Критический анализ обновленных международных рекомендаций по питанию пациентов с муковисцидозом

Цель обзора - проанализировать рекомендации, содержащиеся в обновленной версии Руководства ESPEN-ESPGHAN-ECFS 2024 года по вопросам питания младенцев, детей и взрослых при муковисцидозе (МВ). Предыдущая версия Руководства была опубликована в 2016 году. Рабочая группа экспертов, в которую вошли врачи, диетологи и представители пациентских сообществ, обновила рекомендации по питанию, включая методы его оценки и ведения в любом возрасте. Расширены главы о беременности, заболеваниях печени, диабете, связанном с МВ, заболеваниях костей, пищевых и минеральных добавках, пробиотиках. Появились новые главы, посвященные питанию при использовании высокоэффективной (таргетной) терапии модуляторами CFTR и питанию после трансплантации органов. Главное заключается в изменении концепции питания в эру таргетной терапии от гиперкалорийной высокожировой диеты для всех пациентов до «здорового питания» как основной рекомендации. Руководство ESPEN-ESPGHAN-ECFS 2024 года по вопросам питания при МВ содержит целый ряд важных положений, которые будут полезны практическим врачам педиатрам и терапевтам, пульмонологам, гастроэнтерологам и диетологам, специализирующимся в области МВ. Следует учитывать существенные различия в клинико-эпидемиологической ситуации в РФ при диагностике нарушений нутритивного статуса и определении тактики нутритивной поддержки пациентов с муковисцидозом.

муковисцидоз, рекомендации по питанию, международные, российские, обновление

1. Wilschanski M., Anne Munck A, Carrion E., et al. ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis. Clin Nutr. 2024; 43: 413-445.

2. 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; 35:557-577.

3. Farrell P. M. The prevalence of cystic fibrosis in the European Union. J Cyst Fibros. 2008; 7:450-453.

4. Kashirskaya N. Yu., Kapranov N. I., Kondratyeva E. I. Mucoviscidosis. 2nd edition, revised and expanded. Moscow. MEDPRACTIKA-M. Publ., 2021. 680 p. (in Russ.)@@ Муковисцидоз. Издание 2-е, переработанное и дополненное (под редакцией Каширской Н. Ю., Капранова Н. И., Кондратьевой Е. И.). М.: «МЕДПРАКТИКА-М.»: 2021. 680 с.

5. Krasovsky S.A., Starinova M. A., Voronkova A. Yu., Amelina E. L., Kashirskaya N. Yu., Kondratyeva E. I., Nazarenko L. P. Register of patients with cystic fibrosis in of the Russian Federation. 2021. St. Petersburg, Charitable Foundation ‘Ostrova’, 2023, 81 p. (in Russ.)@@ Регистр пациентов с муковисцидозом в Российской Федерации. 2021 год. Под ред. Красовского С. А., Стариновой М. А., Воронковой А. Ю., Амелиной Е. Л., Каширской Н. Ю., Кондратьевой Е. И., Назаренко Л. П. - СПб.: Благотворительный фонд «Острова», 2023, 81 с.

6. Bischoff S.C., Singer P., Koller M. Standard operating procedures for ESPEN guidelines and consensus papers. Clin Nutr. 2015; 34:1043-1051.

7. Poulimeneas D., Grammatikopoulou M. G., Petrocheilou A. et al.Comparison of international growth standards for assessing nutritional status in cystic fibrosis: the GreeCF study. J Pediatr Gastroenterol Nutr. 2020; 71: 35-39.

8. Calella P, Valerio G, Thomas A, et al. Association between body composition and pulmonary function in children and young people with cystic fibrosis. Nutrition. 2018; 48:73-76

9. Hollander-Kraaijeveld FM, ∙ Lindeman Y, ∙ de Roos NM et al. Non-fasting bioelectrical impedance analysis in cystic fibrosis: implications for clinical practice and research. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2020; 19:153-158

10. Bravo M.P., Balboa P., Torrejon C. et al. Bone mineral density, lung function, vitamin D and body composition in children and adolescents with cystic fibrosis: a multicenter study. Nutr Hosp. 2018; 35:789-795.

