Важные практические аспекты ведения пациентов с синдромом короткой кишки с кишечной недостаточностью

Синдром короткой кишки – расстройство функции желудочно-кишечного тракта – возникает в результате массивной резекции тонкой кишки. Причинами резекции наиболее часто становятся такие заболевания, как острая мезентериальная ишемия, болезнь Крона, травма брюшной полости, спаечная кишечная непроходимость, злокачественные новообразования кишечника, лучевой энтерит. Синдром короткой кишки с кишечной недостаточностью характеризуется тяжелой мальабсорбцией – мальдигестией, сопровождающейся болями в животе, диареей, дегидратацией и прогрессированием белково-энергетической недостаточности. У многих пациентов с синдромом короткой кишки со временем развиваются долгосрочные клинические осложнения, связанные с измененной анатомией и физиологией кишечника или различными лечебными мероприятиями, такими как парентеральное питание и центральный венозный катетер. Данный обзор посвящен наиболее важным аспектам диагностики, лечения и профилактики ряда осложнений, которые могут возникнуть у данного контингента пациентов, включая нарушение баланса макро- и микронутриентов, диарею, дисбаланс жидкости и электролитов, метаболические заболевания костей, нарушения желчеотделения, избыточный рост бактерий в тонком кишечнике, а также осложнений, связанных с венозным доступом. Основными лечебными стратегиями при синдроме короткой кишки с кишечной недостаточностью являются: диетотерапия, энтеральная нутритивная поддержка полуэлементными смесями, парентеральное питание, пероральная регидратация, назначения антисекреторных и антидиаррейных препаратов, ферментов. Введение аналога глюкагоноподобного пептида-2 тедуглутида клинически целесообразно для снижения зависимости от парентерального питания и улучшения качества жизни. Ведение пациентов с синдромом короткой кишки с кишечной недостаточностью является сложным, длительным процессом и требует тщательного динамического клинико-лабораторного мониторинга.

1. Аверьянова ЮВ, Батыршин ИМ, Демко АЕ, Иванова ГЕ, Ивашкин ВТ, Костюченко ЛН и др. Клинические рекомендации Северо-Западной ассоциации парентерального и энтерального питания, Межрегиональной ассоциации по неотложной хирургии, Российской гастроэнтерологической ассоциации, Союза реабилитологов России и Российского трансплантационного общества по диагностике и лечению синдрома короткой кишки с кишечной недостаточностью у взрослых. Российский журнал гастроэнтерологии, гепатологии, колопроктологии. 2022;32(1):60–103. https://doi.org/10.22416/1382-4376-2022-32-1-60-103.

2. Pironi L. Definitions of intestinal failure and the short bowel syndrome. Best Pract Res Clin Gastroenterol. 2016;30(2):173–185. https://doi.org/10.1016/j.bpg.2016.02.011.

3. Nightingale JM, Kamm MA, van der Sijp JR, Ghatei MA, Bloom SR, LennardJones JE. Gastrointestinal hormones in short bowel syndrome. Peptide YY may be the ‘colonic brake’ to gastric emptying. Gut. 1996;39(2):267–272. https://doi.org/10.1136/gut.39.2.267.

4. Parrish CR, DiBaise JK. Managing the Adult Patient With Short Bowel Syndrome. Gastroenterol Hepatol (N Y). 2017;13(10):600–608. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5718176/.

5. Massironi S, Cavalcoli F, Rausa E, Invernizzi P, Braga M, Vecchi M. Understanding short bowel syndrome: current status and future perspectives. Dig Liver Dis. 2020;52(3):253–261. https://doi.org/10.1016/j.dld.2019.11.013.

6. Pape UF, Maasberg S, Pascher A. Pharmacological strategies to enhance adaptation in intestinal failure. Curr Opin Organ Transplant. 2016;21(2):147–152. https://doi.org/10.1097/MOT.0000000000000296.

7. Tappenden KA. Intestinal adaptation following resection. JPEN J Parenter Enteral Nutr. 2014;38(1 Suppl.):23S–31S. https://doi.org/10.1177/0148607114525210.

8. Messing B, Crenn P, Beau P, Boutron-Ruault MC, Rambaud JC, Matuchansky C. Long-term survival and parenteral nutrition dependence in adult patients with the short bowel syndrome. Gastroenterology. 1999;117(5):1043–1050. https://doi.org/10.1016/s0016-5085(99)70388-4.

