View More View Less
  • 1 Jász-Nagykun-Szolnok Megyei Hetényi Géza Kórház és Rendelőintézet, Szolnok
  • 2 Szegedi Tudományegyetem, Általános Orvostudományi Kar, Szeged
  • 3 Észak-Közép-budai Centrum, Budapest
  • 4 Jósa András Kórház, Nyíregyháza
  • 5 Royal Manchester Children’s Hospital, Manchester, United Kingdom
Open access

Absztrakt:

A legújabb definíció szerint rövidbél-szindrómának nevezzük a bél jelentős hosszának elvesztése után kialakuló elégtelen bélműködés tünetegyüttesét, melyben a homeostasis és fejlődés-növekedés csak a hiányzó víz és elektrolit, illetve makrotápanyagok parenteralis pótlásával tartható fenn. A rövid bélben lezajló lassú természetes adaptációs folyamat a veszteséget csak bizonyos mértékben képes kompenzálni. Ennek megfelelően megkülönböztetünk (1) akut, (2) elhúzódó és (3) krónikus típust. A kórkép a leggyakrabban gyermekkorban jelentkezik nekrotizáló enterocolitis, malrotatio, volvulus, hasfalzáródási rendellenesség és ilealis atresia következményeként. A legnagyobb kihívást a krónikus típus ellátása okozza, bár a multidiszciplináris szemléletnek köszönhetően folyamatosan javul a betegek hosszú távú túlélése és életminősége, béltranszplantációra egyre ritkábban van szükség. A szerzők célja az intestinalis rehabilitáció legfontosabb szempontjainak – fokozott gasztrinszekréció, „high-output” stoma, csökkent tranzitidő, a centrális vénás kanülök ápolása, az enteralis, illetve parenteralis táplálás és az adaptáció serkentése – összegzése mellett a legújabb sebészi kezelési lehetőségek, köztük az autológ intestinalis rekonstrukció (AIRS) módszereinek, a passzázslassításnak (az ileocoecalis billentyű pótlása), a „bélhosszabbítás”-nak (LILT, STEP, SILT) és a felszívófelszínt növelő eljárásoknak (kontrollált bélexpanzió) az áttekintése volt. Ezeken túl a szerzők érintik a jelenlegi kutatások (disztrakciós enterogenezis, ’tissue engineering’) legújabb eredményeit. Orv Hetil. 2020; 161(7): 243–251.

If the inline PDF is not rendering correctly, you can download the PDF file here.

  • 1

    Lennard-Jones JE. Indications and need for long-term parenteral nutrition: implications for intestinal transplantation. Transplant Proc. 1990; 22: 2427–2429.

  • 2

    Cserni T, Polonkai E, Török O, et al. In utero incarceration of congenital diaphragmatic hernia. J Paediatr Surg. 2011; 46: 551–553.

  • 3

    Bianchi A, Morabito A. The dilated bowel: a liability and an asset. Semin Pediatr Surg. 2009; 18: 249–257.

  • 4

    Coletta R, Khalil BA, Morabito A. Short bowel syndrome in children: surgical and medical perspectives. Semin Pediatr Surg. 2014; 23: 291–297.

  • 5

    Nightingale J, Woodward JM, 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(Suppl 4): iv1– iv12.

  • 6

    O’Keefe SJ, Haymond MW, Bennet WM, et al. Long-acting somatostatin analogue therapy and protein metabolism in patients with jejunostomies. Gastroenterology 1994; 107: 379–388.

  • 7

    Joly F, Dray X, Corcos O, et al. Tube feeding improves intestinal absorption in short bowel syndrome patients. Gastroenterology 2009; 136: 824–831.

  • 8

    Matarese LE. Nutrition and fluid optimization for patients with short bowel syndrome. J Parenter Enteral Nutr. 2013; 37: 161–170.

  • 9

    Schäfer K, Zachariou Z, Löffler W, et al. Continuous extracorporeal stool-transport system: a new and economical procedure for transitory short-bowel syndrome in prematures and newborns. Pediatr Surg Int. 1997; 12: 73–75.

  • 10

    Schäfer K, Schledt A, Linderkamp O, et al. Decrease of cholestasis under “continuous extracorporeal stool transport (CEST)” in prematures and neonates with stomas. Eur J Pediatr Surg. 2000; 10: 224–227.

  • 11

    Pataki I, Szabó J, Varga P, et al. Recycling of bowel content: the importance of the right timing. J Pediatr Surg. 2013; 48: 579–584.

  • 12

    Rowley S, Clare S. ANTT: a standard approach to aseptic technique. Nurs Times 2011; 107: 12–14.

  • 13

    Zhong L, Wang HL, Xu B, et al. Normal saline versus heparin for patency of central venous catheters in adult patients – a systematic review and meta-analysis. Crit Care 2017; 21: 5.

