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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.


1. Mondoev LG, Birjukova LS. (2007). Application of carnitine in patients with the chronic renal failure, undergoing long-term hemodialysis. Nephrology and dialysis. 9(4): 391–394.

2. Tokarchuk NI, Vyzhga YV. (2016). Prescribtion of the levocarnitin for the treatment of secondary cardiomyopathy in infants. Sovremennaya pediatriya. 5(77): 67–70. https://doi.org/10.15574/SP.2016.77.67.

3. Azevedo VM, Albanesi Filho FM, Santos MA et al. (2013). The role of L-carnitine in nutritional status and echocardiographic parameters in idiopathic dilated cardiomyopathy in children. J Pediatr. 81(5): 368–372. https://doi.org/10.2223/JPED.1387; PMid:16247537

4. Di Liberato L, Arduini A, Rossi C et al. (2014). L-carnitine status in endstage renal disease patients on automated peritoneal dialysis. J Nephrol. 27(6): 699–706. https://doi.org/10.1007/s40620-014-0076-x; PMid:24599831

5. El-Hattab AW, Scaglia F. (2015). Disorders of carnitine biosynthesis and transport. Molecular Genetics and Metabolism. 116(3): 107–112. https://doi.org/10.1016/j.ymgme.2015.09.004; PMid:26385306

6. Fu L, Huang M, Chen S. (2013). Primary carnitine deficiency and cardiomyopathy. Korean Circulation Journal. 43(12): 785–792. https://doi.org/10.4070/kcj.2013.43.12.785; PMid:24385988 PMCid:PMC3875693

7. Jafari A, Khatami M-R, Dashti-Khavidaki S. et al. (2017). Protective effects of L-carnitine against delayed graft function in kidney transplant recipients: a pilot, randomized, double-blinded, placebo-controlled clinical trial. Renal Nutrition. 27(2): 113–126. https://doi.org/10.1053/j.jrn.2016.11.002; PMid:28065453

8. Kidney Disease: Improving Global Outcomes (KDIGO) СKD Work Group. KDIGO 2012. (2013). Clinical Practice Guideline for the Evaluation and Management of Chronic Kidney Disease. Kidney Inter Suppl. 3: 1–150.

9. Lundin U, Weinberger KM. (2018). Towards metabolic biomarkers for the diagnosis and prognosis of CKD. https://www.intechopen.com/books/advances-in-nephropathy-towards-metabolic-biomarkers-for-the-diagnosis-and-prognosis-of-ckd. https://doi.org/10.5772/intechopen.80335

10. Mercadal L, Coudert M, Vassault A et al. (2012). L-carnitine treatment in incident hemodialysis patients: the multicenter, randomized, double-blinded, placebo-controlled CARNIDIAL trial. Clin J Am Soc Nephrol. 7(11): 1836–1842. https://doi.org/10.2215/CJN.12431211; PMid:22935844 PMCid:PMC3488953

11. National Kidney Foundation. (2002). K/DOQI clinical practice guidelines for chronic kidney diseases: evaluation, classification and stratification. Am J Kidney Dis. 39(1): 17–31.

12. Sgambat K, Moudgil A. (2016). Carnitine deficiency in children receiving continuous renal replacement therapy. Hemodial Int. 20(1): 63–67. https://doi.org/10.1111/hdi.12341; PMid:26265013