Surfactant synthesis and kinetics in infants with congenital diaphragmatic hernia.
In animal models of congenital diaphragmatic hernia (CDH), surfactant deficiency contributes to the pathophysiology of the disease; however, information on CDH in humans is limited. We compared surfactant disaturated phosphatidylcholine (DSPC) synthesis and metabolism, by stable isotope technology, in newborn infants with CDH and in control subjects. DSPC amount, total proteins, and surfactant protein-A (SP-A) from tracheal aspirates were also measured. DSPC and SP-A were significantly lower in 14 infants with CDH than in the eight control subjects. Mean DSPC was 2.3 +/- 1.3 mg/ml of epithelial lining fluid (ELF) in infants with CDH and 4.6 +/- 1.5 mg/ml of ELF in control subjects (p = 0.001). Mean SP-A in infants with CDH and in control subjects was 16.2 +/- 9.3 and 61.2 +/- 30.6 microg/ml of ELF, respectively (p = 0.03). DSPC kinetics was measured in 12 of 14 infants with CDH and in 5 of 8 control subjects. Secretion time was 8.3 +/- 5.5 and 8.5 +/- 2.5 hours and peak time 51.9 +/- 15.2 and 51 +/- 13 hours in infants with CDH and in control subjects, respectively. Fractional synthesis rate was not different for infants with CDH and control subjects (p = 0.4). In conclusion, surfactant DSPC synthesis and kinetics were not significantly deranged in infants with CDH compared with control subjects. Other factors, such as lower surface area or increased DSPC catabolism, may contribute to surfactant pool alteration in CDH.
Department of Pediatrics, Division of Pediatric Surgery, and Institute of Internal Medicine, University of Padova, Padova, Italy.
Paola E
PE
Cogo
Luc J I
LJ
Zimmermann
Federica
F
Rosso
Francesca
F
Tormena
Piergiorgio
P
Gamba
Giovanna
G
Verlato
Aldo
A
Baritussio
Virgilio P
VP
Carnielli
D006548:Hernia, Diaphragmatic / Q000378:metabolism; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D010713:Phosphatidylcholines / Q000378:metabolism; D011506:Proteins / Q000378:metabolism; D011510:Proteolipids / Q000378:metabolism; D037662:Pulmonary Surfactant-Associated Protein A; D037661:Pulmonary Surfactant-Associated Proteins; D011663:Pulmonary Surfactants / Q000378:metabolism*; D014132:Trachea / Q000378:metabolism; D014508:Urea / Q000378:metabolism
D010713:Phosphatidylcholines; D011506:Proteins; D011510:Proteolipids; D037662:Pulmonary Surfactant-Associated Protein A; D037661:Pulmonary Surfactant-Associated Proteins; D011663:Pulmonary Surfactants; C017463:lecithins, disaturated; D014508:Urea