable gender and age distribution (mean age 9.8±4.3) were studied. The results for the two groups were compared along with the results of published reports. Result: Control group had higher diastolic and systolic dimentions compared to the patient group in M-mode measurements of left ventricle by conventional echocardiography (p <0.05). There was no significant relationship between the groups in terms of the dimensions of systolic and diastolic measurements of interventricular septum and posterior wall of left ventricle, and ejection fraction. Pulmonary artery systolic pressure was significantly higher in the patient group (p<0.001). Myocardial performance indices of left ventricle free wall and interventricular septum were increased in the patient group compared to the control group (p<0.05). ). As measured by speckle tracking echocardiography, 7 segments in left ventricular myocardial longitudinal strain and 3 segments in left ventricular myocardial circumferential strain showed significant reductions in patients with cystic fibrosis compared to controls (p <0.05). Conclusions: Tissue doppler echocardiographic imaging and speckle tracking echocardiography may help identifying subclinical left ventricular dysfunction in cystic fibrosis patients with unremarkable conventional echocardiography. Its may be considered for the routine follow-up of cystic fibrosis patients.