Cystic fibrosis causes

Tobramycin is similar to gentamicin it is more active against most strains of Pseudomonas aeruginosa and may be less nephrotoxic. It is commonly administered via a nebulizer for treatment of infective exacerbations of cystic fibrosis caused by pseudomonads or Enlerobacteriaceae. 

Disorders caused by misfolded mutant proteins that fail to pass the quality control system of the ER (e.g., mutations of the cystic fibrosis transmembrane regulator protein (CFTR) causing cystic fibrosis). The mutant proteins are retrotranslocated into the cytosol and finally subjected to proteolysis. In some... 

These drug are prescribed as replacement therapy for those with pancreatic enzyme insufficiency. Conditions or diseases that may cause a decrease in or absence of pancreatic digestive enzymes include cystic fibrosis, chronic pancreatitis, cancer of the pancreas,... 

Mutations that affect the structure of membrane proteins (teceptots, ttanspotters, ion channels, enzymes, and stmctutal proteins) may cause diseases examples include cystic fibrosis and familial hypetcholes-terolemia. 

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR), a chloride (CF) channel characterised by chloride permeability and secretion, and also by the regulation of other epithelial ion channels (Eidelman et al, 2001). Mutations in the CFTR gene lead to an impaired or absent Cl conductance in the epithelial apical membrane, which leads to defective Cl secretion and absorption across the epithelium. Genistein (Illek et al, 1995 Weinreich et al, 1997) and other flavonoids (Illek and Fisher, 1998) have been shown, in different animal and tissue models, to activate wild-type CFTR and CFTR mutants by (Eidelman et al, 2001 Roomans, 2001 Suaud et al, 2002) ... 

The incidence of chronic pancreatitis is approximately 1 in 10,000 people. O The most common cause of chronic pancreatitis in adults in Western countries is ethanol abuse. The most common cause in children is cystic fibrosis, due to preexisting pancreatic insufficiency inherent in the disease. Gallstones can occur at the same time as chronic pancreatitis but are not often implicated as the cause. Unlike acute pancreatitis, chronic pancreatitis has an unknown etiology in a significant number of cases (30%).29,3°... 

Damage to connective caused by leakage of elastases leads to damage associated with inflammatory diseases, such as pulmonary emphysema, adult respiratory distress syndrome, septic shock, cystic fibrosis, carcinogenesis, chronic bronchitis, and rheumatoid arthritis. Compounds that directly inhibit elastase or its release from human neutrophils are of enormous pharmaceutical and cosmetological interest in the development of new anti-inflammatory drugs. A possible source for elastase inhibitors are the medicinal Asteraceae and Droseraceae, particularly those used as traditional medicine in Asia. 

The pancreas, in which the mucus blocks its ducts in 85 per cent of cystic fibrosis patients, causing pancreatic insufficiency. This is chiefly characterized by secretion of greatly reduced levels of digestive enzymes into the small intestine. 

Pancreatin is a pancreatic extract usually obtained from the pancrease of slaughterhouse animals. It contains a mixture of enzymes, principally amylase, protease and lipase, and, thus, exhibits a broad digestive capability. It is administered orally mainly for the treatment of pancreatic insufficiency caused by cystic fibrosis or pancreatitis. As it is sensitive to stomach acid, it must be administered in high doses or, more usually, as enteric-coated granules or capsules that may be taken directly or sprinkled upon the food prior to its ingestion. Individual digestive activities, such as papain, pepsin or bromelains (proteases), or a-amylase are sometimes used in place of pancreatin. 

A family medical history can identify people with a higher-than-usual chance of having common disorders, such as heart disease, high blood pressure, stroke, certain cancers, and diabetes. These complex disorders are influenced by a combination of genetic factors, environmental conditions, and lifestyle choices. A family history also can provide information about the risk of rarer conditions caused by mutations in a single gene, such as cystic fibrosis and sickle cell anemia. 

Exhibits 2.2,2.3, and 2.4 provide examples of genetic causes of diseases, for example, cancer, sickle cell anemia, and cystic fibrosis. It should be noted that although some of these diseases are the result of mutations in a single gene (including Huntington s disease and Duchenne muscular dystrophy), most are due to the influence of multiple genes. 

CF is caused by the absence of a protein called cystic fibrosis transmembrane conductance regulator (CFTR). This protein is required for the transport of chloride ions across cell membranes. On the molecular level, there is a mutation in the gene that encodes for CFTR. As a result, CFTR cannot be processed properly by the cell and is unable to reach the exocrine glands to assume its transport function. 

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