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 (A)</p> Signup and view all the answers

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

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

How does defective CFTR affect airway surface liquid?

<p>Decreases its hydration (C)</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 (C)</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 (B)</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 (A)</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 (A)</p> Signup and view all the answers

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

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

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

<p>Production of metalloproteinases (D)</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 (C)</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 (B)</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 (A)</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 (B)</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 (B)</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 (D)</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 (C)</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 (D)</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 (B)</p> Signup and view all the answers

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

<p>Protein A (B)</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 (C)</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 (C)</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 (D)</p> Signup and view all the answers

Which enzyme produced by Staphylococcus aureus contributes to tissue damage?

<p>Protease (C)</p> Signup and view all the answers

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

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

What type of bacteria is Moraxella catarrhalis?

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

Which virulence factor is NOT produced by Stenotrophomonas maltophilia?

<p>Catalase (A)</p> Signup and view all the answers

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

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

Which bacterial species mentioned is fastidious and facultative aerobic?

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

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

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

Which bacterium causes endophthalmitis among other infections?

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

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

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

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

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

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