Surfactant proteins in pediatric interstitial lung disease

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Matthias Griese1, Elke Lorenz1, Meike Hengst1

  • 1Dr von Hauner Children´s Hospital, German Center for Lung Research (DZL), University of Munich, Munich, Germany.

Pediatric Research
|September 17, 2015
PubMed

Insights

Quantifying surfactant proteins SP-B and SP-C in bronchoalveolar lavage fluid (BALF) aids in diagnosing children's interstitial lung diseases (chILD). Low SP-C levels suggest various lung diseases, while SP-B levels help screen for SP-B deficiency.

Area of Science:

  • Pulmonary Medicine
  • Biochemistry
  • Genetics

Background:

  • Children's interstitial lung diseases (chILD) encompass diverse conditions.
  • Many chILD entities affect the alveolar surfactant region, involving surfactant proteins SP-B and SP-C.

Purpose of the Study:

  • To assess the diagnostic value of quantifying SP-B and SP-C levels in bronchoalveolar lavage fluid (BALF) for chILD.
  • To explore the relationship between SP-C levels and genetic factors.

Main Methods:

  • Quantified SP-B and SP-C levels in BALF from 302 children with chILD and controls using western blotting.
  • Genotyped single-nucleotide polymorphisms (SNPs) in the SFTPC promoter via direct sequencing in a subset of patients.

Main Results:

  • A lack of dimeric SP-B was specific to hereditary SP-B deficiency.
  • Low or absent SP-C was observed in surfactant dysfunction disorders and other diffuse parenchymal lung diseases related to the alveolar surfactant region.
  • A single SNP in the SFTPC promoter was associated with SP-C levels.

Conclusions:

  • SP-B levels are useful for screening SP-B deficiency.
  • Low SP-C levels may indicate diseases linked to mutations in TTF1, SFTPC, ABCA3, and other un identified genes.
  • Measuring SP-B and SP-C levels aids in chILD differential diagnosis; gene sequencing is necessary for precise molecular diagnosis.
Abstract

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