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Childhood Interstitial Lung Disease (chILD)

Heterogeneous group of rare diffuse lung disorders presenting with hypoxemia, tachypnea and crackles.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Childhood Interstitial Lung Disease (chILD)?

Childhood interstitial lung disease (chILD) is a heterogeneous group of rare diffuse parenchymal lung disorders that begin in infancy or childhood. It includes entities specific to infancy (neuroendocrine cell hyperplasia of infancy, pulmonary interstitial glycogenosis, surfactant dysfunction syndromes) and disorders also seen in adults (hypersensitivity pneumonitis, sarcoidosis).

Surfactant dysfunction syndromes (SP-B, SP-C, ABCA3 mutations and TTF1) cause severe neonatal respiratory failure or chronic ILD, while NEHI presents with persistent tachypnea, hypoxemia and characteristic HRCT findings (ground glass in right middle lobe and lingula). Aspiration, immune deficiencies and pulmonary vascular disease should be excluded.

Diagnosis follows the chILD pathway: clinical chILD syndrome, HRCT with sedation if needed, genetic testing for surfactant disorders, bronchoscopy/BAL and, when needed, surgical lung biopsy. Treatment depends on entity and includes oxygen, corticosteroids, hydroxychloroquine, azithromycin, and lung transplantation in selected progressive cases.

Symptoms

Persistent tachypnea and dyspnea
Chronic hypoxemia
Crackles on auscultation
Failure to thrive
Recurrent pneumonia
Cyanosis on exertion
Digital clubbing in chronic disease

Risk Factors

Family history of ILD or surfactant disease
Consanguinity
Surfactant gene mutations (SP-B, SP-C, ABCA3)
Premature birth and chronic lung disease
Aspiration risk factors
Immune deficiencies
Environmental antigen exposure

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent tachypnea in an infant
  • Failure to thrive with respiratory symptoms
  • Recurrent pneumonia and crackles
  • Cyanosis or hypoxemia
  • Family history of childhood lung disease

Treatment Methods

01
Multidisciplinary chILD pathway evaluation
02
Oxygen therapy and nutrition support
03
Corticosteroids and hydroxychloroquine
04
Azithromycin in selected cases
05
Genetic counseling for surfactant disorders
06
Lung transplantation in progressive disease

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

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You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.