11. Bellissimo M.P., Zhang I., Ivie E. A. et al. Visceral adipose tissue is associated with poor diet quality and higher fasting glucose in adults with cystic fibrosis. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2019; 18:430-435.

12. Contreras-Bolivar V., Olveira C., Porras N. et al. Assessment of body composition in cystic fibrosis: agreement between skinfold measurement and densitometry. Nutr Hosp. 2022; 39:376-382.

13. Owen E, Williams JE, Davies G, et al. Growth, body composition, and lung function in prepubertal children with cystic fibrosis diagnosed by newborn screening. Nutr Clin Pract. 2021; 36:1240-1246

14. Calella P., Valerio G., Brodlie M. et al. Tools and methods used for the assessment of body composition in patients with cystic fibrosis: a systematic review. Nutr Clin Pract. 2019; 34:701-714.

15. Sokolov I. [Optimisation of nutritional support in children with cystic fibrosis]. diss. med. science. Moscow. 2021, 23 p. (in Russ.)@@ Соколов И. Оптимизация нутритивной поддержки у детей с муковисцидозом, автореф. дисс. … канд. мед. наук. М. 2021, - 23 C. /

16. Szentpetery S., Fernandez G. S., Schechter M. S. et al. Obesity in cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2022; 21:777-83.

17. Bonhoure A., Boudreau V., Litvin M. et al. Overweight, obesity and significant weight gain in adult patients with cystic fibrosis association with lung function and cardiometabolic risk factors. Clin Nutr. 2020; 39:2910-6.

18. Gramegna A., Aliberti S., Contarini M. et al. Overweight and obesity in adults with cystic fibrosis: an Italian multicenter cohort study. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2022;21:111-4.

19. Harindhanavudhi T., Wang Q., Dunitz J. et al. Prevalence and factors associated with overweight and obesity in adults with cystic fibrosis: a single-center analysis. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2020;19: 139-45.

20. Ashkenazi M., Nathan N., Sarouk I. et al. Nutritional status in childhood as a prognostic factor in patients with cystic fibrosis. Lung. 2019;197(3):371-6.

21. Kilinc A.A., Beser O. F., Ugur E. P. et al. The effects of nutritional status and intervention on pulmonary functions in pediatric cystic fibrosis patients. Pediatr Int. 2021; 63:316-22.

22. Ng C., Major G., Smyth A. R. Timing of pancreatic enzyme replacement therapy (PERT) in cystic fibrosis. Cochrane Database Syst Rev. 2021;8: CD013488.

23. Nagy R., Gede N., Ocskay K. et al. Association of body mass index with clinical outcomes in patients with cystic fibrosis: a systematic review and meta-analysis. JAMA Netw Open. 2022;5: e220740.

24. Zazzeron L., Alicandro G., Dacco V. et al. Effects of prolonged proton pump inhibitor treatment on nutritional status and respiratory infection risk in cystic fibrosis: a matched cohort study. Dig Liver Dis. 2022; 55:360-5.

25. Woestenenk J.W., van der Ent C. K., Houwen R. H. Pancreatic enzyme replacement therapy and coefficient of fat absorption in children and adolescents with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2015;61:355-60.

26. Peng Y., Wu M., Alvarez J. A., Tangpricha V. Vitamin D status and risk of cystic fibrosis-related diabetes: a retrospective single center cohort study. Nutrients. 2021; 13:40-48.

27. Saxby N., Painter C., Kench A., King S., Crowder T. In: Bell Scott C, editor. The Australian and New Zealand cystic fibrosis nutrition guideline authorship group. Nutrition guidelines for cystic fibrosis in Australia and New Zealand. Sydney: Thoracic Society of Australia and New Zealand; 2017.

