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CF + TB Flashcards

1
Q

What does the name ‘cystic fibrosis’ refer to?

A

The disease’s effects on the pancreas, leading to cysts and fibrosis.

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2
Q

What type of genetic disorder is cystic fibrosis?

A

Autosomal recessive disorder.

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3
Q

What does the CFTR gene stand for?

A

Cystic fibrosis transmembrane conductance regulator.

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4
Q

What is required to inherit cystic fibrosis?

A

Two mutated CFTR genes, one from each parent.

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5
Q

Who is more commonly affected by cystic fibrosis?

A

People of European descent.

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6
Q

What is the function of the CFTR protein?

A

It pumps chloride ions into various secretions.

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7
Q

What is the most common mutation associated with cystic fibrosis?

A

The ∆F508 mutation.

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8
Q

What does the ∆F508 mutation involve?

A

Deletion of the 508th amino acid, phenylalanine, in the CFTR protein.

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9
Q

What happens to the CFTR protein with the ∆F508 mutation?

A

It gets misfolded and cannot migrate to the cell membrane.

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10
Q

What is a potential consequence of thick secretions in newborns with CF?

A

Meconium ileus, a surgical emergency.

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11
Q

What is pancreatic insufficiency in CF?

A

Thick secretions jam pancreatic ducts, preventing digestive enzymes from reaching the small intestine.

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12
Q

What can result from the lack of pancreatic enzymes in CF?

A

Poor weight gain and failure to thrive.

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13
Q

What is steatorrhea?

A

Fat-containing stools due to fat malabsorption.

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14
Q

What can chronic pancreatic damage in CF lead to?

A

Insulin-dependent diabetes.

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15
Q

What role do cilia play in the lungs?

A

They help clear mucus and debris from the airways.

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16
Q

What happens to mucociliary action in CF?

A

It becomes defective due to thick mucus, leading to chronic bacterial colonization.

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17
Q

What is a CF exacerbation?

A

A sudden increase in bacterial load causing cough, fever, and decreased lung function.

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18
Q

Which bacteria are commonly problematic in CF?

A
  • Staphylococcus aureus (gram positive) * Pseudomonas aeruginosa (gram negative)
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19
Q

What is bronchiectasis?

A

Airway wall damage causing permanent dilation of the bronchi.

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20
Q

What can repeated CF exacerbations lead to?

A

Respiratory failure, the leading cause of death with CF.

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21
Q

What reproductive issue is common in men with CF?

A

Infertility due to lack of vas deferens.

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22
Q

What is digital clubbing?

A

Fingernails that begin to spoon around the fingertips.

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23
Q

What is a common diagnostic test for CF in newborns?

A

Screening for immunoreactive trypsinogen (IRT).

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24
Q

What does a high chloride level in sweat indicate?

A

Presence of cystic fibrosis.

