Background:The acylcarnitine profiles obtained from dried blood spots on "Guthrie cards" have been widely used for the diagnosis and follow-up of children suspected of carrying an inherited error of metabolism, but little attention has been paid to potential age-related variations in the reference values. In this study, we evaluated the variations in free carnitine and acylcarnitine concentrations with age, as measured by tandem mass spectrometry. Methods: Filter-paper blood spots were collected from 433 healthy individuals over a period of 17 months. Eight age groups were defined: cord blood, 3-6 days (control group), 15-55 days, 2-18 months, 19 -59 months, 5-10 years, 11-17 years, and 18 -54 years. Free carnitine and acylcarnitines were measured for each individual. Mean values were calculated for each age group and compared with those for the control group. Results: Free carnitine was significantly higher in older children than in newborns (P <0.05), but the concentrations of several acylcarnitines tended to be significantly lower in cord blood and in groups of older children than in the control group. Only minor sex-related differences were observed. Conclusion: Although the risk of underdiagnosis of fatty acid oxidation disorders with the use of newborn values as reference can be considered as small, in some circumstances the use of age-related reference values
Background: The clinical significance of early transient hypoglycemia (ETH), a frequent event in preterm newborns, is a highly controversial issue. In experimental models, hypoglycemia has been reported to cause oxidative stress. Among the reactive species, early generated peroxynitrite is responsible for protein nitration and lipid peroxidation, a process referred to as nitrative stress. Objectives: The aim of the present study is to investigate whether ETH is associated with protein nitration in the preterm newborn. Methods: Using a novel highly sensitive ELISA, we quantified plasma nitroalbumin (PNA) as a marker of peroxynitrite generation in 72 preterm newborns (28–36 weeks), among which 25 had a glycemia level of <2.5 mmol/l during the first hour of life (H1). Results: PNA was significantly higher in ETH than in normoglycemic infants at H1 [median = 6.3 (3.8–8.8) vs. 3.4 ng/ml (2.1–5.1), p = 0.027] and at day 1 [median = 6.6 (5.6–15.3) vs. 3.9 ng/ml (2.3–4.6), p = 0.014]. PNA was inversely correlated with glycemia at H1 (r = –0.30, p = 0.01) and at day 1 (r = –0.63, p = 0.001). In ETH infants, lactatemia was inversely correlated with PNA. At day 1, PNA was higher in ETH infants treated by gavage than in those treated with intravenous dextrose [median = 8.9 ng/ml (7.1–10.4) vs. 4.4 ng/ml (2.6–5.7), p = 0.008]. Conclusions: These results indicate that ETH is associated with increased peroxynitrite generation resulting in systemic protein nitration in premature newborns. Treatment of ETH with intravenous dextrose is associated with lower PNA levels than gavage.
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