CF and bronciectasis Flashcards
Wat is CF
- 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)
what does the mutation of CF gene cause
- 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
What is the pathogegnsis of cystic fibrosis lung disease/
- CFTR defect
- impaired mucus clearance
- chronic bacterial infection
- chronic neutrophilic inflammation
- bronchiectasis
What are the mains glands that are affected in CF?
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
What is te clincal manifestation of CF?
– Persistent prod. cough – Wheezes – Chronic infections – Cyanosis – Clubbing – Recurrent pneumonia – Hemoptysis – Looks like COPD – May develop bronchiectasis
Wat is some diggestive problem in CF ?
– Fatty (smelly) stool – Fatty liver (cirrhosis) – Gall bladder abnormal – Pancreatic insufficiency (20 % -Malnutrition due to ↓ fat absorption and vitamin deficiency
sinuses problem in CF?
- nasal cogestion
- loss of smell
- nasal polyps
- sinusitits
pancreas problem in CF?
- acute/chronic pancreatitis
- pancreatic insufficiency
GI problem in CF?
- biliary duct obstruction
- chronic constipation
sweat gland problem in CF?
- excessive salt loss
- heat prostration
- high level of sweat chloride
lungg problem in CF?
- bronciectasis
- endobroncial infection and inflammation
reproductive problem in CF?
- infertility
- bilateral absence of vas deferens
Wat are thhe diagnosis of CF?
- sweat chloride test
- ->pilocarpine
- -> if > or equal to 60meq/L on 2 occasion then its likely CF - meconium ileus at birt
- family history and gene detection
- NPD: nasal potential difference
what is CXR finding for CF
- hyperinflation (depressed diaphragm)
- bronchial thickening
- patchy atelectasis/infiltrations; enlarged heart (PA) more upper lobe involvement
AB finding for CF?
hypoxemia and acidosis
PFT finding for CF?
obstructive pattern
SPutum C&S?
mostly gram negative
- P. aeruginosa,
- Klebsiella, E.
Wat is the Gi treatment for CF?
- Diet high in fats, calories due to poor absorption,
2. ++ salt, protein, Zn, Ca, Fe, Vit . E & K
Wat is the secretion removal treatment for CF
3.Control and Removal of Secretions
-chest physio, suction, humidity,
deep breathing techniques, OPEP, postural drainage?
what is the medication treatment for CF
- Vaccines, mucolytics, antibiotics both inhaled and
oral & IV, bronchodilator, anti inflammatories,
whhat are 2 disease modifying med tat help CFTR?
Lumacaftor
–> help CFTR to function better & open correctly
Ivacaftor
–>helps mucus to get to cell surface (Ivacaftor
What is the complication of CF
Diabetes
Recurrent pneumonia
COPD
bronchiectasis
defintion of bronchiectasis?
Abnormal irreversible dilation of the
bronchi due to destructive and
inflammatory changes in the airway walls
(small and medium bronchi)
cause of bronchiectasis
onset: found in childhood
underlying cause often not found
localized bronchiectasis?
- Aspiration with airway blockage
- Aspergillus fumigatus
cause of diffuse bronchiectasis
- congenital or familial factors
• primary ciliary dyskinesia
• tracheobronchomegaly (Mounier-Kuhn Syndrome)
• cartilage deficiency (Williams-Campbell Syndrome)
• CF (50 % of bronchiectasis)
• alpha 1 antitrypsin deficiency - sequelae of toxic inhalation/aspiration
what is the systemic disorder associated with bronchiectasis
RA (rheumatoid arthritis)
Inflammatory bowel disease/ulcerative colitis
what is the pathophyiology of bronchiectasis?
- 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
- decreased ciliary activity may be due to toxic factors released from bacteria and proteolytic enzymes released from neutrophils
- hemoptysis occurs because of the increase blood low to the epithelium layer of the bronchi
Wht are the 3 types of bronchiectasis?
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
what is the result of anatomic alteration of lungs
Chronic dilation and distortion of bronchial airways - Excessive production of often foulsmelling sputum - bronchospasm - hyerpinflation of alveoli - atelectasis, parenchymal fibrosis - hemorrhage
what are the diagnosis and lab findings of bronchiectasis?
sputum:
–> increase WBC and bacteria (anerobic or aerobic)
CT scan
- -> peribronchial fibrosis
- ->mucous plugs and atelectasis
PFT
–>obstructive or restrictive
ABG
–>normal than worsen
How to tell a bilateral extensive bronciectasis on CT or CXR
enlarged hilar lymph nodes
what is the clinical manifestation and complication
productive cough foul smelling mucus clubbing accessory muscle use barrel chest cyanosis
what is the goal of treatment for bronchiectasis?
- control infection
- contrilling airway secretion
- preventing complication
what is the treatment for bronchectasis
1lobectomy/segmentectomy
- bronchdilation
- smoking cessation
- avoidance of irritants
