Oral nutritional supplements in patients with cystic fibrosis: research results

Abstract

BACKGROUND: The nutritional status of patients with cystic fibrosis significantly affects their survival and the function of all organs, particularly the lungs. Due to increased protein and energy needs, these patients often require high-calorie and high-protein oral nutritional supplements (ONS).

AIM: To assess the tolerability, efficacy, and safety of Fresubin Protein® powder, a protein module.

MATERIALS AND METHODS: The data of 14 patients with mixed form of cystic fibrosis and nutritional deficiencies, aged between 6 and 16 years, were analyzed. Five patients received Fresubin Protein® powder as monotherapy (Group 1), four patients received the same therapeutic food together with emulsion of medium-chain triglycerides (Group 2), and five patients received ONS balanced in protein, fat, and carbohydrates (control group). The dynamics between percentile and Z-Scores of body mass index (BMI) at the beginning and at the end of the study period (day 180±2) were used as the main efficacy parameter of the investigated food. Body fat and lean body mass were assessed by caliperometry as additional efficacy parameters.

RESULTS: All patients showed an increase in Z-Score and BMI percentile from baseline while taking Fresubin Protein® powder both as monotherapy and in combination with medium-chain triglyceride emulsion. There were no statistically significant differences from the control group (p >0.05). However, Group 1 (100% of patients) demonstrated positive changes in muscle mass gain, as evidenced by caliperometry data, which was statistically significant (p <0.05) compared with the control group (20%). In Group 2, muscle gain was observed in 75% of the patients (p >0.05).

CONCLUSION: These findings suggest that protein-enriched ONS not only increase BMI but also contribute to muscle mass gain in patients with cystic fibrosis. New therapeutic approaches are needed, such as the inclusion of high-protein ONS in the diet to prevent muscle mass loss, along with the monitoring of body composition to improve control of disease progression.

Keywords

cystic fibrosis / nutritional support / oral nutritional supplements / malnutrition / whey protein

References

[1] Union of Paediatricians of Russia, Association of Medical Geneticists, Russian Respiratory Society, Russian Transplant Society, Association of Children's Doctors of the Moscow Region. Cystic fibrosis (cystic fibrosis): clinical recommendations. In: Rubric clinical guidelines [Internet]. Ministry of Health of the Russian Federation; 2021 [cited 03 Sep 2021]. Available from: https://cr.minzdrav.gov.ru/recomend/372_2 (In Russ.)

[2] Союз педиатров России, Ассоциация медицинских генетиков, Российское Респираторное общество, Российское трансплантологическое общество, Ассоциация детских врачей Московской области. Кистозный фиброз (муковисцидоз): клинические рекомендации. В: Рубрикатор клинических рекомендаций [интернет]. Москва: Министерство здравоохранения Российской Федерации, 2021. Режим доступа: https://cr.minzdrav.gov.ru/recomend/372_2 Дата обращения 03.09.2021.

[3] Kondratieva EI, Kashirskaya NYu, Kapranov NI, editors. National consensus “Cystic fibrosis: definition, diagnostic criteria, therapy”. 2nd ed. Moscow: Boroges Company; 2018, 356 p. (In Russ.)

[4] Национальный консенсус «Муковисцидоз: определение, диагностические критерии, терапия». 2-е изд. 2018 / под ред. Е.И. Кондратьевой, Н.Ю. Каширской, Н.И. Капранова. Москва: Компания БОРГЕС, 2018. 356 с.

[5] Kapranov NI, Kashirskaya NY, Kondratieva EI, editors. Cystic fibrosis. Edition 2nd, revised and supplemented. Moscow: MEDPRACTICA-M; 2021. (In Russ.)

[6] Муковисцидоз. 2-е изд., переработанное и дополненное / под ред. Н.И. Капранова, Н.Ю. Каширской, Е.И. Кондратьевой. Москва: МЕДПРАКТИКА-М, 2021.

[7] Sherman VD, Kondratieva EI, Voronkova AY, et al. Influence of neonatal screening for cystic fibrosis by the example of patients of the Moscow region. Medical Council. 2017;(18):124–128. EDN: ZQTKQN doi: 10.21518/2079-701X-2017-18-124-128

[8] Шерман В.Д., Кондратьева Е.И., Воронкова А.Ю., и др. Влияние неонатального скрининга на течение муковисцидоза на примере групп пациентов Московского региона // Медицинский совет 2017. № 18. С. 124–128. EDN: ZQTKQN doi: 10.21518/2079-701X-2017-18-124-128

