Cystic fibrosis (CF) is a common progressive genetic disorder among children which involves lungs, kidneys, intestine and liver. Apart from the significance of genetic factors, various environmental factors particularly bone mineral density are directly associated with CF. Remarkably, bone disease is appeared as a routine and commo...

The median life expectancy for cystic fibrosis is now over 30 years, and it is projected that in newborn infants it will become more than 40 years. The identification of the cystic fibrosis gene and its product, cystic fibrosis transmembrane conductance regulator (CFTR), has widened the spectrum of the disease from the classical case of the infant with cystic fibrosis to the elderly childless m...

Lymphangioleiomyomatosis (LAM) is a rare disease characterized by progressive cystic destruction of the lungs. We present an unusual radiological presentation of lymphangioleiomyomatosis in a patient followed for 33 years with profuse coarse lung nodules in addition to the classical cystic lesions. We believe that this report might support the case for considering LAM a low-malignant neoplasm.

Cystic fibrosis patients and patients with chronic obstructive pulmonary disease, trauma, burn wound, or patients requiring ventilation are susceptible to severe pulmonary infection by Pseudomonas aeruginosa. Physiological innate defense mechanisms against this pathogen, and their alterations in lung diseases, are for the most part unknown. We now demonstrate a role for the sphingoid long chain...

In order to investigate whether circulating immune complexes containing Pseudomonas aeruginosa antigens mediate pulmonary damage in cystic fibrosis, we studied lung function, serum immune complex levels, and immunoglobulin concentrations in relationship to chronic pseudomonas colonisation in 69 affected children. Sixteen of the children with cystic fibrosis had increased levels of immune comple...

CONTEXT Disease variation can be substantial even in conditions with a single gene etiology such as cystic fibrosis (CF). Simultaneously studying the effects of genes and environment may provide insight into the causes of variation. OBJECTIVE To determine whether secondhand smoke exposure is associated with lung function and other outcomes in individuals with CF, whether socioeconomic status ...

INTRODUCTION Mutations in both alleles of the cystic fibrosis transmembrane conductance regulator gene result in the disease cystic fibrosis, which usually manifests as chronic sinopulmonary disease, pancreatic insufficiency, elevated sodium chloride loss in sweat, infertility among men due to agenesis of the vas deferens and other symptoms including liver disease. CASE PRESENTATION We descri...

For effective clinical management of cystic fibrosis (CF) lung disease it is important to closely monitor the start and progression of lung damage. The aim of this study was to investigate the ability of high-resolution computed tomography (HRCT) scoring systems and pulmonary function tests (PFT) to detect changes in lung disease. CF children (n=48) had two HRCT scans in combination with two PF...

Cystic fibrosis is the most common fatal inherited autosomal recessive disease in Caucasians, affecting approximately one out of every 2,000 births. Survival of patients with cystic fibrosis has significantly improved due to advances in respiratory and nutritional care, and their current average life expectancy is 30-40 years. Development of cystic fibrosis-related diabetes is a comorbidity tha...

Pulmonary surfactant is a surface active material composed of both lipids and proteins that is produced by alveolar type II pneumocytes. Abnormalities of surfactant in the immature lung or in the acutely inflamed mature lung are well described. However, in a variety of subacute diseases of the mature lung, abnormalities of lung surfactant may also be of importance. These diseases include chroni...