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Pediatric Enteral Nutrition in Short Bowel Syndrome 1 Definition of SBS BASED ON FUNCTION Malabsorptive state occurring as a result of the loss of a significant portion of the intestine Based on the need for intervention, such as need for TPN for longer than 1-3 months FUNCTION MORE IMPORTANT THAN LENGTH Amount of resection/remaining bowel resulting in this degree of malabsorption varies 2 Causes of Short Bowel Syndrome 436 patients from 13 series (1972-2000) Necrotizing enterocolitis 29 % Volvulus 27 % Atresia 23 % Gastroschisis 10 % Aganglionosis 4% Other 7% 3 Factors Contributing to Outcome Age at time of injury Amount and site of remaining bowel Function and motility of residual intestine Adaptation Other complicating factors cholestatic liver disease infections further injury to remaining bowel 4 Age at Time of Injury Intestine will grow as the infant grows Potential for growth is greatest in premature infant 19 to 27 weeks gestation: 115 + 21 cm 27 to 35 weeks gestation: 172 + 29 cm over 35 weeks gestation: 248 + 40 cm (length of normal jejunum and ileum at autopsy) 5 Touloukian. J Ped Surg 1983 Amount and Site of Remaining Bowel 6 Intrinsic factor, pepsin, HCl CCK, secretin iron CCK, secretin, GIP, VIP carbohydrates, protein, fats, vitamins, minerals, trace elements water, electrolyte, vitamin B12, bile salt, fat, fat soluble vitamins enterglucagon, GLP-2, peptide YY water, electrolytes, SCFA, oxalates Loss of Any Bowel Decreased surface area for absorption Shorter transit time Hypergastrinemia decreased pancreatic enzyme activity precipitation of bile acids damage to epithelium of proximal small bowel stimulates intestinal motility 8 Loss of Jejunum Initial marked decrease in nutrient absorption Generally better tolerated because of adaptive capacity of ileum Decreased CCK and secretin results in decreased pancreatic enzyme activity 9 Loss of Ileum Large fluid and electrolyte losses Sodium loss can contribute to poor growth Zinc depletion can worsen diarrhea Loss of ileal brake Malabsorption of bile acids impairing fat and fat soluble vitamin absorption Lack of absorption of Vitamin B12 10 Loss of Ileocecal Valve Promotes small bowel bacterial overgrowth 11 Loss of Colon Loss of colonic brake Loss of water and electrolyte resorptive capacity Loss of ability to salvage calories from malabsorbed carbohydrates 12 Function and Motility of Residual Intestine 13 Adaptation Hyperplasia increased surface area Increased crypt cell production Increased crypt depth Increased length of villi Increased number of transporters per cell Increase in enzyme activity 14 hyperplasia Increased surface area dilatation elongation 15 Adaptation Promoted by Luminal nutrients Endogenous secretions Humoral factors 16 Post-Operative Management Use PN to maintain hydration and adequate nutrition for growth and development Promote adaptation with the use of trophic feedings 17 Luminal Nutrients-Enteral Nutrition Fuel for enterocytes – stimulating hyperplasia Promote peristalsis – decreases overgrowth Stimulate flow of gastrointestinal secretions and secretion of humoral factors 18 “The main goal of treatment in SBS is intestinal adaptation while optimizing weight gain and linear growth while trying to maximize enteral nutrition while minimizing parenteral support.” “This is as much an art as it is a science” Quiros-Teijeira et al. J Pediatr 2004 19 Type of Feeding Breast milk Standard formula Protein hydrolysate formula Amino acid formula 