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Bronchiectasis Therapy Decrease inflammation Bronchopulmonary Hygiene Slide 31 Surgery Cystic Fibrosis Epidemiology CF Genetics CFTR cystic fibrosis ... – PowerPoint PPT presentation

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1
Bronchiectasis
  • Dr.Balamugesh

2
  • Chronic dilation of the bronchi marked by fetid
    breath and paroxysmal coughing, with the
    expectoration of mucopurulent matter.

Dorlands Illustrated Medical Dictionary 28th
Ed., W.B. Saunders Company
3
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4
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5
Morphological types
  • Cylindrical or tubular bronchiectasis
  • Varicose
  • saccular or cystic bronchiectasis

6
Bronchiectasis
  • Causes and pathogenesis
  • Microbiology /common pathogens
  • Therapeutic Goals

7
Ct/cxr
8
Ct/cxr
9
CAUSES
10
Bronchiectasis
  • Worldwide, infection is the primary cause
  • M. Tuberculosis
  • Childhood illnesses
  • Rubeola, B. Pertussis

11
Infection
Inflammation
Bronchiectasis
Altered development
12
Inflammation
  • Characteristics across etiologies
  • Persistent
  • Neutrophil dominant
  • Pro-inflammatory cytokines (IL-8, IL-1, TNF-a)
  • Low anti-inflammatory cytokines (IL-10)

13
Airway Damage Failure to Resolve Inflammation
  • Inappropriate inflammation
  • e.g. ABPM, CF (?)
  • Impaired clearance of stimuli
  • Bacteria, mucus, toxins

14
Airway Damage Failure to Resolve Inflammation
  • Altered Airway Milieu
  • Proteolytic damage
  • -e.g. cleaved receptors
  • -impaired macrophage function
  • Oxidant stress
  • -low antioxidants associated with worse disease
  • -dysregulation of signaling and cellular
    function

15
Childhood Infections
M. Tb.
Infection
Impaired Clearance
Inflammation
Bronchiectasis
Altered development
16
Impaired Clearance
  • Altered ciliary Function
  • PCD, smoking, CFTR, Youngs
  • Mucus rheology
  • CFTR, Mucoid Ps. A
  • Dilated or obstructed airways
  • Impaired cough, foreign bodies, aspiration

17
Impaired ImmunityIneffective inflammation
  • Acquired
  • Chemo-immunomodulation
  • Congenital/innate

18
HIV/AIDS
Chemo-Immunosuppression anti-TNF, MTX,
anti-neoplastics
Childhood Infections
M. Tb.
Innate Deficiency CGD, CVID, Igopathy (IFN)
Infection
Impaired Clearance foreign body, CF
Inflammation
Bronchiectasis
Altered development
19
Chemo-Immunosuppression
HIV/AIDS
Childhood Infections
M. Tb.
Innate Deficiency
Infection
Impaired Clearance
Inflammation
Auto-Immune RA, Sjogrens, IBD ABPM
Bronchiectasis
Altered development
20
Chemo-Immunosuppression
HIV/AIDS
Childhood Infections
M. Tb.
Innate Deficiency
Infection
Impaired Clearance
Inflammation
Auto-Immune ABPM
Bronchiectasis
Altered development
21
Chemo-Immunosuppression
HIV/AIDS
Childhood Infections
M. Tb.
Innate Deficiency
Infection
Impaired Clearance
Inflammation
Auto-Immune ABPM
Bronchiectasis
Altered development
22
BronchiectasisEpidemiology and Etiology
  • Who?
  • Patients - with altered immune system
  • - with persistent respiratory symptoms
  • Why?
  • Persistent inflammation in the respiratory system

23
Bronchiectasis TherapyDecrease inflammation
  • antibiotics
  • clearance
  • Flutter, IPPV, Vest, Bronchodilators, hypertonic
    saline
  • (anti-inflammatory chemotherapy)
  • Steroids, macrolides, interferon-gamma, ibuprofen
  • (surgical resection)

24
When to suspect bronchiectasis?
  • Chronic cough, sputum
  • Coarse rales
  • Persistent respiratory symptoms
  • Recurrent pneumonia
  • Progressive obstructive lung disease
  • Funny bugs

25
Clinical Characteristics
  • Focal
  • Sputum production
  • Mild lt15 cc/d
  • Moderate 15-150 cc/d
  • Severe gt150 cc/d
  • Hemoptysis
  • Dyspnea
  • Chest pain
  • Systemic
  • Malnutriton/wasting
  • Chronic Inflammation
  • gammaglobulinemia
  • CRP
  • Sed rate
  • anemia

26
Bronchiectasis TherapyAntibiotics
  • Episodic or suppressive antibiotics?
  • Yes.
  • Selective pressure vs. suppression of damage

27
Bronchiectasis TherapyAntibiotics
  • Pro
  • Decrease inflammation
  • Slow progression
  • Eradication?

Con Select resistance Cost Side effects adherence
difficult (e.g. Huong et al.)
28
Bronchiectasis TherapyAntibiotics when to use?
  • Yes
  • Evidence of exacerbation
  • Progressive decline
  • Frequent exacerbator
  • Active inflammation (?)

