CYSTIC FIBROSIS ESSAYS

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cystic fibrosis Essays
cystic fibrosis Essays
NSG-530-IKC – Advanced Pathophysiology
Module 2: Discussion
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Melissa, a 12-year-old girl with cystic fibrosis comes to the primary care office with complaints of increased cough and productive green sputum over the last week. She also complains of increasing shortness of breath. She denies sore throat or nasal congestion. On physical examination her temperature is 101 and she has inspiratory wheezes bilaterally. Negative lymphadenopathy noted. Posterior pharynx is pink without exudate. BP 112/72 HR 96 RR 28 cystic fibrosis Essays.
In cystic fibrosis, the airway microenvironment favors bacterial colonization. In a minimum of 150 words explain the pathophysiological reason for this occurrence.
Post your initial response by Wednesday at midnight. Respond to one student by Sunday at midnight. Both responses must be a minimum of 150 words, scholarly written, APA formatted, and referenced. A minimum of 2 references are required (other than your text). Refer to grading rubric for online discussion.
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Module 2 DiscussionSubscribe
Hilary Szpara posted Jan 27, 2021 3:28 AM
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The lungs of a person with cystic fibrosis are saturated with sticky mucus, which makes it easier for bacteria to colonize there. The reason that mucus is able to accumulate in the lungs so easily is due to the mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene (Melnik et al., 2019). This mutation of the CFTR gene disrupts the function of the CFTR protein, an ion channel in the lungs which helps to regulate the volume of liquid, in this case mucus, on epithelial cells (Melnik et al., 2019). When the ion channel’s function is disrupted, there is a reduction in chloride secretion and an increase in sodium absorption (Rossi, 2019).  This malfunction in ion movement reduces the thickness of the periciliary layer, which usually acts as a lubricating layer and allows the normally present mucus to move more easily. However, when the thickness of the periciliary layer is decreased, excess mucus is able to build up because the cilia are no longer able to move it out of the lungs (Rossi, 2019). When this excess amount of mucus builds up in the lungs, the ability of the lungs to properly clear out bacteria is diminished (Bhagirath et al., 2016).  Rossi, G. A, Morelli, P., Galietta, L. J., Colin, A. A. (2019). Airway microenvironment alterations and pathogen growth in cystic fibrosis. Pediatric Pulmonology, 54(4). https://doi-org.wilkes.idm.oclc.org/10.1002/ppul.24246less1 UnreadUnread5 ViewsViews
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View profile card for Sheryl Dixon
Last post February 7 at 2:45 PM by Sheryl Dixon
Melnik, A. V., Vazquez-Baeza, Y., Aksenov, A., A., Hyde, E., McAvoy, A. C., Wang, M., da Silva, R. R., Protsyuk, I., Wu, J. V., Bouslimani, A., Lim, Y. W., Luzzato-Knaan, T., Comstock, W., Quinn, R. A., Wong, R., Humphrey, G., Ackermann, G., Spivey, T., Brouha, S. S., Bandeira, N., Lin, G. Y., Rohwer, F., Conrad, D. J., Alexandriv, T., Knight, R., Dorrestein, P. C., Garg, N. (2019). Molecular and microbial microenvironments in chronically diseased lungs associated with cystic fibrosis. mSystems, 4(5), 1-16. https://doi.org/10.1128/ mSystems.00375-19
Bhagirath, A. Y., Li, Y., Somayajula, D., Dadashi, M., Badr, S., & Duan, K. (2016). Cystic fibrosis lung environment and Pseudomonas aeruginosa infection. BMC pulmonary medicine, 16(1), 174. https://doi.org/10.1186/s12890-016-0339-5
In healthy lungs, the mucus layer that is present is thin and works with the cilia to carry bacteria that are inhaled back up to the pharynx so that they can be swallowed (Bhagirath et al., 2016). Defects in CFTR are also associated with less efficient recognition and killing of bacteria, lower expressions of cytokines which promote innate immune responses, and lower production of anti-inflammatory proteins (Rossi, 2019). All of these factors together help to create an environment in which bacteria that would normally be expelled from the body or swallowed, are essentially trapped in the thick, sticky mucus, where they are able to thrive and lead to chronic bacterial infections (Melnik et al., 2019) cystic fibrosis Essays.
