Scedosporium/Pseudallescheria species are frequently seen in cystic fibrosis patients. However, disseminated forms after lung transplantation in these patients are rarely seen, but often with poor outcome. In this case report we describe a lung transplant recipient with cystic fibrosis who developed a spondylodiscitis that was caused by Scedosporium apiospermum. The patient was treated with ant...

The effect of cystic fibrosis on caffeine metabolism was studied in young children using the caffeine breath test. Eight children with cystic fibrosis aged 2-6 years and nine age matched controls were studied on a single occasion, and the cumulative percentage of labelled caffeine exhaled as carbon dioxide measured over two hours. This was significantly higher in the patients with cystic fibros...

In this investigation, the presence of calcitonin and calmodulin has been demonstrated in immunoperoxidase formalin-fixed, paraffin-embedded sections of lung from autopsy tissues of four patients who died as a result of complications resulting from their cystic fibrosis disease. Immunoreactive calcitonin has been stained and quantitated in solitary endocrine cells which are increased in number ...

Cystic and cavitary lung lesions constitute a spectrum of pulmonary diseases diagnosed in both children and adults. Cysts and cavities are commonly encountered abnormalies on chest radiography and chest computed tomography. High-resolution computed tomography (HRCT) of the chest frequently helps define morphologic features that may serve as important clues regarding the nature of cystic and cav...

RATIONALE Intervention for cystic fibrosis lung disease early in its course has the potential to delay or prevent progressive changes that lead to irreversible airflow obstruction. Denufosol is a novel ion channel regulator designed to correct the ion transport defect and increase the overall mucociliary clearance in cystic fibrosis lung disease by increasing chloride secretion, inhibiting sodi...

Abstract While remarkable advances in cystic fibrosis (CF) care have led to improvements survival and quality of life, many individuals with CF are living advanced lung disease (ACFLD) others will face continued progression its associated complex treatments choices. This review provide a summary recently published guidelines for ACFLD transplant referral highlight ongoing work enhance the those...

Cystic fibrosis is the most common lethal autosomal recessive disorder in the Caucasian population. Although the survival rate in patients constantly improves, lung damage is still the major cause of morbidity and mortality in patients with cystic fibrosis. In clinical practice, evaluation of patients' pulmonary state is made by combination of monitoring of lung function and more directly by as...

Cystic fibrosis (CF) is an autosomal recessive inherited multisystem disorder caused by mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Respiratory failure remains the most frequent cause of morbidity and mortality. Lung transplantation is the only option to treat end-stage lung disease. Very few cases of CF occur in Koreans. We report the ...

Progressive lung disease determines the morbidity and mortality of cystic fibrosis (CF) patients. CF lung disease is characterised by endobronchial inflammation sustained by bacterial infections and an ongoing accumulation of airway neutrophils. Activated or necrotic neutrophils liberate proteases that cause damage to structural, cellular and soluble components of the pulmonary microenvironment...

Inflammation is a major component of the vicious cycle characterizing cystic fibrosis pulmonary disease. If untreated, this inflammatory process irreversibly damages the airways, leading to bronchiectasis and ultimately respiratory failure. Antiinflammatory drugs for cystic fibrosis lung disease appear to have beneficial effects on disease parameters. These agents include oral corticosteroids a...