Includes bibliographical references and index.
|Statement||[edited by] Paul N. Leatte.|
|Contributions||Leatte, Paul N.|
|LC Classifications||RC858.C95 C94 2009|
|The Physical Object|
|LC Control Number||2009021727|
ISBN: OCLC Number: Description: xv, pages: illustrations (some color) ; 27 cm. Contents: Newborn screening for cystic fibrosis / Anne Deucher and Iris Schrijver --Cystic fibrosis and respiratory viral infections / Dennis Wat --Cystic fibrosis, pseudomonas aeruginosa infection, and relevance of innate immune response: challenge for . Cystic fibrosis (CF) is the most common life-shortening autosomal recessive disease among Caucasian populations, with a frequency of 1 in to live births. The median predicted survival for CF patients in the United States was years (95% CI, ) according to the Cystic Fibrosis Foundation Registry Report [ 1 ]. The affected gene is the cystic fibrosis transmembrane regulator (CFTR), which when mutated produces an abnormal production of the cystic fibrosis transmembrane regulator protein . Cystic fibrosis (CF) is a severely life-shortening genetic disease resulting from abnormalities in the cystic fibrosis transmembrane conductance regulator (CFTR), a chloride channel found in cells lining the lungs, intestines, pancreatic ducts, sweat glands, and reproductive organs.
Cystic fibrosis; etiology, diagnosis and treatments. Ed. by Paul N. Leatte. Nova Biomedical Books pages $ Hardcover Genetics--research and issues series RC Specialists in the hereditary degenerative disease review recent research findings into a number of aspects relating to it. of these treatments still needs to be established, but early studies look promising for several compounds. This review summarizes our current understanding of the pathophysiology and treat-ment of cystic fibrosis lung disease. Key words: cystic fibrosis, airway surface liquid, cystic fibrosis. ISBN: OCLC Number: Description: 1 online resource (xv, pages): illustrations (some color). Contents: Newborn screening for cystic fibrosis / Anne Deucher and Iris Schrijver --Cystic fibrosis and respiratory viral infections / Dennis Wat --Cystic fibrosis, pseudomonas aeruginosa infection, and relevance of innate immune response: . Cystic fibrosis clearly poses a huge burden to patients and families in terms of the life shortening nature of the disease, the time consuming treatments prescribed, and the ongoing morbidity. Times of particular stress include diagnosis, adolescence (when adherence to treatment can often be poor), and end of by:
Cystic fibrosis is an inherited disease caused by mutations in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR) CFTR gene provides instructions for the CFTR protein. The CFTR protein is located in every organ of the body that makes mucus, including the lungs, liver, pancreas, and intestines, as well as sweat glands.. The CFTR . Diagnosis is by sweat test or identification of 2 cystic fibrosis-causing mutations in patients with a positive newborn screening test result or characteristic clinical features. Treatment is supportive through aggressive multidisciplinary care along with small-molecule correctors and potentiators targeting the cystic fibrosis transmembrane. Cystic Fibrosis What Is Cystic Fibrosis Cystic fibrosis (CF) is a chronic, progressive, and frequently fatal genetic (inherited) dis ease of the body’s mucus glands. CF pri marily affects the respiratory and digestive systems in children and young adults. The sweat glands and the reproductive system are also usually involved. On the average. According to the Cystic Fibrosis Foundation Patient Registry, in the United States: More t people are living with cystic fibrosis (more t worldwide). Approximately 1, new cases of CF are diagnosed each year. More than 75 percent of people with CF are diagnosed by age 2. More than half of the CF population is age 18 or older.