肠衰竭相关肝病儿童脂肪酸谱特征
高剂量的大豆油（SO）脂肪乳剂是肠衰竭相关肝病（IFALD）的风险因素之一。
为了探讨IFALD患儿接受不同浓度SO肠外营养后的脂肪酸谱变化情况，美国波士顿儿童医院回顾分析了54位IFALD患儿，发现PN添加不同浓度SO制剂可导致脂肪酸谱差异：SO剂量约1g/kg/d的患儿与3g/kg/d的患儿相比，亚麻酸、二十碳五烯酸、硬脂酸、ω-3和ω-6脂肪酸水平均显著下降。此外，低剂量SO（1g/kg/d）并不会导致必需脂肪酸的缺乏。
JPEN J Parenter Enteral Nutr. 2017;41(2):295-296.
Characterization of fatty acid profiles in children with intestinal failure-associated liver disease.
Meredith A. Baker, Paul D. Mitchell, Alison A. O'Loughlin, Alexis K. Potemkin, Lorenzo Anez-Bustillos, Duy T. Dao, Gillian L. Fell, Kathleen M. Gura, Mark Puder.
Boston Children's Hospital, Boston, Massachusetts, USA.
PURPOSE: Soy oil-based lipid emulsions (SO), particularly when administered at high doses, are a risk factor for intestinal failure-associated liver disease (IFALD). Our institution transitioned from SO doses of 2-4 g/kg/d to 1 g/kg/d for all parenteral nutrition (PN)-dependent surgical patients in 2008. We aim to characterize fatty acid profiles (FAP) in children with IFALD receiving SO doses of approximately 3 g/kg/d and 1 g/kg/d.
METHODS: A retrospective review of prospectively collected data was performed. Serum FAP of 54 patients <4 years old who developed IFALD (serum direct bilirubin >2 mg/dL) while receiving standard PN with SO were examined before transitioning to a fish oil-based lipid emulsion for IFALD treatment from June 2004 to June 2016. We defined a lipid dose of 3 g/kg/d as >2 g/kg/d and 1 g/kg/d as <1.5 g/kg/d. Data are expressed as median with interquartile range (IQR).
RESULTS: There were no differences in demographics or anthropometrics between patients who received 3 g/kg/d SO (n = 14, range of SO dose 2.06-3.31 g/kg/d) and those who received 1 g/kg/d SO (n = 40, range of SO dose 0.90-1.34 g/kg/d). Patients who received 1 g/kg/d of SO had significantly lower levels of a-linolenic acid, eicosapentaenoic acid, linoleic acid, stearic acid, total ω-3 fatty acids, and total ω-6 fatty acids than patients who received 3 g/kg/d of SO. Although triene:tetraene (T:T) ratios were higher in patients who received 1 g/kg/d of SO (median [IQR] = 0.035 [0.024, 0.055]) than in those who received 3 g/kg/d of SO (0.021 [0.019, 0.025], P = .003), no patients had essential fatty acid deficiency as defined by a T:T ratio >0.2. The maximum T:T ratio was 0.199.
CONCLUSIONS: PN-dependent patients who develop IFALD while receiving SO at a dose of 1 g/kg/d have different FAP than patients receiving SO at a dose of 3 g/kg/d. While patients on 1 g/kg/d of SO do not develop essential fatty acid deficiency as defined by a T:T ratio >0.2, similar lipid doses of newer mixed-oil emulsions with lower long-chain polyunsaturated fatty acid content may result in essential fatty acid deficiency and should be closely monitored.
DOI: 10.1177/0148607116686023