28. McDonald C.M., Alvarez J. A., Bailey J., Bowser E. K., Farnham K., Mangus M. et al. Academy of nutrition and dietetics: 2020 cystic fibrosis evidence analysis center evidence-based nutrition practice guideline. J Acad Nutr Diet. 2021; 121:1591-15636 e3.

29. Papachristou E., Katsagoni C. N., Roussou X. et al. Dietary intake, weight status, pulmonary function and metabolic profile in children with cystic fibrosis with or without pancreatic sufficiency. Nutrition. 2023:112091.

30. Nowak J.K., Wykretowicz A., Madry E., Krauze T., Drzymala-Czyz S., Krzyzanowska-Jankowska P. et al. Preclinical atherosclerosis in cystic fibrosis: two distinct presentations are related to pancreatic status. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2022;21:26-33.

31. Madde A., Okoniewski W., Sanders D. B., Ren C. L., Weiner D. J., Forno E. Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2022;21:769-76.

32. Caley L., Smith L., White H., Peckham D. G. Average rate of lung function decline in adults with cystic fibrosis in the United Kingdom: data from the UK CF registry. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2021;20:86-90.

33. Miller T., Antos N. J., Brock L. A., Wade T., Goday P. S. Lactation consultation sustains breast milk intake in infants with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2019;69:358-62.

34. Tan S.M.J., Coffey M. J., Ooi C. Y. Differences in clinical outcomes of paediatric cystic fibrosis patients with and without meconium ileus. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2019;18:857-62.

35. Jain R., Kazmerski T. M., Zuckerwise L. C. et al. Pregnancy in cystic fibrosis: Review of the literature and expert recommendations. J Cyst Fibros. 2022; 21(3): 387-395.

36. Victoria C.B., Casilda O., Nuria P. et al. Oral nutritional supplements in adults with cystic fibrosis: effects on intake, levels of fat-soluble vitamins, and bone remodeling biomarkers. Nutrients. 2021;13: 1-9.

37. Libeert D., Declercq D., Wanyama S. et al. The effect of enteral tube feeding in cystic fibrosis: a registry based study. J Cyst Fibro: Off J Eur Cystic Fibr Soci. 2018;17:264-70.

38. Shaikhkhalil A.K., Freeman A. J., Sathe M. Variations in nutrition practices in cystic fibrosis: a survey of the digest Program. Nutr Clin Pract. 2021;36: 1247-51.

39. Sankararaman S., Hendrix S. J., Schindler T. Update on the management of vitamins and minerals in cystic fibrosis. Nutr Clin Pract. 2022;37:1074-87.

40. Gettle L.S., Harden A., Bridges M., Albon D. Prevalence and risk factors for iron deficiency in adults with cystic fibrosis. In: Nutrition in clinical practice: official publication of the American society for parenteral and enteral nutrition. 2020. vol. 35, p. 1101-9.

41. Declercq D., Peremans L., Glorieus M. et al. Urinary sodium/creatinine ratio is a predictor for fractional sodium excretion and related to age in patients with cystic fibrosis. J Cyst Fibros. 2022;21(2):136-40.

42. Ciofu O., Smith S., Lykkesfeldt J. A systematic Cochrane Review of antioxidant supplementation lung disease for cystic fibrosis. Paediatr Respir Rev. 2020;33:28-9.

43. Strandvik B. Nutrition in cystic fibrosis-some notes on the fat recommendations. Nutrients. 2022;14.

44. McTavish D., Thornton J. Appetite stimulants for people with cystic fibrosis. Cochrane Database Syst Rev. 2022;9: Cd008190.

45. Chedevergne F., Sermet-Gaudelus I. Prevention of osteoporosis in cystic fibrosis. Curr Opin Pulm Med. 2019;25:660-5.

46. Atlas G., Yap M., Lim A., Vidmar S., Smith N., King L. et al. The clinical features that contribute to poor bone health in young Australians living with cystic fibrosis: a recommendation for BMD screening. Pediatr Pulmonol. 2021;56: 2014-22.

