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 le;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 lt;95 a value of pCO2 gt;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: 267 71
📌 3 Key Takeaways
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Objective & Hypothesis: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 le;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 lt;95 a value of pCO2 gt;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: 267 71
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Key Findings & Data: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 le;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 lt;95 a value of pCO2 gt;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: 267 71
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Conclusion & Impact: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 le;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 lt;95 a value of pCO2 gt;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: 267 71
🔬Design:Araştırma Makalesi
📍Scope:2012 Verileri
⚙️Variables:Çocuk · kistik fibroz · ölüm riski
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 le;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 lt;95 a value of pCO2 gt;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: 267 71
Özet
Amaç : Ağır akciğer hastalığı kistik fibroza bağlı ölümlerin 80 rsquo;inden 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 le;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 Sa02 lt;95 ve pCO2 gt;50 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.
Gökdemir, Y., Erdem, E., Akpınar, İ. N., Ersu, R., Karadağ, B., Karakoç, F. (2012). Other risk factors associated with mortality in moderate and severe cystic fibrosis patients. Türk Pediatri Arşivi, Cilt: 47 Sayı: 4, 267–271. https://doi.org/10.4274/tpa.901