[9] Vitkovskaya IP, Abramov SI, Zelenova OV. Clinical and economic justification for the inclusion of new enteral mixtures in the list of specialized products therapeutic nutrition for disabled children diagnosed with cystic fibrosis. Sovremennye problemy zdravoohranenija i medicinskoj statistiki. 2023;(1):435–449. EDN: MILLZZ doi: 10.24412/2312-2935-2023-1-435-449

[10] Витковская И.П., Абрамов С.И., Зеленова О.В. Клинико-экономическое обоснование включение энтеральных смесей в перечень специализированных продуктов лечебного питания для детей-инвалидов с диагнозом муковисцидоз // Современные проблемы здравоохранения и медицинской статистики. 2023. № 1. С. 435–449. EDN: MILLZZ doi: 10.24412/2312-2935-2023-1-435-449

[11] Turck D, Braegger CP, Colombo C, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016;35:557–77. doi: 10.1016/j.clnu.2016.03.004

[12] Turck D., Braegger C.P., Colombo C., et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis // Clin Nutr. 2016. Vol. 35. P. 557–77. doi: 10.1016/j.clnu.2016.03.004

[13] Li L, Somerset S. Digestive system dysfunction in cystic fibrosis: challenges for nutrition therapy. Dig Liver Dis. 2014;46:865–874. doi: 10.1016/j.dld.2014.06.011

[14] Li L., Somerset S. Digestive system dysfunction in cystic fibrosis: challenges for nutrition therapy // Dig Liver Dis. 2014. Vol. 46. P. 865–874. doi: 10.1016/j.dld.2014.06.011

[15] Calella P, Valerio G, Brodlie M, et al. Cystic fibrosis, body composition and health outcomes: a systematic review. Nutrition. 2018;55-56:131–139. doi: 10.1016/j.nut.2018.03.052

[16] Calella P., Valerio G., Brodlie M., et al. Cystic fibrosis, body composition and health outcomes: a systematic review // Nutrition. 2018. Vol. 55-56. P. 131–139. doi: 10.1016/j.nut.2018.03.052

[17] Culhane S, George C, Pearo B, Spoede E. Malnutrition in cystic fibrosis: a review. Nutr Clin Pract. 2013;28(6):676–683. doi: 10.1177/0884533613507086

[18] Culhane S., George C., Pearo B., Spoede E. Malnutrition in cystic fibrosis: a review // Nutr Clin Pract. 2013. Vol. 28, N. 6. P. 676–683. doi: 10.1177/0884533613507086

[19] Engelen MP, Com G, Deutz NEP. Protein is an important but undervalued macronutrient in the nutritional care of patients with cystic fibrosis. Curr Opin Clin Nutr Metab Care. 2014;17(6):515–520. doi: 10.1097/MCO.0000000000000100

[20] Engelen M.P., Com G., Deutz N.E. Protein is an important but undervalued macronutrient in the nutritional care of patients with cystic fibrosis // Curr Opin Clin Nutr Metab Care. 2014. Vol. 17, N. 6. P. 515–520. doi: 10.1097/MCO.0000000000000100

[21] Geukers VG, Oudshoorn JH, Taminiau JA, et al. Short-term protein intake and stimulation of protein synthesis in stunted children with cystic fibrosis. Am J Clin Nutr. 2005;81(3):605–610. doi: 10.1093/ajcn/81.3.605

[22] Geukers V.G., Oudshoorn J.H., Taminiau J.A., et al. Short-term protein intake and stimulation of protein synthesis in stunted children with cystic fibrosis // Am J Clin Nutr. 2005. Vol. 81, N. 3. P. 605–610. doi: 10.1093/ajcn/81.3.605

[23] Stephenson AL, Mannik LA, Walsh S, et al. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study. Am J Clin Nutr. 2013;97(4):872–877. doi: 10.3945/ajcn.112.051409

[24] Stephenson A.L., Mannik L.A., Walsh S., et al. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study // Am J Clin Nutr. 2013. Vol. 97, N. 4. P. 872–877. doi: 10.3945/ajcn.112.051409

[25] Waterlow JC. Protein-energy malnutrition, 2nd edition. London: Edward Arnold, 1992. 407 p. ISBN-10: 0340600608, ISBN-13: 978-0340600603

[26] Waterlow J.C. Protein-energy malnutrition, 2nd edition. London: Edward Arnold, 1992. ISBN-10: 0340600608, ISBN-13: 978-0340600603

[27] WHO Guideline: Updates on the management of severe acute malnutrition in infants and children. Geneva: World Health Organization; 2013.

[28] WHO Guideline: Updates on the management of severe acute malnutrition in infants and children. Geneva: World Health Organization, 2013.

Funding

Исследование и публикация осуществлены при поддержке ООО «Фрезениус Каби» (Россия)This work was supported by the Fresenius Kabi LLC (Russia)

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