1. Chronic Lung Disease in Children
Dr Sarika Gupta (MD,PhD); Asst. Professor

3. Chronic Lung Disease in Children
Cough, wheeze and dyspnoea
Chest deformity
Stunting and failure to thrive
Clubbing
Halitosis
Sputum production
Cyanosis
Cor pulmonale

4. Chronic Lung Disease in Children Patterns of Onset
Chronic symptoms and signs from birth
Anatomical and developmental disorders
T-O Fistulae Laryngo/tracheo/bronchomalacia
Lung cysts/ dysplasia
Congenital lobar emphysema
Diaphragmatic hernia
Persisting disease after neonatal resp distress
BPD (Chronic lung disease of infancy)

5. Chronic Lung Disease in Children Patterns of Onset
Chronic symptoms and signs with insidious onset after normal early life
Immune-mediated disorders
Asthma
Interstitial disorders
Immune deficiency disorders
Chronic or recurrent infection
Infections per se
Tuberculosis

6. Chronic Lung Disease in Children Patterns of Onset
Chronic symptoms and signs after acute onset
Acute non-febrile onset
Foreign body
Inhalation injury (paraffin, smoke)
Acute febrile onset
Bronchiolitis obliterans
Necrotising pneumonia

7. Chronic Lung Disease in Children Disease Patterns
Relapsing symptoms with periods of apparent normality
Reactive airways eg asthma
Exacerbations of symptoms without normality in between
Episodes of febrile deterioration Super-imposed infection on
Abnormal lung/bronchiectasis
Foreign body
Uncontrolled asthma with acute attacks
Chronic persistent symptoms Cough, wheeze

8. Chronic lung disease : clinical categories
Obstructive lung disease
Mainly airway obstruction with air trapping : XRay shows high lung volume
Restrictive lung disease
Low lung volume, lung cannot expand
Destructive lung disease
Usually associated with infection and pus formation

9. Cystic fibrosis Autosomal recessive Chronic respiratory symptoms Cough
Recurring pneumonia
Chronic URTI
Chronic airway obstruction
Bronchiectasis
Pancreatic deficiency and malabsorption
Diagnosis: Sweat ChlorideTest, Gene Probe Δ508, 72 hr. fecal fat determination, fasting blood sugar, liver function studies, sputum culture, X Ray chest

10. Cystic fibrosis
Factor responsible for manifestations of the disease is mechanical obstruction caused by increased viscosity of mucous gland secretions
Mucous glands produce a thick protein that accumulates and dilates the glands
Passages in organs such as the PANCREAS become obstructed
First manifestation is meconium ileus in newborn

11. Cystic fibrosis
Respiratory goal: removal of secretions (chest physiotherapy with Thairapy vest) by vibrations loosen mucus
Nutritional:
Fat soluble vitamins ADKE
High calorie, high protein, low fat
Maintain Na balance (when sweating and ill)

12. Bronchiectasis
Permanent destruction of bronchial wall and lung tissue due to chronic infection :
Lumen obstruction eg foreign body, lymph nodes
Parenchymal destruction from pneumonia with tissue necrosis
Repeated respiratory infections with Cystic Fibrosis, malnutrition, HIV, recurrent aspiration

13. Bronchiectasis Repeatedly ill with febrile chest infections
Clubbing and halitosis
Discoloured sputum
Widespread crackles and wheezes
Progressive deterioration of lung function
Development of pulmonary hypertension
Management : Physiotherapy, antibiotics, bronchodilators, immunize against “flu”, surgery in localized unilateral disease with good lung function

14. Chronic wheezing Splinting of upper limb girdle Barrel chest
Air trapping on X Ray
Causes:
Asthma
Bronchiolitis obliterans
HIV-associated lung disease

15. Bronchopulmonary Dysplasia
DEFINITION: defined as a need for increased oxygen:
• Infants <32 weeks gestation: oxygen requirement at 36 weeks gestational age or at discharge (whichever comes first)
• Infants ≥32 weeks gestation: oxygen requirement at age >28 d or at discharge (whichever comes first)
INCIDENCE is inversely related to birth weight and GA

16. Bronchopulmonary Dysplasia
ETIOLOGICAL FACTORS include:
• Lung immaturity with (a) ↑ susceptibility to damage from oxygen, barotrauma and volutrauma, (b) surfactant deficiency and (c) immature antioxidant defenses
• Oxygen toxicity
• Barotrauma and volutrauma
• Pulmonary edema (excessive fluid administration, patent ductus arteriosus)
• Inflammation (multiple associated biochemical changes)

