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The effect of metabolic therapy on carnitine status and metabolomic amino acid profile in children with chronic kidney disease

Modern pediatrics. Ukraine. 2019.5(101):31-37; doi 10.15574/SP.2019.101.31
S.V. Kushnirenko, N.V. Olkhovych
Shupyk National Medical Academy of Postgraduate Education, Kyiv, Ukraine
State Enterprise «Institute for Genetic and Regenerative Medicine» National Academy of Medical Sciences of Ukraine, Kyiv

For citation: Kushnirenko SV, Olkhovych NV. (2019). The effect of metabolic therapy on carnitine status and metabolomic amino acid profile in children with chronic kidney disease. Modern Pediatrics.Ukraine. 5(101): 31-37. doi 10.15574/SP.2019.101.31/
Article received: May 17, 2019. Accepted for publication: Sep 07, 2019.

Objective: to study the effect of metabolic therapy with levocarnitine on carnitine status, metabolomic amino acid profile and the functional state of the cardiovascular system in children with chronic kidney disease (CKD) 4–5 st.
Materials and methods. The concentration of acylcarnitines and amino acids in dry blood spots was determined in 38 children with CKD 2–5 st. aged from 2 to 17 by liquid chromatography tandem mass spectrometry. To correct the carnitine status in 20 children with CKD 4–5 st., levocarnitine (Agvantar) was administered orally at the rate of 50 mg/kg daily for 2 months. The efficacy and safety of levocarnitine was evaluated based on the value dynamics of metabolomic amino acid profile, carnitine status, clinical and laboratory values, electrocardiography and echocardiography.
Results. The obtained results showed that oral administration of levocarnitine was accompanied by significant dynamics of an increase in free carnitine (C0) to the level of 46.11±2.9 μm as compared with the data obtained before treatment in patients with CKD 2–5 st. (p<0.05). After 2 months of levocarnitine therapy, the content of C5DC (glutarylcarnitine) and C6DC (3-methylglutaconylcarnitine) decreased twice in comparison with respective values obtained in patients with CKD 5 st. before treatment (p<0.05). Levocarnitine intake did not have a negative effect on the metabolomic amino acid spectrum of the blood. Over 2 months of levocarnitine therapy, improvement of the functional state of the cardiovascular system and an increase in physical endurance was achieved in children with CKD 4–5 st.
Conclusions. Prescription of levocarnitine (Agvantar) to children with CKD 4–5 st. is pathogenetically justified, it can improve the carnitine status values, restore the free carnitine pool and, in combination with complex therapy, achieve stabilization of the functional state of the cardiovascular system.
The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of Kyiv City Children's Clinical Hospital No. 1. The informed consent of the patient was obtained for conducting the studies.
No conflict of interest was declared by the authors.
Key words: chronic kidney disease, children, metabolic therapy, levocarnitine, carnitine status, metabolic amino acid profile.

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