S3-Leitlinie der Deutschen Gesellschaft für Ernährungsmedizin e. V. in Zusammenarbeit mit der AKE, der GESKES und der DGVS

Zusammenfassung

Einleitung: Beim Kurzdarmsyndrom und beim Darmversagen sind enterale und parenterale Ernährung die Basis der Therapie. Die rekonstruktive Chirurgie vermag die anatomische Situation zu verbessern. Die Darmtransplantation stellt gegenwärtig eine Therapieoption beim drohenden Scheitern der parenteralen Ernährung dar.

Methodik: Es wurde eine systematische Literatursuche zum Kurzdarmsyndrom und zum Darmversagen, gekoppelt mit einer Literatursuche zur enteralen und parenteralen Ernährung, zur rekonstruktiven Chirurgie und zur Darmtransplantation durchgeführt. In der Arbeitsgruppe wurden auf dieser Basis Empfehlungen formuliert und hinsichtlich der Empfehlungsstärke bewertet. Sie wurden anschließend in einem Delphi-Verfahren und einer Konsensuskonferenz vorgestellt, diskutiert und verabschiedet.

Ergebnisse: Die Leitlinie bezieht sich spezifisch auf das Kurzdarmsyndrom bei Erwachsenen. Sie enthält einen allgemeinen Teil mit Definitionen sowie Empfehlungen zur Dokumentation der anatomischen Situation und des Ernährungszustands, zur Indikation, Zusammensetzung und Durchführung einer parenteralen Ernährung (unter besonderer Berücksichtigung der meist im Vordergrund stehenden Flüssigkeits- und Elektrolytverluste), zu den Prinzipien der spezifischen Diät, zu den Kathetern und deren Infektionsmanagement, zur spezifischen und symptomatischen Pharmakotherapie, zur rekonstruktiven Chirurgie und zur Darmtransplantation.

Schlussfolgerung: Kontrollierte Studien sind beim Darmversagen spärlich wegen der Seltenheit des Krankheitsbilds und der großen individuellen Unterschiede. Die prognostizierte Kurzdarmsituation auf der Basis einer möglichst detailliert beschriebenen anatomischen Ausgangssituation stellt die Indikation zur Ernährungsintervention dar. Die ergänzend intendierte, individualisierte parenterale Ernährung, die Prophylaxe und Therapie der Komplikationen und die rekonstruktiv-operativen Ansätze sollen konsequent umgesetzt werden. Spezifische und symptomatische pharmakologische Ansätze können ebenfalls genutzt werden. Die Darmtransplantation stellt eine Option beim drohenden Scheitern der parenteralen Ernährung dar.

Abstract

Introduction: Enteral and parenteral nutrition are the cornerstone for the therapy of short bowel syndrome and intestinal failure. The underlying anatomic situation may be amendable to reconstructive surgery. Intestinal transplantation is currently an option in case of failure of parenteral nutrition.

Methods: A systematic literature search for short bowel syndrome or intestinal failure coupled with a literature search for enteral or parenteral nutrition, reconstructive surgery or intestinal transplantation was performed. Based on this the working group developed recommendations, which were presented, discussed and confirmed in a consensus conference.

Results: The guideline addresses only short bowel syndrome (intestinal failure) in adults. It contains a general part with definitions and a specific part with recommendations for the documentation of the postoperative anatomy and the nutritional status, for the indication, the compounding and the administration of parenteral nutrition (with special emphasis on the predominant fluid and electrolyte losses), for the principles of a specific diet, for the catheters and the management of their infectious complications, for the specific and symptomatic pharmacotherapy, for reconstructive surgery and for intestinal transplantation.

Conclusion: Controlled studies addressing intestinal failure are sparse due to the orphan nature of the disease and due to the large interindividual variation. The anticipated short bowel situation (based on a detailed description of the postoperative situation) is the indication for nutritional intervention. Individually tailored (compounded) parenteral nutrition, prophylaxis and treatment of complications and reconstructive surgery should all be applied. Specific and symptomatic pharmacologic approaches can be used as well. Intestinal transplantation is a therapeutic option if parenteral nutrition fails.

