Predicting inadequate long-term lung development in children with congenital diaphragmatic hernia: an analysis of longitudinal changes in ventilation and perfusion.

PURPOSE: Infants born with congenital diaphragmatic hernia (CDH) demonstrate a wide variability in postnatal catch-up lung growth. The goals of this study were to assess the pulmonary development of children born with CDH using sequential ventilation-perfusion (V/Q) scintigraphy and to identify the perinatal factors that correspond to a progressive V/Q mismatch. METHODS: The records of 137 patients seen between 1990 and 2005 in a multidisciplinary CDH clinic were reviewed. Changes in the ipsilateral V/Q quotient were compared in 46 patients who had 2 or more studies with the following variables: sex, patch repair, laterality, gestational age, and use of extracorporeal membrane oxygenation. An abnormal V/Q quotient was defined as greater than 1.2 (reference range, 0.8-1.2). RESULTS: Abnormal V/Q scans were identified in 28 (61%) of the 46 patients at the time of the last V/Q study. Patients who underwent a patch repair had nearly 7 times the risk (P < .001) of developing an ipsilateral V/Q mismatch. The use of extracorporeal membrane oxygenation had a variable effect on the probability of an abnormal V/Q study finding. No other variable was significant. CONCLUSIONS: Many children with CDH develop significant and progressive V/Q mismatches. Although some perinatal variables appear to be predictive of this phenomenon, they may simply be surrogates for a greater degree of pulmonary hypoplasia present at birth. This subpopulation of CDH survivors is an identifiable group clearly at risk and thus requires long-term follow-up.

Glutamine supplementation in infants with gastrointestinal disease: a randomized, placebo-controlled pilot trial.

OBJECTIVE: Glutamine (Gln) is a non-essential amino acid that plays an important role in energy metabolism for gastrointestinal epithelia and other cells with rapid turnover. We evaluated the effects of enteral supplementation with Gln in infants undergoing surgery for congenital or acquired gastrointestinal disease. METHODS: This was a randomized, double-masked, controlled clinical trial. RESULTS: Twenty infants were randomly assigned to receive Gln (n = 9) or placebo amino acid (n = 11), with a goal of supplemental amino acid intake of 0.4 g.kg(-1).d(-1). Infants were weaned from parenteral nutrition, and enteral feeds were started according to a standardized feeding protocol. Median (interquartile range) durations of parenteral nutrition were 39 d (12 to 99) in the Gln group and 21 d (6 to 59) in the control group (P = 0.201). Median (interquartile range) durations needed to reach 80% of the US recommended dietary allowance for energy with enteral nutrition were 24 d (8 to 55) in the Gln group and 12.5 d (5 to 32) in the control group (P = 0.313). There were no differences in the occurrence of infections between groups. Among all infants enrolled, significant correlations were found between duration of parenteral nutrition and residual small bowel length, peak concentrations of direct bilirubin, and alanine aminotransferase. Peak direct bilirubin was associated with longer duration of parenteral nutrition, shorter gestation, older age before feeds were started, shorter bowel length, and larger amounts of parenteral energy and protein intake. CONCLUSIONS: In this pilot trial, enteral Gln supplementation was well tolerated among infants with surgical gastrointestinal disease. There was no effect observed on the duration of parenteral nutrition, tolerance of enteral feeds, or intestinal absorptive or barrier function. Larger, multicenter trials in infants with surgical gastrointestinal disease are needed due to the variability in important outcome measurements.