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25
What are the main goals of cystic fibrosis treatment?
* Nutrition and healthy weight gain * Pulmonary function maintenance
26
What are fat-soluble vitamins that may need supplementation in CF?
* A * D * E * K
27
What is the purpose of chest physiotherapy in CF treatment?
To loosen mucus.
28
What medications are used to thin mucus in CF?
* N-acetylcysteine * Dornase alfa
29
What type of lung disease is CF classified as?
Obstructive lung disease.
30
What is a potential treatment for advanced CF?
Lung transplant.
31
What is lumacaftor's role in CF treatment?
It acts as a chaperone to bring the mutated ∆F508 CFTR to the cell membrane.
32
What is ivacaftor's original target mutation?
G551D mutation. ## Footnote this is a type 3 mutations: dysregulation of CFTR, diminished atp binding and hydrolysis --- channel is delivered to the epitheliim normally, but is not functioning
33
What is the effect of ivacaftor on the ∆F508 CFTR?
It improves its function. | metabolised by cyp450, so there are pharmacy considerations ## Footnote improves chloride flow through the CFTR
34
What is the outlook for patients with CF regarding genetic technologies?
Aimed at correcting specific gene mutations.
35
Cystic fibrosis primarily affects which two organs?
* Lungs * Pancreas
36
Kaftrio drug | THE GAME CHANGER
ivacaftor + tezefactor + elexefactor | BALL OF SPUTUM WASHED UP, V GOOD DRUG, REVOLUTIONISING TREATEMNT ## Footnote SIDE EFFECTS: liver sos --> monitor LFT (liver function tests) - death from liver failure
37
What shape are Mycobacteria?
Slender, rod-shaped ## Footnote Mycobacteria are classified as strict aerobes.
38
What unique component contributes to the waxy cell wall of Mycobacteria?
Mycolic acid ## Footnote This waxy cell wall makes them acid-fast.
39
What staining technique is used to identify Mycobacterium tuberculosis?
Ziehl–Neelsen stain ## Footnote This technique leaves the bacteria bright red despite alcohol exposure.
40
How is Mycobacterium tuberculosis primarily transmitted?
Inhalation ## Footnote It gains entry into the lungs through the respiratory system.
41
What is the role of macrophages in the immune response to TB?
They phagocytize foreign cells, digest, and destroy them ## Footnote They recognize foreign proteins on TB's surface.
42
What happens when TB survives inside macrophages?
It proliferates and creates a localized infection ## Footnote TB inhibits the fusion of phagosomes with lysosomes.
43
What does primary tuberculosis indicate?
Signs of infection soon after exposure to TB ## Footnote Most people at this stage are asymptomatic or have mild flu-like symptoms.
44
What is a granuloma?
A structure formed by immune cells surrounding TB infection ## Footnote It attempts to wall off the bacteria and prevent its spread.
45
What is caseous necrosis?
A type of tissue death that resembles cheese ## Footnote It occurs in the tissue inside the granuloma.
46
What is a Ghon focus?
An area of caseous necrosis caused by TB ## Footnote It is located at the site of TB infection.
47
What characterizes a Ghon complex?
The Ghon focus and affected hilar lymph node ## Footnote This complex is typically subpleural and occurs in the lower lobes of the lungs.
48
What is a Ranke complex?
A calcified Ghon complex seen on X-ray ## Footnote It indicates previous TB infection.
49
What happens if the immune system becomes compromised regarding TB?
The Ghon focus can reactivate ## Footnote This can lead to spread to the upper lobes of the lungs.
50
What is systemic miliary TB?
TB that spreads to almost every tissue in the body ## Footnote It can occur when bacteria disseminate from lung cavities.
51
What common complication occurs when TB spreads to the kidneys?
Sterile pyuria ## Footnote This refers to high levels of white blood cells in the urine.
52
What is the purpose of the PPD intradermal skin test?
To test for previous exposure to TB ## Footnote A localized reaction indicates a positive test.
53
What does a positive tuberculin test indicate?
Previous exposure to TB ## Footnote It does not differentiate between active and latent TB.
54
What is an IGRA?
Interferon gamma release assay ## Footnote It detects evidence of previous exposure to TB proteins in the blood.
55
What is the standard treatment for latent TB infection?
Isoniazid for 9 months ## Footnote This is a single drug treatment approach.
56
What combination of antibiotics is typically used to treat active TB disease?
* Isoniazid * Rifampin * Ethambutol * Pyrazinamide ## Footnote These combinations help make patients non-infectious within weeks.
57
What is directly observed therapy (DOT)?
A treatment method where someone confirms medication intake ## Footnote It ensures adherence to the TB medication regimen.
58
What does MDR-TB stand for?
Multi-drug resistant tuberculosis ## Footnote It refers to strains resistant to standard TB treatments.
59
What is the breakthrough medication approved for XDR-TB?
Pretomanid ## Footnote It is part of a combination therapy effective for almost 90% of XDR-TB cases.
60
What are the symptoms of active TB disease?
* Fevers * Night sweats * Weight loss * Hemoptysis ## Footnote These symptoms indicate the need for further testing.
61
The classic colonisation pathway for CF patients (immunocompromised patietns), is
Haemophilues Staph aureus pseudomonas ## Footnote 1. Haemophilus (e.g., Haemophilus influenzae) Gram-negative (-) Shape: Small coccobacilli (between cocci and rods) Oxygen Requirement: Facultative anaerobe Staphylococcus aureus Gram-positive (+) Shape: Cocci in clusters (grape-like appearance) Oxygen Requirement: Facultative anaerobe Special Features: Catalase-positive (differentiates from Streptococcus) Coagulase-positive (differentiates from other Staph species) Produces toxins (e.g., enterotoxins, toxic shock syndrome toxin) Diseases Caused: Skin infections: Cellulitis, abscesses, impetigo Systemic infections: Sepsis, endocarditis, osteomyelitis Toxin-mediated diseases: Toxic Shock Syndrome (TSS), food poisoning Pseudomonas (e.g., Pseudomonas aeruginosa) Gram-negative (-) Shape: Rod (bacillus) Oxygen Requirement: Obligate aerobe Diseases Caused: Opportunistic infections in immunocompromised patients Hospital-acquired infections: Pneumonia (ventilator-associated), sepsis Cystic fibrosis patients: Chronic lung infections Burn wound infections
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Respiratory (9 decks)

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