Schlüsselwörter

Kurzdarmsyndrom - Darmversagen - parenterale Ernährung - rekonstruktive Chirurgie - Darmtransplantation

Keywords

short bowel syndrome - intestinal failure - parenteral nutrition - reconstructive surgery - intestinal transplantation

^* DGEM Steering Committee: Bischoff SC, Lochs H, Weimann A sowie das DGEM-Präsidium

• Literatur

• 1 Stingel K, Schütz T, Koller M et al. Leitlinie der Deutschen Gesellschaft für Ernährungsmedizin (DGEM). Aktuel Ernahrungsmed 2013; 38: 90-96
• 2 Koller M, Schutz T, Valentini L et al. Outcome models in clinical studies: implications for designing and evaluating trials in clinical nutrition. Clin Nutr 2013; 32: 650-657
• 3 O'Keefe SJ, Buchman AL, Fishbein TM et al. Short bowel syndrome and intestinal failure: consensus definitions and overview. Clin Gastroenterol Hepatol 2006; 4: 6-10
• 4 Lal S, Teubner A, Shaffer JL. Review article: intestinal failure. Aliment Pharmacol Ther 2006; 24: 19-31
• 5 Sundaram A, Koutkia P, Apovian CM. Nutritional management of short bowel syndrome in adults. J Clin Gastroenterol 2002; 34: 207-220
• 6 Van Gossum A, Cabre E, Hebuterne X et al. ESPEN Guidelines on Parenteral Nutrition: gastroenterology. Clin Nutr 2009; 28: 415-427
• 7 Messing B, Crenn P, Beau P et al. Long-term survival and parenteral nutrition dependence in adult patients with the short bowel syndrome. Gastroenterology 1999; 117: 1043-1050
• 8 Wilmore DW, Lacey JM, Soultanakis RP et al. Factors predicting a successful outcome after pharmacologic bowel compensation. Ann Surg 1997; 226: 288-292
• 9 Shatari T, Clark MA, Lee JR et al. Reliability of radiographic measurement of small intestinal length. Colorectal Dis 2004; 6: 327-329
• 10 Nightingale JM, Bartram CI, Lennard-Jones JE. Length of residual small bowel after partial resection: correlation between radiographic and surgical measurements. Gastrointest Radiol 1991; 16: 305-306
• 11 Amiot A, Messing B, Corcos O et al. Determinants of home parenteral nutrition dependence and survival of 268 patients with non-malignant short bowel syndrome. Clin Nutr 2013; 32: 368-374
• 12 Crenn P, Coudray-Lucas C, Thuillier F et al. Postabsorptive plasma citrulline concentration is a marker of absorptive enterocyte mass and intestinal failure in humans. Gastroenterology 2000; 119: 1496-1505
• 13 Luo M, Fernandez-Estivariz C, Manatunga AK et al. Are plasma citrulline and glutamine biomarkers of intestinal absorptive function in patients with short bowel syndrome?. JPEN J Parenter Enteral Nutr 2007; 31: 1-7
• 14 Picot D, Garin L, Trivin F et al. Plasma citrulline is a marker of absorptive small bowel length in patients with transient enterostomy and acute intestinal failure. Clin Nutr 2010; 29: 235-242
• 15 Nightingale JM, Lennard-Jones JE, Gertner DJ et al. Colonic preservation reduces need for parenteral therapy, increases incidence of renal stones, but does not change high prevalence of gall stones in patients with a short bowel. Gut 1992; 33: 1493-1497
• 16 Nordgaard I, Hansen BS, Mortensen PB. Importance of colonic support for energy absorption as small-bowel failure proceeds. Am J Clin Nutr 1996; 64: 222-231
• 17 Atia A, Girard-Pipau F, Hebuterne X et al. Macronutrient absorption characteristics in humans with short bowel syndrome and jejunocolonic anastomosis: starch is the most important carbohydrate substrate, although pectin supplementation may modestly enhance short chain fatty acid production and fluid absorption. JPEN J Parenter Enteral Nutr 2011; 35: 229-240
• 18 Jeppesen PB, Mortensen PB. Colonic digestion and absorption of energy from carbohydrates and medium-chain fat in small bowel failure. JPEN J Parenter Enteral Nutr 1999; 23: S101-S105
