Section of Pediatric Surgery, Children's Hospital, University of Helsinki, Helsinki, Finland.
J Pediatr Gastroenterol Nutr. 2012 Jun;54(6):803-11. doi: 10.1097/MPG.0b013e3182474118.
We prospectively evaluated incidence of prolonged (>28 days) parenteral nutrition (PN), associated complications, and significance of parenteral plant sterols (PS) in neonatal intestinal failure-associated liver disease (IFALD) compared with children.
We recruited 28 neonates (mean age 50 days, range 28-126) and 11 children (6.9 y, 2.1-16.6) in all of Finland. Patients underwent repeated measurements of serum cholesterol, noncholesterol sterols, including PS, cholestanol and cholesterol precursors, and liver biochemistry during and 1 month after discontinuation of PN. Healthy matched neonates (n=10) and children (n=22) served as controls.
IFALD occurred more frequently among neonates (63%) than children (27%; P<0.05). Ratios of serum PS, including stigmasterol, sitosterol, avenasterol, and campesterol, and total PS were increased among neonates compared with healthy controls and children on PN by 2- to 22- and 2- to 5-fold (P<0.005), respectively. Neonates with IFALD had significantly higher ratios of serum PS and cholestanol compared with neonates without IFALD (P<0.05). Total duration of PN associated with serum cholestanol, stigmasterol, avenasterol, alanine aminotransferase, and aspartate aminotransferase (r=0.472-0.636, P<0.05). Cholestanol and individual serum PS, excluding campesterol, reflected direct bilirubin (r=0.529-0.688, P<0.05). IFALD persisted after discontinuation of PN in 25% of neonates with 4.2- and 2.2-times higher ratios of serum stigmasterol and cholestanol compared with neonates without IFALD (P<0.05).
Frequent occurrence of IFALD among neonates on PN displays an association to duration of PN and markedly increased serum PS, especially stigmasterol, in comparison to healthy neonates and children on PN. Striking accumulation of parenteral PS may contribute to IFALD among neonates.
我们前瞻性评估了与儿童相比，肠衰竭相关肝病（IFALD）新生儿中肠外营养（PN）持续时间（>28 天）、相关并发症以及肠外植物固醇（PS）的意义。
我们招募了芬兰的 28 名新生儿（平均年龄 50 天，范围 28-126 天）和 11 名儿童（6.9 岁，2.1-16.6 岁）。在停止 PN 期间和之后 1 个月，患者接受了血清胆固醇、非胆固醇甾醇（包括 PS、胆甾烷醇和胆固醇前体）和肝功能的重复测量。健康匹配的新生儿（n=10）和儿童（n=22）作为对照组。
IFALD 在新生儿中更常见（63%），而在儿童中则较少（27%；P<0.05）。与健康对照组和 PN 治疗的儿童相比，新生儿的血清 PS 比值（包括豆甾醇、谷甾醇、菜油甾醇和菜油固醇）和总 PS 分别增加了 2-22 倍和 2-5 倍（P<0.005）。与无 IFALD 的新生儿相比，IFALD 新生儿的血清 PS 和胆甾烷醇比值显著更高（P<0.05）。PN 持续时间与血清胆甾烷醇、豆甾醇、菜油甾醇、丙氨酸氨基转移酶和天冬氨酸氨基转移酶呈正相关（r=0.472-0.636，P<0.05）。胆甾醇和除菜油固醇外的个体血清 PS 与直接胆红素呈正相关（r=0.529-0.688，P<0.05）。在停止 PN 后，25%的新生儿 IFALD 持续存在，其血清豆甾醇和胆甾烷醇比值分别比无 IFALD 的新生儿高 4.2 倍和 2.2 倍（P<0.05）。
PN 治疗的新生儿 IFALD 发生率较高，与 PN 持续时间有关，并与 PN 治疗的健康新生儿和儿童相比，血清 PS 尤其是豆甾醇明显增加。肠外 PS 的大量积累可能导致新生儿 IFALD。
