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Types of Childhood Interstitial Lung Disease

The broad term "childhood interstitial lung disease" (chILD) refers to a group of rare lung diseases that can affect babies, children, and teens. Some of these diseases are more common in certain age groups.

Diseases more common in infancy include:

  • Surfactant (sur-FAK-tant) dysfunction mutations
  • Developmental disorders, such as alveolar capillary dysplasia
  • Lung growth abnormalities
  • Neuroendocrine cell hyperplasia of infancy (NEHI)
  • Pulmonary interstitial glycogenosis (PIG)

Diseases more common in children older than 2 years of age and teens include:

  • Idiopathic interstitial pneumonias:
    • Nonspecific interstitial pneumonia
    • Cryptogenic organizing pneumonia
    • Acute interstitial pneumonia
    • Desquamative interstitial pneumonia
    • Lymphocytic interstitial pneumonia
  • Other primary disorders:
    • Alveolar hemorrhage syndromes
    • Aspiration syndromes
    • Hypersensitivity pneumonitis
    • Infectious or postinfectious disease (bronchiolitis obliterans)
    • Eosinophilic pneumonia
    • Pulmonary alveolar proteinosis
    • Pulmonary infiltrates with eosinophilia
    • Pulmonary lymphatic disorders (lymphangiomatosis, lymphangiectasis)
    • Pulmonary vascular disorders (haemangiomatosis)
  • ILD associated with systemic disease processes:
    • Connective tissue diseases
    • Histiocytosis
    • Malignancy-related lung disease
    • Sarcoidosis
    • Storage diseases
  • Disorders of the compromised immune system:
    • Opportunistic infection
    • Disorders related to therapeutic intervention
    • Lung and bone marrow transplant-associated lung diseases
    • Diffuse alveolar damage of unknown cause

The various types of chILD can affect many parts of the lungs, including the alveoli (air sacs), bronchial tubes (airways), and capillaries. (Capillaries are the tiny blood vessels that surround the air sacs.) The structures of the lung that chILD may affect are shown in the illustration below.

Normal Lungs and Lung Structures

The photo shows how spirometry is done. The patient takes a deep breath and then blows hard into a tube connected to a spirometer. The spirometer measures the amount of air breathed out. It also measures how fast the air was blown out.

Figure A shows the location of the lungs and airways in the body. Figure B is a detailed view of the lung structures that childhood interstitial lung disease may affect, such as the bronchioles, neuroendocrine cells, alveoli, capillary network, surfactant, and interstitial space.

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January 1, 2011