Depending on parenteral nutrition to survive. Chronic intestinal failure due to short bowel syndrome

A case report and literature review

Keywords: SHORT BOWEL SYNDROME, PARENTERAL NUTRITION, TEDUGLUTIDE, INTESTINAL FAILURE, CHRONIC DISEASE

Abstract

Short bowel syndrome consists of intestinal failure after a massive intestinal resection. It is an unusual condition, ranging from a mild and reversible malfunction to a severe condition causing inability. Short bowel syndrome has high morbimortality, high medical costs and a significant impact on quality of life. 50% of patients with short bowel syndrome do not spontaneously adapt and neither does absorption improve by the remaining intestine, thus they depend on parenteral nutrition for the rest of their lives. Specialized nutritional therapy and focalized hormone therapy in the intestine are pillars in rehabilitation. They promote intestine adaptation and achieve independence from parenteral nutrition and avoid intestinal transplant. Today, Teduglutide is the reference treatment for these patients, although it is still not available in Uruguay due to its high cost. The study presents a 23 year old patient carrier of chronic intestinal failure due to short bowel syndrome who has depended on parenteral nutrition for 17 years and presents severe complications associated to this technique.

References

(1) Pironi L, Arends J, Baxter J, Bozzetti F, Peláez RB, Cuerda C, et al. ESPEN endorsed recommendations. Definition and classification of intestinal failure in adults. Clin Nutr 2015; 34:171-80.

(2) Jeppesen PB. Short bowel syndrome – characterisation of an orphan condition with many phenotypes. Expert Opin Orphan Drugs 2013; 1:515-25.

(3) Wanden-Berghe C, Virgili Casas N, Ramos Boluda E, Cuerda Compés C, Moreno Villares JM, Pereira Cunill JL, et al. Informe del Grupo de Nutrición Artificial Domiciliaria y Ambulatoria (NADYA)-Nutrición parenteral domiciliaria en España, 2016. Nutr Hosp 2017; 34:1497-501.

(4) Pironi L, Konrad D, Brandt C, Joly F, Wanten G, Agostini F, et al. Clinical classification of adult patients with chronic intestinal failure due to benign disease: an international multicenter cross-sectional survey. Clin Nutr 2018; 37:728- 38.

(5) Neelis EG, Olieman JF, Hulst JM, de Koning BA, Wijnen RM5, Rings EH. Promoting intestinal adaptation by nutrition and medication. Best Pract Res Clin Gastroenterol 2016; 30(2):249-61.

(6) Pironi L, Arends J, Bozzetti F, Cuerda C, Gillanders L, Jeppesen PB, et al. ESPEN guidelines on chronic intestinal failure in adults. Clin Nutr 2016; 35(2):247-307.

(7) Beyer-Berjot L, Joly F, Maggiori L, Corcos O, Bouhnik Y, Bretagnol F, et al. Segmental reversal of the small bowel can end permanent parenteral nutrition dependency: an experience of 38 adults with short bowel syndrome. Ann Surg 2012; 256:739-44.

(8) Layec S, Beyer L, Corcos O, Alves A, Dray X, Amiot A, et al. Increased intestinal absorption by segmental reversal of the small bowel in adult patients with short-bowel syndrome: a case-control study. Am J Clin Nutr 2013; 97:100-8.

(9) Grant D, Abu-Elmagd K, Mazariegos G, Vianna R, Langnas A, Mangus R, et al. Intestinal transplant registry report: global activity and trends. Am J Transplant 2015; 15:210-9.

(10) Semrad C. Aproximación al paciente con diarrea y malabsorción. En: Goldman-Cecil. Tratado de medicina interna. 25ª ed. Barcelona: Elsevier, 2017:918-35.

(11) Pironi L. Definitions of intestinal failure and the short bowel syndrome. Best Pract Res Clin Gastroenterol 2016; 30(2):173-85. DOI: 10.1016/j.bpg.2016.02.011.

(12) Tappenden KA. Pathophysiology of short bowel syndrome: considerations of resected and residual anatomy. JPEN J Parenter Enteral Nutr 2014; 38(1 Suppl):14S-22S.

(13) Nightingale J, Woodward JM. Guidelines for management of patients with a short bowel. Gut 2006; 55(4 Suppl):iv1-12.

