Bronchiectasis/cystic fibrosis/lung abscess Flashcards
what is the definition of bronchiectasis
irreversible dilation of one or more bronchi in a focal or diffuse manner due to inflammation
what is the classification of bronchiactasis
cylindrical or tubular
varicose
cystic
what is the etiology of the two types of bronchiactasis?
focal -> obstruction
diffuse -> infections, bacterial
what are other causes of bronchictasis?
immuno def
Kartageners syndrome
explain shortly the pathogenesis of bronchictasis
decreased mucociliary clearance casues secretion retention -> increased risk of infection -> infllamtion causing weakening wall -> bronchictasis -> deficitary secretion clearance ->
what are the clinical manifestations ?
persistent orductive cough
with production of thick, tenacious sputum
acute exacerbations
- changes in the nature of sputum production
- increased volume and purulence
explain the physical examination findings
crackles
wheezing
clubbing of the digits
explain the diagnosis of bronchiactasis
specific findings of bronchiectasis
persistent chronic cough and sputum production
radiography - tram tracks
CT - tree bud
wall thickening
cysts from the wall
signet ring sign
what is the bronchoscopy in bronchicatsis used for?
to asses forcal bronchiectasis
what is pulmonary fucntional testing for in bronchiectasis?
obstruction dysfunction - early
obstructive/restrctive - later
what are the treatment for bronchictasis ?
antibiotics
bronchial hygiene
anti inflammatory
surgery
transplnatation
what are the complications of bronchictasis ?
hemoptysis due to bronchial artery embolism
respiratory failure
what is the definition of cystic fibrosis
autosomal recessive transmitted disease affecting the secretion of exocrine glands
resulting in production of an inspissated secretion
explain the clinical manifestations of CF
bronchiectasis with collateral tissue injury
pancreatic insufficiency
intestinal obstruction
What is CFTR and what does it do?
Cystic Fibrosis Transmembrane Conductance Regulator
It’s a protein that acts as an anion channel in the cell membranes of epithelial cells (cells lining passageways in the body).
It regulates the movement of salt and water, which is essential for the consistency of secretions like mucus.
The gene for CFTR is located on chromosome 7.
How does a faulty CFTR protein lead to the thick mucus seen in cystic fibrosis?
Impaired Ion Transport: In CF, the CFTR protein doesn’t work properly, so chloride ions can’t move correctly across cell membranes. This disrupts the balance of salt and water in secretions.
Dehydrated Mucus: The mucus layer becomes dehydrated and thick because water is drawn towards the higher salt concentration outside the cells.
Reduced Mucociliary Clearance: The thick mucus can’t be easily cleared by the cilia (tiny hairs that sweep mucus out of the airways), leading to blockages and infections.
What are the effects of thick mucus accumulation in the airways of someone with CF?
Airway Obstruction: Thick mucus plugs the airways, making it difficult to breathe.
Chronic Infection: Bacteria thrive in the stagnant mucus, leading to repeated lung infections.
Inflammation and Damage: Ongoing infection and inflammation damage the airways, eventually causing bronchiectasis (permanent widening).
Submucosal Gland Hypertrophy: The glands that produce mucus become enlarged and filled with thick secretions.
Epithelial Damage: The airway lining is damaged and buried under layers of mucus and debris.
what are the clinical manifestions of CF?
sputum secretion, like bronchiectasis, clubbing
dyspnea
what are the rare respiratory manifestations of CF?
spontaneous pneumothorax
asthma
ABPA
what are the GI manifesations?
pancreatic insufficiency
if not treated it causes steatorrhea
biliary duct obstruction -> biliary cirrhosis ->
- hepatic failure
- splenomegaly
- hemmorhage from esophageal carices
what are the distal intestional obstruction syndrome in CF?
ingestion of large quantity of fats
in the absence of enzyme substitution therapy
what is the diagnosis of CF?
lab tests -> confirmation
- DNA analysis - most valuable
- sweat analysis - shows elevated salt level
- electric potential diff - hgiher in CF
- screening - DNA test, increased trypsin
what is the treatment of CF?
deit
postural drainage
what is the definition of lung abscess ?
necrosis and cavitation of the lung following a microbial infection
can be single or multiple