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CFTR Mutation Pathophysiology

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36 Questions

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

Decreased secretion of chloride

Which system is the most affected in cystic fibrosis?

Respiratory system

What is the major cause of morbidity and mortality in cystic fibrosis?

Respiratory infections

What happens to the viscosity of secretions in cystic fibrosis?

Viscosity increases

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

Staphylococcus aureus

How does defective CFTR affect airway surface liquid?

Decreases its hydration

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

Progressive lung disease & CF-related diabetes

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

It contains an abundance of nutrients

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

B. multivorans

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

B. cepacia

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

Flagellated motile

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

Production of metalloproteinases

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

S. maltophilia

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

B. cepacia

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

Production of melanin-like pigment

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

Staphylococcus aureus

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

MRSA

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

Pseudomonas aeruginosa

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

Extensive number of virulence factors

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

Pseudomonas aeruginosa

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

Elastase

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

Protein A

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

Siderophores

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

Pseudomonas aeruginosa

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

Pyocyanin

Which enzyme produced by Staphylococcus aureus contributes to tissue damage?

Protease

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

Promoting biofilm formation

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

Burkholderia cepacia complex

What type of bacteria is Moraxella catarrhalis?

Gram-negative diplococci

Which virulence factor is NOT produced by Stenotrophomonas maltophilia?

Catalase

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

Otitis media and sinusitis

Which bacterial species mentioned is fastidious and facultative aerobic?

Hemophilus influenzae

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

Presence of capsule

Which bacterium causes endophthalmitis among other infections?

Acromobacter xylosoxidans

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

$eta$-lactamase

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

Capsule production

Learn about the pathophysiology of CFTR mutations and how they lead to decreased secretion of chloride and increased reabsorption of sodium and water across epithelial cells. Understand the impact on airway surface liquid, mucus hydration, bacterial infections, and inflammation in cystic fibrosis.

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