17. Bronchopulmonary Dysplasia
CLINICAL FEATURES:
• Hypoxia due to V/Q mismatch
• ↑ work of breathing
• Abnormal chest radiograph
• ↑ airway resistance is late feature
• Pulmonary hypertension
• Cor pulmonale (late)

18. Bronchopulmonary Dysplasia
PREVENTIVE MEASURES:
• Minimize barotrauma & volutrauma
• Minimize oxygen toxicity
• Careful attention to intake of fluid and Na+
TREATMENT of ESTABLISHED CLD:
Adequate caloric intake
After 36 weeks GA, maintain O2 saturation >95%
Restrict intake of fluid and Na+
Diuretics
Bronchodilators
Steroids are almost never indicated
Infection prevention

19. Interstitial lung disease
Interstitial lung disease consists of a diverse group of disorders that involve the pulmonary parenchyma and interfere with gas exchange
"diffuse parenchymal lung disease" or “diffuse lung disease”
 ILD is rare in childhood
Children with ILD may present with respiratory failure, or with more indolent or chronic symptoms including tachypnea, hypoxemia, retractions, cough, exercise intolerance, failure to thrive, gastroesophageal reflux

20. Interstitial lung disease
 ILD should be considered in any neonate who presents with unexplained respiratory failure,
or in infants and children with a normal birth history who present with persistent tachypnea, crackles, hypoxemia, chronic cough, or clubbing of the digits
ILD should also be considered in late preterm or preterm infants who present with chronic lung disease out of proportion to the degree of prematurity or other known comorbidities

21. Interstitial lung disease
 Diagnostic studies in children with suspected interstitial lung disease
To exclude more common causes of chronic respiratory symptoms:
Sweat chloride testing
Evaluation for gastroesophageal reflux and recurrent aspiration (such as barium swallow, pH/impedance probe and others)
Bronchoscopy
Cultures and testing for infectious etiologies
Echocardiogram
Testing for HIV and other immunodeficiencies

22. Interstitial lung disease
 Diagnostic studies in children with suspected interstitial lung disease to assess extent and severity of disease:
Chest radiographs
Chest computed tomography scan
Electrocardiogram/ Echocardiogram
Pulmonary function studies

23. Interstitial lung disease
 Diagnostic studies in children with suspected interstitial lung disease to identify systemic disorders predisposing to interstitial lung disease:
Immune studies - Immunodeficiency/immune dysfunction
Studies for connective tissue disease-ANA, ANCA (Autoimmune disease, Sarcoidosis, Vasculitis syndromes including granulomatosis with polyangiitis [Wegener's], Anti-glomerular basement membrane disease)
Hypersensitivity pneumonitis panel
Serum and urine amino acids
Genetic studies for surfactant dysfunction

24. Interstitial lung disease
TREATMENT — supportive therapy and pharmacologic interventions, tailored to the type of ILD:
Supportive therapy
Limiting exposure to cigarette smoke and other inhaled irritants
Oxygen therapy for hypoxemia
Supervised exercise
Bronchodilators for reversible airway obstruction
Aggressive treatment of intercurrent infections
Standard childhood vaccinations
RSV Immunoprophylaxis

25. Interstitial lung disease
Specific treatment — Specific treatment is available for some ILD disorders
Antimicrobials for certain infections, management of swallowing dysfunction and/or reflux in patients with chronic aspiration, avoidance of the offending antigen in hypersensitivity pneumonitis, and whole lung lavage for older children with pulmonary alveolar proteinosis
Glucocorticoids are the mainstay of therapy for many children with ILD because inflammation and inappropriate cellular proliferation are thought to play an important role in pathogenesis of many ILD subtypes
Lung transplantation

26. Chronic lung disease : Management
In patients with long standing symptoms consider the possibility of chronic lung disease rather than just prescribing antibiotic treatment
Diagnosis : XRay, TB skin test, special studies-sweat test, CT scan
Functional assessment : Lung function, 02, CO2
Airway management: bronchodilator
Specific therapy (anti-inflammatory)
Antibiotic for infection and fever
Physiotherapy : postural drainage
Monitor for pulmonary hypertension and cor pulmonale

27. Summary