Cystic Fibrosis

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.

  1. 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 medicine16(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 FibrosisSubscribe
  • 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 reviews12, 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).
  • Cystic FibrosisSubscribe
  • 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
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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. 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).
  • Discussion Post 2 – Cystic FibrosisSubscribe
  • 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 ViewsViews
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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.
  • Dennies JonesSubscribe
  • 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 Pesquisa25(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 & Therapeutics42(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.
  • Melissa-Module 2 Cystic FibrosisSubscribe
  • 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.     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 HealthResearch 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. 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.
  • Eleany Yasein, Module 2Subscribe
  • 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 Disease12(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 ViewsViews
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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).
  • Module 2 DiscussionSubscribe
  • 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
  • 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).
  • Module 2 DiscussionSubscribe
  • 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). 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 channel, which affects the exchange of chloride and bicarbonate. This exchange is also shown to help regulate the pH levels and overall homeostasis of the epithelial cells in the ASL, as an imbalance would result in worsening of bacterial colonization in the airways of cystic fibrosis (Borowitz, 2015).Borowitz, D. (2015). CFTR, bicarbonate, and the pathophysiology of cystic fibrosis. Pediatric Pulmonology50(S40), S24 – S25. https://dx.doi.org/10.1002/ppul.23247 Haq, I.J., Gray, M.A., Garnett, J.P., Ward, C., & Brodlie, M. (2016). Airway surface homeostasis in cystic fibrosis: Pathophysiology and therapeutic targets. Thorax71(3), 284 – 287. http://dx.doi.org/10.1136/thoraxjnl-2015-207588  less1 UnreadUnread5 ViewsViews
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  • View profile card for Jazmin Jerez-Rivera
  • Last post January 30 at 12:29 PM by Jazmin Jerez-Rivera
  • De Palma, F.D.E., Raia, V., Kroemer, G., Maiuri, M.C. (2020). The multifaceted roles of microRNAs in cystic fibrosis. Diagnostics (Basel, Switzerland)10(1102), 1 – 19. https://dx.doi.org/10.3390/diagnostics10121102
  • References
  • Cystic FibrosisSubscribe
  • Jazmin Jerez-Rivera posted Jan 26, 2021 7:14 PM
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  • Cystic fibrosis is a genetic disorder that affects the cystic fibrosis transmembrane conductance regulator (CFTR) gene (CDC, 2020). A mutation to this gene causes mucus to buildup and thicken in different parts of the body. The systems primarily affected are gastrointestinal, respiratory, and genitourinary systems This thickened mucus can begin to clog pancreatic and biliary ducts which blocks enzymes to break down food and can interfere with nutrient absorption (Brown et.al., 2017). It can also cause greasy and bulky stool.ReferencesKeep them breathing, Journal of the American Academy of Physician Assistants, Volume 30, https://www.cdc.gov/genomics/disease/cystic_fibrosis.htmless1 UnreadUnread4 ViewsViews
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  • View profile card for Dennies Jones
  • Last post January 29 at 7:20 PM by Dennies Jones
  • Centers for Disease Control and Prevention. (2020). Cystic Fibrosis.
  • Issue 5, 23-27 doi: 10.1097/01.JAA.0000515540.36581.92
  • Brown, Sheena D. PhD, MSCR; White, Rachel MMSc, PA-C; Tobin, Phil DHSc, PA-C. (2017).
  • In the respiratory system mucus can increase in the airway of the lungs which can be difficult to clear. This is a constant breeding ground for bacteria which produces a hyperactive inflammatory response (Brown et.al., 2017). In the long run, chronic infections persist causing pulmonary exacerbations, and the continual inflammatory response can lead to airway destruction (CDC, 2020).
