1. NORMAL GROWTH AND ESSENTIAL FATTY ACID STATUS IN CHILDREN WITH INTESTINAL FAILURE ON LIPID LIMITATION Gillian Tam, MD; Irina Strogach, MS, RD, CDN; Nicole Baron, RN, MS, CPNP ; John F. Thompson, M.D Gtam@montefiore.org, Jothomps@montefiore.org Poster No. 192 BACKGROUND: Lipid limitation, i.e., ≤ 1g/kg/day of soy oil lipid emulsion (SOLE), has been suggested as a method to reduce risk of intestinal failure (IF) associated liver disease (ILALD). There are limited data as to the effects of this strategy on growth and essential fatty acid (EFA) status METHODS: Medical records were retrospectively reviewed from 9 patients with intestinal failure requiring chronic parenteral nutrition (PN) support for 14 months to 8 years Table 1. Patient Characteristics PatientAgeEtiologyAnatomy 17.9 years Mid-gut volvulus, malrotation15 cm bowel left: duodenum, jejunum to ascending colon, (-) ICV 23.9 years Gastroschisis, Intestinal atresia, NEC Resected 6cm jejunum and 15 cm ileum, (-) ICV, (+) Ascending Colon 33.6 years Mid-gut volvulus2 cm jejunum to ICV, (+)ICV, (+) entire colon 42.8 years NEC with perforated ileum, pseudocyst resection 10 cm jejunum and 7 cm ileum, (+) ICV, (+) entire colon 58.3 years Gastroschisis, NEC86 cm: duodenum with some jejunum and ileum, (+) ICV, (+) entire colon 62.3 years Mid-gut volvulus, Gastroschisisduodenum to left descending colon 72.3 years Intrauterine bowel perforation, Small intestinal atresia 68 cm, jejunum to colon, (-) ICV, (+) entire colon 82.1 years NEC with perforated boweljejunum to sigmoid colon, (-) ICV, (+) sigmoid colon, 30 ->70 cm s/p STEP, 9 1.6 years Mid-gut volvulus, Malrotation2nd part of duodenum to 2cm prox to ICV, (+) ICV, (+) entire colon Table 2. TPN Characteristics PatientPercentage of Calories via PNFat Emulsion Dose (g/kg/day)Glucose Infusion Rate (mg/kg/min) MeanAt StartAt EndMeanMinMaxMeanMinMax 141.072300.5701.512.32.421.5 239.1100190.540110.9017 342.259230.810.70.913.012.213.8 424.026240.530.40.815.01416 537.262340.7601.212.2016.8 685.690850.660112.2015.4 749.474350.40018.9015 879.389820.550.2114.810.317 982.087730.730.60.816.21517 Mean53.373.2245.00.620.211.0212.85.9916.6 Table 3. Laboratory Monitoring Patient Direct Bilirubin (mg/dL) ALT/SGPT (U/L) AST/SGOT (U/L) Alkaline Phosphatase (U/L) Triene:Tetraene Ratio α- Linolenic Acid Mean (nmol/mL) Linoleic Acid Mean (nmol/mL) MeanMaxMeanMaxMeanMaxMeanMaxMeanMax 10.1 59.611140.463239.82800.0660.1560.61853 20.080.140.3855193282.83850.0180.0351.02781 30.1 43.7713668236.32380.1700.0273.02999 40.1 33.0404852226.02330.0220.0365.53123 50.1 60.593122227163.01640.0260.0396.02586 60.130.2106.316576.395758.79680.0290.0364.72199 70.1 41.0433542261.33310.0300.03101.73551 80.851.590.511580.592459.55760.0710.1347.71761 9 0.1 81.512465.596164.51750.0240.0237.01245 Mean 0.180.2760.79461.6492.0310.2372.20.0300.0567.52455 RESULTS: No patient developed IFALD as defined by a direct bilirubin > 2 mg/dL No patient developed EFA deficiency as defined by a triene:tetraene ratio > 0.2 Mean Height and Weight Z-Scores improved Figure 1. Z-Scores for Weight and Height Normal range for α-Linolenic acid: 20-120nmol/mL. Normal range for linoleic acid: 1600-3500nmol/mL CONCLUSION: In this case series, lipid limitation allowed normal growth while preventing the development of cholestasis and fatty acid deficiency