Transcript Chronic Lung Disease in Children

CHRONIC LUNG
DISEASE IN CHILDREN
Dr Sarika Gupta (MD,PhD); Asst. Professor
Chronic Lung Disease in Children
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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
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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)
Chronic Lung Disease in Children
Patterns of Onset
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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
Chronic Lung Disease in Children
Patterns of Onset
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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
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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
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
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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
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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
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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
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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
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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
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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
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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)
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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)
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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
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Interstitial lung disease
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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
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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
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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
Interstitial lung disease
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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
Interstitial lung disease
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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
Interstitial lung disease
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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
Interstitial lung disease
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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
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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