Poster No. 192
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
Table 1. Patient Characteristics
Patient
Age
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,
1.6
years
Mid-gut volvulus, Malrotation
2nd part of duodenum to 2cm prox to
ICV, (+) ICV, (+) entire colon
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
9
Etiology
Anatomy
Figure 1. Z-Scores for Weight and Height
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
Normal range for α-Linolenic acid: 20-120nmol/mL. Normal range for linoleic acid: 1600-3500nmol/mL
Table 3. Laboratory Monitoring
Patient
Direct Bilirubin
(mg/dL)
ALT/SGPT
(U/L)
AST/SGOT
(U/L)
Alkaline
Phosphatase (U/L)
Triene:Tetraene
Ratio
αLinoleic
Linolenic
Acid
Acid Mean
Mean
(nmol/mL) (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
CONCLUSION:
In this case series, lipid limitation
allowed normal growth while preventing the
development of cholestasis and fatty acid
deficiency