Nutritional Management Of Pediatric Short Bowel Syndrome

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NUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #12Series Editor: Carol Rees Parrish, M.S., R.D., CNSDNutritional Management ofPediatric Short Bowel SyndromeAna Abad-SindenJames SutphenPediatric short bowel syndrome (SBS), usually caused by massive intestinal resection,presents a significant nutritional challenge to the pediatric clinician. The overall clinical course and nutritional outcomes of SBS are impacted by various factors including remaining intestinal length and site, functional differences between the proximaland distal small intestine, and the presence of the colon. Nutritional management ofSBS can be variable from patient to patient and can be divided into three stages: parenteral nutrition, enteral nutrition, and introduction of solid foods. Optimization ofparenteral nutrition with a balanced fuel mix of carbohydrate, protein and fat shouldbe provided to meet energy needs and promote growth during the first few weeks ofnutritional management. Following fluid and electrolyte stability and demonstratedgrowth on parenteral nutrition, enteral nutrition with semi-elemental or elementalformulas should be initiated in a timely manner to promote intestinal adaptation.Because electrolyte and fat-soluble vitamin loss can impair optimal growth, infantsand children with SBS often require supplementation with sodium and pediatric multivitamins and also benefit from supplementation with fiber and glutamine. Introduction of age appropriate low simple carbohydrate, high protein foods using smallfrequent feedings during the day further promote intestinal adaptation as well asoral-motor skills. The following article will present a comprehensive overview ofnutritional management in the pediatric patient with SBS with practical guidelinesfor the clinician.(continued on page 30)Ana Abad-Sinden, MS, RD, CNSD, Director, Dietetic Internship Program, Pediatric Nutrition Support Specialistand James Sutphen, M.D., Assistant Professor, UVA Health System, Department of Nutrition Services,Charlottesville, VA.28PRACTICAL GASTROENTEROLOGY DECEMBER 2003

Nutritional Management of Pediatric Short Bowel SyndromeNUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #12(continued from page 28)INTRODUCTION AND ETIOLOGYPATHOPHYSIOLOGYediatric short bowel syndrome (SBS), a disordercharacterized by diarrhea, malabsorption, fluidand electrolyte disturbances, and eventually malnutrition, is usually caused by massive intestinal resection (1–4). However, infants and children may exhibitmalabsorption as well as the infectious and metaboliccomplications associated with SBS, even with minimalor relatively modest resections of the small bowel (2).As such, the overall functional prognosis and complications associated with SBS do not always strictly correlate with the length of the remaining small intestine.Additional factors such as the presence of the ileocecalvalve, the presence or amount of remaining colon, andthe physiological status of the remaining intestine significantly impact the overall management and functional status of the child with SBS (3,4).Short bowel syndrome in neonates may be presentat birth due to a congenital anomaly or may develop inolder infants and children as a result of disease ortrauma, occurring later in life. Infants may be bornwith intestinal atresias, which may occur along anyportion of the small intestine within an isolated area oralong multiple segments. The obstruction caused bythe atretic portions of the gut can lead to gastrointestinal (GI) ischemia, necrosis and resection (3,5). Gastroschisis, which results from an abdominal walldefect, can result in significant bowel injury orischemia, necessitating bowel resection (3). Infantswho are born with normal GI anatomy may experienceintestinal injury and resection as a result of necrotizingenterocolitis (NEC) (2), which usually occurs in thepreterm infant. While the exact cause is unknown, thepremature GI tract is more susceptible to intestinalhypoxic injury and ischemia (5,6). Additional factorssuch as the presence of pathogenic bacteria, aggressivefeeding advancement with hyperosmolar formulations,and intrauterine cocaine exposure may also increasethe risk of NEC (6). In older infants and children