No Minimal disease without organism Patient
Intolerant Unaffordable
29
Bronchiectasis TherapyDecrease inflammation
  • Clearance
  • Immunomodulatory chemotherapy
  • proposed therapies
  • Steroids
  • Macrolides, tetracyclines
  • interferon-gamma
  • ibuprofen

30
Bronchopulmonary Hygiene
  • removal of respiratory secretions is beneficial
  • chest percussion and postural drainage
  • chest clapping or cupping
  • inflatable vests or mechanical vibrators
  • Oral devices that apply positive end-expiratory
    pressure maintain the patency of the airway
    during exhalation

31
  • Maintaining adequate systemic hydration, enhanced
    by nebulization with saline,
  • Acetylcysteine delivered by nebulizer thins
    secretions
  • aerosolized recombinant human DNase (rhDNase) in
    patients with cystic fibrosis

32
Surgery
  • Localised bronchiectasis
  • Proximal obstructive lesion
  • Massive hemoptysis
  • Recurrent infections

33
Cystic FibrosisEpidemiology
  • 1/2500 caucasian births (3-5 carrier rate)
  • Autosomal recessive defect of CFTR
  • Gene identified 1989 on Cr. 7
  • 30,000 affected in USA
  • 12,000 adults
  • survival 31 years

34
CF Genetics
  • Autosomal recessive, non-lethal
  • gt900 identified aberrations
  • Genotypephenotype correlation poor
  • Survival benefit (?)
  • Heterozygote-phenotypes postulated (pancreatitis,
    ABPA, MOTT bronchiectasis)

35
CFTRcystic fibrosis transmembrane regulator
  • chloride channel
  • epithelial cells
  • variable genotype - phenotype linkage
  • various defects identified
  • ion transport
  • regulation
  • processing
  • production

36
Results of Decreased CFTR Function
  • Primary
  • Increased water resorption increased mucus
    viscosity
  • Altered cellular signaling intra/trans(?)
  • (?) altered cholera toxin effects
  • Secondary
  • Obstruction
  • Inflammation
  • Dysfunction

37
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38
Major Clinical Manifestations
  • Sinusitis, polyps
  • Lung
  • Chronic infections
  • Obstructive disease
  • GI
  • Insufficiency
  • CFRDM
  • Liver disease
  • Reproductive
  • Decreased fertility

39
22/yo male ja Fev1 deltaF508/deltaF508
40
CF respiratory pathology
  • Inflammation starts as early as 4 weeks of age
  • Culture negative
  • BAL increased IL-8, LTB4, neutrophils,
    macrophages

41
CF pathologic changes
  • Chronic bronchiectasis
  • Obstructive lung disease
  • Respiratory failure
  • Transplantation/death

42
The CF Diagnosis earlyor typical presentation
  • Newborn screening
  • Serum trypsinogen with confirmatory sweat
    chloride testing
  • Meconium Ileus
  • Failure to thrive/malabsorption
  • Recurrent sino-pulmonary disease

43
Diagnosis of CF
  • Clinical setting
  • Chronic sino-pulmonary disease
  • GI / Nutritional disease
  • Salt loss syndromes
  • CFTR abnormality
  • Abnormal sweat test
  • gt 60
  • Nasal potential
  • Mutational analysis
  • gt 2

44
Vicious loops
Infection
Bronchial Obstruction
Inflammation
Bronchiectasis
45
Summary
management
diagnosis
  • History
  • Prior infections, exposures
  • Time course
  • Other manifestations?
  • Chest Imaging
  • Define region, pattern
  • Sputum culture
  • Determine causal disease
  • sweat testing
  • immune testing
  • serologic testing
  • Treat bronchiectasis
  • Clearance
  • Antibiotics
  • Immune-modulation
  • Balance burden of disease vs burden of therapy
  • (Sputum, Symptoms, PFTs, Weight, X-rays)
  • Other
  • Underlying disease therapy
  • Transplantation
  • Management of complications
  • Collapse, plugs, hemoptysis

46
Vicious loop
Antibiotics tobramycin
Infection
Bronchial Obstruction
Inflammation
Clearance Albuterol, DNase, Therapy vest
Anti-inflammatory Inhaled steroids
Bronchiectasis
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