  • Module II Discussion ISubscribe
  • Beth Certain posted Jan 27, 2021 10:14 PM
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  • In cystic fibrosis, the airway microenvironment favors bacterial colonization, explains the pathophysiological reason for this occurrence. The patient, Melissa, has a respiratory infection caused by the complication of thick, sticky mucous from CF. Treatment for these infections varies depending on the patient and their history of CF. Various antibiotics are used to treat respiratory infections. These are used for acute infections. Some patients will be on prophylactic therapy to help prevent life-threatening infections.Bhagirath, A.Y., Somayajula, D., Dadashi, M., Badr. S., & Duan, K. (2016). Cystic fibrosis lungRosenstein, B. (2020). Cystic Fibrosis. Merke Manual Professional Versionless1 UnreadUnread3 ViewsViews
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  • Last post January 29 at 5:56 PM by Cassie Fritzinger
  •             https://www.merckmanuals.com/professional
  • environment and Pseudomonas aeruginosa infection. BMC Pulmonary Medicine, 16(1), 174. https://doi.org/10.1186/s12890-016-0339-5
  • References
  • Module 2Subscribe
  • Joanne Hogan posted Jan 26, 2021 6:12 PM
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  • Module 2            Being an autosomal recessive disease, the gene is often seen equally in male and female populations. Parents can carry the gene and not always express it but can still pass it on to their children. Roughly ¼ of children from carrier parents will be affected with the disorder (Heuther et al., 2020, pp 54-55). Huether, S. E., McCance, K. L., & Brashers, V. L. (2020). Understanding pathophysiology (7th ed.). Elsevier.Smith, W. D., Bardin, E., Cameron, L., & Edmondson, C. (2017). Current and future therapies for Psuedomonas aeruginosa infection in patients with cystic fibrosis. FEMS Microbiology Letters364(14). Retrieved January 25, 2021, from https://doi.org/10.1093/femsle/fnx121less1 UnreadUnread6 ViewsViews
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  • Last post January 28 at 2:33 PM by Tallona Boddy
  • Rout-Pitt, N., Farrow, N., Parsons, D., & Donnelley, M. (2018). Epithelial mesenchymal transition (emt): A universal process in lung diseases with implications for cystic fibrosis pathophysiology. Respiratory Research19(1). Retrieved January 25, 2021, from https://doi.org/10.1186/s12931-018-0834-8
  • References
  •             Cystic fibrosis is a genetic disorder that primarily occurs due to mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTCR) (Rout-Pitt et al., 2018). This gene is responsible for chloride ion transport out of epithelial cells and the mutation that occurs in cystic fibrosis disrupts this transport of chloride ions leading to dehydration of the respiratory tract (Rout-Pitt et al., 2018). Sodium is also absorbed in unregulated amounts by the epithelial sodium channel further leading to dehydration in the respiratory tract (Smith, et al., 2017). Dehydration in airway epithelium leads to chronic inflammation, risk of infection, and the formation of fibrotic tissues (Rout-Pitt et al., 2018).  The dehydrated epithelium causes an increase in viscous secretions that become difficult to clear (Smith et al., 2017). Because the airways have such a high mucus content, this inhibits the body’s ability to clear pathogens from the respiratory tract. One of the more common infections in patients with cystic fibrosis is Pseudomonas aeruginosa, which often occurs in children with CF by the age of five (Smith et al., 2017). According to Smith et al. (2017), the normal response of neutrophils during infection often leads to an increased inflammatory response, reducing clearance of the pathogen and creating a cycle that promotes a difficulty to clear the infection and increase the likelihood of reinfection by the same organism.
  •             Cystic fibrosis is an autosomal recessive disease that primarily effects the respiratory and gastrointestinal systems in the body (Huether et al., 2020, pp 54-55).  The lungs and pancreas are primarily affected due to an increase in mucus production leading to clogged pancreatic ducts and lung infections (Huether et al., 2020, pp 54-55). Patients may experience early death due to heart failure or lung disease (Heuther et al., 2020, pp 54-55).
