Abstract
Electrospray-tandem mass spectrometry represents a powerful method for detection of inborn errors of fatty acid metabolism. In the present study, it was used to examine neonatal carnitine metabolism, which reflects fatty acid metabolism. In 70 healthy neonates, blood samples were taken from the umbilical cord and by heel-stick puncture in full-term neonates on postnatal d 5. Cord blood specimens were also obtained from 15 preterm and 10 small-for-gestational-age infants. Acylcarnitine concentrations were measured in dried blood spots by electrospray tandem mass spectrometry. Compared with cord blood, the levels of nearly all acylcarnitine species were significantly higher on the postnatal d 5, whereas free carnitine remained unchanged. Total acylcarnitine/free carnitine-ratio increased, whereas the free carnitine/total carnitine-ratio (0.54 ± 0.05;p < 0.01) further decreased. A reduced availability of free carnitine in the early neonatal period may affect fatty acid oxidation and thus be of potential pathophysiological relevance under conditions with higher energy demands, e.g. in sepsis. Cord blood concentrations of free carnitine, total carnitine, and total acylcarnitines were strongly related to birth weight (p < 0.01). Lower umbilical artery pH, i.e. mild hypoxia, caused accumulation of mainly long-chain acylcarnitines. This implicates that long-chain acylcarnitines could serve as a parameter of perinatal asphyxia.
Similar content being viewed by others
Log in or create a free account to read this content
Gain free access to this article, as well as selected content from this journal and more on nature.com
or
Abbreviations
- FC:
-
free carnitine
- m/z:
-
mass to charge ratio of ions
- tAC:
-
total acylcarnitines
- TC:
-
total carnitine
References
Hahn P, Novak M 1985 How important are carnitine and ketones for the newborn infant?. Fed Proc 44: 2369–2373
Ramsay RR 1994 Carnitine and its role in acyl group metabolism. Essays Biochem 28: 47–61
Shenai JP, Borum PR 1984 Tissue carnitine reserves of newborn infants. Pediatr Res 18: 679–682
Shenai JP, Borum PR, Mohan P, Donlevy SC 1983 Carnitine status at birth of newborn infants of varying gestation. Pediatr Res 17: 579–582
Chace DH, Hillman SL, Van HJ, Naylor EW 1997 Rapid diagnosis of MCAD deficiency: quantitatively analysis of octanoylcarnitine and other acylcarnitines in newborn blood spots by tandem mass spectrometry. Clin Chem 43: 2106–2113
Rashed MS, Ozand PT, Bucknall MP, Little D 1995 Diagnosis of inborn errors of metabolism from blood spots by acylcarnitines and amino acids profiling using automated electrospray tandem mass spectrometry. Pediatr Res 38: 324–331
Penn D, Dolderer M, Schmidt-Sommerfeld E 1987 Carnitine concentrations in the milk of different species and infant formulas. Biol Neonate 52: 70–79
Schoderbeck M, Auer B, Legenstein E, Genger H, Sevelda P, Salzer H, Marz R, Lohninger A 1995 Pregnancy-related changes of carnitine and acylcarnitine concentrations of plasma and erythrocytes. J Perinat Med 23: 477–485
Novak M, Monkus EF, Chung D, Buch M 1981 Carnitine in the perinatal metabolism of lipids. I. Relationship between maternal and fetal plasma levels of carnitine and acylcarnitines. Pediatrics 67: 95–100
Bargen-Lockner C, Hahn P, Wittmann B 1981 Plasma carnitine in pregnancy. Am J Obstet Gynecol 140: 412–414
Schmidt-Sommerfeld E, Penn D, Wolf H 1981 The influence of maternal fat metabolism on fetal carnitine levels. Early Hum Dev 5: 233–242
Giannacopoulou C, Evangeliou A, Matalliotakis I, Relakis K, Sbirakis N, Hatzidaki E, Koumandakis E 1998 Effects of gestation age and of birth weight in the concentration of carnitine in the umbilical plasma. Clin Exp Obstet Gynecol 25: 42–45
Bernardini I, Evans MI, Nicolaides KH, Economides DL, Gahl WA 1991 The fetal concentrating index as a gestational age-independent measure of placental dysfunction in intrauterine growth retardation. Am J Obstet Gynecol 164: 1481–1487
Böhles HJ, Evangeliou A, Bervoets K, Sewell AC 1994 Carnitine esters in metabolic disease. J Pediatr 153: S57–S61
Winter SC, Zorn EM, Hugh Vance W 1990 Carnitine deficiency. Lancet 335: 981–982
Johnson DW 1999 Inaccurate measurement of free carnitine by the electrospray tandem mass spectrometry screening method for blood spots. J Inherit Metab Dis 22: 201–202
Campoy C, Bayes R, Peinado JM, Rivero M, Lopez C, Molina-Font JA 1998 Evaluation of carnitine nutritional status in fullterm newborn infants. Early Hum Dev 53: S149–S164
De Sousa C, English NR, Stacey TE, Chalmers RA 1990 Measurement of L-carnitine and acylcarnitines in body fluids and tissues in children and adults. Clin Chim Acta 187: 317–328
Penn D, Schmidt-Sommerfeld E, Pascu F 1981 Decreased tissue carnitine concentrations in newborn infants receiving total parenteral nutrition. J Pediatr 98: 976–978
Sachan DS, Smith RB, Plattsmier J, Lorch V 1989 Maternal, cord, and neonatal carnitine correlations and lipid profiles of various birthweight infants. Am J Perinatol 6: 14–17
Yamada KA, McHowat J, Yan GX, Donahue K, Peirick J, Kleber AG, Corr PB 1994 Cellular uncoupling induced by accumulation of long-chain acylcarnitine during ischemia. Circ Res 74: 83–95
Hütter JF, Soboll S 1992 Role of fatty acid metabolites in the development of myocardial ischemic damage. Int J Biochem 24: 399–403
Knabb MT, Saffitz JE, Corr PB, Sobel BE 1986 The dependence of electrophysiological derangements on accumulation of endogenous long-chain acyl carnitine in hypoxic neonatal rat myocytes. Circ Res 58: 230–240
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Meyburg, J., Schulze, A., Kohlmueller, D. et al. Postnatal Changes in Neonatal Acylcarnitine Profile. Pediatr Res 49, 125–129 (2001). https://doi.org/10.1203/00006450-200101000-00024
Received:
Accepted:
Issue date:
DOI: https://doi.org/10.1203/00006450-200101000-00024
This article is cited by
-
Fatal pitfalls in newborn screening for mitochondrial trifunctional protein (MTP)/long-chain 3-Hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency
Orphanet Journal of Rare Diseases (2018)
-
Analysis and interpretation of acylcarnitine profiles in dried blood spot and plasma of preterm and full-term newborns
Pediatric Research (2015)
-
Amino Acid and Acylcarnitine Profiles in Premature Neonates: A Pilot Study
The Indian Journal of Pediatrics (2013)
-
Postprandial changes of amino acid and acylcarnitine concentrations in dried blood samples
Journal of Inherited Metabolic Disease (2010)
-
Effect of methanol on endogenous and exogenous carnitine levels in rat plasma
Archives of Toxicology (2005)