CHRONIC LUNG DISEASE IN CHILDREN

Dr Sarika Gupta (MD,PhD); Asst. Professor

Chronic Lung Disease in Children

Cough, wheeze and dyspnoea Chest deformity Stunting and failure to thrive Clubbing Halitosis Sputum production Cyanosis Cor pulmonale

Chronic Lung Disease in Children

Patterns of Onset Chronic symptoms and signs from birth Anatomical and developmental disorders

T-O Fistulae Laryngo/tracheo/bronchomalaciaLung cysts/ dysplasiaCongenital lobar emphysemaDiaphragmatic hernia

Persisting disease after neonatal resp distress

BPD (Chronic lung disease of infancy)

Chronic Lung Disease in Children

Patterns of Onset Chronic symptoms and signs with insidious

onset after normal early life Immune-mediated disorders

AsthmaInterstitial disorders

Immune deficiency disordersChronic or recurrent infection

Infections per seTuberculosis

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

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 deteriorationSuper-imposed infection on

Abnormal lung/bronchiectasis

Foreign body

Uncontrolled asthma with acute attacks Chronic persistent symptoms

Cough, wheeze

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

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

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

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)

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

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

Chronic wheezing

Splinting of upper limb girdle Barrel chest Air trapping on X Ray

Causes: Asthma Bronchiolitis obliterans HIV-associated lung disease

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

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)

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)

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

 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

 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

 Interstitial lung disease

 Diagnostic studies in children with suspected interstitial lung disease

To exclude more common causes of chronic respiratory symptoms:

1. Sweat chloride testing

2. Evaluation for gastroesophageal reflux and recurrent aspiration (such as barium swallow, pH/impedance probe and others)

3. Bronchoscopy

4. Cultures and testing for infectious etiologies

5. Echocardiogram

6. Testing for HIV and other immunodeficiencies

 Interstitial lung disease

 Diagnostic studies in children with suspected interstitial lung disease to assess extent and severity of disease:

1. Chest radiographs

2. Chest computed tomography scan

3. Electrocardiogram/ Echocardiogram

4. Pulmonary function studies

 Interstitial lung disease

 Diagnostic studies in children with suspected interstitial lung disease to identify systemic disorders predisposing to interstitial lung disease:

1. Immune studies - Immunodeficiency/immune dysfunction

2. Studies for connective tissue disease-ANA, ANCA (Autoimmune disease, Sarcoidosis, Vasculitis syndromes including granulomatosis with polyangiitis [Wegener's], Anti-glomerular basement membrane disease)

3. Hypersensitivity pneumonitis panel

4. Serum and urine amino acids

5. Genetic studies for surfactant dysfunction

 Interstitial lung disease

TREATMENT — supportive therapy and pharmacologic interventions, tailored to the type of ILD:

Supportive therapy

1. Limiting exposure to cigarette smoke and other inhaled irritants

2. Oxygen therapy for hypoxemia

3. Supervised exercise

4. Bronchodilators for reversible airway obstruction

5. Aggressive treatment of intercurrent infections

6. Standard childhood vaccinations

7. RSV Immunoprophylaxis

 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

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