Department of Gastroenterology, New Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Pediatric Liver and Gut Research Group, New Children's Hospital, Helsinki University Hospital, Helsinki, Finland.
JPEN J Parenter Enteral Nutr. 2023 Jan;47(1):41-50. doi: 10.1002/jpen.2416. Epub 2022 Jun 19.
Steatosis is a common feature of intestinal failure-associated liver disease (IFALD) in adult and older pediatric patients receiving long-term parenteral nutrition (PN). There are limited clinical data concerning steatosis in infants with short bowel syndrome (SBS). We investigated early histopathological steatosis and its association to PN.
In this retrospective study, 31 patients with SBS had a diagnostic liver biopsy taken at the median age of 5 (IQR 3-8) months. Follow-up biopsy was available for 24 patients at the median age of 29 (IQR 14-52) months. We evaluated the biopsies for steatosis and other histopathological signs of IFALD and compared results with patient characteristics, PN composition, and liver biochemistry.
Diagnostic biopsies revealed steatosis in 8 (26%) patients. At the age of 3 months, patients with steatosis had received higher amounts of parenteral glucose: median 15.1 (IQR 12.4-17.2) vs 12.3 (8.7-14.4) g/kg/d (P = 0.04), amino acids: 2.9 (2.5-3.4) vs 2.2 (1.6-2.7) g/kg/d (P = 0.03), and energy: 87 (80-98) vs 73 (54-79) kcal/kg/d (P = 0.01) than those without steatosis. We detected no significant differences in parenteral lipid intake between the groups. Steatosis also associated with increased serum bile acid (P = 0.02), alanine aminotransferase (P = 0.0002), and aspartate aminotransferase (P = 0.001) levels.
In this cohort, high parenteral glucose, amino acid, and energy provision associated with liver steatosis in infants with SBS. We recommend monitoring of bile acid and transaminase levels while aiming for PN with balanced macronutrient supply according to current recommendations to protect the liver from steatosis.
在接受长期肠外营养（PN）的成人和老年儿科患者中，脂肪变性是肠衰竭相关肝病（IFALD）的常见特征。关于短肠综合征（SBS）婴儿的脂肪变性，临床数据有限。我们研究了早期组织病理学脂肪变性及其与 PN 的关系。
在这项回顾性研究中，31 名 SBS 患者在中位年龄为 5（IQR 3-8）个月时接受了诊断性肝活检。24 名患者在中位年龄为 29（IQR 14-52）个月时可获得随访活检。我们评估了活检的脂肪变性和 IFALD 的其他组织病理学征象，并将结果与患者特征、PN 成分和肝功能生化指标进行了比较。
诊断性活检显示 8 例（26%）患者有脂肪变性。在 3 个月时，有脂肪变性的患者接受了更高剂量的肠外葡萄糖：中位数 15.1（IQR 12.4-17.2）比 12.3（8.7-14.4）g/kg/d（P=0.04），氨基酸：2.9（2.5-3.4）比 2.2（1.6-2.7）g/kg/d（P=0.03），和能量：87（80-98）比 73（54-79）kcal/kg/d（P=0.01）。两组间肠外脂质摄入无显著差异。脂肪变性还与血清胆汁酸（P=0.02）、丙氨酸氨基转移酶（P=0.0002）和天冬氨酸氨基转移酶（P=0.001）水平升高相关。
在本队列中，SBS 婴儿高剂量的肠外葡萄糖、氨基酸和能量与肝脏脂肪变性相关。我们建议在根据当前建议提供平衡的宏量营养素供应以保护肝脏免受脂肪变性的同时，监测胆汁酸和转氨酶水平。
