4) Bronchiectasis and CF Flashcards

1
Q

What is Bronchiectasis?

A

-Chronic infection of the bronchi/bronchioles giving permanent dilatation of these airways

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2
Q

Main organisms colonising bronchiectasis pts?

A
Strep. pneumoniae
H influenzae
Moraxella catarr
Pseudomonas
S. aureus- consider CF/ABPA
non tb mycobacteria
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3
Q

Causes of Bronchiectasis?

A

Congenital:

  • CF
  • Karageners/PCD/Youngs

post infectious

  • measles/pertussis
  • bronchiolits
  • Pneumonia
  • TB/HIV

Other:

  • Bronchial obstruction (foreign body/tumour)
  • crohns
  • RA
  • Immunodeficiency (hypergammaglobuliaemia)
  • ABPA
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4
Q

Types of dilation in bronchiectasis?

A

Cyllindrical
Varicose
cystic/saccular

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5
Q

Clinical features of Bronchiectasis?

A

Persistant productive cough, dyspnoea, fatigue, intermittent haemoptysis

Clubbing, coarse crackles, wheeze?

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6
Q

Complications of bronchiectasis?

A

Pneumonia, pleural effusion? haemoptysis, empyema, amyloid?

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7
Q

Ix in Bronchiectasis?

A

Sputum culture
CXR- thickened bronchial walls (tram tracks)
CT- signet ring
Spirometry- often obstructive
Bronchoscopy
ig, CF sweat test, aspergillus, ciliary fx

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8
Q

Management of Bronchiectasis?

A

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

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9
Q

Which lobe usually gets bronchiectasis in TB?

A

Middle as smallest lume, get hilar gland swelling which obstruct which then gives bronchiectasis

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10
Q

Features suggesting PCD?

A

Ent issues in childhood (grommets, sinusitis etc), born with bronchitis

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11
Q

Why do you get laterality disorders with PCD?

A

Ciliary dysfuntion also affects those that orient your organs, if theyre buggered only 50% chance itll be right

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12
Q

Easy test for PCD?

A

Nasal NO: if low, likely to be PCD, then can do ciliary brushings for microscopy

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13
Q

What does IBD related bronchiectasis often respond to?

A

Steroid therapy

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14
Q

1o immunodeficiency that can present in adults?

A

CVID, suspect in those with dysregulated immune responses

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15
Q

When to suspect other cause of Bronchiectasis in COPD?

A

Normally have lower lobe cylindrical disease, if it is bad upper lobe consider a different aetiology

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16
Q

What is the mutation in CF?

A

CFTR gene on chromosome 7. Leads to deficient Cl- secretion and excess NA+ secretion

17
Q

Presentation of CF in a neonate?

A

FTF, meconium ileus, rectal prolapse

18
Q

Presentation of CF later on?

A

Chest: Cough, infections, bronchiectasis, pneumothorax, failure, cor pulmonale

GI: Pancreatic insufficency- DM, gallstones, cirrhosis

Other: infertility, polyps, HPOA

Cyanosis, clubbing, coarse crackles

19
Q

Diagnosis of CF?

A

Sweat test, genetic testing

Fecal elastase is a good marker of pancreatic exocrine fx

20
Q

Testing in CF?

A

All, clotting, vitamins( fat absorbed ones) GTT
cultures
cxr
abdo US for fatty liver/chronic pancreatitis
aspergillus testing

21
Q

Management of CF?

A

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

22
Q

Prognosis in CF now?

A

Median survival now 40