withnormal GI anatomy, malrotation can lead to volvulus,resulting in occlusion of the mesenteric artery andischemia (3,5). Additional conditions such as herniasand intussusception can lead to intestinal hypoxicinjury and resection. Crohn's disease, abdominaltumors and radiation enteritis resulting from radiationtherapy are also common causes of intestinal resectionand SBS in older children and adults (3).A basic review of normal neonatal GI anatomy andphysiology is essential to an understanding of theimpact of intestinal resection on the clinical outcomeand management of pediatric SBS. Both remainingintestinal length and functional differences between theproximal and distal small intestine have a major impacton the overall clinical course and nutritional management of SBS. In the term neonate, the small bowel isestimated to be 240 cm, while the colon is about 40 cm(7). The length of the jejunum, ileum and colon doublesin the last trimester of pregnancy, thus giving a preterminfant a better outcome potential in terms of gut growth(2). Good clinical outcome, which can be defined asbeing able to eventually meet nutrition and growthneeds on enteral nutrition (EN), can be achieved withas little as 15 cm of jejunum and ileum with the presence of the ileocecal valve, but 40 cm without the presence of the valve (8). These outcomes, however, presume normal functioning of the remaining bowel.The degree and extent of malabsorption and metabolic complications seen in SBS also depends on thesite of intestinal resection. The jejunum, which haslonger villi and a large absorptive surface area, has ahigh concentration of digestive enzymes as well asenzyme carrier proteins and is the site of greatest nutrient absorption. The duodenum and jejunum are the primary sites for carbohydrate, protein, fat, as well asmineral absorption. Although the ileum has shortervilli and less overall absorptive capacity than thejejunum, it is the only site for B12 and bile salt absorption through site specific receptor-mediated transport.While the ileum can adapt and compensate for jejunalloss, the duodenum and jejunum cannot take on theabsorptive functions of the ileum. Ileal resectionresults in decreased enterohepatic circulation and vitamin B12 and bile salt malabsorption, impairing normalmicelle formation and efficient fat and fat-soluble vitamin absorption. Ileal resection can also lead to anincreased risk of renal oxalate stones, bilious diarrheaand an increased potential for cholelithiasis. Additionally, ileal resection impairs the normal regulation ofgut motility, as the ileum is a site of GI hormone production, particularly those that affect small bowelmotility such as enteroglucagon and peptide YY (4).P30PRACTICAL GASTROENTEROLOGY DECEMBER 2003(continued on page 32)

Nutritional Management of Pediatric Short Bowel SyndromeNUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #12(continued from page 30)Overall, gut adaptation and functional prognosis ismore of a challenge following ileal resection comparedto jejunal resection.Resection of the ileocecal valve and length of theremaining colon also impact the course of nutritional management and overall clinical outcomes in pediatric SBS.As the ileocecal valve slows the emptying of chyme fromthe small intestine into the colon, its resection increasesthe emptying rate of small intestinal contents (5). Fluidand electrolyte loss are also increased due to rapid transittime. Bacteria can also migrate upward to the small intestine, resulting in small bowel bacterial overgrowth. Bacterial colonization of the small bowel leads to deconjugationof the remaining bile acids, altering micelle formation, andexacerbating steatorrhea (9). Furthermore, bacterial overgrowth can also result in D-lactic acid production, whichcannot be metabolized by humans; consequently, D-lacticacidosis with neurological symptoms such as ataxia,dysarthria and confusion can result (10). When steatorrhearesults from SBS, fluid and electrolyte homeostasis can bebetter maintained if the colon has been preserved. Energybalance can also be improved through colonic preservation as short chain fatty acids, a by-product of bacterialcarbohydrate fermentation in the colon, can provide up toan additional 500 kcal/day (11).NUTRITIONAL MANAGEMENT INSHORT BOWEL SYNDROMENutritional management