TY - JOUR
T1 - Impaired surfactant protein B synthesis in infants with congenital diaphragmatic hernia
AU - Cogo, Paola E.
AU - Simonato, Manuela
AU - Danhaive, Olivier
AU - Verlato, Giovanna
AU - Cobellis, Giovanna
AU - Savignoni, Francesco
AU - Peca, Donatella
AU - Baritussio, Aldo
AU - Carnielli, Virgilio P.
PY - 2013/3/1
Y1 - 2013/3/1
N2 - 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
AB - 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
KW - Isotope labelling
KW - Metabolism
KW - Pulmonary surfactants
UR - http://www.scopus.com/inward/record.url?scp=84875275057&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84875275057&partnerID=8YFLogxK
U2 - 10.1183/09031936.00032212
DO - 10.1183/09031936.00032212
M3 - Article
C2 - 22700843
AN - SCOPUS:84875275057
VL - 41
SP - 677
EP - 682
JO - European Journal of Respiratory Diseases
JF - European Journal of Respiratory Diseases
SN - 0903-1936
IS - 3
ER -