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Sheryl Dixon posted Jan 27, 2021 5:40 PM
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Cystic Fibrosis (CF) is an autosomal recessive disorder that affects the lungs, digestive tract and reproductive organs .CF affects mostly the gastrointestinal (GI) and respiratory systems. The hallmark pathophysiology triad of CF is obstruction, inflammation and infection that are evident throughout the GI tract and within the airways (Heuther, McCane, &Brashers, 2020).         With Melissa complaining of increased coughing and productive green sputum and increased shortness of breath, I saw where in Stanford, Ryan & Solis-Mayo ,2020, stated that CF affects the cells that produce mucus, sweat and digestive juices .It causes these fluids to become thick and sticky which plug the tubes ,ducts and passageways ,traps germs and makes infections more likely.                                                                                                ReferencesHuether, S. E., & McCance, K. L. & Brashers, V.L. (2020). Understanding Pathophysiology 7th ed. Elsevier Mosby.Stanford, G., Ryan, H., & Solis-Moya, A. (2020). Respiratory muscle training for cystic fibrosis. The Cochrane database of systematic reviews, 12, CD006112. Retrieved from https://doi.org/10.1002/14651858.CD006112.pub5less3 UnreadUnread12 ViewsViews
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View profile card for Caroline Otto
Last post January 31 at 11:12 PM by Caroline Otto
Rossi A.G, Morrelli, P., Galietta ,L.J & Collin, A.A. (2019) Airway microenvironment alterations and pathogen growth in cystic fibrosis. Retrieved from https://doi.org/10.1002/ppul.24246
        In CF Treatment can help but there is no cure .Respiratory training for CF is one of the intervention, but study still think the intervention is insufficient and that it should be a case by case basis .Treatment is primarily focus on pulmonary health and nutrition and prevention of persistent cycles of lung infection and inflammation ( Heuther, McCane, &Brashers, 2020).
     The complex interplay between the virulent organisms & predominantly Pseudomonas aeruginosa including up regulation of virulence genes and utilization of mucin as a nutrient source, modulates the action of pathogens, modifies the CF airway milieu and contributes to the processes leading to airway derangement (Rossi, et.al.2019).
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Caroline Otto posted Jan 27, 2021 10:52 PM
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At the center of Cystic fibrosis is the gene mutation CFTCR (cystic fibrosis transmembrane conductance regulator protein), which prevents chloride and water from not being transported correctly across epithelial membranes, specifically found within the lining the inside of our lungs.  The gene mutation causes the lungs to have the inability of clear secretions effectively, thereby speeding up the disease process of Pneumonia.  Huether, S. E., McCance, K. L. & Brashers, V. L. (2020). Understanding Pathophysiology 7th ed. Elsevier Mosby. less2 UnreadUnread5 ViewsViews cystic fibrosis Essays
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View profile card for Caroline Otto
Last post January 31 at 10:01 PM by Caroline Otto
Rossi, G. A., Morelli, P., Galietta, L. J., & Colin, A. A. (2019). Airway microenvironment alterations and pathogen growth in cystic fibrosis. Pediatric pulmonology, 54(4), 497–506. https://doi.org/10.1002/ppul.24246
Ciofu, O., Hansen, C. R., & Høiby, N. (2013). Respiratory bacterial infections in cystic fibrosis. Current opinion in pulmonary medicine, 19(3), 251–258. https://doi.org/10.1097/MCP.0b013e32835f1afc
References:
The majority of Cystic Fibrosis patients have a bacterial colonization of Pseudomonas (Ciofu, et.al. 2013). Pseudomonas bacterial lies dominantly within these patients’ lungs, and during an activated cycle these patients will produce green sputum which is the clinical sign for Pseudomonas (Rossi, et.al. 2019).   Aggravated external conditions and overgrowth of the bacterial neighbors produce broncho-constriction and friction causing difficulty breathing and further inflammation.
Our lungs are an enclosed organ filled with little pockets, small airways cystic fibrosis Essays. The inability of the cilia to move in unison with ventilation prevents the clearance of mucous and secretions thereby causing an environment that is warm, wet and favoring of a microenvironment colonized by opportunistic bacterias  Since this environment is one that is continuously imunno-activated, because of induced inflammatory mediators, that destroy immunoglobulin G (IgG), there is an abundance of neutrophils which release oxidants and proteases that directly damage the lung proteins. This direct damage is reflected by a bacterial film that develops over time that resists beta-lactam antibiotics, and causes a constant infection, which feeds the bacterial community now living inside the lungs (Huether, et.al. Pg. 708, 2020).