of SBS, which can be variablefrom patient to patient depending on a number of physiological and clinical factors, can generally be dividedinto three phases or stages (4, 5). Advancement fromone stage to another is individualized depending on clinical course, and for pediatric patients, on the efficiencyand quality of their growth. Stage 1, which generallylasts from one to three weeks, involves initial hydrationand electrolyte management to achieve hemodynamicstability, and initiation of parenteral nutrition (PN) usingthe central venous route for optimal nutritional support.Stage 2, which is characterized by a reduction in diarrhea and improved intestinal adaptation, involves thejudicious initiation of EN using a continuous feedingschedule, while gradually weaning PN delivery toachieve caloric and protein requirements. Successful PNweaning is predicated by EN tolerance, ostomy output32PRACTICAL GASTROENTEROLOGY DECEMBER 2003and growth outcomes. Stage 3 of nutritional management is characterized as a period of complete boweladaptation when EN is well tolerated and oral feedingscan be initiated. The time required to arrive at stage 3can take a few months to greater than a year, dependingon the child's clinical course, extent of metabolic complications and growth outcomes.Stage 1: Parenteral NutritionThe initial mode of nutrition support for pediatric SBS isPN using the central venous route in order to meet caloricand nutrient requirements. Generally, a balanced fuel mixcontaining carbohydrate, protein and fat should be provided to meet caloric requirements and promote growth.In infants, dextrose should be started at 5–7 mg of glucose/kg/min and advanced by 1–3 mg of glucose/kg/minto an endpoint goal of 12–14 mg/kg/min of glucose (2).This stepwise advancement promotes the gradualresponse of endogenous insulin, thus preventing hyperglycemia and glycosuria (2–4). Furthermore, provision ofadequate, but not excessive carbohydrate infusion, canhelp prevent immune dysfunction, hepatic steatosis, andexcess CO2 production and retention (4). Parenteral lipidscan be initiated at 1 gm/kg/day and advanced by 1 gm/kgper day to an endpoint goal of 3 gm/kg in infants and 1–2gm/kg/day in children. Generally, lipid provision shouldnot exceed 30%–40% of total calories to minimize thepotential for immune dysfunction and hyperlipidemia,particularly in small for gestational age or stressedneonates (13). Optimal fatty acid oxidation requires carnitine, which may become a conditionally essential aminoacid in preterm and infants with SBS (14). Parenteralamino acids can be started liberally at 1.5–2 gm/kg/dayand advanced to goal by day two or three of PN. Even inneonates, recent studies have clearly demonstrated thatprogressive protein initiation and advancement promotesimproved nitrogen retention and is well tolerated (15, 16).Infants less than two years of age should be managed witha pediatric amino acid formulation such as TrophAmine(B.Braun, Irvine, CA, website: www.bbraunusa.com).Pediatric amino acid solutions provide numerous advantages in the management of infants with SBS including areduction in the concentration of hepatotoxic amino acidswhile providing essential amino acids including taurine,tyrosine and histidine (17,18). The addition of 30–40 mg

Nutritional Management of Pediatric Short Bowel SyndromeNUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #12of cysteine HCl per gram of amino acids, recommendedfor use with pediatric amino acid formulations, decreasesthe pH of the PN solution thus increasing calcium andphosphorus solubility. In addition, supplementation ofcysteine, an essential amino acid in neonates, in PN solutions serves as a precursor to taurine production, leading toplasma concentrations in the neonatal target range (19).Provision of adequate electrolyte, vitamin and mineral levels is essential for optimal parenteral nutritionsupport of the infant or child with SBS. Calcium andphosphorus delivery should be optimized as patientswith SBS, particularly infants, have a higher requirement. Pediatric multivitamin solutions, which includevitamin K, and trace element solutions including copper, zinc, chromium, manganese, and selenium shouldbe provided daily. As patients with SBS often haveexcessive zinc losses through ostomy output and diarrhea, an additional 