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Harizah mohd yaacob short bowel syndrome updates

Harizah mohd yaacob short bowel syndrome updates






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    Harizah mohd yaacob short bowel syndrome updates Harizah mohd yaacob short bowel syndrome updates Presentation Transcript

    • CASE BACKGROUND Patient H, born at 33 weeks gestation Birth weight – 1053 gram Developed necrotizing enterocolitis (NEC) that required a small-bowel resection  45 cm of residual small bowel and  an intact ileocecal valve Post-op – was maintained with  parenteral nutrition  slow advancement of enteral nutrition, Patient developed Parenteral Nutrition Associated Liver Disesase (PNALD)
    • NEC Risk factors :  Extreme prematurity  Birth weight < 2.0 kg  Formula feeding  History of asphyxia / respiratory distress / exchange transfusion / congenital cardiac disease
    • SMALL BOWEL LENGTH VS PNSeminars in Fetal & Neonatal Medicine 16 (2011) 157-163
    • CITRULLINE Assessment tool for neonatal SBS Citrulline → non-structural amino acid that is primarily synthesized in the intestinal mucosa and reflects mucosal mass, act as a biomarker of intestinal functions Serum citrulline is highly positively correlated with intestinal length and the ability to wean from PN Intestinal failure patients with a serum citrulline level persistently < 12 mmol/L are usually unable to wean from PN
    • CASE PROGRESSION Attempt was made to improve hepatic function by using standard techniques 1. maximizing enteral feedings 2. reducing the amount of soy lipid emulsion (SLE), cycling PN 3. treating bacterial overgrowth 4. SLE was eliminated when no significant improvement in hepatic function was observed
    • PARENTERAL NUTRITION Hepatoprotective PN regimens Soy lipid-based PN formulas administered at < 0.5 g/kg/day have been shown to be effective in delaying or preventing cholestasis Fish oil PN formulas at 1 g/kg/day has been associated with the reversal of hyperbilirubinemia and few apparent side-effects in neonates with intestinal failure Puder M, Valim C, Meisel JA, et al. Ann Surg 2009
    • FISH OIL IN PN,PEDIATRICS Volume 121, Number 3, March 2008 IS IT SAFE ?
    • JPEN © 2012 American Society for Parenteral and Enteral Nutrition
    • CASE PROGRESSION  Patients were tolerating variable amounts of daily calories from elemental formulas containing approximately 40% of calories as fat at the time of SLE removal AGE PN EN WT TOTAL NOTES (kg) BILIRUBIN9 months 65 % 35 % 6.011 months 60 % 40 % 6.5 7.9 mg/dl SLE was reduced and enteral feeding was maximized15 months 20 % 80 % 6.75 SLE was eliminated19 months 0% 100 % 7.0 1.2 mg/dl PNALD completely reversed
    • ENTERAL NUTRITION Prompt transition to enteral nutrition  most important intervention – obviates intestinal failure associated liver disease (IFALD) & central access blood stream infection (CABSI) Vitamins and trace elements deficiency maybe evident only after several months weaning to full enteral nutrition (previously provided in PN) Zinc deficiency may also occur – losses are accentuated by diarrhea or excessive stomal output
    • ELEMENTAL VS SEMI-ELEMENTAL Infants have a “leaky gut” – sensitive to cow’s milk or soy protein ↓ in intestinal permeability in four infants with SBS after switched from a protein hydrolysate formula → formula containing free amino acids (Bines et al) Often given elemental formula (< fat & < LCPUFA than human milk)  concern of fat malabsorption in SBS LCPUFA (compared to CHO & protein)  are very large molecules that create the least osmotic load and contain the highest calorie density  unlikely to cause osmotic diarrhea  increase the amount of calories absorbed
    • LCT VS MCT LCT is a potent stimulator of enterogastrone MCT provide lesser stimulation of mucosal adaptation If ileum resection done – bile acid concentrations may not be sufficient to absorb large quantities of long-chain fat LCT : MCT ratio in  Elemental formula – 80 : 20  Semi-elemental formula – 40 : 60
    • STOMA OUTPUT Advancement of EN are allowed  stool or stomal output is < 1.5 to 2 ml/kg/h (40 to 50 ml/kg/d) EN should not be advanced  ↑ in stool loss (50% of previous volume / amounts > 40 to 50 ml/kg/d) Continuous EN tend to cause less diarrhea Loperamide (anti-diarrheal) may be used to decrease stool or stomal output Stomal refeeding is also an effective strategy in patients with a long mucous fistula
    • PROBIOTICS Use of enteral probiotics (restore more normal flora to the GI tract) is theoretically appealing Unfortunately, PN-dependent patients – administered probiotics have developed CABSI with the organism administered The probiotic translocates and infects the central line. So, the use of probiotics is not recommended Land MH, Rouster-Stevens K, Woods CR, et al. Pediatrics 2005
    • Nutrition in Clinical Practice Volume 25 Number 2 April 2010 199-204 © 2010 ASPEN patients received enteral fish oil (250 mg/kg/d)
    • Neonatology 2010;98:348–353
    • FISH OIL VIA ENTERAL ?Pharmacotherapy. 2011;31(5):503-509
    • F ISH OIL VIA ENTERAL ? Starting dose of fish oil is based on the human uterine accretion of DHA (50 mg/day) The maximal dose is based on the fact that DHA < 315 mg/day appears to be safe in breast-fed infants 1–6 months of age who solely consume human milk with a DHA content at the high end of normal (1.0 % total fatty acids)
    • SERIAL TRANSVERSE ENTEROPLASTY PROCEDURE (STEP)  Recent compilation of >100 STEP patients – by International STEP Registry (2010 Annual Meeting of AAP)  50% of all patients were successfully converted to full enteral tolerance & median time – 2 years
    • CURRENT APPROACHCurrent approach to managing patients with SBS is : a multidisciplinary effort focused on nutritional, pharmacologic & surgical interventions that achieve full enteral nutrition while minimizing the complications of PN therapy