• 19 Egger NG, Carlson GL, Shaffer JL. Nutritional status and assessment of patients on home parenteral nutrition: anthropometry, bioelectrical impedance, or clinical judgment?. Nutrition 1999; 15: 1-6
• 20 Messing B, Joly F. Guidelines for management of home parenteral support in adult chronic intestinal failure patients. Gastroenterology 2006; 130: S43-S51
• 21 Drozdowski L, Thomson AB. Intestinal mucosal adaptation. World J Gastroenterol 2006; 12: 4614-4627
• 22 Buchman AL. Use of percutaneous endoscopic gastrostomy or percutaneous endoscopic jejunostomy in short bowel syndrome. Gastrointest Endosc Clin N Am 2007; 17: 787-794
• 23 Nightingale JMD, Lennard-Jones JE, Walker ER et al. Jejunal efflux in short bowel syndrome. The Lancet 1990; 336: 765-768
• 24 Staun M, Pironi L, Bozzetti F et al. ESPEN Guidelines on Parenteral Nutrition: home parenteral nutrition (HPN) in adult patients. Clin Nutr 2009; 28: 467-479
• 25 Bischoff SC, Arends J, Dörje F et al. S3-Guideline of the German Society for Nutritional Medicine (DGEM) in Cooperation with the GESKES and the AKE: Nutritional support in the homecare and outpatient sector. Aktuel Ernahrungsmed 2013; 38: 323-344
• 26 Nightingale J, Woodward JM and on behalf of the Small Bowel and Nutrition Committee of the British Society of Gastroenterology. Guidelines for management of patients with a short bowel. Gut 2006; 55: iv1-12
• 27 Ladefoged K, Olgaard K. Sodium homeostasis after small-bowel resection. Scand J Gastroenterol 1985; 20: 361-369
• 28 Lauverjat M, Hadj AA, Vanhems P et al. Chronic dehydration may impair renal function in patients with chronic intestinal failure on long-term parenteral nutrition. Clin Nutr 2006; 25: 75-81
• 29 Vetter T, Lohse MJ. Magnesium and the parathyroid. Current Opinion in Nephrology and Hypertension 2002; 11: 403-410
• 30 Fleming CR, George L, Stoner GL et al. The importance of urinary magnesium values in patients with gut failure. Mayo Clin Proc 1996; 71: 21-24
• 31 Arasaradnam RP, Bolton RP. Hypomagnesaemia due to malabsorption is not always responsive to oral magnesium oxide supplementation alone. Gut 2002; 50: 897
• 32 Zeki S, Culkin A, Gabe SM et al. Refeeding hypophosphataemia is more common in enteral than parenteral feeding in adult in patients. Clin Nutr 2011; 30: 365-368
• 33 Kushner RF. Total parenteral nutrition-associated metabolic acidosis. JPEN J Parenter Enteral Nutr 1986; 10: 306-310
• 34 Tsai IC, Huang JW, Chu TS et al. Factors associated with metabolic acidosis in patients receiving parenteral nutrition. Nephrology (Carlton) 2007; 12: 3-7
• 35 Stewart PA. Modern quantitative acid-base chemistry. Can J Physiol Pharmacol 1983; 61: 1444-1461
• 36 Fencl V, Leith DE. Stewart’s quantitative acid-base chemistry: applications in biology and medicine. Respir Physiol 1993; 91: 1-16
• 37 Berkelhammer CH, Wood RJ, Sitrin MD. Acetate and hypercalciuria during total parenteral nutrition. Am J Clin Nutr 1988; 48: 1482-1489
• 38 Schloerb PR. Glucose in parenteral nutrition: a survey of U.S. medical centers. JPEN J Parenter Enteral Nutr 2004; 28: 447-452
• 39 Guenst JM, Nelson LD. Predictors of total parenteral nutrition-induced lipogenesis. Chest 1994; 105: 553-559
• 40 Wolfe RR, O'Donnell Jr TF, Stone MD et al. Investigation of factors determining the optimal glucose infusion rate in total parenteral nutrition. Metabolism 1980; 29: 892-900
• 41 Jeejeebhoy K. Zinc: an essential trace element for parenteral nutrition. Gastroenterology 2009; 137: S7-12
• 42 Wolman SL, Anderson GH, Marliss EB et al. Zinc in total parenteral nutrition: requirements and metabolic effects. Gastroenterology 1979; 76: 458-467
