CF and bronciectasis

Question 1

Wat is CF

Answer 1

• 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)

Question 2

what does the mutation of CF gene cause

Answer 2

• 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

Question 3

What is the pathogegnsis of cystic fibrosis lung disease/

Answer 3

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

Question 4

What are the mains glands that are affected in CF?

Answer 4

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

Question 5

What is te clincal manifestation of CF?

Answer 5

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

Question 6

Wat is some diggestive problem in CF ?

Answer 6

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

Question 7

sinuses problem in CF?

Answer 7

• nasal cogestion
• loss of smell
• nasal polyps
• sinusitits

Question 8

pancreas problem in CF?

Answer 8

• acute/chronic pancreatitis

- pancreatic insufficiency

Question 9

GI problem in CF?

Answer 9

• biliary duct obstruction

- chronic constipation

Question 10

sweat gland problem in CF?

Answer 10

• excessive salt loss
• heat prostration
• high level of sweat chloride

Question 11

lungg problem in CF?

Answer 11

• bronciectasis

- endobroncial infection and inflammation

Question 12

reproductive problem in CF?

Answer 12

• infertility

- bilateral absence of vas deferens

Question 13

Wat are thhe diagnosis of CF?

Answer 13

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

Question 14

what is CXR finding for CF

Answer 14

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

Question 15

AB finding for CF?

Answer 15

hypoxemia and acidosis

Question 16

PFT finding for CF?

Answer 16

obstructive pattern

Question 17

SPutum C&S?

Answer 17

mostly gram negative

• P. aeruginosa,
• Klebsiella, E.

Question 18

Wat is the Gi treatment for CF?

Answer 18

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

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

Question 19

Wat is the secretion removal treatment for CF

Answer 19

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

Question 20

what is the medication treatment for CF

Answer 20

• Vaccines, mucolytics, antibiotics both inhaled and

oral & IV, bronchodilator, anti inflammatories,

Question 21

whhat are 2 disease modifying med tat help CFTR?

Answer 21

Lumacaftor
–> help CFTR to function better & open correctly

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

Question 22

What is the complication of CF

Answer 22

Diabetes
Recurrent pneumonia
COPD
bronchiectasis

Question 23

defintion of bronchiectasis?

Answer 23

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

Question 24

cause of bronchiectasis

Answer 24

onset: found in childhood
underlying cause often not found

localized bronchiectasis?

• Aspiration with airway blockage
• Aspergillus fumigatus

Question 25

cause of diffuse bronchiectasis

Answer 25

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

Question 26

what is the systemic disorder associated with bronchiectasis

Answer 26

RA (rheumatoid arthritis) | Inflammatory bowel disease/ulcerative colitis

Question 27

what is the pathophyiology of bronchiectasis?

Answer 27

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 ``` 3. decreased ciliary activity may be due to toxic factors released from bacteria and proteolytic enzymes released from neutrophils 4. hemoptysis occurs because of the increase blood low to the epithelium layer of the bronchi

Question 28

Wht are the 3 types of bronchiectasis?

Answer 28

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

Question 29

what is the result of anatomic alteration of lungs

Answer 29

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

Question 30

what are the diagnosis and lab findings of bronchiectasis?

Answer 30

sputum: --> increase WBC and bacteria (anerobic or aerobic) CT scan - -> peribronchial fibrosis - ->mucous plugs and atelectasis PFT -->obstructive or restrictive ABG -->normal than worsen

Question 31

How to tell a bilateral extensive bronciectasis on CT or CXR

Answer 31

enlarged hilar lymph nodes

Question 32

what is the clinical manifestation and complication

Answer 32

``` productive cough foul smelling mucus clubbing accessory muscle use barrel chest cyanosis ```

Question 33

what is the goal of treatment for bronchiectasis?

Answer 33

1. control infection 2. contrilling airway secretion 3. preventing complication

Question 34

what is the treatment for bronchectasis

Answer 34

1lobectomy/segmentectomy 2. bronchdilation 3. smoking cessation 4. avoidance of irritants