  • Fritzinger, NSG 530 Wk 2Subscribe
  • Cassie Fritzinger posted Jan 27, 2021 12:56 PM
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  • Cystic Fibrosis (CF) is an autosomal recessive disease and is the most common fatal genetic disorder. For a person to develop cystic fibrosis both parents must either have or be a carrier of the mutated gene. The disease is characterized as a single gene mutation of the Transmembrane Conductance Regulator Gene (CFTR). The gene protein is located on every body organ which produces mucus, and the mutation inhibits the protein preventing the release of chloride.  Increased chloride levels cause the mucus produced to be thick and sticky, which in turn blocks passages commonly in the lungs and digestive system. This mucociliary clearance is hindered in clearing particles such as bacteria found in the nose and mouth out of the body to get rid of them (NIH, 2020). This disease causes severe damage to the lungs, digestive system and other organs of the body, and improved care has increased the life expectancy of the patient with CF with some living into their 50s (Mayo Clinic, 2020).Patients who have CF develop a biofilm which can be resistant to antibiotics thus any bacteria which would normally be killed by the antibiotics now collect in this localized area causing a chronic local inflammatory response and causing damage to the airway tissue which is second line of defense to infection (Heuther, 2020). While there is no cure for CF, some of the treatment goal is to prevent infections by loosening the mucus from the lungs, treating and preventing intestinal blockages which can be accomplished by many different means such as gene mutation medications,  improved hydration, and organ/disease specific medications. Keeping active, and maintaining a healthy diet and lifestyle is also very important in the prevention of infection for the CF patient (Mayo Clinic, 2020). Heuther, S. E., McCance, K. L., & Brashers, V. L. (2020). Understanding Pathophysiology (7th ed.). Elsevier.NIH. (2020). National Heart, Lung, and Blood Institute, Cystic fibrosis. Retrieved January 27, 2021. From https://www.nhlbi.nih.gov/health-topics/cystic-fibrosisless1 UnreadUnread6 ViewsViews
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  • Last post January 28 at 12:49 PM by Candice Russell
  • Mayo Clinic. (2020). Cystic Fibrosis. Retrieved January 27, 2021. From https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700
  • References
  •  
  • “Colonization is the ability of a pathogenic microorganism to survive and multiply on or within the human environment” (Heuther, 2020, pg. 197). Microorganisms which are infectious to humans and affect the mucus membranes such as the lungs include bacteria, fungi, and protozoa. Normal microbiome is present on our body surfaces and are colonized. These normal microbiomes normally do not cause disease. However, under certain circumstances they can produce an infectious reaction known as an opportunistic infection. This is when the normally ‘normal bacteria/fungi’ become infectious secondary to a breakdown in the normal body defenses or in an immunocompromised individual (Heuther, 2020).
  • Module 2 CF Discussion PostSubscribe
  • Candice Russell posted Jan 27, 2021 2:26 PM
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  • Cystic Fibrosis (CF) is an autosomal recessive inherited disease that results from defective epithelial chloride (Cl) ion transport. CF is described as a “multiorgan disease, affecting the lungs, digestive tract, and reproductive organs” (Brashers, 2020, p. 708). CF is caused by mutations, or errors, in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CFTR functions as a channel on the cell surface, allowing movement of chloride in and out of the cell. Proper functioning of the CFTR protein, allows for normal balance of Cl and fluid at the cell surface. Mutation of the CFTR gene results in abnormal expression of cystic fibrosis transmembrane conductance regulator (CFTCR) protein (Brashers, 2020, p. 709).In a review of inflammatory mechanism in airway diseases, Aghasafari et al. (2019) stated “Mutations in CFTR influence the lung epithelial innate immune function that leads to exaggerated and ineffective airway inflammation that fails to abolish pulmonary pathogens.” Many resources emphasized the importance of understanding the airway environment and its structure, in order to understand CF respiratory symptoms. Surette (2014) stated upper