PHYTOSTEROLEMIA IN CHILDREN WITH PARENTERAL-NUTRITION ASSOCIATED CHOLESTATIC LIVER-DISEASE
PHYTOSTEROLEMIA IN CHILDREN WITH PARENTERAL-NUTRITION ASSOCIATED CHOLESTATIC LIVER-DISEASE
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DOI：
10.1016/0016-5085(93)91079-w
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发表时间：
1993-12-01
期刊：
影响因子：
29.4
通讯作者：
MILLA, PJ
中科院分区：
文献类型：
--
作者：
CLAYTON, PT;BOWRON, A;MILLA, PJ
背景：用于肠外营养（PN）的脂质乳液含有植物甾醇。我们的假设是，这些植物甾醇可以积累并导致胆汁淤积性肝病和 PN 的其他并发症，例如血小板减少症（发生在遗传性植物甾醇血症中）。方法：使用气相色谱-质谱法，测量 29 名接受 PN 的 2 个月至 9 岁儿童和 29 名年龄匹配的儿童的血浆甾醇浓度 控制。接受 PN 的儿童分为两个亚组：5 名患有严重 PN 相关胆汁淤积性肝病（胆红素水平 >100 μmol/L；天冬氨酸转氨酶 [AST]水平 >200 U/L）和 24 名胆红素水平<100 μmol/L 和/或 AST 水平<200 U/L。 结果：5 名患有严重 PN 相关的儿童 肝病患者的植物甾醇和谷甾烷醇血浆浓度与遗传性植物甾醇血症患者的血浆浓度一样高（总植物甾醇1.3-1.8 mmol/L）。所有 5 人均患有间歇性血小板减少症。两名患者的脂肪乳摄入量减少至<50 mL·kg−1·wk−1 与血浆植物甾醇浓度降低以及肝功能测试和血小板计数改善相关。与 PN 相关肝病较轻的儿童相比，患有严重疾病的 5 名儿童血浆植物甾醇浓度较低。结论：接受 PN 治疗且血浆植物甾醇浓度较高的儿童也患有胆汁淤积性肝病和血小板减少症；植物甾醇血症可能是 PN 并发症的发病机制之一。
Background:Lipid emulsions used for parenterai nutrition (PN) contain phytosterols. Our hypothesis was that these phytosterols can accumulate and contribute to cholestatic liver disease and other complications of PN, e.g., thrombocytopenia (which occurs in hereditary phytosterolemia).Methods:Using gas chromatography-mass spectrometry, plasma concentrations of sterols were measured in 29 children aged 2 months to 9 years receiving PN and in 29 age-matched controls. The children receiving PN fell into two subgroups: 5 with severe PN-associated cholestatic liver disease (bilirubin level, >100 μmol/L; aspartate aminotransferase [AST]level, >200 U/L) and 24 with a bilirubin level of <100 μmol/L and/or AST level of <200 U/L.Results:The 5 children with severe PN-associated liver disease had plasma concentrations of phytosterols and sitostanol that were as high as those seen in patients with hereditary phytosterolemia (total phytosterols 1.3–1.8 mmol/L). All 5 had intermittent thrombocytopenia. A reduction in intake of lipid emulsion to <50 mL·kg−1·wk−1was associated with a decrease in plasma phytosterol concentrations and an improvement in liver function tests and platelet counts in two patients. Children with less severe PN-associated liver disease had lower plasma phytosterol concentrations than the 5 with severe disease.Conclusions:Children receiving PN who have high plasma phytosterol concentrations also have cholestatic liver disease and thrombocytopenia; phytosterolemia might contribute to the pathogenesis of complications of PN.