Lisa C. Grossman is a first-year medical student at Georgetown University,Washington, DC. in patients who have 10% or more CFTR function. Patients with 1% or less functioning of CFTR generally have a more classic type of cystic fibrosis; however, no definitive correlation exists between the genotype and the phenotype. In patients with 1% or less functioning of the gene, the patients’ cells have...

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 ...

We have previously shown that a decreased level of phosphatidylglycerol in cystic fibrosis (CF) respiratory mucus is partly responsible for its marked adhesiveness and stickiness, which impair mucus transport, and that distearoyl phosphatidylglycerol (DSPG) was the most efficient form of phosphatidylglycerol in the enhancement of respiratory mucus clearance. The aim of our study was to analyse ...

BACKGROUND Inflammation plays a critical role in lung disease development and progression in cystic fibrosis. Azithromycin is used for the treatment of cystic fibrosis lung disease, although its mechanisms of action are poorly understood. We tested the hypothesis that azithromycin modulates lung inflammation in cystic fibrosis mice. METHODS We monitored cellular and molecular inflammatory mar...

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...

Background Dermatoglyphics could assist in the diagnosis of congenital abnormalities. The aim of this study was to identify the dermatoglyphic patterns (finger print pattern type, total ridge count of each finger, a-b ridge count, and articulotrochanteric distance [ATD angles]) in the parents of cystic fibrosis children. Materials and Methods We recruited 75 parents of children with cystic fibr...

Purpose of review Over the past four decades, outcomes for patients with cystic fibrosis have improved dramatically. Major contributors to this improvement are a better understanding of disease pathogenesis and the systematic conduct of clinical trials evaluating new therapies designed to address these defects. This review describes recent developments in cystic fibrosis pulmonary therapies int...

Cystic fibrosis in patients over 40 is rare. We report a 52-year-old woman in whom cystic fibrosis was confirmed by sweat analysis. This patient represents the oldest cystic fibrosis patient (with confirmatory sweat chlorides) ever described. We conclude that any patient with the appropriate clinical presentation, regardless of age, should be investigated for cystic fibrosis.

Cystic fibrosis airways are frequently colonised with fungi. However, the interaction of these fungi with immune cells and the clinical relevance in cystic fibrosis lung disease are incompletely understood.We characterised granulocytes in airway fluids and peripheral blood from cystic fibrosis patients with and without fungal colonisation, non-cystic fibrosis disease controls and healthy contro...