  • 14

    Chong CY, Ong RY, Seah VX, et al. Taurolidine-citrate lock solution for the prevention of central line-associated bloodstream infection in paediatric haematology-oncology and gastrointestinal failure patients with high baseline central-line associated bloodstream infection rates. J Paediatr Child Health 2020; 56: 123–129. [Epub 2019 May 30]

  • 15

    Bueloni TN, Marchi D, Caetano C, et al. Cefazolin–gentamicin versus taurolidine–citrate for the prevention of infection in tunneled central catheters in hemodialysis patients: a quasi-experimental trial. Int J Infect Dis. 2019; 85: 16–21.

  • 16

    Zens T, Nichol P, Leys C, et al. Fractured pediatric central venous catheters – repair or replace? J Pediatr Surg. 2019; 54: 165–169.

  • 17

    Van Gossum A, Cabre E, Hébuterne X, et al. ESPEN guidelines on parenteral nutrition: gastroenterology. Clin Nutr. 2009; 28: 415–427.

  • 18

    Jeppesen PB, Hartmann B, Thulesen J, et al. Glucagon-like peptide 2 improves nutrient absorption and nutritional status in short-bowel patients with no colon. Gastroenterology 2001; 120: 806–815.

  • 19

    Yazbeck R. Teduglutide, a glucagon-like peptide-2 analog for the treatment of gastrointestinal diseases, including short bowel syndrome. Curr Opin Mol Ther. 2010; 12: 798–809.

  • 20

    Shamir R, Kolacek S, Koletzko S, et al. Oral insulin supplementation in paediatric short bowel disease: a pilot observational study. J Pediatr Gastroenterol Nutr. 2009; 49: 108–111.

  • 21

    Folaranmi S, Rakoczy G, Bruce J, et al. Ileocaecal valve: how important is it? Pediatr Surg Int. 2011; 27: 613–615.

  • 22

    McBride KD, Fisher R, Warnock N, et al. A comparative analysis of radiological and surgical placement of central venous catheters. Cardiovasc Intervent Radiol. 1997; 20: 17–22.

  • 23

    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.

  • 24

    Bianchi A. Intestinal loop lengthening – a technique for increasing small intestinal length. J Pediatr Surg. 1980; 15: 145–151.

  • 25

    Khalil BA, Ba’ath ME, Aziz A, et al. Intestinal rehabilitation and bowel reconstructive surgery: improved outcomes in children with short bowel syndrome. J Pediatr Gastroenterol Nutr. 2012; 54: 505–509.

  • 26

    Kőnig R. Overview of non-transplant surgical treatment management of short bowel syndrome in children. Paediatr Today 2015; 11: 85–92.

  • 27

    Hutcher NE, Mendez-Picon G, Salzberg AM. Prejejunal transposition of colon to prevent the development of short bowel syndrome in puppies with 90 per cent small intestine resection. J Pediatr Surg. 1973; 8: 771–777.

  • 28

    Glick PL, de Lorimier AA, Adzick NS. Colon interposition: an adjuvant operation for short-gut syndrome. J pediatr Surg. 1984; 19: 719–725.

  • 29

    Garcia VF, Templeton JM, Eichelberger MR, et al. Colon interposition for the short bowel syndrome. J Pediatr Surg. 1981; 16: 994–995.

  • 30

    Folaranmi SE. Clinical pathology of the ileocaecal junction in childhood and justification of its reconstruction as part of the autologous intestinal reconstructive surgery. PhD thesis. University of Debrecen, Doctoral School of Clinical Medicine, Debrecen, 2014. Available from: https://dea.lib.unideb.hu/dea/bitstream/handle/2437/181723/Folaranmi_Semiu_tezis_angol_impresszum-t.pdf?sequence=7&isAllowed=y [accessed: August 31, 2019].

  • 31

    Cserni T, Pap Szekeres J, Furka I, et al. Hydrostatic characteristics of the ileocolic valve and intussuscepted nipple valves: an animal model. J Invest Surg. 2005; 18: 185–191.

  • 32

    Littmann I, Berentey Gy. (eds.) Operative techniques. [Sebészeti műtéttan.] Medicina Könyvkiadó, Budapest, 1988. [Hungarian]

  • 33

    Cserni T, Paran S, Kanyári Z, et al. New insight into the neuromuscular anatomy of the ileocaecal valve. Anat Rec. 2009; 292: 254–261.

  • 34

    Jones BA, Hull MA, McGuire MM, et al. Autologous intestinal reconstruction surgery. Semin Pediatr Surg. 2010; 19: 59–67.

  • 35

    Thompson JS, Pinch LW, Murray N. Experience with intestinal lengthening for the short-bowel syndrome. J Pediatr Surg. 1991; 26: 721–724.

  • 36

    Hosie S, Loff S, Wirth H. Experience of 49 longitudinal intestinal lengthening procedures for short bowel syndrome. Eur J Pediatr Surg. 2006; 16: 171–175.

