BACKGROUND The loss of cystic fibrosis transmembrane conductance regulator (CFTR) mediated chloride conductance does not fully explain the diverse pathologies evident in patients with cystic fibrosis (CF). Bicarbonate (HCO(3)(-)) secretion is also impaired in CFTR expressing tissues and CFTR is thought to regulate HCO(3)(-) secretion at the apical membrane of epithelial cells. We hypothesised t...

Ultrasound technique was used to produce native and acetylated cassava starch particles containing potassium sorbate (KS). In order obtain an active packaging, films with addition of KS (NKSPF) or added (AKSPF) were formulated. As control systems, without (CF) that not retained in (KSF), produced. The NKSPF AKSPF microstructure consistent composite materials. Tensile test revealed CF KSF ductil...

Non-invasive positive pressure ventilation (NIPPV) for respiratory failure in cystic fibrosis (CF) is frequently used in adults and has been shown to be of benefit to patients with advanced disease in terms of stabilisation of lung function, reduction in symptoms and increased exercise capacity. When used as an adjunct to physiotherapy, NIPPV increases oxygen saturations (SpO2), tidal volume, m...

INTRODUCTION Death in childhood from cystic fibrosis (CF) is now an uncommon event in the U.K. We wished to assess the circumstances surrounding deaths (and lung transplantation) in the modern era of CF care. METHODS A retrospective review was carried out pooling data from two large paediatric specialist CF units in London for the 10-year period 2000-2009 inclusive. RESULTS There were 11 de...

Neural circuits are shaped by activity-dependent elimination of redundant synapses during postnatal development. In many systems, postsynaptic activity is known to be crucial, but the precise mechanisms remain elusive. Here, we report that the immediate early gene Arc/Arg3.1 mediates elimination of surplus climbing fiber (CF) to Purkinje cell (PC) synapses in the developing cerebellum. CF synap...

Cystic fibrosis (CF) is the most common autosomal recessive disease of the Caucasian population. Among the various CF mutations, p.F508del is the most frequent, accounting for two-thirds of the global CF chromosomes, although showing great variability among populations. We have studied 115 unrelated CF patients from a mixed population of Minas Gerais (Brazil). To evaluate part of the DNA sequen...

OBJECTIVE Over the last decades improvements in medical therapies have delayed the progression of lung disease in cystic fibrosis (CF). However, lung disease is still the most common cause of premature death, and lung transplantation today is the only treatment for end-stage lung disease in patients with CF. We present a retrospective review of the outcome of CF patients transplanted in Denmark...