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Jennifer Bryant posted Jan 26, 2021 2:32 PM
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Cystic fibrosis (CF) is a progressive genetic disease that causes persistent airway infection. This is caused by a gene mutation that affects the functional deficiency of the cystic fibrosis transmembrane regulator (CFTR). Mutations in the CFTR gene affects epithelial innate immune function and airway clearance of the lung. CFTR functions as a chloride channel where it is highly expressed where is regulates chloride ion movement. Defective transport leads to an imbalance, creating thick dehydrated mucus (Useckaite et al, 2020).References     Pathophysiology (7th ed.).  Elsevier.     neutrophilic inflammation in cystic fibrosis. J Leukoc Biol. 2020; 108: 1777– 1785.     Cassidy, H., Dillon, E. T., Brennan, K., Doyle, S. L., Carter, S., Donnelly, S., Linnane,vesicles mediate inflammatory signalling in cystic fibrosis. Thorax, 75(6), 449-458.less1 UnreadUnread4 Views Views cystic fibrosis Essays
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View profile card for Melissa Morgan
Last post January 31 at 9:57 PM by Melissa Morgan
     B., McKone, E. F., McNally, P., & Coppinger, J. A. (2020). Increased extracellular
Useckaite, Z., Ward, M. P., Trappe, A., Reilly, R., Lennon, J., Davage, H., Matallanas, D.,
Ng, HP, Jennings, S, Wellems, D, et al. Myeloid CFTR loss‐of‐function causes persistent
Heuther, S., McCance, K., and Brashers, V.  (2020).  Understanding
            In CF lungs, the predominant inflammatory cells are neutrophils. Neutrophils are dedicated microbial killer cells that phagocytose invading organisms. An excessive presence of neutrophils mounts an excessive inflammatory response, a vicious cycle causing damage to the airways (Ng et al, 2020). This microenvironment favors bacteria colonization by creating a bacterial biofilm that promotes chronic endobroncial infection (Huether et al, 2020). Melissa presents with common clinical manifestations of bronchiectasis: increased cough, productive green sputum, wheezing and fever.
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Dennies Jones posted Jan 25, 2021 7:39 PM
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Cystic fibrosis, the most common lethal recessive disease in white children, occurs in about 1 in 2500 births. Approximately 1 in 25 whites carry a copy of a mutation that causes cystic fibrosis (Huether et al. 2020). Defective transport of chloride ions leads to a salt imbalance, which results in secretions of abnormally thick, dehydrated mucus (Huether et al.2020). According to Andrade et al.(2018), cystic fibrosis is a progressive disease with lung function deterioration, malnutrition, and progressive exercise limitation. Moore and Mastoridis (2017) state that P.aeruginosa infection in the lower airways correlates with declining lung function. Andrade, R. C., da Silva e Silva, C. M., Diniz da Silva, A. L. L., Haun, S. R., de Souza, V. A., & Ezequiel, D. J. S. (2018). Comparison of respiratory muscle strength, quality of life, and functional capacity among adolescents with cystic fibrosis with different bacteriological profiles. Fisioterapia e Pesquisa, 25(2), 143–150. https://doi-org.wilkes.idm.oclc.org/10.1590/1809-2950/16862525022018Huether, Sue E., McCance, Kathryn L… Understanding Pathophysiology – E-Book (p. 169). Elsevier Health Sciences. Kindle Edition.Moore, J. E., & Mastoridis, P. (2017). Clinical implications of Pseudomonas aeruginosa location in the lungs of patients with cystic fibrosis. Journal of Clinical Pharmacy & Therapeutics, 42(3), 259–267. https://doi-org.wilkes.idm.oclc.org/10.1111/jcpt.12521less1 UnreadUnread8 ViewsViews
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View profile card for Aina Oluwo
Last post January 31 at 7:48 PM by Aina Oluwo
                    Inflammation of the airway obstructs the airflow, which causes inadequate ventilation. The impairment of pulmonary function is characterized by respiratory infection followed by colonization by bacteria (Andrade et al., 2018). Bacterial colonization is favorable due to its decreased lung function, nutrient-rich mucous pooled in oxygenated areas such as the respiratory tract, thus creating an optimal breeding environment for bacteria. This is a vicious cycle of bacterial infection in cystic fibrosis patients.
