Fazilet KARAKOÇ, İhsan Nuri AKPINAR, Bülent KARADAĞ, Refika ERSU, Yasemin GÖKDEMİR, Ela ERDEM

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

Amaç: Ağır akciğer hastalığı kistik fibroza bağlı ölümlerin %80inden sorumludur. Bu çalışmanın amacı orta ve ağır akciğer hastalığı olan kistik fibrozlu hastalarda ölüm ile ilişkili olabilecek diğer risk etmenlerini araştırmaktır. Gereç ve Yöntem: Kliniğimizde izlenen kistik fibrozlu 200 hastadan, orta ve ağır akciğer hastalığı olan (FEV1 beklenen değer %≤60) 35 hasta çalışmaya alındı. Demografik veriler, akciğer işlevleri, yüksek çözünürlüklü göğüs tomografisi, değiştirilmiş Bhalla skoru, kan gazı ve beslenme durumu incelendi. Bulgular: Orta ve ağır akciğer hastalığı olan kistik fibrozlu hastaların solunum desteğine gereksinimi %31,4 idi. Düşük akciğer işlevleri, girişimsel olmayan ventilasyon ihtiyacı, diabetes mellitus, Sa0250 mmHg olması ve son bir yılda sık sık damardan antibiyotik gereksinimi olmasının ölüm riskinde artış ile ilişkili olduğu saptandı. Çıkarımlar: Bu çalışmada tek bir merkezde izlenen; farklı mutasyonlara sahip ve akciğer nakli yapılmamış orta ve ağır akciğer hastalığı olan kistik fibroz hastaları değerlendirilmiş ve ölümle ilişkili diğer etmenler bildirilmiştir.

Other risk factors associated with mortality in moderate and severe cystic fibrosis patients

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 Sa02 value of 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.

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