4) Bronchiectasis and CF Flashcards
What is Bronchiectasis?
-Chronic infection of the bronchi/bronchioles giving permanent dilatation of these airways
Main organisms colonising bronchiectasis pts?
Strep. pneumoniae H influenzae Moraxella catarr Pseudomonas S. aureus- consider CF/ABPA non tb mycobacteria
Causes of Bronchiectasis?
Congenital:
- CF
- Karageners/PCD/Youngs
post infectious
- measles/pertussis
- bronchiolits
- Pneumonia
- TB/HIV
Other:
- Bronchial obstruction (foreign body/tumour)
- crohns
- RA
- Immunodeficiency (hypergammaglobuliaemia)
- ABPA
Types of dilation in bronchiectasis?
Cyllindrical
Varicose
cystic/saccular
Clinical features of Bronchiectasis?
Persistant productive cough, dyspnoea, fatigue, intermittent haemoptysis
Clubbing, coarse crackles, wheeze?
Complications of bronchiectasis?
Pneumonia, pleural effusion? haemoptysis, empyema, amyloid?
Ix in Bronchiectasis?
Sputum culture
CXR- thickened bronchial walls (tram tracks)
CT- signet ring
Spirometry- often obstructive
Bronchoscopy
ig, CF sweat test, aspergillus, ciliary fx
Management of Bronchiectasis?
Postural drainage +physio
ABX according to sensitivity, if known pseudomonas can try eradication with ciprofloxacin/neb colistin (3 weeks)
excessive exacerbations try long term abx
steroids for ABPA
surgery if isolated disease
immunisations
Which lobe usually gets bronchiectasis in TB?
Middle as smallest lume, get hilar gland swelling which obstruct which then gives bronchiectasis
Features suggesting PCD?
Ent issues in childhood (grommets, sinusitis etc), born with bronchitis
Why do you get laterality disorders with PCD?
Ciliary dysfuntion also affects those that orient your organs, if theyre buggered only 50% chance itll be right
Easy test for PCD?
Nasal NO: if low, likely to be PCD, then can do ciliary brushings for microscopy
What does IBD related bronchiectasis often respond to?
Steroid therapy
1o immunodeficiency that can present in adults?
CVID, suspect in those with dysregulated immune responses
When to suspect other cause of Bronchiectasis in COPD?
Normally have lower lobe cylindrical disease, if it is bad upper lobe consider a different aetiology
What is the mutation in CF?
CFTR gene on chromosome 7. Leads to deficient Cl- secretion and excess NA+ secretion
Presentation of CF in a neonate?
FTF, meconium ileus, rectal prolapse
Presentation of CF later on?
Chest: Cough, infections, bronchiectasis, pneumothorax, failure, cor pulmonale
GI: Pancreatic insufficency- DM, gallstones, cirrhosis
Other: infertility, polyps, HPOA
Cyanosis, clubbing, coarse crackles
Diagnosis of CF?
Sweat test, genetic testing
Fecal elastase is a good marker of pancreatic exocrine fx
Testing in CF?
All, clotting, vitamins( fat absorbed ones) GTT
cultures
cxr
abdo US for fatty liver/chronic pancreatitis
aspergillus testing
Management of CF?
MDT
chest: physio, abx for exacerbation, mucolytics and bronchodilators may be helpful
GI: replace pancreatic enzymes, fat soluble vitamin supplements, uroxodecholyic acid for liver
Manage diabetesm screeen, fertiliy and genetic counselling
Advancced: o2, diuretics for cor Pulm. NiV, transplant
Prognosis in CF now?
Median survival now 40
