Russian Journal of Woman and Child Health
ISSN 2618-8430 (Print), 2686-7184 (Online)

Clinical efficacy of dry nutritional treatment products for phenylketonuria in children, adults, and pregnant women: study results

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DOI: 10.32364/2618-8430-2019-2-4-355-360

N.A. Semenova1, G.V. Baydakova1, N.V. Nikitina2, V.K. Podolina2, E.Yu. Belyashova3V.N. Kuznetsova3, E.M. Kochegurova4, L.P. Andreeva5, O.N. Khaylova5, S.I. Kutsev1

1Research Centre for Medical Genetics, Moscow, Russian Federation

2Clinical and Diagnostic Center “Protection of Health of Mother and Child”,
Yekaterinburg, Russian Federation

3Orenburg Regional Clinical Hospital No. 2, Orenburg, Russian Federation

4E.M. Bakunina Regional Clinical Perinatal Center, Tver, Russian Federation

5Saratov Regional Children’s Clinical Hospital, Saratov, Russian Federation

Abstract

Aim: to assess the efficacy of domestic nutritional treatment products for phenylketonuria (PKU) in children, adults, and pregnant women.

Patients and Methods: 46 patients with verified PKU were enrolled in this open-label multicenter prospective non-controlled study. Patients were allocated to the groups depending on nutritional treatment products which were prescribed according to the Clinical Guidelines of the Ministry of Health of Russian Federation. Clinical efficacy of the products was assessed according to the Good Clinical Practice guidelines based on clinical and laboratory tests.

Results: all patients taken nutritional treatment products throughout the study. No dyspepsia, abnormal poop, or allergic reactions were reported. Most patients reported on more pleasant smell and odor of the products as compared with the products taken previously. At baseline, mean phenylalanine levels were within normal limits. After the treatment, phenylalanine levels were stable  in all patients. Clinical and biochemical tests were within normal limits.

Conclusion: dry nutritional treatment products are highly effective in children, adults, and pregnant women with PKU.

Keywords: phenylketonuria, specialized food, phenylalanine, mixture, products.

For citation: Semenova N.A., Baydakova G.V., Nikitina N.V. et al. Clinical efficacy of dry nutritional treatment products for phenylketonuria in children, adults, and pregnant women: study results. Russian Journal of Woman and Child Health. 2019;2(4):355–360.



About the authors:

1Nataliya A. Semenova – MD, PhD, geneticist, Senior Researcher, ORCID iD 0000-0001-7041-045X;

1Galina V. Baydakova – PhD (biology), Leading Researcher, ORCID iD 0000-0001-8806-5287; 

2 Nataliya V. Nikitina – MD, geneticist, Head of the Department, ORCID iD 0000-0002-2811-4718;

2Valentina K. Podolina – MD, pediatrician, ORCID iD 0000-0003-1854-0040;

3Elena Yu. Belyashova – MD, Head of the Department, ORCID iD 0000-0002-7289-9532;

3Vera N. Kuznetsova – MD, PhD, geneticist, ORCID iD 0000-0002-7624-0639;

4Elena M. Kochegurova – MD, PhD, geneticist, Head of the Department, ORCID iD 0000-0003-4473-528X;

5Liliya P. Andreeva – MD, PhD, geneticist, ORCID iD 0000-0001-8700-7325;

5Olga N. Khaylova – MD, geneticist, ORCID iD 0000-0002-7428-1488;

1Sergey I. Kutsev – MD, PhD, Professor, Corresponding Member of RAS, Director, ORCID iD 0000-0002-3133-8018.

1Research Centre for Medical Genetics. 1, Moskvorechie  str., Moscow, 115522, Russian Federation.

2Clinical and Diagnostic Center “Protection of Health of Mother and Child”. 52, Flotskaya str., Yekaterinburg, 620041, Russian Federation.

3Orenburg Regional Clinical Hospital No. 2. 24, Nevelskaya str., Orenburg, 460000, Russian Federation.

4E.M. Bakunina Regional Clinical Perinatal Center. 115 build. 3, St. Petersburg highway, Tver, 170036, Russian Federation.

5Saratov Regional Children's Clinical Hospital. 6, Vol-skaya str., Saratov, 410028, Russian Federation.

Contact information: Nataliya A. Semenova, e-mail: semenova@med-gen.ruFinancial Disclosure: no author has a financial or property interest in any material or method mentioned. There is no conflict of interests. Received 31.06.2019.




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