47. Emiralioglu N., Ademhan Tural D., Hizarcioglu Gulsen H., Ergen Y. M., Ozsezen B., Sunman B. et al. Does cystic fibrosis make susceptible to celiac disease? Eur J Pediatr. 2021;180:2807-13.

48. Putman M.S., Anabtawi A., Le T. et al. Cystic fibrosis bone disease treatment: current knowledge and future directions. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2019;18(Suppl 2): S56-65.

49. Ullal J., Kutney K., Williams K. M., Weber D. R. Treatment of cystic fibrosis related bone disease. J Clin Transl Endocrinol. 2022;27:100291.

50. Jeffery T.C., Chang A. B., Conwell L. S. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev. 2023;1: CD002010.

51. Olesen H.V., Drevinek P., Gulmans V. A., Hatziagorou E., Jung A., Mei-Zahav M. et al. Cystic fibrosis related diabetes in Europe: prevalence, risk factors and outcome; Olesen et al. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2020;19: 321-7.

52. Alves C., Della-Manna T., Albuquerque C. T.M. Cystic fibrosis-related diabetes: an update on pathophysiology, diagnosis, and treatment. J Pediatr Endocrinol Metab. 2020;33:835-43.

53. Prentice B.J., Chelliah A., Ooi C. Y. et al. Peak OGTT glucose is associated with lower lung function in young children with cystic fibrosis. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2020;19:305-9.

54. Scully K.J., Sherwood J. S., Martin K. et al. Continuous glucose monitoring and HbA1c in cystic fibrosis: clinical correlations and implications for CFRD diagnosis. J Clin Endocrinol Metab. 2022;107: e1444e54.

55. Racine F., Shohoudi A., Boudreau V. et al. Glycated hemoglobin as a first-line screening test for cystic fibrosis- related diabetes and impaired glucose tolerance in children with cystic fibrosis: a validation study. Can J Diabetes. 2021;45:768e74.

56. Onady G.M., Stolfi A. Drug treatments for managing cystic fibrosis-related diabetes. Cochrane Database Syst Rev. 2020;10: CD004730.

57. Thavamani A., Salem I., Sferra T. J., Sankararaman S. Impact of altered gut microbiota and its metabolites in cystic fibrosis. Metabolites. 2021;11:123.

58. Bruzzese E., Raia V., Ruberto E., Scotto R., Giannattasio A., Bruzzese D. et al. Lack of efficacy of Lactobacillus GG in reducing pulmonary exacerbations and hospital admissions in children with cystic fibrosis: a randomised placebo controlled trial. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2018;17:375-82.

59. Van Biervliet S., Hauser B., Verhulst S., Stepman H., Delanghe J., Warzee J. P. et al. Probiotics in cystic fibrosis patients: a double blind crossover placebo controlled study: pilot study from the ESPGHAN Working Group on Pancreas/CF. Clin Nutr ESPEN 2018;27:59-65.

60. Duckers J., Lesher B., Thorat T., Lucas E., McGarry L.J., Chandarana K. et al. Real-world outcomes of ivacaftor treatment in people with cystic fibrosis: a systematic review. J Clin Med. 2021;10:1527.

61. Southern K.W., Murphy J., Sinha I. P., Nevitt S. J. Corrector therapies (with or without potentiators) for people with cystic fibrosis with class II CFTR gene variants (most commonly F508del). Cochrane Database Syst Rev. 2020;12: CD010966.

62. Middleton P.G., Mall M. A., Drevínek P. et al. Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. N Engl J Med. 2019;381:1809-19.

63. Carnovale V., Iacotucci P., Terlizzi V. et al. Effectiveness and safety of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease with the Phe508del/minimal function genotype. Respir Med. 2021;189:106646.

64. Burgel P-R., Munck A., Durieu I. et al. Real-life safety and effectiveness of lumacaftor-ivacaftor in patients with cystic fibrosis. Am J Respir Crit Care Med. 2020;201:188-97.

