Abstract
Aim: to assess the efficacy and tolerability of a specialized dietary therapeutic product in long-chain and very long-chain fatty acid metabolism disorders in children. Patients and Methods: this open-label prospective uncontrolled study enrolled 5 children aged 35 ± 14 months who were diagnosed with longchain and very long-chain fatty acid metab-olism disorders. Diet therapy using the tested product (“Monogen”) was prescribed and corrected after the analysis of actual diet. Physical development and neurological status were evaluated, blood levels of specific acyl-carnitines (С16-ОН, С18-ОН, С18:1-ОН, С14:1, С14) were measured. Tolerability was assessed by the rate and severity of adverse events (including allergic reactions) and the changes in vital functions compared to baseline. Patient and doctor satisfaction was evaluated using a questionnaire. Results: children received the tested product throughout the study (30±2 days). The lev-els of acyl-carnitines were within normal ranges in all children. No dyspepsia, allergic reactions, somatic dysfunction, or neurological deviations were reported. Physical exam consistently fell in the range of normal for age. Parents and doctors were fully satisfied with the tested product (“Monogen”). Conclusions: “Monogen” is highly effective for therapeutic feeding of children with long-chain and very long-chain fatty acid metabolism disorders. KEYWORDS: long-chain fatty acid oxidation disorders, β-oxidation of fatty acids, long-chain acyl-coA dehydrogenase deficiency, biallelic mutation, therapeutic feeding. FOR CITATION: Semenova N.A., Shestopalova E.A., Kutsev S.I. Efficacy and tolerability of a specialized dietary therapeutic product in longchain and very long-chain fatty acid metabolism disorders in children. Russian Journal of Woman and Child Health. 2021;4(1):98–102. DOI: 10.32364/2618-8430-2021-4-1-98-102.
Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
