Abstract
Cystic Fibrosis (CF) is a genetic disorder which results in abnormal Cystic Fibrosis Transmembrane Regulator (CFTR) protein. CFTR protein actively transports chloride and bicarbonate out of cells which produce mucus in epithelium of various organs. However, when there is an abnormality in this protein then ion and water transport across airway surface is affected and form viscous mucus due to dehydration. This disease affects lung, pancreases, intestine and hepatobiliary system. Infection of lungs is main reason of high morbidity and mortality. Patients with CF are more prone to bacterial infection of the respiratory tract. Mainly four bacterial pathogens are found in the respiratory tract of CF patient viz. Staphylococcus aureus, Haemophilus influenzae, Burkholderia cepacia, Pseudomonas aeruginosa. Mucoid P. aeruginosa is the most dangerous pathogen among them due to ability to form biofilm. There are many conventional and new treatment options available for CF patients. New therapies are used to correct the CFTR protein and antibiotic treatment is used to prevent or early infection or suppression of chronic infection. However, when P. aeruginosa turn into mucoid form, formation of extracellular polysaccharide biofilm starts with alginate as major constituent. The antibiotics fail to reach the target as EPS of the biofilm shield the microbe from antibiotics action. Alginate lyase, an alginate degrading has the ability to break the alginate and subsequently increases the efficacy of antibiotics therapy. The co-administration of alginate lyase and Dnase along with antibiotics has proved to be very efficient in the management of patients with CF.
Highlights
Cystic Fibrosis is single gene autosomal recessive genetic and life threatening disorder which is characterized by damage to the respiratory and digestive system [1]
Functional Cystic Fibrosis Transmembrane Regulator (CFTR) is important for the proper hydration of the Airways Surface Liquid (ASL) in respiratory tract and this is important for ciliary movement which helps in the proper functioning of lungs and removal of inhaled microorganisms and particles
In early Cystic Fibrosis (CF) infection most of the P. aeruginosa is characterized as non mucoid strain but in the deficiency of oxygen bacteria come under stress and non-mucoid strain transformed into mucoid strain [20]
Summary
Cystic Fibrosis is single gene autosomal recessive genetic and life threatening disorder which is characterized by damage to the respiratory and digestive system [1]. Clinical features of CF include salty flavor skin, continuous coughing and dyspnea, viscous mucous (sputum), unhealthy or breathe audibly, non-cancerous growth on the lining of sinuses foul smelling and greasy stools, body weight imbalance, intestine blockage especially in new born and acute constipation This disease occurs due to mutation in cystic fibrosis trans-membrane conductance regulator protein which is called CFTR [4]. In early CF infection most of the P. aeruginosa is characterized as non mucoid strain but in the deficiency of oxygen bacteria come under stress and non-mucoid strain transformed into mucoid strain [20] This stress generates the mutation to happen on the genes which are related to the alginate production and controlled activation of the excess of the capsule like alginate. CFTR modulators theratyping is an innovative and fast growing field that has power to recognize rare CFTR variants that are reactive to drugs in development [30]
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