9. DiBaise JK, Parrish CR, Thompson JS (eds.). Short Bowel Syndrome: Practical Approach to Management. Boca Raton: CRC Press; 2016. 458 p. https://doi.org/10.1201/b20188.

10. Mermel LA, Allon M, Bouza E, Craven DE, Flynn P, O’Grady NP et al. Clinical practice guidelines for the diagnosis and management of intravascular catheter-related infection: 2009 Update by the Infectious Diseases Society of America. Clin Infect Dis. 2009;49(1):1–45. https://doi.org/10.1086/599376.

11. Leiberman D, Stevenson RP, Banu FW, Gerasimidis K, McKee RF. The incidence and management of complications of venous access in home parenteral nutrition (HPN): A 19 year longitudinal cohort series. Clin Nutr ESPEN. 2020;37:34–43. https://doi.org/10.1016/j.clnesp.2020.03.025.

12. Johnson E, Vu L, Matarese LE. Bacteria, Bones, and Stones: Managing Complications of Short Bowel Syndrome. Nutr Clin Pract. 2018;33(4):454–466. https://doi.org/10.1002/ncp.10113.

13. Wall EA. An overview of short bowel syndrome management: adherence, adaptation, and practical recommendations. J Acad Nutr Diet. 2013;113(9):1200–1208. https://doi.org/10.1016/j.jand.2013.05.001.

14. Lakkasani S, Seth D, Khokhar I, Touza M, Dacosta TJ. Concise review on short bowel syndrome: Etiology, pathophysiology, and management. World J Clin Cases. 2022;10(31):11273–11282. https://doi.org/10.12998/wjcc.v10.i31.11273.

15. Rudziński M, Ławiński M, Gradowski Ł, Antoniewicz AA, Słodkowski M, Bedyńska S et al. Kidney stones are common in patients with short-bowel syndrome receiving long-term parenteral nutrition: A predictive model for urolithiasis. JPEN J Parenter Enteral Nutr. 2022;46(3):671–677. https://doi.org/10.1002/jpen.2133.

16. Nightingale J, Woodward JM. Guidelines for management of patients with a short bowel. Gut. 2006;55(4 Suppl.):iv1–12. https://doi.org/10.1136/gut.2006.091108.

17. Kumar PR, Fenton TR, Shaheen AA, Raman M. Prevalence of vitamin D deficiency and response to oral vitamin D supplementation in patients receiving home parenteral nutrition. JPEN J Parenter Enteral Nutr. 2012;36(4):463–469. https://doi.org/10.1177/0148607111416483.

18. Uzzan M, Kirchgesner J, Poupon J, Corcos O, Pingenot I, Joly F. Antioxidant trace elements serum levels in long-term parenteral nutrition (PN): Prevalence and infectious risk associated with deficiencies, a retrospective study from a tertiary home-PN center. Clin Nutr. 2017;36(3):812–817. https://doi.org/10.1016/j.clnu.2016.05.008.

19. Btaiche IF, Carver PL, Welch KB. Dosing and monitoring of trace elements in long-term home parenteral nutrition patients. JPEN J Parenter Enteral Nutr. 2011;35(6):736–747. https://doi.org/10.1177/0148607111413902.

20. Petersen C. D-lactic acidosis. Nutr Clin Pract. 2005;20(6):634–645. https://doi.org/10.1177/0115426505020006634.

21. Matarese LE. Nutrition and fluid optimization for patients with short bowel syndrome. JPEN J Parenter Enteral Nutr. 2013;37(2):161–170. https://doi.org/10.1177/0148607112469818.

22. Parrish CR, DiBaise JK. Short Bowel Syndrome in Adults – Part 3. Hydrating the Adult Patient with Short Bowel Syndrome. Pract Gastroenterol. 2015;39(2):10–18. Available at: https://med.virginia.edu/ginutrition/wp-content/uploads/sites/199/2014/06/Parrish_Feb_15.pdf.

23. Baker ML, Williams RN, Nightingale JM. Causes and management of a high-output stoma. Colorectal Dis. 2011;13(2):191–197. https://doi.org/10.1111/j.1463-1318.2009.02107.x.

