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Surfactant Synthesis and Kinetics in Infants with Congenital Diaphragmatic Hernia

Department of Pediatrics, Division of Pediatric Surgery, and Institute of Internal Medicine, University of Padova, Padova, Italy; Department of Pediatrics, Erasmus University, Rotterdam, The Netherlands; and Institute of Child Health and Great Ormond Street Hospital, London, United Kingdom

Correspondence and requests for reprints should be addressed to Virgilio P. Carnielli, M.D., Ph.D., Senior Lecturer, Pediatric Nutrition, Institute of Child Health, Great Ormond Street Hospital, 30 Guilford Street, MRC CNRC Room W.4.03, London WC1N 1EH, UK. E-mail: v.carnielli{at}ich.ucl.ac.uk

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 µg/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.

Key Words: surfactant • congenital diaphragmatic hernia • stable isotopes

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