Cystic Fibrosis and Tuberculosis Flashcards

1
Q

What is the most common lethal genetic disease in Caucasians

A

Cystic fibrosis

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2
Q

how is cystic fibrosis inherited

A

autosomal recessive

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3
Q

what chromosome is cystic fibrosis on

A

chromosome 7

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4
Q

what is the gene product for cystic fibrosis?

A

CF transmembrane conductance regulator ( CFTR)

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5
Q

How do 70% of mutations occur in cystic fibrosis

A

3 nucleotide deletion

  • delete a single amino acid
  • delta F508
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6
Q

What are the major organs affected in cystic fibrosis

A

lung and exocrine glands ( pancreas and small intestines)

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7
Q

what are lung and upper respiratory findings in cystic fibrosis

A
  • chronic cough with sputum secretion
  • chronic wheezing
  • chronic pansinusitis
  • nalal polyposis
  • strep. pneumo as an infant
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8
Q

what are GI problems and hormonla probelms with cystic fibrosis

A
  • meconium ileus (MI) intestinal blockage
  • pancreatic insufficiency
  • liver disease
  • delayed puberty
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9
Q

what is seen in X-ray of cystic fibrosis

A

accumulation of neutrophils

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10
Q

what is the most common bacteria to cause cystic fibrosis

A

P. aeruginosa

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11
Q

Is p. aeruginosa gram positive or negative

A

negative

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12
Q

what is the shape of p. aeruginosa

A

rod shape

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13
Q

What does p. aeruginosa produce

A
"grape-like odor"
produces biofilm (polysacharide)
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14
Q

Alginate produces what

A

mucoidy

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15
Q

what is required for alginate to replicate

A

lot of cellular energy

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16
Q

what encloses bacteria in biofilm? Where is biofilm found?

A

exopolysaccharide matrix enclosed bacterial COMMUNITY, different from bacteria in suspension
-biofilms is found every UBIQUITOUS

17
Q

what are clinical manifestations of CF (3)

A
  1. localized lung infection: excessive neutrophil infiltration
  2. infection around bronchi and bronchioles
  3. malnutrition
18
Q

What is used to treat cystic fibrosis? how is the pseudo treated

A
  • inhaled tobramycin and oral azithromycin

- combination therapy for pseudo: penicillin, ticarcillin or piperacillin, plus aminoglycosdie ( gentamicin or amikacin)

19
Q

is Psuedo resistant to many durgs

A

YES

20
Q

What is the second and first most common infectious cause of death in adults worldwide

A
  • Tuberculosos 2

- HIV 1

21
Q

where does mycoplasma tuberculosis reside in body

A

macrophages and monocytes

22
Q

Where does secondary TB usually occur in lung

A

apex of lung

23
Q

tuberculosis describe the bacteria

A
  • gram + like cell wall

- aerobic rod `

24
Q

what is tuberculosis bacteria resistant to

A

drying
chemical
germicides

25
Q

what is located in cell of tuberculosis

A

mycolic acid waxes

26
Q

how do you stain for tuberculosis

A

acid fast

27
Q

how fast does tuberculosis grow

A

slow

28
Q

How can tuberculosis live a macrophage

A
  • prevents phagocytic/lysosome vesicle fursion
  • LLO homologue (hemolysin)
  • prevents acidification of phagosome
  • produce NH4
29
Q

What causes lung damage by tubercuosis

A

TNF alpha provoked by cell wall components

30
Q

what role does mycolic acids have in tuberculosis

A

stimulates immune response

31
Q

What medium does tuberculosis grow on

A

Lowenstein-Jenson

Middlebrook

32
Q

What is caseous granuloma

A
  • central core-multinucleated giant cells ( fused macrophages)
  • epithelioid cell- large macrophages
  • CD4 positive T cells
33
Q

What is ghon comples

A

hilar lymphadenopathy

Langhan’s cells

34
Q

what is Miliary TB

A

white nodules

35
Q

relate TB and AIDS

A

TB kills more rapidly in AIDS

36
Q

Aids and TB patients are more likely to have what clinical symptoms

A
  • extrapulmonary disease
  • lymph nodes
  • genitourinary
  • CNS
37
Q

AIDS and TB are susceptible to what infection

A

MAI

38
Q

What test is used to detect TB

A

PPD

39
Q

Who may not show a positive PPD test even though they have TB

A

AIDS
cross- reactive to other mycobacteria
BCG