airways, particularly the oral cavity, represent the most diverse microbiome site in the body (Stearns et al., 2011, as cited by Surette 2014). The mutation of the CFTR gene, which results in reducing airway surface fluids, compromises normal airway function. Surette (2014) states altered airway milieu results in a thick mucus secretion that impairs mucociliary clearance, “consequently the lower airways become colonized by bacteria” (p.61). Bhagirath et al. (2016) state CF patients present with abnormalities in the size and shape of the trachea, imaging of children displayed early structural defects. Pulmonary imaging of airways in infants and young children, with CF, displayed thicker walls, with higher dilation (Bhagirath et al., 2016). Bhagirath et al. (2016) further state these airways make “excellent reservoir for chronic and relapsing lower respiratory tract infections.Neutrophils, a type of white blood cell (WBC), has many functions, one being, protecting against infections. Neutrophils are considered the first line of defense when protecting us from infection. Margaroli and Tirouvanziam (2016) state “Neutrophils are generally considered to be rigid, pre-programmed effector leukocytes, in the case of CF, neutrophiles, “show complex properties that come in stark contrast with rigid pre-programmed phenotype.” Margaroli and Tirouvanziam (2016) described CF lungs to be a “hostile environment”, the presence of bacterial toxins may induce rapid necrosis of incoming neutrophils. Laval et al. (2016) documented “absence or dysfunction of CFTR in neutrophils impairs bacterial killing” (Painter et al., 2006; Bonfield et al., 2012, Ng et al., 2014; as cited by Laval et al., 2016).                                   Rossi, GA, Morelli, P, Galietta, LJ, Colin, AA. Airway microenvironment alterations and pathogen growth in cystic fibrosis. Pediatric Pulmonology. 2019; 54: 497– 506. https://doi-org.wilkes.idm.oclc.org/10.1002/ppul.24246     less0 UnreadUnread
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  • Surette MG. The cystic fibrosis lung microbiome. Ann Am Thorac Soc. 2014 Jan;11 Suppl 1:S61-5. doi: 10.1513/AnnalsATS.201306-159MG. PMID: 24437409.
  • Margaroli, C., Tirouvanziam, R. Neutrophil plasticity enables the development of pathological microenvironments: implications for cystic fibrosis airway disease. Mol Cell Pediatr 3, 38 (2016). https://doi.org/10.1186/s40348-016-0066-2
  • Laval, J., Ralhan, A., & Hartl, D. (2016). Neutrophils in cystic fibrosis, Biological Chemistry397(6), 485-496. doi: https://doi.org/10.1515/hsz-2015-0271
  • Huether, S. E., McCance, K. L., & Brashers, V. L. (2020). Understanding pathophysiology / Sue E. Huether, Kathryn L. McCance ; section editor, Valentina L. Brashers (7th ed.). Elsevier.
  • 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, 16http://dx.doi.org.wilkes.idm.oclc.org/10.1186/s12890-016-0339-5
  • Aghasafari, P., George, U. & Pidaparti, R. A review of inflammatory mechanism in airway diseases. Inflamm. Res. 68, 59–74 (2019). https://doi-org.wilkes.idm.oclc.org/10.1007/s00011-018-1191-2
  • Reference:
  •             Rossi et al. (2019) in a review of airway microenvironment in CF, it was stated that a combination of factors, including impaired mucociliary clearance, and dysregulation of defense function, bacterial infection follows.
  • Aghasafari et al. (2018) stated “inflammatory mechanisms employ a group of pattern recognition receptors (PRPs) to recognize molecular patterns expressed by invading pathogens” (p. 59). Toll-like receptors (TLRs), which is a type of PRP, are found on membrane surfaces. TLRs recognize molecular patterns shared by pathogens and activate inflammatory cells to start the resolution process. Many factors affect the inflammatory response including tissue microenvironment and disease (Aghasafari et al., 2018). In CF there is an excessive inflammatory response, which plays a significant role in lung damage.
  • Deficiency in the CFTR gene leads to thick and viscose mucus that clogs small airways, contributing to the development of persistent lung inflammation, which increases the risk of lung infections (Aghasafari et al., 2019). In a journal article discussing CF and infection, Bhagirath et al. (2016) stated “the dominant chronic inflammation is generated by the failure of microbial clearance and the creation of a toxic pro-inflammatory local microenvironment, which damages the lung and the innate immunity, further facilitating infections” (p. 3).
