Cystic fibrosis respiratory problems

An additional transferase that uses acetyl-CoA as a substrate sometimes acetylates one mannuronate unit. The disaccharide irnits are then inserted into the growing chain. An additional modification, which occurs after polymerization, is random C5 epimeriza-tion of unacetylated D-mannuronate residues to l-guluronate.136 182 Formation of alginate is of medical interest because infections by alginate-forming bacteria are a major cause of respiratory problems in cystic fibrosis.182... 

Children with cystic fibrosis lose excessive amounts of salt in perspiration and become dehydrated readily. A salty taste of the skin and an elevated chloride concentration of sweat are traditional diagnostic symptoms.3 More serious problems arise from progressive respiratory failure and inadequate pancreatic secretion. Lung infections with Pseudomonas aeruginosa are the major cause of death. The CFTR gene is expressed in many tissues, especially those of the mucous membranes. 

Carmen is a pale 20-year-old lady. Like many cystic fibrosis patients, she was diagnosed when she was a baby her problem was suspected when she suffered repeated respiratory infections and failed to gain weight as expected. Patients with cystic fibrosis secrete a high concentration of NaCl in their sweat and this forms the basis of an early diagnostic test for the condition. 

Drug formulations In February 2010, a solution of aztreonam for inhalation, formulated with lysine, was approved by the US FDA for the treatment of respiratory symptoms in patients with cystic fibrosis and infected with Pseudomonas aeruginosa [75 ], a bacterium that has always been difficult to treat with antibiotics, especially when it grows in biofilm, as is the case in the airways. So far, no new types of adverse effects have been found [76 ]. A key problem will be the development of resistance to this new formulation [77 ]. 

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