Chronic Lung Disease in Children

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

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

3. Chronic Lung Disease in ChildrenPatterns 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)

4. Chronic Lung Disease in ChildrenPatterns 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

5. Chronic Lung Disease in ChildrenPatterns 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

6. Chronic Lung Disease in ChildrenDisease 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

7. 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

8. 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

9. 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

10. 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)

11. 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

12. 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

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

14. 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

15. 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)

16. 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)

17. 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

18.  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

19.  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

20.  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

21.  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

22.  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

23.  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

24.  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

25. 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