• 43 Atia AN, Buchman AL. Oral rehydration solutions in non-cholera diarrhea: a review. Am J Gastroenterol 2009; 104: 2596-2604
• 44 Matarese LE. Nutrition and fluid optimization for patients with short bowel syndrome. JPEN J Parenter Enteral Nutr 2013; 37: 161-170
• 45 Dreesen M, Foulon V, Spriet I et al. Epidemiology of catheter-related infections in adult patients receiving home parenteral nutrition: A systematic review. Clin Nutr 2013; 32: 16-26
• 46 Reimund JM, Arondel Y, Finck G et al. Catheter-related infection in patients on home parenteral nutrition: results of a prospective survey. Clin Nutr 2002; 21: 33-38
• 47 Shirotani N, Iino T, Numata K et al. Complications of central venous catheters in patients on home parenteral nutrition: an analysis of 68 patients over 16 years. Surg Today 2006; 36: 420-424
• 48 Pironi L, Paganelli F, Labate AM et al. Safety and efficacy of home parenteral nutrition for chronic intestinal failure: a 16-year experience at a single centre. Dig Liver Dis 2003; 35: 314-324
• 49 Bozzetti F, Mariani L, Bertinet DB et al. Central venous catheter complications in 447 patients on home parenteral nutrition: an analysis of over 100.000 catheter days. Clin Nutr 2002; 21: 475-485
• 50 Santarpia L, Alfonsi L, Tiseo D et al. Central venous catheter infections and antibiotic therapy during long-term home parenteral nutrition: an 11-year follow-up study. JPEN J Parenter Enteral Nutr 2010; 34: 254-262
• 51 Santarpia L, Pasanisi F, Alfonsi L et al. Prevention and treatment of implanted central venous catheter (CVC) – related sepsis: a report after six years of home parenteral nutrition (HPN). Clin Nutr 2002; 21: 207-211
• 52 Howard L, Ashley C. Management of complications in patients receiving home parenteral nutrition. Gastroenterology 2003; 124: 1651-1661
• 53 American Gastroenterological Association medical position statement: short bowel syndrome and intestinal transplantation. Gastroenterology 2003; 124: 1105-1110
• 54 Schaffler H, Daraban AM, Roggenbrod S et al. Characterization of refractory port-related blood stream infections in intestinal failure patients on parenteral nutrition. Z Gastroenterol 2011; 49: 335-339
• 55 Pironi L, Forbes A, Joly F et al. Survival of Patients Identified as Candidates for Intestinal Transplantation: A 3-Year Prospective Follow-Up. Gastroenterology 2008; 135: 61-71
• 56 Pittiruti M, Hamilton H, Biffi R et al. ESPEN Guidelines on Parenteral Nutrition: central venous catheters (access, care, diagnosis and therapy of complications). Clin Nutr 2009; 28: 365-377
• 57 Bisseling TM, Willems MC, Versleijen MW et al. Taurolidine lock is highly effective in preventing catheter-related bloodstream infections in patients on home parenteral nutrition: A heparin-controlled prospective trial. Clinical Nutrition 2010; 29: 464-468
• 58 Toure A, Lauverjat M, Peraldi C et al. Taurolidine lock solution in the secondary prevention of central venous catheter-associated bloodstream infection in home parenteral nutrition patients. Clin Nutr 2012; 31: 567-570
• 59 Hartl WH, Jauch KW, Parhofer K et al. Complications and monitoring – Guidelines on Parenteral Nutrition, Chapter 11. Ger Med Sci 2009; 7 Doc17
• 60 Jeppesen PB, Pertkiewicz M, Messing B et al. Teduglutide reduces need for parenteral support among patients with short bowel syndrome with intestinal failure. Gastroenterology 2012; 143: 1473-1481
• 61 Jeppesen PB, Gilroy R, Pertkiewicz M et al. Randomised placebo-controlled trial of teduglutide in reducing parenteral nutrition and/or intravenous fluid requirements in patients with short bowel syndrome. Gut 2011; 60: 902-914