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Lois Chappell posted Jan 30, 2021 7:24 PM
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     Cystic fibrosis is an example of an inherited recessive disease; this means a person must have a mutation in both copies of the cystic fibrosis transmembrane conductance regulator (CFTR) gene (Cystic Fibrosis Foundation, 2020) to demonstrate the disease.  If a person is born with just one mutated CFTR gene, they are considered a carrier and may give the defective gene to their children (Cystic Fibrosis Foundation, 2020). References     Foundation cystic fibrosis Essays.     https://www.nih.gov/news-events/nih-research-matters/how-cystic-fibrosis-promotes-lung-infections.less1 UnreadUnread5 ViewsViews
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View profile card for Sheryl Dixon
Last post January 31 at 4:26 PM by Sheryl Dixon
Wein, H.  (2016).  How cystic fibrosis promotes lung infections.  National  Institutes of Health, Research Matters.  Retrieved from
Cystic Fibrosis Foundation (2020).  Cystic fibrosis genetics: the basics.  Retrieved from https://www.cff.org/About-Us/About-the-Cystic-Fibrosis-
     In people with cystic fibrosis disease, the CFTR protein, responsible for the regulation of salt and fluid movement in different areas of the human body is malfunctioning, or, not produced at all, which can  lead to a buildup of thick mucus, that is more acidic and prone to infections (Wein, 2016).  Destruction and malfunction of organs, as well as persistent lung infections can be seen in those diagnosed with cystic fibrosis (Cystic Fibrosis Foundation, 2020).  Medical therapy is aimed at thinning secretions, opening the airway, fighting infections, and aiding digestion (National Institutes of Health, 2020).  There is no cure for cystic fibrosis; pre-pregnancy screening, early diagnosis and treatment of affected individuals is essential to improve clinical outcomes and quality of life.
Module II Discussion Post: CFSubscribed
Gisselle Mustiga posted Jan 27, 2021 11:32 PM
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Cystic fibrosis affects at least 30,000 people in the United States. Between 900 -1000 new cases are detected every year cystic fibrosis Essays. One out of 29 people of Caucasian descent is a carrier not affected by the CF gene mutation. In the United States, cystic fibrosis occurs in 1 in 3,400 live births. It occurs in people of racial and ethnic backgrounds, but whites of Nordic descent are generally are more predisposed to this ailment. It is very common for Caucasians of North European origin.ReferencesFinn, K. M., & Mendenhall, F. (2020). Diagnosis of cystic fibrosis: consensus guidelines from the Cystic Fibrosis Foundation. The Journal of pediatrics, 181, S4-S15.less1 UnreadUnread4 ViewsViews
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View profile card for Jennifer Bryant
Last post January 31 at 4:06 PM by Jennifer Bryant
Trivedi, B. P. (2020). Breath from Salt: A Deadly Genetic Disease, a New Era in Science, and the Patients and Families Who Changed Medicine Forever. BenBella Books.
Historically, half of the affected people were diagnosed when they were five months old, but the average diagnosis age is five years, and some people are not diagnosed until they are adults (Finn & Mendenhall, 2020). Early in life, CF patients become infected with a limited spectrum of bacteria, especially P. aeruginosa. New studies now show that decreased depth of periciliary fluid and abnormal hydration of mucus, which impede mucociliary clearance, contribute to initial infection (Trivedi, 2020). Reduced production of the antibacterial molecule nitric oxide, increased bacterial binding sites (e.g., asialo GM-1) on CF airway epithelial cells, and adaptations made by the bacteria to the airway microenvironment, including the production of virulence factors and the ability to organize into a biofilm, contribute to susceptibility to initial bacterial infection. In short, in healthy lungs, there is a thin layer of mucus that helps your body move dirt and bacteria out of the lungs, while in people patients with CF, this mucus is much thicker and clogs the lungs, creating the perfect environment for harmful bacteria cystic fibrosis Essays.