10 mcg/mL of output is frequentlyrecommended (2,3). In infants and children withcholestasis, however, copper and manganese should bereduced as these trace elements are excreted throughthe biliary route. Either decreasing the pediatric traceelements to 0.5 mL/day or holding them from the PNsolution while adding the chromium and zinc as separate additives may accomplish this. Excessive fluidlosses from diarrhea and ostomy output can also resultin fluid and sodium deficits. Fluid and electrolytes maybe repleted over and above what is provided in the PNusing a separate intravenous solution. Often, sodiummay need to be provided up to 8–10 mEq/kg/daydepending on the degree of ostomy output and diarrhea.The duration of, and continued reliance on, PNtherapy in the management of SBS depends on a number of factors including physiological factors such aslength and site of remaining bowel, presence of bacterial overgrowth, degree of hepatobiliary disease, ability to hydrate, tolerance to EN and quality of growth.Stage 2: Enteral NutritionOnce the patient is stable from a fluid and electrolyte perspective and growing on PN therapy, EN can be initiatedto help promote intestinal adaptation and growth. Intestinal adaptation refers to the processes of cellular hyperplasia, villous hypertrophy, intestinal lengthening, andenhanced hormonal response, which result in increasedabsorptive surface area (5). Intestinal adaptation occurssooner with earlier initiation of EN. The ileum has greaterpotential for adaptation than does the duodenum andjejunum (20). While the process of intestinal adaptationcan begin as soon as 24 to 48 hours post-resection, theprocess can take over one year to occur depending on thecourse of EN therapy and clinical response based onnumerous physiological and metabolic factors (2,3,5).Enteral nutrition is best administered using continuous infusion, gradually advancing the rate based on anumber of outcome indicators (4, 5). In general, continuous feedings are better tolerated and allow for better totalnutrient absorption. Emesis, increased abdominal distention, and significant increases in ostomy output are alsominimized with gradual advancements in the continuousfeeding rate. As enteral calories are advanced, therebyincreasing the intestinal load, additional adaptation is promoted which may reduce the duration of reliance on PN.However, a significant increase in stool output by morethan 50 percent, or output significantly positive in reducing substances, is a contraindication to feeding advancement. Eventually, as continuous EN is tolerated, the PNdelivery can be transitioned to a cyclic schedule, graduallyincreasing the time off of PN by 2 to 4 hour incrementsuntil PN is provided over an 8 to 12 hour nocturnal infusion schedule.Controversy exists regarding the optimal enteralnutrition formula for use in infants and children with SBS.Historically, elemental, semi-elemental or peptide basedformulas have been considered to be the products ofchoice for use in patients with SBS. In adult patients comparing these products to polymeric formulas have demonstrated similar caloric absorption with all products (21).Complex nutrients do appear to stimulate intestinal adaptation better than simple nutrients such as amino acids,peptides and monosaccharides, and is thought to beexplained by the "functional workload" hypothesis, whichessentially states that the greater the bowel must work todigest a nutrient, the greater the inducement to adapt (1).While in adults complex proteins are thought to promotebetter intestinal adaptation than amino acids, in infants andchildren, protein hydrolysate or even amino acid containing formulas are still predominantly used for a number oftheoretical reasons. Allergic response is more prevalent ininfants and children who have experienced a disruption oftheir mucosal barrier. Frequently, infants develop allergicPRACTICAL GASTROENTEROLOGY DECEMBER 200333