24. Arenas Villafranca JJ, Abilés J, Moreno G, Tortajada Goitia B, Utrilla Navarro P, Gándara Adán N. Ostomías de alto débito: detección y abordaje. Nutr Hosp. 2014;30(6):1391–1396. https://doi.org/10.3305/nh.2014.30.6.7894.

25. Chan LN, Limketkai BN. Electrolyte and acid‐base disturbances. In: DiBaise JK, Parrish CR, Thompson JS (eds.). Short Bowel Syndrome: Practical Approach to Management. Boca Raton: CRC Press; 2016, pp. 67–80. https://doi.org/10.1201/b20188.

26. Iyer K, DiBaise JK, Rubio-Tapia A. AGA Clinical Practice Update on Management of Short Bowel Syndrome: Expert Review. Clin Gastroenterol Hepatol. 2022;20(10):2185–2194.e2. https://doi.org/10.1016/j.cgh.2022.05.032.

27. Лукьянец ОБ, Петрова МВ, Яковлева АВ, Шестопалов АЕ. Роль кишечного лаважа в нормализации функций желудочно-кишечного тракта у пациентов в хроническом критическом состоянии. Клиническое питание и метаболизм. 2022;3(3):132–139. https://doi.org/10.17816/clinutr111148.

28. Киселев ВВ, Петриков СС, Жигалова МС, Новиков СВ, Шаврина НВ, Ярцев ПА. Восстановление пропульсивной функции кишечника у пациентов с тяжелым острым панкреатитом в условиях отделения реанимации и интенсивной терапии. Журнал им. Н.В. Склифосовского «Неотложная медицинская помощь». 2023;12(2):210–216. https://doi.org/10.23934/2223-9022-2023-12-2-210-216.

29. Jeppesen PB. Spectrum of short bowel syndrome in adults: intestinal insufficiency to intestinal failure. JPEN J Parenter Enteral Nutr. 2014;38(1 Suppl.):8S–13S. https://doi.org/10.1177/0148607114520994.

30. Баранская ЕК, Шульпекова ЮО. Принципы ведения пациентов с синдромом короткого кишечника. РМЖ. 2010;(13):789–794. Режим доступа: https://www.rmj.ru/articles/bolezni_organov_pishchevareniya/Principy_vedeniya_pacientov__s_sindromom_korotkogo_kishechnika/.

31. Dibb M, Teubner A, Theis V, Shaffer J, Lal S. Review article: the management of long-term parenteral nutrition. Aliment Pharmacol Ther. 2013;37(6):587–603. https://doi.org/10.1111/apt.12209.

32. Hortencio TDR, Arendt BM, Teterina A, Jeejeebhoy KN, Gramlich LM, Whittaker JS et al. Changes in Home Parenteral Nutrition Practice Based on the Canadian Home Parenteral Nutrition Patient Registry. JPEN J Parenter Enteral Nutr. 2017;41(5):830–836. https://doi.org/10.1177/0148607115609289.

33. Wanten G, Calder PC, Forbes A. Managing adult patients who need home parenteral nutrition. BMJ. 2011;342:d1447. https://doi.org/10.1136/bmj.d1447.

34. Duerksen DR, Papineau N, Siemens J, Yaffe C. Peripherally inserted central catheters for parenteral nutrition: a comparison with centrally inserted catheters. JPEN J Parenter Enteral Nutr. 1999;23(2):85–89. https://doi.org/10.1177/014860719902300285.

35. Mullady DK, O’Keefe SJ. Treatment of intestinal failure: home parenteral nutrition. Nat Clin Pract Gastroenterol Hepatol. 2006;3(9):492–504. https://doi.org/10.1038/ncpgasthep0580.

36. Dibb MJ, Abraham A, Chadwick PR, Shaffer JL, Teubner A, Carlson GL, Lal S. Central Venous Catheter Salvage in Home Parenteral Nutrition CatheterRelated Bloodstream Infections: Long-Term Safety and Efficacy Data. JPEN J Parenter Enteral Nutr. 2016;40(5):699–704. https://doi.org/10.1177/0148607114549999.

37. Buchman AL, Opilla M, Kwasny M, Diamantidis TG, Okamoto R. Risk factors for the development of catheter-related bloodstream infections in patients receiving home parenteral nutrition. JPEN J Parenter Enteral Nutr. 2014;38(6):744–779. https://doi.org/10.1177/0148607113491783.

