Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D....

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Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief, Pediatric Pulmonary Director, Cystic Fibrosis Center Director, Pediatric Sleep Disorders Center

Transcript of Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D....

Page 1: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Bronchiectasis in Children

Catherine Kier, M.D.Professor of Clinical Pediatrics

Stony Brook Medicine, Stony Brook, New York

Division Chief, Pediatric Pulmonary

Director, Cystic Fibrosis Center

Director, Pediatric Sleep Disorders Center

Page 2: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Objectives•Objective #1

discuss the specific etiologies and clinical presentation of bronchiectasis in childhood

•Objective #2

review approaches to diagnosis and management and current therapies for bronchiectasis in childhood

Page 3: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Bronchiectasis

Page 4: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Pathophysiology

chronic or repeated episodes of environmental insults+

genetic vulnerability

bronchial injury and dilatation

Chronic infiltrates and atelectasisChronic productive or wet cough

Page 5: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Radiological imaging

Chest x-ray HRCT scan

Page 6: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Pathology

Gross specimen classic saccular enlargement

of the bronchi

Histology/pathologydilated lumen of the bronchus and

chronic inflammation in the bronchial wall

Page 7: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Causes of bronchiectasis• Cystic fibrosis

• Impaired immune function

• Ciliary dyskinesia

• Clinical syndromes

• Congenital tracheobronchomegaly

• Aspiration syndromes

• Obstructive bronchiectasis

• Other pulmonary diseases

Page 8: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

10-year-old boy with chronic infiltrates

Page 9: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

12-year-old boy with cystic bronchiectasis since infancy

Page 10: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

30-year-old male with recurrent pneumonia

Page 11: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

25-year-old male diagnosed with asthma as a child

Page 12: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

14-year-old male with situs inversus

Page 13: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Bronchiectasis in childhood• cystic fibrosis (CF) is the most common cause of bronchiectasis in

childhood

• non-CF bronchiectasis (systematic review)

- specific etiologies on non-CF bronchiectasis in childhood (prevalence)• infectious (17%)

• primary immunodeficiency (16%),

• aspiration (10%)

• ciliary dyskinesia (9

• congenital malformation (3%)

• secondary immunodeficiency (3%)

Brower et al. BMC Pediatrics 2014, 14:299

Page 14: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Underestimate of cases

•Misdiagnosis• “difficult asthma”• Chronic obstructive pulmonary disease (COPD)

• 29% of adults with COPD have underlying bronchiectasis

Page 15: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Genetics

• Interplay between genotype and environment = phenotypic expression of respiratory disease

• Frequency of CFTR genotypes

• Turkish study:• Consanguinity

• Transporter associated with Antigen Presentation (TAP) gene polymorphisms in cohort of children with bronchiectasis

Page 16: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Innate pulmonary immune mechanisms

• Pro-inflammatory cytokine

• Adhesion molecule production and receptor expression

• Exaggerated neutrophilic response

• Metalloproteinases (MMP-2 and -9)• Isolated in sputum and BAL

• Airway destruction by galtinases and collagenases

Page 17: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Reid’s subtypes(bronchographic findings)

• Cylindrical

• Varicose

• Cystic

Varicose and cystic changesCylindrical

Page 18: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

HRCT scoring systems(more recent)

Markers of disease severity

•Cylindrical

• Saccular

Page 19: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Varicose Cystic Cylindrical

Page 20: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

saccular bronchiectasis

Page 21: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Increased bronchoarterial ratio

Diameter of the bronchial lumen divided by the diameter of accompanying artery

Page 22: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

normal bronchoarterial ratio < 5 years of age = 0.5< 18 years of age = 0.8

Page 23: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

fluid-filled dilated bronchi

Page 24: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

bronchial wall thickening

Page 25: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Peripheral airwayLack of Bronchial Tapering

Page 26: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Vascular changes

• Total bronchial arterial blood flow is increased

• Extensive precapillary anastomoses between the two arterial systems (shunt between pulmonary and systemic systems)

• Vascular remodeling of the pulmonary arteries and arterioles (pulmonary hypertension and cor pulmonale)

Page 27: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Airway mucosa abnormality (bronchoscopy)

Page 28: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Sputum markers

• Neutrophilic airway inflammation• CF sputum

• Viscous• Elastic• Adhesive

Page 29: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Mucus-filled saccular airway changes

Page 30: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Respiratory pathogens

• Streptococcus pneumoniae

• Haemophilus influenza non-type b

• Moraxella catarrhalis

• Pseudomonas aeruginosa

Page 31: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,
Page 32: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Mechanisms of lung destruction

• Exaggerated or persistent pulmonary inflammation

• Balance between proteases and anti-proteases

• Collagenase activity (from neutrophils and bacteria)

• Metalloproteinases

• Impaired removal of apoptotic inflammatory cells

• Adhesion molecules

Page 33: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Management Principles

• Aggressive management of infections (antimicrobials)

• Airway clearance methods

• Attention to nutrition

• Vigilant monitoring of clinical trends

• Proactive care

Page 34: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Philosophy of antibiotic use

• Maintenance

• Intermittent

• Regular hospitalizations

Page 35: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Airway Clearance Techniques

• Mechanical “valve” devices (Flutter, Pep, Acapella, and others)

• Postural drainage and chest physiotherapy• Therapeutic vest

Page 36: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Mucus alteration agents

• Inhaled β2-agonists and/or anticholinergic bronchodilators• Hypertonic saline or mannitol inhalation• Dornase alfa (Pulmozyme)• N-Acetylcysteine (Mucomyst)

Page 37: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

New therapies

• Macrolides• Anti-inflammatory• Anti-oxidant• Anti-secretagogue effects• Statins

Page 38: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Other therapies/management

• Asthma therapy• Environmental modification• Prevention: Vaccines

Page 39: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Complications related to bronchiectasis

• Hemoptysis

• Lung abscess

• Pulmonary hypertension

• Sleep disorders

• Reactive airway disease

Page 40: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Lobectomy

• Indications• Poor control of symptoms• Poor growth inspite of optimal medical therapy• Severe and recurrent hemoptysis uncontrolled by bronchial artery

embolization

• Relative indication• Localized disease with moderate persistent symptoms

• Contraindications• Widespread bronchiectasis• Young child (<6 years of age)• Minimally symptomatic disease

Page 41: Bronchiectasis in Children - foocus.com · Bronchiectasis in Children Catherine Kier, M.D. Professor of Clinical Pediatrics Stony Brook Medicine, Stony Brook, New York Division Chief,

Bronchiectasis and health care

• Identifying children for appropriate referral

• Confirm diagnosis and investigate etiology

• Assess severity

• Develop management plan

• Multidisciplinary team approach to chronic care

• Public health issues