Nutritional Management of Pediatric Short Bowel SyndromeNUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #12problems during the course of their management of SBS,particularly if exposed to macromolecules. Bacterial overgrowth, which is common in infants and children withSBS, also predisposes them to GI allergies (1). Enteralfats, particularly long chain fatty acids, have the greatestpotential for exerting trophic effects in the promotion ofintestinal adaptation (22,23). While medium chain triglycerides are more water soluble and have improved absorption in children with SBS, they also have a higher osmoticeffect and less of a trophic effect on the gut (22). The provision of excessive levels of simple carbohydrates canhave significant osmotic effects, exacerbating diarrhea.Optimally, no more than 40% of the total calories provided should be from carbohydrates, particularly in infantswith SBS. In summary, the current standard of practice isto use either a protein hydrolysate formula, or on rareoccasion for infants and children with persistent intolerance, an amino acid based formula with a high percentageof fat, mostly in the form of long chain fatty acids, as theoptimal enteral feeding formula for infants and childrenwith SBS.Despite these established practice guidelines forenteral management of pediatric SBS, there is a lack ofrandomized clinical trials to support the practice ofexclusively using semi-elemental or amino acid basedformulas. A recent prospective, randomized, crossover,double-blind study in pediatric patients compared theeffects of two formulas, differing only in their nitrogensource, on infants with SBS (24), a hydrolyzed and nonhydrolyzed protein source. The study concluded thatintestinal permeability, weight gain and energy andnitrogen balance in the SBS patients did not differbetween the formulas regardless of hydrolysis of enteralnitrogen source. Depending on the extent and site ofintestinal resection and function of the remaining gut,some infants and children may be effectively managedwith standard polymeric infant and pediatric formulas.BREAST MILK AND ENTERAL FORMULASAlthough at first glance, breast milk (BM) may not beconsidered the enteral feeding of choice for infants withSBS as the nutrients are intact and not hydrolyzed, the literature indicates that the use of BM results in improvedGI tolerance and reduced duration of PN in comparisonto protein hydrolysate formulas (4,25). Andorsky, et al34PRACTICAL GASTROENTEROLOGY DECEMBER 2003(25) concluded that several components of BM mighthave been responsible for these observations. Breast milkcontains immunoglobulins as well as growth factors suchas growth hormone and epidermal growth factor, whichmay result in improved intestinal adaptation.Formulas used for the nutritional management ofinfants and children with SBS as well as formula costand manufacturer are summarized in Table 1. Becauseof the semi-elemental nature of the protein and fatsources, the formulas, Pregestimil (Mead Johnson,Evansville, IN) and Alimentum (Abbott Laboratories,Ross Labs Division, Columbus, OH) are commonlyused in the nutritional management of infants withSBS. Pregestimil and Alimentum contain caseinhydrolysate with added amino acids as the proteinsource, and a blend of vegetable oils and MCT oil asthe fat source. The carbohydrate in Pregestimil is provided by corn syrup solids (85 percent) and modifiedtapioca starch (15 percent), while Alimentum containsa blend of tapioca starch and sucrose. Alimentum isalso corn free, which may be valuable in infants withmultiple food allergies. At a concentration of 20 kcalper ounce, Pregestimil and Alimentum have osmolalities of 320 mOsm and 370 mOsm per kilogram,respectively, and cost more than twice that of standardinfant formula. Both Pregestimil and Alimentum areavailable in powdered and ready-to-feed forms. Additionally, Pregestimil also makes a ready-to-feed 24kcal per ounce formula for use in the hospital setting.Infants with SBS who demonstrate persistent GIsymptoms when managed with a casein hydrolysateformula may benefit from use of a purely elementalformula (26). NeoCate (SHS North America), which isavailable in powdered form, is comprised of 100% freeamino acids, is lactose free, and has a blend of 5%MCT oil and 95% long chain fats from vegetable oil.Pediatric patients over the age of one with SBS,who experience continued impaired GI function, orsevere allergic complications may require EN supportwith a semi-elemental or elemental pediatric formula.Over the past 10 years, a variety of semi-elemental andelemental products have entered the medical nutritionals market. Semi-elemental pediatric formulas includePeptamen Junior (Nestle Clinical Nutrition) and PepditeOne (SHS North America). Peptamen Junior contains(continued on page 41)