38. Staun M, Pironi L, Bozzetti F, Baxter J, Forbes A, Joly F et al. ESPEN Guidelines on Parenteral Nutrition: home parenteral nutrition (HPN) in adult patients. Clin Nutr. 2009;28(4):467–479. https://doi.org/10.1016/j.clnu.2009.04.001.

39. Amiot A, Messing B, Corcos O, Panis Y, Joly F. Determinants of home parenteral nutrition dependence and survival of 268 patients with non-malignant short bowel syndrome. Clin Nutr. 2013;32(3):368–374. https://doi.org/10.1016/j.clnu.2012.08.007.

40. Bering J, DiBaise JK. Short Bowel Syndrome in Adults. Am J Gastroenterol. 2022;117(6):876–883. https://doi.org/10.14309/ajg.0000000000001763.

41. Chan LN, Parrish CR, DiBaise JK. Short Bowel Syndrome in Adults – Part 4A A Guide to Front Line Drugs Used in the Treatment of Short Bowel Syndrome. Pract Gastroenterol. 2015;39(3):28–43. Available at: https://practicalgastro.com/2015/03/06/short-bowel-syndrome-in-adults-part-4a-a-guide-to-frontline-drugs-used-in-the-treatment-of-short-bowel-syndrome/.

42. Davila J, Konrad D. Metabolic Complications of Home Parenteral Nutrition. Nutr Clin Pract. 2017;32(6):753–768. https://doi.org/10.1177/0884533617735089.

43. Gompelman M, Paus C, Bond A, Akkermans RP, Bleeker-Rovers CP, Lal S, Wanten GJA. Comparing success rates in central venous catheter salvage for catheter-related bloodstream infections in adult patients on home parenteral nutrition: a systematic review and meta-analysis. Am J Clin Nutr. 2021;114(3):1173–1188. https://doi.org/10.1093/ajcn/nqab164.

44. Kumpf VJ. Pharmacologic management of diarrhea in patients with short bowel syndrome. JPEN J Parenter Enteral Nutr. 2014;38(1 Suppl.):38S–44S. https://doi.org/10.1177/0148607113520618.

45. Amiot A, Messing B, Corcos O, Panis Y, Joly F. Determinants of home parenteral nutrition dependence and survival of 268 patients with non-malignant short bowel syndrome. Clin Nutr. 2013;32(3):368–374. https://doi.org/10.1016/j.clnu.2012.08.007.

46. De Vries FEE, Reeskamp LF, van Ruler O, van Arum I, Kuin W, Dijksta G et al. Systematic review: pharmacotherapy for high-output enterostomies or enteral fistulas. Aliment Pharmacol Ther. 2017;46(3):266–273. https://doi.org/10.1111/apt.14136.

47. Martin GR, Wallace LE, Hartmann B, Holst JJ, Demchyshyn L, Toney K, Sigalet DL. Nutrient-stimulated GLP-2 release and crypt cell proliferation in experimental short bowel syndrome. Am J Physiol Gastrointest Liver Physiol. 2005;288(3):G431–438. https://doi.org/10.1152/ajpgi.00242.2004.

48. Jeppesen PB, Gilroy R, Pertkiewicz M, Allard JP, Messing B, O’Keefe SJ. Randomised placebo-controlled trial of teduglutide in reducing parenteral nutrition and/or intravenous fluid requirements in patients with short bowel syndrome. Gut. 2011;60(7):902–914. https://doi.org/10.1136/gut.2010.218271.

49. Drucker DJ, Yusta B. Physiology and pharmacology of the enteroendocrine hormone glucagon-like peptide-2. Annu Rev Physiol. 2014;76:561–583. https://doi.org/10.1146/annurev-physiol-021113-170317.

50. Jeppesen PB. Teduglutide, a novel glucagon-like peptide 2 analog, in the treatment of patients with short bowel syndrome. Therap Adv Gastroenterol. 2012;5(3):159–171. https://doi.org/10.1177/1756283X11436318.

51. O’Keefe SJ, Jeppesen PB, Gilroy R, Pertkiewicz M, Allard JP, Messing B. Safety and efficacy of teduglutide after 52 weeks of treatment in patients with short bowel intestinal failure. Clin Gastroenterol Hepatol. 2013;11(7):815–823.E3. https://doi.org/10.1016/j.cgh.2012.12.029.