  • CFTCR protein is an activated Cl channel present on the surface of many epithelial cells. These cells include the lining of the airways, bile ducts, the pancreas, sweat ducts, paranasal sinuses, and vas deferens. Improper functioning of the CFTCR protein inhibits the appropriate transport of Cl and fluid across epithelial membranes. Thick, dehydrated mucus secretions are the outcome of inadequate CFTCR protein functioning.
  • Melissa CFSubscribe
  • Tallona Boddy posted Jan 25, 2021 9:41 AM
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  • The airway’s microenvironment in a patient such as, Melissa who does have a diagnosis of cystic fibrosis is most likely not a healthy microenvironment, unfortunately (as mentioned in the discussion prompt).  Melissa’s diagnosis of cystic fibrosis means that she does have a defective system of transport of her chloride ions in her epithelial cells (Huether et al., 2020, p.54). Our chloride ions form from a negatively charged part of salt, chloride is one of our essential electrolytes needed to help regulate fluids in and out of cells (Chloride ion, 2021). When the chloride transport is not functioning correctly, imbalances take place.  For this scenario it is essentially (in the simplest terms) a salt/water ratio imbalance.  Melissa has thick, sticky mucus which is typically (for a patient that does not have cystic fibrosis) thin and more like a lubricant.  Melissa’s lungs have become clogged with this thick sticky mucus, essentially helping the bacteria become trapped in the lungs where they can colonize and thrive creating a biofilm (Infections n.d.).   Cystic Fibrosis Foundation. Infections. Cystic Fibrosis Foundation. https://www.cff.org/Research/Research-Into-the-Disease/Research-into-CF-Complications/Infections/.U.S. National Library of Medicine. (2021, January 23). Chloride ion. National Center for Biotechnology Information. PubChem Compound Database. https://pubchem.ncbi.nlm.nih.gov/compound/Chloride-ion.less1 UnreadUnread9 ViewsViews
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  • View profile card for Cassie Fritzinger
  • Last post January 27 at 1:12 PM by Cassie Fritzinger
  • Huether, S. E., McCance, K. L., & Brashers, V. L. (2020). Understanding pathophysiology (7th ed.).  Elsevier.
  • References
  • Week 2 discussion by Aina OluwoSubscribe
  • Aina Oluwo posted Jan 26, 2021 11:54 PM
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  • According to Brown et al. (2017), Cystic fibrosis (CF) affects a great number of Caucasians both in the United States and across the world. It also occurs in about 1 out of 3,500 births per year in whites and northern Europeans. This life-threatening genetic autosomal recessive disorder causes severe respiratory discomfort by allowing a buildup of thick, viscous mucus secretions particularly in the pulmonary system thus eliciting a response in its patient with severe fatigue, coughing and shortness of breath. To the untrained eye, this can be mis-diagnosed as asthma and patients can sometimes be prescribed inhaler medication that sometimes work to clear airways but more often, this is only a temporary fix as the pathophysiology behind CF calls for more than inhalers to elicit open airway response.According to Brown et al. (2017) these pathogens cause an overwhelming inflammatory response which can be assessed in Melissa’s clinical presentation temperature of 101F, RR-28, increased coughing & SOB, productive green sputum. Ultimately, chronic infection and this repetitive inflammatory response can lead to airway destruction.Referencesfibrosis lung environment and Pseudomonas aeruginosa infection. BMC PulmonaryBrown, S. D., White, R., & Tobin, P. (2017). Keep them breathing: Cystic fibrosisAcademy of Physician Assistants30(5), 23–27.Heuther, S., McCance, K., and Brashers, V.  (2020).  Understandingless0 UnreadUnread
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  • Pathophysiology (7th ed.).  Elsevier.