Nutritional Management of Pediatric Short Bowel SyndromeNUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #12(continued from page 34)Table 1.Pediatric Elemental or Semi-Elemental Formulas for Management of Short Bowel Syndrome*ProductKcal/ccDescriptionInfant Formulas ( 1 year old)Pregestimil0.67Semi-elemental: Peptides & amino acidsReady-to-feed and powdered formAlimentum0.67Semi-elemental: Peptides & amino acidsReady-to-feed and powdered formNeocate0.67Elemental: free amino acidsPowdered formPediatric Formulas (1 year old)Peptamen Junior 1.0Semi-elemental: 100% whey based peptidesReady-to-feedPepdite One 1.0Semi-elemental: 56% soy hydrolysatepeptides & 44% free amino acidsPowdered formNeocate One 1.0Elemental: 100% free amino acidsPowdered formEO281.0Elemental: 100% free amino acidsReady to feed, flavored, available in tetrapacksElecare1.0Elemental: 100% free amino acidsPowdered formCost per 100 Kcal **Manufacturer*** 1.21 (powdered)Mead Johnson 1.33 (ready-to-feed) 1.21 (powdered) 1.71Ross LabsSHS North America 2.13 (unflavored) 2.42 (flavored) 2.19Nestle 1.90SHS North America 1.50SHS North America 2.65Ross LabsSHS North America*Used with permission from the University of Virginia Health System Nutrition Support Traineeship Syllabus (43)**Cost based on pricing for October 2003. Price is highly variable among stores, however.Manufacturer***Mead-JohnsonNestleRoss LaboratorySHS North AmericaPhone 7354100% whey based peptides, while Pepdite One contains 56% low molecular weight peptides from pork andsoy hydrolysates and 44% free amino acids. PeptamenJunior provides 33% of the calories from fat with a60:40 ratio of MCT to long chain triglycerides and anessential fatty acid content of 5% of total calories.Pepdite provides 46% of the calories from fat while providing a 35:65 ratio of MCT to long chain triglycerides.Both formulas are lactose free and sucrose free withcorn syrup solids and/or maltodextrins comprising thecarbohydrate source. These formulas may be indicatedfor pediatric patients with SBS who experience persistent intolerance to intact polymeric formulas. calnutrition.comwww.ross.comwww.shsna.compediatric formulas such as Neocate One (SHS NorthAmerica, Gaithersburg, MD), EleCare (Ross Division,Abbott Laboratories) and Vivonex Pediatric (NovartisNutrition) are indicated for the child with SBS withsevere protein allergy and unable to tolerate thehydrolysate formulas. While their indicated use may berare, they are useful for the nutritional support of thechild with persistent malabsorption and malnutrition.Neocate One and Pediatric EO28 (SHS North America), which are both 1 kcal per mL, contain 100% freeamino acids and a 65:35 ratio of long chain triglyceridesto MCT, providing 32% of the calories from fat. Theyare also lactose and fructose free with a blend of malPRACTICAL GASTROENTEROLOGY DECEMBER 200341

Nutritional Management of Pediatric Short Bowel SyndromeNUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #12Table 2.Pediatric Liquid Multivitamin Supplements*Product and DistributorDoseA(IU)DEB1B2B3B5B6B12C Folate Fluo Other(IU) (IU) (mg) (mg) (mg) (mg) (mg) (mcg) (mg) (mg) (mg) ContentVi-Daylin Multivitamin Liquid (Ross) 1 ml 15004005.50.50.6800.41.535———Poly-vi-sol Multivitamin Liquid(Mead Johnson)1 ml 15004005.20.50.6800.4235———Poly-vi-Flor Multivitamin Liquid(Mead Johnson)1 ml 150040050.50.6800.4235—0.25—ADEK Pediatric Drops(Scandipharm)www.axcanscandipharm.com1 ml 1500400400.50.6630.6445Baby Vitamin Drops (Goldline)1 ml 15004005.20.50.6800.41.535Multivitamins for Older ChildrenVi-Daylin Multivitamin Liquid (Ross) 5 ml 25001.05 1.24001513.501.054.560Thera Multivitamin Liquid (Major)5 ml 10000 4000101010021.44.15200ADEKs tablets (Scandipharm)1 tab. 4,000 4001501.21.310101.51260—0.1 mg K,15 mcg—biotin,5 mg zinc————0.2——*Used with permission from the University of Virginia Health System Nutrition Support Traineeship Syllabus (43)Note: Brand names are representative of products purchased by the University of Virginia.todextrin and sucrose as the carbohydrate source. Neocate One is available in powdered form for use in tubefeedings, while EO28 is flavored and packaged in tetrapacks for oral intake. EleCare, available in powderedform, also provides 100% free