52. Gondolesi GE, Doeyo M, Echevarria Lic C, Lobos F, Rubio S, Rumbo C et al. Results of Surgical and Medical Rehabilitation for Adult Patients With Type III Intestinal Failure in a Comprehensive Unit Today: Building a New Model to Predict Parenteral Nutrition Independency. JPEN J Parenter Enteral Nutr. 2020;44(4):703–713. https://doi.org/10.1002/jpen.1686.

53. Seidner DL, Fujioka K, Boullata JI, Iyer K, Lee HM, Ziegler TR. Reduction of Parenteral Nutrition and Hydration Support and Safety With Long-Term Teduglutide Treatment in Patients With Short Bowel Syndrome-Associated Intestinal Failure: STEPS-3 Study. Nutr Clin Pract. 2018;33(4):520–527. https://doi.org/10.1002/ncp.10092.

54. Jeppesen PB, Pertkiewicz M, Messing B, Iyer K, Seidner DL, O’Keefe SJ et al. Teduglutide reduces need for parenteral support among patients with short bowel syndrome with intestinal failure. Gastroenterology. 2012;143(6):1473–1481.e3. https://doi.org/10.1053/j.gastro.2012.09.007.

55. Schwartz LK, O’Keefe SJ, Fujioka K, Gabe SM, Lamprecht G, Pape UF et al. Long-Term Teduglutide for the Treatment of Patients With Intestinal Failure Associated With Short Bowel Syndrome. Clin Transl Gastroenterol. 2016;7(2):e142. https://doi.org/10.1038/ctg.2015.69.

56. Jeppesen PB, Gabe SM, Seidner DL, Lee HM, Olivier C. Factors Associated With Response to Teduglutide in Patients With Short-Bowel Syndrome and Intestinal Failure. Gastroenterology. 2018;154(4):874–885. https://doi.org/10.1053/j.gastro.2017.11.023.

57. Seidner DL, Gabe SM, Lee HM, Olivier C, Jeppesen PB. Enteral Autonomy and Days Off Parenteral Support With Teduglutide Treatment for Short Bowel Syndrome in the STEPS Trials. JPEN J Parenter Enteral Nutr. 2020;44(4):697–702. https://doi.org/10.1002/jpen.1687.

58. Pevny S, Maasberg S, Rieger A, Karber M, Blüthner E, Knappe-Drzikova B et al. Experience with teduglutide treatment for short bowel syndrome in clinical practice. Clin Nutr. 2019;38(4):1745–1755. https://doi.org/10.1016/j.clnu.2018.07.030.

59. Seidner DL, Schwartz LK, Winkler MF, Jeejeebhoy K, Boullata JI, Tappenden KA. Increased intestinal absorption in the era of teduglutide and its impact on management strategies in patients with short bowel syndrome-associated intestinal failure. JPEN J Parenter Enteral Nutr. 2013;37(2):201–211. https://doi.org/10.1177/0148607112472906.

60. Pironi L, Sasdelli AS, Venerito FM, Musio A, Pazzeschi C, Guidetti M. Candidacy of adult patients with short bowel syndrome for treatment with glucagon-like peptide-2 analogues: A systematic analysis of a single centre cohort. Clin Nutr. 2021;40(6):4065–4074. https://doi.org/10.1016/j.clnu.2021.02.011.

61. Pironi L. Translation of Evidence Into Practice With Teduglutide in the Management of Adults With Intestinal Failure due to Short-Bowel Syndrome: A Review of Recent Literature. JPEN J Parenter Enteral Nutr. 2020;44(6):968–978. https://doi.org/10.1002/jpen.1757.

62. Pironi L, Allard JP, Joly F, Geransar P, Genestin E, Pape UF. Use of teduglutide in adults with short bowel syndrome-associated intestinal failure. Nutr Clin Pract. 2024;39(1):141–153. https://doi.org/10.1002/ncp.11015.

63. Klek S, Szczepanek K, Scislo L, Walewska E, Pietka M, Pisarska M, Pedziwiatr M. Intravenous lipid emulsions and liver function in adult chronic intestinal failure patients: Results after 5 y of home parenteral nutrition. Nutrition. 2021;82:111029. https://doi.org/10.1016/j.nut.2020.111029.