Disorders of surfactant metabolism

Description

Primary interstitial lung disease specific to childhood due to pulmonary surfactant protein anomalies is a group of interstitial lung diseases (ILD) induced by genetic mutations disrupting surfactant function and gas exchange in the lung. The disorders caused by these mutations affect full-term infants and older children and exhibit considerable overlap in their clinical and histologic presentation

Index terms

Disorders of surfactant metabolism
Chronic lung disease with surfactant disorder
Late onset surfactant deficiency

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Neonatal acute respiratory distress with surfactant disorder
Surfactant protein B (SP-B) deficiency
Surfactant ABCA3 gene mutation
Surfactant protein C gene mutation

Exclusions from above levels

01 – Certain infectious or parasitic diseases
05 – Endocrine, nutritional or metabolic diseases
18 – Complications of pregnancy, childbirth and the puerperium

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19 – Certain conditions originating in the perinatal period
20 – Congenital malformations, deformations and chromosomal abnormalities
22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

CB04.0 – Diffuse pulmonary developmental disorders
CB04.1 – Pulmonary lymphatic dysplasia syndromes
CB04.3 – Alveolar or peri-alveolar conditions
CB04.4 – Pulmonary capillaritis
CB04.5 – Brain-lung-thyroid syndrome
CB04.6 – Chronic pneumonitis of infancy
CB04.7 – Neuroendocrine cell hyperplasia of infancy
CB04.Y – Other specified primary interstitial lung diseases specific to infancy or childhood
CB04.Z – Primary interstitial lung diseases specific to infancy or childhood, unspecified

Foundation entity

Disorders of surfactant metabolism – http://id.who.int/icd/entity/1387730200

Part of: CB04: Primary interstitial lung diseases specific to infancy or childhood › 12: Diseases of the respiratory system