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This is particularly problematic for the lungs because the thick mucus impairs clearance of invasive particles and infectious agents to maintain a sterile environment.
Microbial contamination of airway surfaces triggers an inflammatory response, including a massive invasion by neutrophils (Rodrigues et al., 2008). As the neutrophils react to bacterial contamination of the lung tissue they generate considerable amounts of cellular debris that increases the viscosity of the mucous, primarily by the deposition of genomic DNA and elastase proteins. The result of this process is chronic microbial lung infection, chronic immune-mediated inflammation, and progressive tissue damage. Because cystic fibrosis lung disease is so severe the life expectancy used to be late teens or early adulthood, but the development of more effective methods for disease management has almost doubled life expectancy in recent years (Cystic Fibrosis Foundation, n.d.).
Haematology
There is increasing evidence that CFTR is expressed in polymorphonuclear leukocytes and may be required for proper phagosome acidification and bacterial killing (Conese et al., 2011). Impaired phagolysosomal function would explain why microbial lung infections are resistant to an immune response. In addition, platelets play an important role in haemostasis and may mediate some aspects of an inflammatory response. In individuals with cystic fibrosis, platelets display a number of defects and secrete proteins that signal the end of an inflammatory response. These findings have caused some researchers to debate whether stem cell therapy might be an effective treatment for cystic...
Currently, the most promising approach would be to use bone marrow mesenchymal cells to reconstitute 'wild type' function to the lung epithelium. The benefits would be reducing mucus viscosity and inflammation, and promoting tissue repair and an effective immune response.
References
Conese, Massimo, Ascenzioni, Fiorentina, Boyd, a. Christopher, Coutelle De Fino, Charles, de Smedt, Stefaan, Rejman, Joanna et al. (2011). Gene and cell therapy of cystic fibrosis: From bench to bedside. Journal of Cystic Fibrosis, 10, S114-S128.
Cystic Fibrosis Foundation. (n.d.). About Cystic Fibrosis: What you need to know. CFF.org. Retrieved 26 Feb. 2012 from http://www.cff.org/AboutCF/.
Li, Chunying and Naren, Anjaparavanda P. (2005). Macromolecular complexes of cystic fibrosis transmembrane regulatory and its interacting partners. Pharmacology & Therapeutics, 108, 208-223.
Rodrigues, Roberta, Gabetta, Carmen S., Pedro, Karla P., Valdetaro, Fabio, Fernandes, Maria I.M., Magalhaes, Patricia K.R. et al. (2008). Cystic fibrosis and neonatal screening. Cadernos Saude Publica, 24, s475-484.
Cystic Fibrosis (CF) is genetically inherited through a defective gene, which results in the body producing "abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb food." (PubMed Health, 2011) Reports state that millions of Americans carry the defective Cystic Fibrosis gene however; most do not have any symptoms since
Cystic Fibroids Cystic fibrosis Cystic fibrosis is a disease that can be passed down from one generation to the other. It affects secretary glands that produce mucus and sweat. The disease results after the fibrosis transmembrane conductance regulator (CFTR) gene that is found on chromosome 7 has undergone some sort of mutation. Mutation on chromosome 7 alters the production and function of CFTR glycoprotein (Scott, 2013). Studies have identified more than 1600
Cff.org/will each be the source of information and professional peer reviewed articles will be cited from these sources and identified by source as they cited. There is a wealth of available information, data and studies on CF. What it all means to the patients who suffer from this debilitating and life-threatening disease will be understood as this essay proceeds. Chapter One Diagnosis and the Anatomy and Physiology of a Life Threatening Disease: Cystic
cystic fibrosis. There are eleven references used for this paper. There are a number of fatal diseases which a person can be born with in the world today. One of the most debilitating is Cystic Fibrosis. It is important to examine its history, symptoms, diagnostic procedures, treatments and prognosis in order to gain a better understanding of this disease. Cystic fibrosis is a "recessive disorder common among Caucasians. Although the disease
cystic fibrosis. The writer takes the reader on an exploratory journey about cystic fibrosis and its causes, treatments and future. The writer outlines many aspects of the disease including the enzyme treatments that are currently being studied. There were five sources used to complete this paper. The medical community has made many amazing discoveries in the last few decades. People are living longer than ever before and the quality of
Introduction Cystic fibrosis is a genetic condition with a primary symptom of excessive mucous production and resulting lung infections. It is “the most common fatal hereditary lung disease,” (Mall & Hartl, 2014, p. 1042). Symptoms and severity of the disease varies, but all cases are inherited. On the genetic level, cystic fibrosis is an “autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR),”