Impaired surfactant protein B synthesis in infants with congenital diaphragmatic hernia

Paola E. Cogo, Manuela Simonato, Olivier Danhaive, Giovanna Verlato, Giovanna Cobellis, Francesco Savignoni, Donatella Peca, Aldo Baritussio, Virgilio P. Carnielli

Research output: Contribution to journalArticlepeer-review


Pulmonary hypoplasia and hypertension account for significant morbidity and mortality in neonates with congenital diaphragmatic hernia (CDH). Whether CDH is associated with surfactant dysfunction remains controversial. Therefore, we measured disaturated phosphatidylcholine (DSPC) and surfactant protein (SP)-B concentration in tracheal aspirates and their synthesis rate in infants with CDH compared to infants without lung disease. 2H2O as a precursor of DSPC and 1-13C-leucine as a precursor of SP-B were administered to 13 infants with CDH and eight controls matched for gestational age. DSPC and SP-B were isolated from tracheal aspirates, and their fractional synthesis rate was derived from 2H and 13C enrichment curves obtained by mass spectrometry. DSPC and SP-B amounts in tracheal aspirates were also measured. In infants with CDH, SP-B fractional synthesis rate and amount were 62±27% and 57±22% lower, respectively, than the value found in infants without lung disease (p

Original languageEnglish
Pages (from-to)677-682
Number of pages6
JournalEuropean Respiratory Journal
Issue number3
Publication statusPublished - Mar 1 2013


  • Isotope labelling
  • Metabolism
  • Pulmonary surfactants

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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