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 Patient Age Etiology Anatomy

1 7.9 years

Mid-gut volvulus, malrotation 15 cm bowel left: duodenum, jejunum to ascending colon, (-) ICV

2 3.9 years

Gastroschisis, Intestinal atresia, NEC

Resected 6cm jejunum and 15 cm ileum, (-) ICV, (+) Ascending Colon

3 3.6 years

Mid-gut volvulus 2 cm jejunum to ICV, (+)ICV, (+) entire colon

4 2.8 years

NEC with perforated ileum, pseudocyst resection

10 cm jejunum and 7 cm ileum, (+) ICV, (+) entire colon

5 8.3 years

Gastroschisis, NEC 86 cm: duodenum with some jejunum and ileum, (+) ICV, (+) entire colon

6 2.3 years

Mid-gut volvulus, Gastroschisis duodenum to left descending colon

7 2.3 years

Intrauterine bowel perforation, Small intestinal atresia

68 cm, jejunum to colon, (-) ICV, (+) entire colon

8 2.1 years

NEC with perforated bowel jejunum to sigmoid colon, (-) ICV, (+) sigmoid colon, 30 ->70 cm s/p STEP,

91.6

yearsMid-gut volvulus, Malrotation 2nd part of duodenum to 2cm prox to

ICV, (+) ICV, (+) entire colon

Table 2. TPN Characteristics

Patient Percentage of Calories via PN Fat Emulsion Dose (g/kg/day) Glucose Infusion Rate (mg/kg/min)Mean At Start At End Mean Min Max Mean Min Max

1 41.0 72 30 0.57 0 1.5 12.3 2.4 21.5

2 39.1 100 19 0.54 0 1 10.9 0 17

3 42.2 59 23 0.81 0.7 0.9 13.0 12.2 13.8

4 24.0 26 24 0.53 0.4 0.8 15.0 14 16

5 37.2 62 34 0.76 0 1.2 12.2 0 16.8

6 85.6 90 85 0.66 0 1 12.2 0 15.4

7 49.4 74 35 0.40 0 1 8.9 0 15

8 79.3 89 82 0.55 0.2 1 14.8 10.3 17

9 82.0 87 73 0.73 0.6 0.8 16.2 15 17

Mean 53.3 73.22 45.0 0.62 0.21 1.02 12.8 5.99 16.6

Table 3. Laboratory Monitoring

PatientDirect Bilirubin

(mg/dL)ALT/SGPT

(U/L)AST/SGOT

(U/L)Alkaline

Phosphatase (U/L)Triene:Tetraene

Ratioα-

Linolenic Acid Mean (nmol/mL)

Linoleic Acid Mean

(nmol/mL)Mean Max Mean Max Mean Max Mean Max Mean Max

1 0.1 0.1 59.6 111 40.4 63 239.8 280 0.066 0.15 60.6 1853

2 0.08 0.1 40.3 85 51 93 282.8 385 0.018 0.03 51.0 2781

3 0.1 0.1 43.7 71 36 68 236.3 238 0.170 0.02 73.0 2999

4 0.1 0.1 33.0 40 48 52 226.0 233 0.022 0.03 65.5 3123

5 0.1 0.1 60.5 93 122 227 163.0 164 0.026 0.03 96.0 2586

6 0.13 0.2 106.3 165 76.3 95 758.7 968 0.029 0.03 64.7 2199

7 0.1 0.1 41.0 43 35 42 261.3 331 0.030 0.03 101.7 3551

8 0.85 1.5 90.5 115 80.5 92 459.5 576 0.071 0.13 47.7 1761

9 0.1 0.1 81.5 124 65.5 96 164.5 175 0.024 0.02 37.0 1245

Mean 0.18 0.27 60.7 94 61.64 92.0 310.2 372.2 0.030 0.05 67.5 2455

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