  • 37

    Jones BA, Hull MA, Potanos KM, et al. Report of 111 consecutive patients enrolled in the International Serial Transverse Enteroplasty (STEP) Data Registry: a retrospective observational study. J Am Coll Surg. 2013; 216: 438–446.

  • 38

    Kim HB, Fauza D, Garza J, et al. Serial transverse enteroplasty (STEP): a novel bowel lengthening procedure. J Pediatr Surg. 2003, 38: 425–429.

  • 39

    Walker SR, Nucci A, Yaworski JA, et al. The Bianchi procedure: a 20-year single institution experience. J Pediatr Surg. 2006; 41: 113–119.

  • 40

    Kang KH, Gutierrez IM, Zurakowski D, et al. Bowel re-dilation following serial transverse enteroplasty (STEP). Pediatr Surg Int. 2012, 28: 1189–1193.

  • 41

    Cserni T, Takayasu H, Muzsnai Z, et al. New idea of intestinal lengthening and tailoring. Pediatr Surg Int. 2011, 27: 1009–1013.

  • 42

    King B, Carlson G, Khalil BA, et al. Intestinal bowel lengthening in children with short bowel syndrome: systematic review of the Bianchi and STEP procedures. World J Surg. 2013; 37: 694–704.

  • 43

    Cserni T, Varga G, Erces D, et al. Spiral intestinal lengthening and tailoring – first in vivo study. J Pediatr Surg. 2013; 48: 1907–1913.

  • 44

    Cserni T, Biszku B, Guthy I, et al. The first clinical application of the spiral intestinal lengthening and tailoring (SILT) in extreme short bowel syndrome. J Gastrointest Surg. 2014; 18: 1852–1857.

  • 45

    Coletta R, Aldeiri B, Morabito A. Institutional experience with spiral intestinal lengthening and tailoring. Eur J Pediatr Surg. 2019; 29: 412–416.

  • 46

    Cervellione RM, Hajnal D, Varga G, et al. New alternative Mitrofanoff channel based on spiral intestinal lengthening and tailoring. J Pediatr Urol. 2015; 11: 131.e1–131.e5.

  • 47

    Mehrabi V, Mehrabi A, Jamshidi SH, et al. Modified spiral intestinal lengthening and tailoring for short bowel syndrome. Surg Innov. 2016; 23: 30–35.

  • 48

    Alberti D, Boroni G, Giannotti G, et al. “Spiral intestinal lenghtening and tailoring (SILT)” for a child with severely short bowel. Pediatr Surg Int. 2014; 30: 1169–1172.

  • 49

    Shekherdimian S, Panduranga MK, Carman GP, et al. The feasibility of using an endoluminal device for intestinal lengthening. Pediatr Surg. 2010; 45: 1575–1580.

  • 50

    Huynh N, Rouch JD, Scott A, et al. Spring-mediated distraction enterogenesis in-continuity. J Pediatr Surg. 2016; 51: 1983–1987.

  • 51

    Dubrovsky G, Huynh N, Thomas AL, et al. Double plication for spring-mediated in-continuity intestinal lengthening in a porcine model. Surgery 2019; 165: 389–392.

  • 52

    Bitar KN, Raghavan S. Intestinal tissue engineering: current concepts and future vision of regenerative medicine in the gut. Neurogastroenterol Motil. 2012; 24: 7–19.

  • 53

    Choi RS, Vacanti JP. Preliminary studies of tissue-engineered intestine using isolated epithelial organoid units on tubular synthetic biodegradable scaffolds. Transplant Proc. 1997; 29: 848–851.

  • 54

    Grikscheit TC, Siddique A, Ochoa ER, et al. Tissue-engineered small intestine improves recovery after massive small bowel resection. Ann Surg. 2004; 240: 748–754.

  • 55

    Nakase Y, Hagiwara A, Nakamura T, et al. Tissue engineering of small intestinal tissue using collagen sponge scaffolds seeded with smooth muscle cells. Tissue Eng. 2006; 12: 403–412.

  • 56

    Zakhem E, Raghavan S, Gilmont RR, et al. Chitosan-based scaffolds for the support of smooth muscle constructs in intestinal tissue engineering. Biomaterials 2012; 33: 4810–4817.

  • 57

    Pan WK, Zheng BJ, Gao Y, et al. Transplantation of neonatal gut neural crest progenitors reconstructs ganglionic function in benzalkonium chloride-treated homogenic rat colon. J Surg Res. 2011; 167: e221–e230.

  • 58

    Cserni T, Rákóczy Gy. Pediatric surgery in the neonatal period. In: Textbook of Perinatology. Ed. Papp Z., Budapest: Medicina Kiadó, 2nd edition, 2018. [Az újszülöttek fejlődési rendellenességeinek sebészi kezelése. In: A perinatológia kézikönyve. Szerk. Papp Z., Második kiadás. Budapest: Medicina Kiadó, 2018.] pp. 530-584. [Hungarian]