65. Aalbers B.L., de Winter-de Groot K. M., Arets H. G.M. et al. Clinical effect of lumacaftor/ivacaftor in F508del homozygous CF patients with FEV(1) >/1⁄4 90% predicted at baseline. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2020;19:654-8.

66. Bailey J., Rozga M., McDonald C.M. et al. Effect of CFTR modulators on anthropometric parameters in individuals with cystic fibrosis: an evidence analysis center systematic review. J Acad Nutr Diet. 2021;121:1364-13678 e2.

67. Petersen M.C., Begnel L., Wallendorf M., Litvin M. Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2022;21:265-71.

68. King S.J., Tierney A. C., Edgeworth D. et al. Body composition and weight changes after ivacaftor treatment in adults with cystic fibrosis carrying the G551 D cystic fibrosis transmembrane conductance regulator mutation: a double-blind, placebo-controlled, randomized, crossover study with open-label extension. Nutrition. 2021;85: 111124.

69. Sommerburg O., Hammerling S., Schneider S. P. et al. CFTR modulator therapy with lumacaftor/ivacaftor alters plasma concentrations of lipid-soluble vitamins A and E in patients with cystic fibrosis. Antioxidants. 2021;10:483.

70. Francalanci M., Terlizzi V., Fevola C. et al. Nutritional status and circulating levels of fat-soluble vitamins in cystic fibrosis patients: a cohort study and evaluation of the effect of CFTR modulators. Basel, Switzerland: Children. 2023. p. 10.

71. Wisniewski B.L., Aylward S. C., Jordan C. O. et al. Hypervitaminosis A with fulminant secondary intracranial hypertension following personal ized medicine-based Elexacaftor/Tezacaftor/Ivacaftor initiation in a preadolescent with cystic fibrosis. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2022;21:217-20.

72. Hutchinson I., McNally P. Appearance of pancreatic sufficiency and discontinuation of pancreatic enzyme replacement therapy in children with cystic fibrosis on ivacaftor. Ann Am Thorac Soc. 2021;18:182-3.

73. Tetard C., Mittaine M., Bui S. et al. Reduced intestinal inflammation with lumacaftor/ivacaftor in adolescents with cystic fibrosis. J Pediatr Gastroenterol Nutr. 020;71:778-81.

74. Dagenais R.V.E., Su V. C.H., Quon B. S. Real-world safety of CFTR modulators in the treatment of cystic fibrosis: a systematic review. J Clin Med. 2020;10.

75. Scully K.J., Marchetti P., Sawicki G. S., Uluer A., Cernadas M., Cagnina R. E. et al. The effect of elexacaftor/tezacaftor/ivacaftor (ETI) on glycemia in adults with cystic fibrosis. J Cyst Fibros: Off J Eur Cystic Fibr Soci. 2022;21:258-63.

76. Levine H., Prais D., Raviv Y. et al. Lung transplantation in cystic fibrosis patients in Israel: the importance of ethnicity and nutritional status. Clin Transplant. 2017;31.

77. Pryor J.B., Bradford M. C., Jennerich A. L. et al. Body mass index recovery after lung transplant for cystic fibrosis. Ann Am Thorac Soc. 2022;19:1130-8.

78. Cheng K., Rosenthal P., Roberts J. P., Perito E. R. Liver transplant in children and adults with cystic fibrosis: impact of growth failure and nutritional status. Am J Transplant. 2022;22:177-86.

79. Flemming G., Baumann U., Richter N. et al. Survival benefits following liver transplantation: a matched-pair analysis in pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2021;73:385-90.

80. [Clinical guidelines Cystic fibrosis (mucoviscidosis)]. Age group: children/adults. Year of approval: 2021. CD 372. (in Russ.)@@ Клинические рекомендации Кистозный фиброз (муковисцидоз). Возрастная группа: дети/взрослые. Год утверждения: 2021. КР 372.