TABLE 5.
Results.
| References | PN before treatment | EN before treatment | PN after treatment | Weaned | PN after discontinuation | EN after discontinuation |
Weight after | Height after | Stool improvement | Citrulline levels |
| Carter et al. (18)x | U | 29 (69%) | SOC: −0 (−0.3, 1.4) L/week, −0 (−2.0, 0.6) hours per day, −1 (−5, 5) kcal/day/kg |
|
After 4 weeks of suspension, 2 patients resumed PN | SOC | U | U | U | U |
| 0.0125: −0 (−2.5, 0) L/week, −0 (0,2.0) hours per day, −2 (−12, 3) kcal/day/kg | 0.0125: +1,1 (0–12.5) L/week |
0.0125: + 1 μmol/L (−0.8, 22.9) |
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| 0.025: −2,3 L/week (−6.9−0), −4 (−9.0−2.0) hours per day, −17 (−39, 2) kcal/day/kg | 1 at week 11 | 0.025: +2,3 (−0.9–8.8) L/week |
0.025: + 5.4 μmol/L (1.1, 17.2) |
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| 0.05: −1,3 L/week (−11 −1) at 12 weeks, −3 h per day (−12.0−0.8), −17 (−45, 53) kcal/day/kg | 3 at week 4, 8, 12 | 0.05: +0.7 (0–3.9) L/week | 0.05: +7.5 μmol/L (−13.9, 56.5) | |||||||
| Busoni et al. (19)y | 3,000 ml/week | U | 1 patient: −100% at 10 weeks | 1 at week 10 | U | U | +1,8 kg (1.3–2) | +5,7 cm (4,5–6,5) | Yes | U |
| 7,000 ml/week | 1 patient: −30% at 4 weeks, −71% at 8 weeks (reduced infusions at 5 nights) | |||||||||
| 12,600 ml/week | 1 patient: −28% at 45 weeks | |||||||||
| Ferreiro et al. (20) | 16,100 ml/week, 25 Kcal/kg | U | 0 ml/day at 30 weeks | 1 at week 30 | U | U | +4,4 kg | +11 cm | Yes | Increased |
| Hill et al. (22)z | U | U | −3.7 ± −15.72 kcal/kg/day at Cycle 1Day1 (n = 15) and −21.6 ± 17.90 kcal/kg/day at Cycle 1 Week 24 (n = 13). Number of days per week: −0.4 ± 1.92 days per week at C1D1 (n = 14) and −0.9 ± 2.23 days per week at C1W24 (n = 12) | 0 | U | U | Stable | Stable | U | U |
| Kinberg (23)x | U | U | Six patients: −1.8 (0–2.4) L/week One patient reduced number of hours per day |
0 | U | U | U | U | U | U |
| Lambe (25)z | NPEI/REE index was 95 ± 28% |
U | At 12 weeks: all patients > 20% decrease of PN requirements (−33%, NPEI/REE index 64 ± 22%) At 24 weeks: mean NPEI/REE index 57.5 ± 25% |
8 at 48 weeks | U | U | Stable | Stable | Y | 15.2 ± 9 mol/l (mean) at baseline to 26.5 ± 21.6 mol/l at week 12, 31.6 ± 21.6 mol/l at week 24 and 36 mol/l ± 25.2 at week 48 |
| Kocoshis et al. (24)z | SOC: 79.6 ± 31.12 mL/kg/d, 44.6 ± 22.53 kcal/kg/d, 6.6 ± 1.33 days per week, 12.6 ± 5.50 h per day | 52 (88%) |
SOC: > 20% reduction in 1 patient at week 24, –6.0 ± 4.55 mL/kg/d, −0.5 ± 4.95 kcal/kg/d, −0 days per week, –0.2 ± 0.69 h per day | 0 | SOC: stable | Still reduced | U | SOC: height z-score change from baseline: −0.23 +/−0.26 | U | Baseline SOC: 12.6+/−8.43 (8 patients) 0.025: 17.9 ±12.64 (21 pts) 0.05: 16.0 + −11.54 (24 pts) Week 24 SOC: 12.3 + −6.57 0.025: 25.5 + −15.90 0.05: 29.0 + −15.23 |
