The leading cause of mortality and morbidity in humans with cystic fibrosis is lung disease. Advances in our understanding of the pathogenesis of the lung disease of cystic fibrosis, as well as development of innovative therapeutic interventions, have been compromised by the lack of a natural animal model. The utility of the CFTR -knockout mouse in studying the pathogenesis of cystic fibrosis h...

The publisher regrets that in the printed issue of Journal Cystic Fibrosis (Volume 19, Issue 6, November 2020 author name Barry Linnane is missing from editorial (page 850 to 851) “From micro macro; joining dots early CF lung disease”. would like apologise for any inconvenience caused. From diseaseJournal FibrosisVol. 19Issue 6PreviewRespiratory failure most common cause death young adults with...

Although the activity of nitric oxide (NO) synthases are increased in lung tissue of patients with cystic fibrosis, the concentrations of nasal and exhaled NO have recently been found to be decreased in cystic fibrosis. This could either be due to reduced NO formation or metabolism of NO within airway fluids. In this study, the stable NO metabolites, nitrate and nitrite, were determined in the ...

Cystic hamartoma refers to rare benign lung tumors. The literature describes 17 cases of this disease. tumor may look like multiple bilateral cysts or a solitary single-chamber multi-chamber cyst large size, which makes it difficult diagnose. disease can be complicated by spontaneous pneumothorax. itself often looks An observation 52-year-old man complaining chest pain and shortness breath is p...

hydatid cyst (hc) is a parasitic disease that may involve many organs, especially the lung and the liver. pulmonary artery location of the hydatid cyst is extremely rare, but it may cause life-threatening complications. we report a case of a hydatid cyst that completely filled the left main pulmonary artery and its distal part without cardiac involvement. thoracic computed tomography showed fil...

Sarcoidosis in an adult patient with cystic fibrosis lung disease was diagnosed on the basis of pulmonary function and radiographic data. It should be considered in the differential diagnosis of new diffuse interstitial infiltrates or hilar adenopathy in a patient with cystic fibrosis; biopsy of lung, lymph node, or skin lesions and interleukin-2 receptor levels may help to obtain a diagnosis.

Chronic lung diseases, such as chronic obstructive pulmonary disease (COPD), asthma, cystic fibrosis (CF) and interstitial lung diseases (ILD), affect many individuals worldwide. Patients with these chronic lung diseases are susceptible to respiratory lung infections and some of these viral infections can contribute to disease pathogenesis. This review highlights the associations of lung infect...

Abstract Mycobacterium abscessus appears to be increasing cause of pulmonary infection in children with underlying risk factors including cystic fibrosis, chronic lung disease and immunodeficiency syndromes. We present a case M. pediatric patient primary ciliary dyskinesia he was successfully treated parenteral amikacin, linezolid oral clarithromycin combined inhaled amikacin. Clinical improvem...

hydatid disease is a rare parasitic disease, which mainly involves liver then lung tissues. cardiac involvement is very rare, especially when there is not hepatic involvement. we describe a 47-year-old woman with a history of a lung hydatid cyst who was referred to rajaei heart center, tehran, iran in 2012. her chest computed tomographic scan showed a cardiac mass. echocardiographic examination...