amino acids and containsa caloric distribution of 15% protein, 43% carbohydrateas corn syrup solids, and 42% fat in a 67:33 blend oflong chain triglycerides and MCT.ELECTROLYTE, VITAMIN ANDMINERAL SUPPLEMENTATIONExcess sodium losses from excessive ostomy output ordiarrhea may result in impaired growth despite adequate caloric intake and often requires sodium supplementation in the enteral formula. Infants may requireup to 4–8 mEq/kg/day to achieve adequate growth(2–4). Infants who experience metabolic acidosis as acomplication of bacterial overgrowth may also requiresupplementation with sodium bicarbonate.Due to the loss of absorptive surface area andsteatorrhea, children with SBS usually have impaired42PRACTICAL GASTROENTEROLOGY DECEMBER 2003vitamin and mineral status. Fat-soluble vitamins A, D,and E should be supplemented daily using a pediatricmultivitamin preparation (refer to Table 2). As intestinal bacteria primarily synthesize vitamin K, its deficiency is rare unless a child is on long term broad spectrum antibiotic therapy. Water-soluble forms of the fatsoluble vitamins are recommended for optimal absorption. Due to the potential toxicity of the fat-soluble vitamins, annual monitoring of levels is recommendedfor children with SBS. In children with ileal resection,monthly B12 injections are eventually needed to maintain optimal nutrient status.SBS MANAGEMENT WITH FIBER AND GLUTAMINEFiber is now being successfully used in older infants andchildren with an intact colon as a valuable adjunct in themanagement of pediatric SBS. Water-soluble fibers,such as soy polysaccharide and pectin, can helplengthen transit time and thus may help increase absorption by enhancing nutrient contact time with the intesti(continued on page 44)

Nutritional Management of Pediatric Short Bowel SyndromeNUTRITION ISSUES IN GASTROENTEROLOGY, SERIES #12(continued from page 42)nal mucosa (2). An additional benefit of fiber forpatients with a preserved colon is that the undigestedfiber can be metabolized to short chain fatty acids bycolonic bacteria. The short chain fatty acids induce atrophic effect on the colon and are also a primary energysource for colonocytes. Hawkins, et al (27) demonstrated a reduction in stool reducing substances, a reduction in fecal fat loss from 21% to 10% and a resolutionin metabolic acidosis within two weeks of adding1%–3% pectin to the semi-elemental formulas of formerpreterm infants following bowel resection. Liquidpectin may be added to formula using Certo LiquidPectin (Kraft Foods, website: www.kraft.com). Feedings were also advanced more rapidly and both infantswere able to maintain appropriate growth rates. Potential risks of exceedingly high fiber intake (greater than0.5 gm/kg or upper limit of 35 gm) in children, however,include malabsorption of zinc, iron and magnesium.Glutamine, the most abundant amino acid in thebody, is considered to be an important fuel source forrapidly dividing cells such as the cells of the intestinalmucosa. While glutamine is not considered an essential amino acid, it may become conditionally essentialin catabolic illness and in prematurity. Neu, et al (28)found that 24–32 week preterm infants fed pretermformula supplemented with glutamine tolerated enteralfeedings better than unsupplemented infants. Additionally, as glutamine is absent from commercial PNformulations, it is believed that parenteral supplementation with glutamine may help intestinal adaptation ininfants with intestinal immaturity or with short bowelsyndrome (2). While diet modification with glutamineand growth hormone has been demonstrated to havepositive effects on promoting intestinal adaptation inadults (29), this remains controversial. Similar studieshave not been conducted in infants or children.Stage 3: Introduction of Solid FoodsIn infants with short bowel syndrome, solid foodsshould generally be introduced fairly early in their therapy, usually between 4 to 6 months of age or when thechild is developmental

Pediatric short bowel syndrome (SBS), usually caused by massive intestinal resection, presents a significant nutritional challenge to the pediatric clinician. The overall clin-ical course and nutritional outcomes of SBS are impacted by various factors includ-ing remaining intestinal len

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