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.
Highlights
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 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 reduced (21.55 – 22.01 μmol/l, 21.7 ± 0.366 μmol/l)
Определение концентрации свободного и общего карнитина, суммы и спектра ацилкарнитинов позволяет выявить среди недоношенных детей с отягощенным течением неонатального периода (респираторный дистресс-синдром, «вентилятор-ассоциированная» пневмония, гипотрофия, гипоксически-ишемическое поражение ЦНС, кардиомиопатия) пациентов с предполагаемыми врожденными дефектами обмена карнитина и жирных кислот (в нашем исследовании – это дефицит митохондриального трифункционального белка и дефицит транспортера карнитина)
Summary
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. С учетом изложенного целью настоящего исследования было сравнительное изучение особенностей показателей карнитинового обмена у недоношенных новорожденных с «вентилятор-ассоциированной» пневмонией и условно здоровых недоношенных детей. Показатели нормы карнитинового обмена в крови у условно здоровых недоношенных находились в следующих пределах: общий карнитин – 25–60 мкмоль/л, свободный карнитин – 12–30 мкмоль/л, сумма ацилкарнитинов – 12–30 мкмоль/л.
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