20 Breast Milk Bolster immune system Contain growth factors Induce protective colonic flora Shorter duration of parenteral nutrition (Andorsky et al, 2001) 21 Standard Formula Increased permeability to intact proteins with mucosal injury SBS - dilated intestine, poor motility, bacterial overgrowth Allergic reactions to cow’s milk or soy protein is common Carbohydrate source (lactose) 22 Protein Hydrolysate Formula Lower antigenicity Contain MCT oil – does not require bile acids or micelles for absorption Lower peak bilirubin (Andorsky et al, 2001) 23 Amino Acid Based Formula Two infants weaned from TPN using a dilute elemental formula - Christie and Ament J Pediatr 1975 Four patients were able to wean from TPN after change to amino acid based formula - Bines et al JPGN 1998 Shorter duration of TPN -Andorsky et al 2001 24 EleCare® Protein (15% of Kcal) Amino acid-based medical food and infant formula Amino acid profile based on breast milk “Clinically” hypoallergenic (Sicherer et al, J Pediatr, 2001) 25 Al an in e Ar g As i pa nin e rti c ac id Cy G lut s tin am e ic ac G id ly c in e Hi st i Is d ine ole uc in Le e uc ine Ly M si n e e Ph th io e n ni n yl a e lan in e Pr ol ine Se r Th i ne re on Tr y p in e to ph Ty an ro s in e Va l in e per 70 Kcal Amino Acid Profile per 70 Kcal Breast Milk and EleCare EleCare HM 0.600 0.500 0.400 0.300 0.200 0.100 0.000 26 EleCare Fat (42% of Kcal) 33% of fat as MCT 30% MCT diet increased (MCT + LCT) absorption from: • • 23 to 58% preserved colon 46 to 58% no colon (Jeppesen and Mortense Gut, 1998) 27 EleCare Fat (42% of Kcal) Provides essential fatty acids • • Linoleic (LA) - 8% of total energy Linolenic (LNA) - 0.9% of total energy Dietary Reference Intakes (DRI’s) • LA Infants 0- 6 mos Infants 7 - 12 mos Children 1-18 yr • 8% of Kcal 6% of Kcal 5-10% of Kcal LNA Infants 0- 6 mos Infants 7 - 12 mos Children 1-18 yr 1% of Kcal 1% of Kcal 0.7-1.2% of Kcal 28 EleCare Carbohydrate (43% of Kcal) Corn syrup solids Osmolality at 20 Kcal/oz = 350 mOsm/kg water Used in chronic diarrhea due to SBS (Saavedra, et al, 2000) 29 Route of Delivery/Advancing Continuous drip continuous saturation of transporters Advance (slowly) as long as output < 25-50 ml/kg/day perianal area intact 30 Other Strategies to Improve Feeding Tolerance Addition of soluble fibers to the feeding Acid blockade Zinc Sodium chloride Loperamide 31 Comparisons of Amino Acid-Based Products Product Age Indication Fat Blend Osmolality (mOsm/kg water) Infants and Children 42% of total cal – 33% MCT 350 at 20 cal/fl oz 560 at 30 cal/fl oz Infant Formula One+ Junior (unflavored) EO28 (RTF) Infants Children > 1 yr Children > 1 yr Children > 1 yr 41% of total cal - 5% MCT 32% of total cal - 35% MCT 46% of total cal - 35% MCT 32% of total cal - 35% MCT 375 at 20cal/fl oz 610 at 30cal/fl oz 607 at 30cal/fl oz 820 at 30cal/fl oz Vivonex® Pediatric Children > 1 yr 25% of total cal - 60% MCT 360 at 24 cal/ fl oz EleCare® Neocate® 32 Product Cost per 100 cal Cost savings per month compared to EleCare EleCare® $1.60 NA Neocate® Infant Formula $1.90 4 month old- $67 Neocate® 1+ $2.15 15 month old- $214 3 year old- $280 Neocate® Jr. $1.82 15 month old- $86 3 year old- $112 Pediatric EO28® $1.67 15 month old- $27 3 year old- $36 Vivonex® Pediatric $1.80 15 month old- $78 3 year old- $102 33 Summary Enteral nutrition is the key to bowel adaptation Breast milk and amino acid formula are associated with shorter duration of PN EleCare is the only infant amino acid formula with MCT oil. 34