Infant pulmonary function testing in chronic pneumonitis of infancy due to surfactant protein C mutation

Pediatr Pulmonol. 2015 Jun;50(6):E17-23. doi: 10.1002/ppul.23166. Epub 2015 Mar 9.

Abstract

Pulmonary function testing is a vital tool in evaluation and management of adult ILD patients and is rarely overlooked during workup. However, there is paucity of data regarding its usefulness in management of infants with suspected interstitial lung disease. In this paper, we present the contribution of infant pulmonary function testing (iPFT) to the management of two infants with biopsy confirmed chronic pneumonitis of infancy due to surfactant protein C mutation. We have productively and safely used serial iPFT for decision making both during diagnosis and follow-up of these infants.

Keywords: child interstitial lung disease (chILD); chronic pneumonitis of infancy (CPI); diffuse lung disease (DLD); infant pulmonary function testing (iPFT); surfactant protein C deficiency (SFTPC).

Publication types

  • Case Reports

MeSH terms

  • Female
  • Humans
  • Infant
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / genetics
  • Lung Diseases, Interstitial / physiopathology
  • Mutation*
  • Pneumonia / diagnosis*
  • Pneumonia / genetics
  • Pneumonia / physiopathology
  • Pulmonary Surfactant-Associated Protein C / genetics*
  • Respiratory Function Tests*

Substances

  • Pulmonary Surfactant-Associated Protein C