• 62 Nightingale JM, Walker ER, Farthing MJ et al. Effect of omeprazole on intestinal output in the short bowel syndrome. Aliment Pharmacol Ther 1991; 5: 405-412
• 63 Jeppesen PB, Staun M, Tjellesen L et al. Effect of intravenous ranitidine and omeprazole on intestinal absorption of water, sodium, and macronutrients in patients with intestinal resection. Gut 1998; 43: 763-769
• 64 Ladefoged K, Christensen KC, Hegnhøj J et al. Effect of a long acting somatostatin analogue SMS 201-995 on jejunostomy effluents in patients with severe short bowel syndrome. Gut 1989; 30: 943-949
• 65 Cooper JC, Williams NS, King RFGJ et al. Effects of a long-acting somatostatin analogue in patients with severe ileostomy diarrhoea. Br J Surg 1986; 73: 128-131
• 66 Rosen GH. Somatostatin and its analogs in the short bowel syndrome. Nutr Clin Pract 1992; 7: 81-85
• 67 McDoniel K, Taylor B, Huey W et al. Use of clonidine to decrease intestinal fluid losses in patients with high-output short-bowel syndrome. JPEN J Parenter Enteral Nutr 2004; 28: 265-268
• 68 Buchman AL, Fryer J, Wallin A et al. Clonidine reduces diarrhea and sodium loss in patients with proximal jejunostomy: a controlled study. JPEN J Parenter Enteral Nutr 2006; 30: 487-491
• 69 Matarese LE, Steiger E. Dietary and medical management of short bowel syndrome in adult patients. J Clin Gastroenterol 2006; 40 (Suppl. 02) S85-S93
• 70 Layer P, Keller J, Lankisch PG. Pancreatic enzyme replacement therapy. Curr Gastroenterol Rep 2001; 3: 101-108
• 71 Kelly DA. Intestinal failure-associated liver disease: what do we know today?. Gastroenterology 2006; 130: S70-S77
• 72 Goulet O, Joly F, Corriol O et al. Some new insights in intestinal failure-associated liver disease. Current Opinion in Organ Transplantation 2009; 14: 256-261
• 73 Gura KM, Lee S, Valim C et al. Safety and Efficacy of a Fish-Oil-Based Fat Emulsion in the Treatment of Parenteral Nutrition-Associated Liver Disease. Pediatrics 2008; 121: e678-e686
• 74 Diamond IR, Sterescu A, Pencharz PB et al. Changing the paradigm: omegaven for the treatment of liver failure in pediatric short bowel syndrome. J Pediatr Gastroenterol Nutr 2009; 48: 209-215
• 75 Jurewitsch B, Gardiner G, Naccarato M et al. Omega-3-enriched lipid emulsion for liver salvage in parenteral nutrition-induced cholestasis in the adult patient. JPEN J Parenter Enteral Nutr 2011; 35: 386-390
• 76 Xu Z, Li Y, Wang J et al. Effect of omega-3 polyunsaturated fatty acids to reverse biopsy-proven parenteral nutrition-associated liver disease in adults. Clin Nutr 2012; 31: 217-223
• 77 Fishbein TM. Intestinal transplantation. N Engl J Med 2009; 361: 998-1008
• 78 Leitlinien der Deutschen Gesellschaft für Gefäßchirurgie – Akuter Intestinalarterienverschluss. 2008
• 79 Pironi L, Goulet O, Buchman A et al. Outcome on home parenteral nutrition for benign intestinal failure: A review of the literature and benchmarking with the European prospective survey of ESPEN. Clin Nutr 2012; 31: 831-845
• 80 Pironi L, Joly F, Forbes A et al. Long-term follow-up of patients on home parenteral nutrition in Europe: implications for intestinal transplantation. Gut 2011; 60: 17-25
• 81 Grant D, Abu-Elmagd K, Reyes J et al. 2003 report of the intestine transplant registry: a new era has dawned. Ann Surg 2005; 241: 607-613
• 82 Mazariegos GV, Steffick DE, Horslen S et al. Intestine transplantation in the United States, 1999–2008. Am J Transplant 2010; 10: 1020-1034
• 83 Lopushinsky SR, Fowler RA, Kulkarni GS et al. The optimal timing of intestinal transplantation for children with intestinal failure: a Markov analysis. Ann Surg 2007; 246: 1092-1099