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MDRS > CF and bronciectasis > Flashcards

CF and bronciectasis Flashcards

1
Q

Wat is CF

A
  • An inherited autosomal recessive disease
  • characterized by dysfunction of exocrine glands
  • primarily affecting the GI and
    respiratory systems.
  • 2 genes on chromosome 7
  • Mutation causes production of abnormal protein cystic fibrosis trans membrane
    regulator (CFTR)
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2
Q

what does the mutation of CF gene cause

A
  • abnormal expression of CFTR protein
  • result in a defect in
    chloride transport and
    sodium absorption across
    epithelial cells
  • results in abnormal
    regulation of exocrine
    glands
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3
Q

What is the pathogegnsis of cystic fibrosis lung disease/

A
  1. CFTR defect
  2. impaired mucus clearance
  3. chronic bacterial infection
  4. chronic neutrophilic inflammation
  5. bronchiectasis
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4
Q

What are the mains glands that are affected in CF?

A

sweat

  • unable to conserve salt from sweat
  • salty skin, dehydration
pancreas
- lack of digestive enzymes
- difficulty digesting fat
- vitamin deficiency (A,D,E,K)
lung

lung
- increased amount & thickness of mucus
– impairs cilia causing airway obstruction
– chronic infection/inflammation of tissues

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5
Q

What is te clincal manifestation of CF?

A 
– Persistent prod. cough
– Wheezes
– Chronic infections
– Cyanosis
– Clubbing
– Recurrent pneumonia
– Hemoptysis
– Looks like COPD
– May develop
bronchiectasis
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6
Q

Wat is some diggestive problem in CF ?

A 
– Fatty (smelly) stool
– Fatty liver (cirrhosis)
– Gall bladder abnormal
– Pancreatic insufficiency
(20 %
-Malnutrition due to ↓
fat absorption and
vitamin deficiency
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7
Q

sinuses problem in CF?

A
  • nasal cogestion
  • loss of smell
  • nasal polyps
  • sinusitits
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8
Q

pancreas problem in CF?

A
  • acute/chronic pancreatitis

- pancreatic insufficiency

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9
Q

GI problem in CF?

A
  • biliary duct obstruction

- chronic constipation

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10
Q

sweat gland problem in CF?

A
  • excessive salt loss
  • heat prostration
  • high level of sweat chloride
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11
Q

lungg problem in CF?

A
  • bronciectasis

- endobroncial infection and inflammation

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12
Q

reproductive problem in CF?

A
  • infertility

- bilateral absence of vas deferens

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13
Q

Wat are thhe diagnosis of CF?

A
  1. sweat chloride test
    - ->pilocarpine
    - -> if > or equal to 60meq/L on 2 occasion then its likely CF
  2. meconium ileus at birt
  3. family history and gene detection
  4. NPD: nasal potential difference
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14
Q

what is CXR finding for CF

A
  1. hyperinflation (depressed diaphragm)
  2. bronchial thickening
  3. patchy atelectasis/infiltrations; enlarged heart (PA) more upper lobe involvement
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15
Q

AB finding for CF?

A

hypoxemia and acidosis

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16
Q

PFT finding for CF?

A

obstructive pattern

17
Q

SPutum C&S?

A

mostly gram negative

  • P. aeruginosa,
  • Klebsiella, E.
18
Q

Wat is the Gi treatment for CF?

A
  1. Diet high in fats, calories due to poor absorption,

2. ++ salt, protein, Zn, Ca, Fe, Vit . E & K

19
Q

Wat is the secretion removal treatment for CF

A

3.Control and Removal of Secretions
-chest physio, suction, humidity,
deep breathing techniques, OPEP, postural drainage?

20
Q

what is the medication treatment for CF

A
  • Vaccines, mucolytics, antibiotics both inhaled and

oral & IV, bronchodilator, anti inflammatories,

21
Q

whhat are 2 disease modifying med tat help CFTR?

A

Lumacaftor
–> help CFTR to function better & open correctly

Ivacaftor
–>helps mucus to get to cell surface (Ivacaftor

22
Q

What is the complication of CF

A

Diabetes
Recurrent pneumonia
COPD
bronchiectasis

23
Q

defintion of bronchiectasis?

A

Abnormal irreversible dilation of the
bronchi due to destructive and
inflammatory changes in the airway walls
(small and medium bronchi)

24
Q

cause of bronchiectasis

A

onset: found in childhood
underlying cause often not found

localized bronchiectasis?

  • Aspiration with airway blockage
  • Aspergillus fumigatus
25
Q

cause of diffuse bronchiectasis

A
  1. congenital or familial factors
    • primary ciliary dyskinesia
    • tracheobronchomegaly (Mounier-Kuhn Syndrome)
    • cartilage deficiency (Williams-Campbell Syndrome)
    • CF (50 % of bronchiectasis)
    • alpha 1 antitrypsin deficiency
  2. sequelae of toxic inhalation/aspiration
26
Q

what is the systemic disorder associated with bronchiectasis

A

RA (rheumatoid arthritis)

Inflammatory bowel disease/ulcerative colitis

27
Q

what is the pathophyiology of bronchiectasis?

A
  1. the bronchi of the lower lobe is usually affected

2.impaired clearance of bronchial
secretions &  thickness of secretions

causing:
- stagnant mucous
- infection of airways
- further destruction of bronchial walls
via inflammatory mediator release
  1. decreased ciliary activity may be due to toxic factors released from bacteria and proteolytic enzymes released from neutrophils
  2. hemoptysis occurs because of the increase blood low to the epithelium layer of the bronchi
28
Q

Wht are the 3 types of bronchiectasis?

A

Cylindrical
– regular and uniform dilation of airways

Varicose
- irregular pattern (alternating areas of
constriction and dilation)

Cystic (saccular) – most severe form
– progressive, distal enlargement of airways ending in sac like dilations

29
Q

what is the result of anatomic alteration of lungs

A 
Chronic dilation and distortion of
bronchial airways
- Excessive production of often foulsmelling sputum
- bronchospasm
- hyerpinflation of alveoli
- atelectasis, parenchymal fibrosis 
- hemorrhage
30
Q

what are the diagnosis and lab findings of bronchiectasis?

A

sputum:
–> increase WBC and bacteria (anerobic or aerobic)

CT scan

  • -> peribronchial fibrosis
  • ->mucous plugs and atelectasis

PFT
–>obstructive or restrictive

ABG
–>normal than worsen

31
Q

How to tell a bilateral extensive bronciectasis on CT or CXR

A

enlarged hilar lymph nodes

32
Q

what is the clinical manifestation and complication

A 
productive cough
foul smelling mucus
clubbing
accessory muscle use
barrel chest
cyanosis
33
Q

what is the goal of treatment for bronchiectasis?

A
  1. control infection
  2. contrilling airway secretion
  3. preventing complication
34
Q

what is the treatment for bronchectasis

A

1lobectomy/segmentectomy

  1. bronchdilation
  2. smoking cessation
  3. avoidance of irritants

MDRS (23 decks)

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