Disorders of carnitine metabolism in premature infants with fan-associated pneumonia
https://doi.org/10.21508/1027-4065-2019-64-4-45-51
Abstract
We studied the concentration of carnitine and its fractions in peripheral blood in 22 premature infants with fan-associated pneumonia and in 20 conditionally healthy premature infants by tandem mass spectrometry. The birth body weight of children was 2,086.32 ± 117.13 and 2,140.9 ± 74.4 g, gestational age was 33.7 ± 0.41 and 34.16 ± 0.51 weeks, respectively. The concentration of total carnitine was at the lower limit or decreased in 10 (45%) children in the acute period of the disease. 4 newborns with fan-associated pneumonia demonstrated persistent carnitine deficiency: the content of free carnitine was very low: 7.47 – 8, 37 μmol/l (7.97 ± 0.197 μmol/l), the concentration of total carnitine was also reduced (21.55 – 22.01 μmol/l, 21.7 ± 0.366 μmol/l). The fractions of acylcarnitines varied widely throughout the disease. One child had high rates of C18OH (0.282 μmol/l; norm 0–0.110 μmol/l) and C18:1OH (0.282 μmol/l; norm 0–0.180 μmol/l) during the entire neonatal period. It could be associated with mitochondrial trifunctional protein deficiency. The study of total carnitine and its fractions in premature infants with fan-associated pneumonia allowed us to identify violations of its metabolism, both secondary and hereditary at early stage.
About the Authors
V. V. Garmaeva
Children’s Clinics MEDSI;
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
G. M. Dementieva
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
M. V. Kushnareva
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
G. V. Baydakova
Medical Genetics Research Center
Russian Federation
Moscow
Russian Federation
Moscow
V. S. Sukhorukov
Pirogov Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
E. S. Keshishyan
Veltischev Research and Clinical Institute for Pediatrics of the Pirogov Russian National Research Medical University
Russian Federation
Moscow
Russian Federation
Moscow
E. Yu. Zakharova
Medical Genetics Research Center
Russian Federation
Moscow
Russian Federation
Moscow
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Review
For citations:
Garmaeva V.V., Dementieva G.M., Kushnareva M.V., Baydakova G.V., Sukhorukov V.S., Keshishyan E.S., Zakharova E.Yu. Disorders of carnitine metabolism in premature infants with fan-associated pneumonia. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2019;64(4):45-51. (In Russ.) https://doi.org/10.21508/1027-4065-2019-64-4-45-51
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