Lecture 61 - Bronchiectasis, CF, and Bronchopulmonary Dysplasia Flashcards
what is the differential dx for “holes” on lung imaging (ct)?
○ Cysts – usually well defined
○ Emphysema – no true walls; surrounded by normal septa
○ Masses with cavitation
○ Abscess
○ Chronic cavity – from prior to TB; thick walled
Bronchiectasis
what is broncheictasis? what is it associated with?
characterized by permanent dilation of bronchi and bronchioles caused by destruction of the muscle and elastic tissue of the bronchus
○ Usually results from, or associated with, chronic infections and resulting chronic inflammation
what are some notable symptoms and physical exam findings of bronchiectasis?
what pattern would PFTs indicate?
Symptoms – lots of think mucus production, SOB
Exam – Bronchial breath sounds; hypoxia, productive cough, rhonchi, wheezing,
Classical Radiological findings of Bronchiectasis?
CXR:
CT:
CXR: airway wall thickening; dilated airways
CT: thickened airway with mucoid impaction
+ SIGNET RING (dilated airway appears much larger than adjacent blood vessel)
List the different Etiology of Bronchiectasis
Psst Infectious – Necrotizing bacterial PNA, TB, non TB
Congenital/hereditary – CF, A1AT def,
Primary Ciliary Dyskinesia (+/- Kartagener’s Syndrome)
Immunodeficiencies: HIV, Ig Def
Autoimmune d/o – IBD, UC, Sjogren’s, RA
Aspiration/GERD
ABPA (fungal hypersensitivity rxn)
What is Primary Ciliary Dyskinesia – PCD
What is Kartagener’s Syndrome:
Mutation to the Dynein Arm of Cilia; poorly functioning
leading to poor airway clearance
Kartagener’s Syndrome:
- Situs Inversus, Sinusitis, and Bronchiectasis
what is Allergic Bronchopulmonary Aspergillosis?
what immune factors/cells would you find?
how is it treated ?
§ Hypersensitivity reaction of airways to colonized aspergillus
§ Characterized by – bronchoeictasis, high IgE, eosinophilia (therefore allergic)
§ Treatment: Corticosteroids, antifungals (itra, voriconazole)
what labs should you order for suspected bronchiectasis?
sputum culture – if concerned for infection or TB etiology
CBC with differnetial – for ABPA
aspergillus antibodies
Sweat CHloride – for CF
what is traction bronchiectasis?
what is seen on CT?
what might be seen on PFTs?
Bronchiectasis in the setting of a pulmonary fibrosis (a restrictive pulmonary disease)
CT - Honeycombing = Fibrosis
PFTs – may be restrictive pattern (if fibrosis dominant)
how is bronchiectasis treated?
Treat the underlying condition
Bronchodilators (sometimes)
Anti-Inflammatory (azithromycin has an anti-inflam compoennet)
Airway clearnace – chest physiotherapy, percussion vests to break up sputum
Abx
what is cystic fibrosis ?
describe the pathophysiology of the respiratory tract
AUtosomal recessive disease
mutation of the CFTR gene (an epithelial chloride channel protein)
affects fluid secretion in exocrine glands and epithelial lining of respiratory tract, GI and reproductive tracts
Chloride channel is defective. Normally secretes chloride ion, while Na is being absrobed. Water follows suit
With no Chloride secretion – much more Water is absorbed into the cell, resulting in a dehydrated mucous that is hard to clear
what is the most common mutation in CF? what is the most affected demographic ?
ΔF508: most common mutation in Caucasians
Common manifestations of CF
Chronic Pulm Disease; can’t clear mucus; leads to a lot of infections
Pancreatic insufficeincy – leading to malnutrtiion etc
high Nacl in sweat (cl is not reabsorbed)
How is CF diagnosed ?
- clinical findings?
- laab findings?
Clinical – □ Chronic cough and sputum production
Sputum culture: ® S . Aureus, Pseudomonas, Burkholderia capecia
Bronchiectasis on imaging –
Progressive decline in FEV1
Lab findings:
- Sweat test
CFTR mutations
what infections are common in peds CF patients?
what infections are common in adult CF patients?
Peds: S. Aureus
Adults: Pseudomonas
