Impaired surfactant protein B synthesis in infants with congenital diaphragmatic hernia.
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. (2)H2O as a precursor of DSPC and 1-(13)C-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 (2)H and (13)C 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<0.01 and p<0.05, respectively). There were no significant group differences in DSPC fractional synthesis rate and amount. Infants with CDH have a lower rate of synthesis of SP-B and less SP-B in tracheal aspirates. In these infants, partial SP-B deficiency could contribute to the severity of respiratory failure and its correction might represent a therapeutic goal.
Terapia Intensiva Cardiochirurgica, Ospedale Paediatrico Bambino Gesu, Giustiniani, Rome, Italy. paola.cogo@opbg.net
Paola E
PE
Cogo
Manuela
M
Simonato
Olivier
O
Danhaive
Giovanna
G
Verlato
Giovanna
G
Cobellis
Francesco
F
Savignoni
Donatella
D
Peca
Aldo
A
Baritussio
Virgilio P
VP
Carnielli
D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D016022:Case-Control Studies; D005865:Gestational Age; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000378:metabolism; D065630:Hernias, Diaphragmatic, Congenital; D013058:Mass Spectrometry; D010713:Phosphatidylcholines / Q000378:metabolism; D011649:Pulmonary Alveolar Proteinosis / Q000150:complications / Q000151:congenital; D037701:Pulmonary Surfactant-Associated Protein B / Q000172:deficiency / Q000378:metabolism; D012121:Respiration, Artificial; D014132:Trachea / Q000378:metabolism; D017563:Lung Diseases, Interstitial
C017463:lecithins, disaturated; D010713:Phosphatidylcholines; D037701:Pulmonary Surfactant-Associated Protein B