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Eleany Yasein posted Jan 26, 2021 11:17 PM
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      Cystic fibrosis is an autosomal recessive disorder that can lead to impairment in different organs including lungs, intestines, as well as the pancreas (Fagundes, Vendrusculo & Perez-Ruiv, 2020). It’s also known to be a disease that tends to progress at a fast rate, and is a result of a gene mutation located in the chromosome 7. This chromosome is responsible for encoding the protein cystic fibrosis transmembrane regulator (CFTR). CFTR regulates the movement of many ions including sodium, chloride, and water across the cell membrane (Fagundes et al., 2020). The chromosome mutation affects the function of CFTR. This leads to the imbalance of osmolality and disrupted movement of ions, which affects the intracellular and extracellular movement of salt and water (Kulkarni, Kansra, & Karande, 2019). In the lung, the defective CFTR affects the normal lining of epithelial tissue and cilia that are there to protect the lung. As a result of that, thick mucus that’s sticky will build up, which makes it easier for bacteria to colonize, and difficult for airway clearance. Furthermore, normal inflammatory processes can not occur due to the imbalance of osmolality and the pH (Kiedrowski & Bomberger, 2018).   Fagundes Donadio, M. V., Vendrusculo, F. M., & Pérez-Ruiz, M. (2020). Scoring tools to monitor risk of disease progression in patients with cystic fibrosis. Journal of Thoracic Disease, 12(8), 3940–3943. https://doi-org.wilkes.idm.oclc.org/10.21037/jtd.2020.03.121Kulkarni, H., Kansra, S., & Karande, S. (2019). Cystic fibrosis revisited. Journal of Postgraduate Medicine. 65(4), 193-196. doi: 10.4103/jpgm.JPGM_263_18less1 UnreadUnread7 Views Views cystic fibrosis Essays
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View profile card for Beth Certain
Last post January 31 at 12:33 AM by Beth Certain
Kiedrowski, R. M., & Bomberger, M. J. (2018, December 20). Viral-Bacterial Co-infections in the Cystic Fibrosis Respiratory Tract. Frontiers in Immunology, 9(3067), doi: 10.3389/fimmu.2018.03067
                                                                             References
         Cystic fibrosis can cause pulmonary decline due to the cause of inflammation, hyperventilation, mucus being retained, as well as impediment of the airway. Moreover, this disorder can lead to chronic infections caused by bacteria including Staphylococcus aureus and Pseudomonas aeruginosa (Fagundes et al., 2020). The productive green sputum, and fever Melissa is having indicates signs of infection, that are caused by bacteria in the lung, due the progressive of cystic fibrosis. Lastly, the wheezing she is having also indicates airway obstruction, due to air trapping in the lung (Kulkarine et al., 2019).
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Alfonsina Perez posted Jan 26, 2021 9:59 PM
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             In patients with cystic fibrosis, the airway microenvironment favors bacteria colonization because the mutation in the CFTR can lead to several malfunctions at the cellular level. First, CFTR malfunction can lead to collapse of the cilia which are in charge of moving fluid through the cells (Ribeiro & Lubamba, 2017). Second, thick mucus may adhere to the airways which can then result in chronic inflammation (Ribeiro & Lubamba, 2017). Constant inflammation in the airways and the presence of thick mucus which is not easily moved in and out of the airway provides an ideal environment for bacteria to colonize in the airway of a person with cystic fibrosis.      less1 UnreadUnread6 ViewsViews
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View profile card for Steven Bartos
Last post January 30 at 11:41 PM by Steven Bartos
doi:http://dx.doi.org.wilkes.idm.oclc.org/10.3390/ijms18010118 cystic fibrosis Essays
inflammation. International Journal of Molecular Sciences, 18(1), 118.
Ribeiro, C. M. P., & Lubamba, B. A. (2017). Role of IRE1α/XBP-1 in cystic fibrosis airway
doi:10.1016/S0140-6736(16)00576-6
Elborn, J. S., Prof. (2016). Cystic fibrosis. The Lancet (British Edition), 388(10059), 2519-2531.
References
            In our case study for this week, we learn about a 12 year old girl with cystic fibrosis. Elborn (2016) defines cystic fibrosis as an autosomal recessive genetic disorder which affects mainly the respiratory system but can also affect other areas of the body including, pancreas, liver and sweat glands. Cystic fibrosis is caused by a gene mutation, this particular gene is in charge of encoding a chloride conducting membrane which regulates mucus in the airways (Elborn, 2016). As a result of the gene mutation, the cystic fibrosis transmembrane conductance regulator (CFTR) does not effectively regulate mucus production and inflammation which can lead to an over production of mucus affecting epithelial cells (Elborn, 2016).
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Steven Bartos posted Jan 27, 2021 3:22 PM
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The pathophysiology of the microenvironment that favors bacterial colonization in cystic fibrosis can be traced down to the genetic level. Cystic fibrosis is a monogenetic autosomal recessive disease caused by a mutated gene, called the cystic fibrosis transmembrane conductance regulator (CFTR). This gene regulates the chloride ion channel in epithelial cells. When the CFTR dysregulates the chloride ion channel, this leads to an imbalance of salt and subsequent thickened mucus and viscous secretions in the bronchi, pancreas, biliary tract, and intestine. Although mucus provides antibacterial defense for the body, its increased viscosity in cystic fibrosis contributes to the harboring of bacteria (De Palma et al., 2020) cystic fibrosis Essays. The airway surface liquid (ASL) is fluid that covers the surface of the airway epithelium. It assists in clearing out bacteria of the airway and overall homeostasis of that environment. When the volume, pH, ionic or nutrient content of the ASL is impaired, this results in dysregulation of antimicrobial activity and leads to subsequent bacterial infection (Haq et al., 2016). New research is looking at how CFTR also regulates a bicarbonate chan

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