|
0.025: 56.8 ± 25.24 ml/kg/day, 43.3 ± 21.10 kcal/kg/d, 6.5 ± 1.10 days per week, 11.7 ± 3.03 h per day |
0.025: > 20% reduction in 13 patients at week 24, –16.2 ± 10.52 mL/kg/d, –14.9 ± 8.29 kcal/kg/d, –0.9 ± 1.78 days per week, –2.5 ± 2.73 h per day | 0.025: increased in 23 patients, + 76.9% ± 117.19% in volume, +82.7% ± 136.27% in calories | 0.025: height z-score change from baseline: −0.09± 0.3 | |||||||
| 0.05: 60 ± 29.19 ml/kg/day, 43.3 ± 16.52 kcal/kg/d, 6.6 ± 0.79 days per week, 11.2 ± 2.99 h per day | 0.05: > 20% reduction in 18 patients at week 24, –23.3 ± 17.50 mL/kg/d, –19.0 ± 14.28 kcal/kg/d, –1.3 ± 2.24 days per week, –3.0 ± 3.84 h per day | 0.05: increased in 26 patients, + 79.5% ± 134.49% in volume, + 86.47% ± 128.11% in calories | 0.05: height z-score change from baseline: −0.04 ± 0.24 | |||||||
| Martìnez et al. (26)z | 11.55 ± 3.5 L/week | 15% ± 8.8% (of total intake) | 1: 12.9 L/week at week 14 1: 11.7 L/week at week 8 |
2 at weeks 44, 101 | U | U | U | U | U | U |
| Ramos-Boluda et al. (16)y | 55 ml/kg/d (8–210) 33 kcal/kg/d (0–65) 2 patients only IV fluids |
U | 4 decreased PN requirements 1 No change 1 discontinuation Response rate (< 20% of PN requirements) of 47% at month 3, 87% at 6 months, and 93% at 1 year. |
3 at month 3 4 at month 6 3 at month12 |
U | U | U | U | Y | Baseline: 20 mmol/l (7.8 −51) 12 months: 37.9 mmol/l (9–67) |
| Ribeiro-Mourão et al. (15) | 3 patients: 145% PN/REE, 1 patient 97% PN/REE; |
U | 1 month: reduction of at least 1 day/week (all patients) 6 months: 1 patient 2 days reduction (1 patient discontinued after 1 month) |
2 at 6 months | U | Increased by 10% at month 1, 73% at month 6 | + 1.3 kg at month 1 + 2.8 kg at month 6 |
+1.6 cm at month 1 +5.6 cm at month 6 |
Y | U |
| Rumbo et al. (17) | 6 days/week | U | 0 ml/kg/day at week 25 | 1 at week 25 | U | Improved (weaned) | Improved BMI/A 1.32 at start, 0.54 at week 50) | Improved H/A-3.66 at start, −2.51 at week 50) | Y | U |
| Sigalet et al. (9)z | 66% of calories intake ± 16 | 34% of calories intake ± 16 | 54% of calories intake | 0 | 25% ± 18 of calories intake | 75% ± 46 of calories intake | Baseline: 4.7 Kg ± 1.6 42 days: 5.3 kg ±1.2 |
U | Y | Baseline: 7.8 ± 1.7 42 days: 10.5 ± 3.4 μmol/L |
| Sigalet et al. (12) | U | U | Stable | 0 | Stable | Stable | Increased | Increased | Y | Increased |
| Mercer et al. (21)z | U | U | −42.4% ± 29.19 at C1D1 (n = 33), and −49.6% ± 32.57 at C1W24 (n = 25), −1.0 ± 1.89 days per week at C1D1 (n = 33) and −1.4 ± 2.62 days per week at C1W24 (n = 25). | 7 at C1W24 | U | U | Stable | Stable | U | U |
| Total | 36 |
PN, Parenteral Nutrition; EN, Enteral Nutrition; U, Unknown; PN/REEI, PN intake in calories/resting energy expenditure.
xMedian (range), yMean (range), zMean ± Standard Deviation.