  • https://doi.org/10.1097/01.JAA.0000515540.36581.92
  • pathophysiology, diagnosis, and treatment. JAAPA: official journal of the American
  • Medicine, 16http://dx.doi.org.wilkes.idm.oclc.org/10.1186/s12890-016-0339-5
  • Bhagirath, A. Y., Li, Y., Somayajula, D., Dadashi, M., Badr, S., & Duan, K. (2016). Cystic
  • Understanding the pathogenesis of the disease is key to management and prevention. In people with cystic fibrosis, these excess secretion of mucus and chronic bacterial infection result in a special type of chronic obstructive pulmonary disease that can further leave the airways become badly damaged and scarred. One of the ways to manage this disease is the application of pulmonary therapies such as chest percussions to loosen up mucus and administration of mucus liquefying solutions aerosolized dornase alfa (Huether et al, 2020).
  •  The pathophysiology of CF is caused by a mutation in the CF transmembrane conductance regulator (CFTR) gene. The CFTR protein produced by this gene regulates the movement of chloride and sodium ions across epithelial cell membranes thus a defect in this transport chain elicits a salt imbalance (Huether et al., 2020). This imbalance results in the secretions of thick mucus throughout the body causing obstructions to systems such as pulmonary and digestive. In a healthy person, the respiratory system have mucins which is secreted by goblet and submucosal glands. Bhagirath et al. (2016) further explained the unfolding of the mucins by bicarbonates makes them slippery to trap microorganisms and remove them from epithelial surfaces. Thus in a CF patient, the thick mucus in the respiratory tract hampers the bacterial mucociliary clearance and allows for microbial colonization with pathogens such as Staph aureus or Pseudomonas aeruginosa to mention a few (Bhagirath et al., 2016)  .
  • Amandeep KaurSubscribe
  • Amandeep Kaur posted Jan 26, 2021 5:37 PM
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  • Melissa is subject to a genetic disorder that renders her vulnerable to many respiratory infections, such as what she is experiencing right now. The pathophysiology for this disorder stems from her genetic makeup. Cystic Fibrosis is an autosomal recessive condition, this indicates that two copies of the defected gene must be present for the individual to present with the condition (Kelsey et al., 2019). The mutation lies within the CFTR gene, found on the long arm of Chromosome 7, and spans 230kb with 27 coding exons. There have been over 2000 mutations that have identified, 127 of them disease-causing (Bhagirath et al., 2016). The mutation leads to the improper formation of the CFTR protein, which can regulate many ions including, sodium, potassium, chloride, and sodium bicarbonate. This protein is expressed in many organs; kidneys, pancreas, intestine, heart, vans deferens, and the lungs. Kelsey, R., Manderson Koivula, F. N., McClenaghan, N. H., & Kelly, C. (2019). Cystic fibrosis-related diabetes: Pathophysiology and therapeutic challenges. Clinical Medicine Insights: Endocrinology & Diabetes12, N.PAG.Bhagirath, A. Y., Li, Y., Somayajula, D., Dadashi, M., Badr, S., & Duan, K. (2016). Cystic fibrosis lung environment and Pseudomonas aeruginosa infection. BMC pulmonary medicine16(1), 174.
  • References
  • The most lethal defective pathway is the CFTR-dependent water-secreting in the epithelial tissue of the lungs (Bhagirath et al., 2016). CTFR protein dysfunction caused there to be a hindrance to inadequate water section to the cell’s surface. This is due to the inability of CTFR protein to secrete chloride and bicarbonate, since these ions cannot be secreted water will not follow suit. This leads to the accumulation of thick sticky mucus in the respiratory tract. This will trap the infection causing microbials and there will be ineffective clearance in the lungs. Some of the most commonly found pathogens in cystic fibrosis are Pseudomonas aeruginosaStaphylococcus aureusHaemophilus influenzae, and Burkholderia cepacia complex. The toxic environment will prime for there to be chronic respiratory infections, which will damage the lungs and innate immunity. Chronic use of antibiotic therapies to combat these infections can transpire resistant variant strains of pathogens, which can lead to death. 
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