CFTR Mutation Pathophysiology
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Questions and Answers

What is the main consequence of defective CFTR in cystic fibrosis?

  • Increased secretion of chloride
  • Decreased reabsorption of sodium
  • Decreased secretion of chloride (correct)
  • Increased reabsorption of water
  • Which system is the most affected in cystic fibrosis?

  • Nervous system
  • Respiratory system (correct)
  • Cardiovascular system
  • Skeletal system
  • What is the major cause of morbidity and mortality in cystic fibrosis?

  • Liver diseases
  • CF-related diabetes
  • Respiratory infections (correct)
  • Intestinal obstruction
  • What happens to the viscosity of secretions in cystic fibrosis?

    <p>Viscosity increases</p> Signup and view all the answers

    What is the most common pathogen causing disease in CF patients?

    <p>Staphylococcus aureus</p> Signup and view all the answers

    How does defective CFTR affect airway surface liquid?

    <p>Decreases its hydration</p> Signup and view all the answers

    What are the major long-term complications of cystic fibrosis?

    <p>Progressive lung disease &amp; CF-related diabetes</p> Signup and view all the answers

    What happens to sputum in cystic fibrosis that facilitates pathogen colonization?

    <p>It contains an abundance of nutrients</p> Signup and view all the answers

    What is the most common Burkholderia cepacia complex species found in people with cystic fibrosis?

    <p>B. multivorans</p> Signup and view all the answers

    Which Burkholderia cepacia complex species produces a melanin-like pigment that helps evade oxidative killing in the host?

    <p>B. cepacia</p> Signup and view all the answers

    What is the unique characteristic of Stenotrophomonas maltophilia among the mentioned bacteria?

    <p>Flagellated motile</p> Signup and view all the answers

    What makes Burkholderia cepacia complex resistant to antibiotics according to the text?

    <p>Production of metalloproteinases</p> Signup and view all the answers

    Which bacterium is described as an environmental bacteria that lives in water, aquatic areas, and wet surfaces?

    <p>S. maltophilia</p> Signup and view all the answers

    Which Burkholderia cepacia complex species contributes more to Cepacia syndrome due to its inflammatory nature?

    <p>B. cepacia</p> Signup and view all the answers

    What characteristic of B. cepacia contributes to its survival in the lungs?

    <p>Production of melanin-like pigment</p> Signup and view all the answers

    Which pathogen is a common clinical pathogen causing a wide range of infections in skin, soft tissue, and deep tissues?

    <p>Staphylococcus aureus</p> Signup and view all the answers

    What is the leading cause of bloodstream and lower respiratory tract infections according to the text?

    <p>MRSA</p> Signup and view all the answers

    Which pathogen is a dominant pathogen in people with cystic fibrosis contributing to morbidity and mortality?

    <p>Pseudomonas aeruginosa</p> Signup and view all the answers

    What is attributed to the adaptability and flexibility of Pseudomonas aeruginosa in causing chronic infections?

    <p>Extensive number of virulence factors</p> Signup and view all the answers

    Which pathogen has been observed to undergo pathoadaptative changes in response to the stressful environment of the CF airway?

    <p>Pseudomonas aeruginosa</p> Signup and view all the answers

    What is the main virulence factor produced by Pseudomonas aeruginosa that can break down lung tissue?

    <p>Elastase</p> Signup and view all the answers

    Which virulence factor of Staphylococcus aureus binds to antibodies, preventing bacterial opsonization?

    <p>Protein A</p> Signup and view all the answers

    What type of virulence factor does Burkholderia cepacia complex produce to scavenge iron from the host?

    <p>Siderophores</p> Signup and view all the answers

    Which pathogen associated with CF lung disease is known for producing biofilms that protect it from the host immune response and antibiotics?

    <p>Pseudomonas aeruginosa</p> Signup and view all the answers

    What is the specific toxin produced by Pseudomonas aeruginosa that contributes to inflammation and tissue damage in the lungs?

    <p>Pyocyanin</p> Signup and view all the answers

    Which enzyme produced by Staphylococcus aureus contributes to tissue damage?

    <p>Protease</p> Signup and view all the answers

    What is the primary effect of alginate produced by Pseudomonas aeruginosa?

    <p>Promoting biofilm formation</p> Signup and view all the answers

    Which bacterial group produces toxins and enzymes that contribute to tissue damage and inflammation?

    <p>Burkholderia cepacia complex</p> Signup and view all the answers

    What type of bacteria is Moraxella catarrhalis?

    <p>Gram-negative diplococci</p> Signup and view all the answers

    Which virulence factor is NOT produced by Stenotrophomonas maltophilia?

    <p>Catalase</p> Signup and view all the answers

    What infections can be caused by Moraxella catarrhalis according to the text?

    <p>Otitis media and sinusitis</p> Signup and view all the answers

    Which bacterial species mentioned is fastidious and facultative aerobic?

    <p>Hemophilus influenzae</p> Signup and view all the answers

    What characteristic distinguishes Hemophilus influenza type B (HiB) from Stenotrophomonas maltophilia?

    <p>Presence of capsule</p> Signup and view all the answers

    Which bacterium causes endophthalmitis among other infections?

    <p>Acromobacter xylosoxidans</p> Signup and view all the answers

    In penicillin-resistant infections, which enzyme is commonly produced by Moraxella catarrhalis?

    <p>$eta$-lactamase</p> Signup and view all the answers

    What distinguishes Stenotrophomonas maltophilia from Acromobacter xylosoxidans based on the text?

    <p>Capsule production</p> Signup and view all the answers

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