[New therapeutic developments in cystic fibrosis]. / Nouvelles thérapeutiques ciblant le canal chlorure dans la mucoviscidose.

Since the discovery of chloride secretion by the Cystic Fibrosis Transport regulator CFTR in 1983, and CFTR gene in 1989, knowledge about CFTR synthesis, maturation, intracellular transfer and function has dramatically expanded. These discoveries have led to the distribution of CF mutations into 6 classes with different pathophysiological mechanisms. In this article we will explore the state of art on CFTR synthesis and its chloride secretion function. We will then explore the consequences of the 6 classes of mutations on CFTR protein function and we will describe the new therapeutic developments aiming at correcting these defects.

[Update on the management of acute viral bronchiolitis: proposed guidelines of Grand Ouest University Hospitals]. / Prise en charge de la bronchiolite aiguë du nourrisson de moins de 1 an : actualisation et consensus médical au sein des hôpitaux universitaires du Grand Ouest (HUGO).

BACKGROUND AND OBJECTIVES: While our European and North American colleagues have recently updated their recommendations, the 2000 Consensus Conference remains the main guideline on management of acute viral bronchiolitis in France. We aimed to establish an updated inter-regional protocol on management of acute viral bronchiolitis in infants. METHOD: Pediatricians, pediatric pulmonologists, and emergency physicians of the Grand Ouest University Hospitals (France) gathered to analyze the recent data from the literature. RESULTS: Criteria to distinguish childhood asthma from acute viral bronchiolitis were established, then prescriptions of diagnostic tests, antibiotics, and chest physiotherapy were defined and reserved for very limited situations. Similarly, the modalities of oxygen therapy prescription and nutritional support were proposed. Finally, other therapeutics such as nebulized hypertonic saline seem promising, but their place in the treatment of acute bronchiolitis in infants remains unclear. CONCLUSION: This work has provided new proposals for management of acute viral bronchiolitis and helped standardize practices within the Grand Ouest University Hospitals. This local organization could lay the keystone for working toward guidelines initiated by learned societies at the national level.