(14) Parrish CR, DiBaise JK. Managing the adult patient with short bowel syndrome. Gastroenterol Hepatol (N Y) 2017; 13(10):600-8.

(15) Chan LN, DiBaise JK, Parrish CR. Short bowel syndrome in adults-Part 4A. A guide to front line drugs used in the treatment of short bowel syndrome. Practical Gastroenterol 2015; 39(3):28-42.

(16) Winkler MF, Smith CE. Clinical, social, and economic impacts of home parenteral nutrition dependence in short bowel syndrome. JPEN J Parenter Enteral Nutr 2014; 38(1 Suppl): 32S-7S.

(17) Canovai E, Ceulemans LJ, Peers G, De Pourcq L, Pijpops M, De Hertogh G, et al. Cost analysis of chronic intestinal failure. Clin Nutr 2018; 38(4):1729-36. DOI:10.1016/j. clnu.2018.07.032.

(18) 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):1043-50.

(19) 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; 32:368-74.

(20) Jeppesen PB. The non-surgical treatment of adult patients with short bowel syndrome. J Expert Opin Orphan Drugs 2013; 1:527-38.

(21) Solar H, Crivelli A, Echevarria C, Ramisch D, Luis Moulin, Fabio Nashman, et al. Autologous gastro-intestinal reconstruction in adult patients with severe intestinal failure. Report of long term outcomes and its correlation with anatomical predicting factors. Report from a single center. Abstracts from the XIV International Small Bowel Transplant Symposium. Transplant 2015; 99(6S-1 Suppl):S107. Abstract nº 655.

(22) Pape UF, Maasberg S, Pascher A. Pharmacological strategies to enhance adaptation in intestinal failure. Curr Opin Organ Transplant 2016; 21:147-52.

(23) Gondolesi GE, Pattín F, Nikkoupur H. Management of intestinal failure in middle-income countries, for children and adults. Curr Opin Organ Transplant 2018; 23(2):212-8.

(24) Hvistendahl M, Brandt CF, Tribler S, Naimi RM, Hartmann B, Holst JJ, et al. Effect of liraglutide treatment on jejunostomy output in patients with short bowel syndrome: an open-label pilot study. JPEN J Parenter Enteral Nutr 2016; 42(1):112-21.

(25) Jeppesen PB, Sanguinetti EL, Buchman A, Howard L, Scolapio JS, Ziegler TR, et al. Teduglutide (ALX-0600), a dipeptidyl peptidase IV resistant glucagon-like peptide 2 analogue, improves intestinal function in short bowel syndrome patients. Gut 2005; 54:1224-31.

(26) 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; 143:1473-81.e3.

(27) 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:e142.

(28) 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; 154:874-85.

(29) Billiauws L, Bataille J, Boehm V, Corcos O, Joly F. Teduglutide for treatment of adult patients with short bowel syndrome. Expert Opin Biol Ther 2017; 17:623-32.

(30) Ring LL, Nerup N, Jeppesen PB, Svendsen LB, Achiam MP. Glucagon like peptide-2 and neoplasia; a systematic review. Expert Rev Gastroenterol Hepatol 2018; 12:257-64.

(31) Kochar B, Herfarth HH. Teduglutide for the treatment of short bowel syndrome - a safety evaluation. Expert Opin Drug Saf 2018; 17(7):733-9.

(32) Naimi RM, Hvistendahl M, Enevoldsen LH, Madsen JL, Fuglsang S, Poulsen SS, et al. Glepaglutide, a novel long-acting glucagon-like peptide-2 analogue, for patients with short bowel syndrome: a randomised phase 2 trial. Lancet Gastroenterol Hepatol 2019; 4(5):354-63. DOI:/10.1016/S2468-1253(19)30077-9.

Published
2019-11-04
How to Cite
1.
Moreira E, López P, Silva L, Olano E. Depending on parenteral nutrition to survive. Chronic intestinal failure due to short bowel syndrome. Rev. Méd. Urug. [Internet]. 2019Nov.4 [cited 2024Nov.25];35(4):316-24. Available from: http://www2.rmu.org.uy/ojsrmu311/index.php/rmu/article/view/311