Orta ve ağır akciğer tutulumu olan kistik fibrozlu hastalarda ölüme etki eden diğer risk etmenleri

Abstract

Aim:Severe pulmonary disease is responsible for over 80% of deaths associated with cystic fibrosis. Our aim was to evaluate the other risk factors associated with mortality in patients with cystic fibrosis who have moderate and severe lung disease. Material and Method: Among 200 patients with cystic fibrosis who were followed up in our clinic, 35 patients with moderate and severe lung disease (%FEV1 predicted ≤60) were included in the study. Demographic data, pulmonary function tests, high resolution thorax tomography, modified Bhalla score, blood gas analysis and nutritional status of the patients were evaluated. Results: Requirement for respiratory support was 31.4% in moderate and severe lung disease. Lower pulmonary function, requirement for non invasive ventilation, presence of diabetes mellitus, a Sa0 2 value of <95, a value of pCO 2 >50 mm Hg and requirement for frequent IV antibiotics were significantly related with increased mortality. Conclusions: This is a single centre study conducted in patients with cystic fibrosis who had moderate and severe lung disease and who had not received lung transplantation. We evaluated the clinical outcome and other factors that were associated with mortality. (Turk Arch Ped 2012; 47: 263-267)

Examination of CFTR Gene Mutations in Patient Groups with Borderline Sweat Test and Clinical Preliminary Diagnosis of Cystic Fibrosis by Next-Generation Sequencing Method in Sanliurfa Province

“…Expectations and predictions about CF shown as a serious problem in European countries and in studies on molecular treatment methods are that the number of adults with CF will increase by 70%, and the disease will gain more importance (15). In the first decade of life, the disease presents with bronchiectasis, exocrine pancreatic insufficiency, abnormal liver function tests, and meconium ileus; after the second decade, hemoptysis, pneumothorax, progressive lung failure, diabetes, cirrhosis, portal hypertension, and osteoporosis can be observed (1,9,15,16). Multidisciplinary approach and age-specific care services are important in patients with CF because of the effects of the disease on many systems and changes in this effect with age.…”