bronchiectasis and cystic fibrosis

Question 1

what is bronchiectasis ?

Answer 1

permenant dilation of bronchi

may be focal or diffuse

Question 2

what is the pathology of bronchiectasis ?

Answer 2

the constant airway inflammation causes neutrophils to be released - resulting in up regulation of neutrophils and metric metalloproteinase

the normal structures of the wall including cartilage , muscle and elastic tissue are destroyed and replaced by fibrous tissue

contains pools of thick purulation

there can be squamous metaplasia of the bronchial wall due to the constant inflammation and infection it receives

vasculairty in the bronchial wall increases as a result of inflammation
anatsamoses of bronchial and pulmonary arterial circulation can occur

Question 3

what is the bronchial inflammation usually caused by ?

Answer 3

pseudomonas auriginosa , hemophilux influenza - injury the respiratory epithelium and the mucocillary clearance

smoking

cytic fibrosis

COPD

adenovirus and influenza virus

staph areus

no infectious - exposure to toxic substances - eg ammonia

GERD

Question 4

how does bronchiectasis clinically present itself ?

Answer 4

persistant reoccurring cough
purulent sputum production
hemoptysisi
pneumonia

Question 5

what is the diagnosis method to bronchiectasis ?

Answer 5

HRCT

if focal bronchiectasis - consider bronchoscopy
induced sputum for AFB - acid fast bacilli - tuberculosis

diffuse 
consider - CF - sweat chloride test
quantitative immunoglobin test
induced sputum for AFB 
studies for colliery morphology

Question 6

what is the treatment of infection in bronchiectasisi ?

Answer 6

amoxicillin , levofloxacin

pseudomnas - quinolone , aminoglycoside carbapenem , 3rd gen cephalosporins

Question 7

what is cystic fibosis ?

Answer 7

multi system disease

characterised by chronic bacterial infection in the airways ultimately leading to bronchiectasis

Question 8

what s the pathogenesis of CF ?

Answer 8

autosomal recessive disease - CFTR gene mutated
abnormal ion transport function
faulty regulation of NA reabsorption and inability to secrete cl by cftr
REDUCE THE VOLUME OF LIQUId in mucosa

and becomes thick due to lack of fluid

adhesion of mucus to airways

Question 9

what is the diagnostic hallmark of CF in the lung

Answer 9

airway epithelial increased transepithelilal electric potential difference
exhibit abnormalities in NA absorption and CL secretions

Question 10

what is the effect of CF in other parts of the body except for lung

Answer 10

sinusitis commonly

retention of enzymes in pancreas- pancreatitis due to blocked pancreatic duct and blocked billary bile ducts - leading to intestines cannot not fully absorbing nutrients
foul smelly stools - leading to poor growth

reproductive organs

meconium ileus