Persistent abnormal liver fibrosis after weaning off parenteral nutrition in pediatric intestinal failure
Persistent abnormal liver fibrosis after weaning off parenteral nutrition in pediatric intestinal failure
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DOI：
10.1002/hep.26360
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发表时间：
2013-08-01
期刊：
影响因子：
13.5
通讯作者：
Pakarinen, Mikko P.
中科院分区：
文献类型：
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作者：
Mutanen, Annika;Lohi, Jouko;Pakarinen, Mikko P.
本研究的目的是评估儿童肠衰竭（IF）对肝脏组织学的长期影响。总共有38例IF患者（中位年龄：7.2岁；范围：0.2-27岁）接受了肝活检、胃镜检查、腹部超声检查和实验室检查。16例患者在74个月后接受肠外营养（PN）（范围2.5-204）。22例提前8.8年（范围0.3-27）停用PN， 35个PN月（范围0.7-250）停用。15个移植供体肝脏作为对照。94%的PN患者和77%的PN断奶患者肝脏组织学异常（P = 0.370）。在胰十二指肠切除术期间，肝脏组织学以胆汁淤积为主（胰十二指肠切除术患者占38%，断奶患者占0%，P = 0.003），门静脉炎症（胰十二指肠切除术患者占38%，胰十二指肠切除术患者占9%，P = 0.050）。纤维化（88%对64%，P = 0.143；美他韦分期：1.6[范围，0-4]对1.1[范围，0-2];P = 0.089）和脂肪变性（50%对45%，P = 1.000）在断奶期间和之后同样常见。血浆丙氨酸转氨酶（78 U/L[范围，19-204]对34[范围，9-129],P = 0.009）和结合胆红素（43 mol/L[范围，1-215]对4[范围，1-23],P = 0.037）在断奶后显著高于断奶后。1例患者在停用PN后出现食管静脉曲张。Metavir分期与小肠长度（r = -0.486; P = 0.002）和脓毒症发作次数（r = 0.480; P = 0.002）相关。在多变量分析中，年龄调整后的小肠长度（ss = -0.533, P = 0.001）、门静脉炎症（ss = 0.291, P = 0.030）和回盲瓣缺失（ss = 0.267, P = 0.048）是纤维化分期的预测指标。结论：尽管胆汁淤积和门静脉炎症得到了缓解，但断奶后肝纤维化和脂肪变性仍然存在。广泛小肠切除术是肝纤维化分期的主要预测因素。(肝脏病学58:729 2013;738)
The aim of this study was to evaluate the long-term effects of pediatric intestinal failure (IF) on liver histology. Altogether, 38 IF patients (median age: 7.2 years; range, 0.2-27) underwent liver biopsy, gastroscopy, abdominal ultrasound, and laboratory tests. Sixteen patients were on parenteral nutrition (PN) after 74 PN months (range, 2.5-204). Twenty-two had weaned off PN 8.8 years (range, 0.3-27) earlier, after 35 PN months (range, 0.7-250). Fifteen transplant donor livers served as controls. Abnormal liver histology was found in 94% of patients on PN and 77% of patients weaned off PN (P = 0.370). During PN, liver histology weighted with cholestasis (38% of patients on PN versus 0% of patients weaned off PN; P = 0.003) and portal inflammation (38% versus 9%; P = 0.050) were found. Fibrosis (88% versus 64%; P = 0.143; Metavir stage: 1.6 [range, 0-4] versus 1.1 [range, 0-2]; P = 0.089) and steatosis (50% versus 45%; P = 1.000) were equally common during and after weaning off PN. Plasma alanine aminotransferase (78 U/L [range, 19-204] versus 34 [range, 9-129]; P = 0.009) and conjugated bilirubin (43 mol/L [range, 1-215] versus 4 [range, 1-23]; P = 0.037) were significantly higher during than after weaning off PN. Esophageal varices were encountered in 1 patient after weaning off PN. Metavir stage was associated with small bowel length (r = -0.486; P = 0.002) and number of septic episodes (r = 0.480; P = 0.002). In a multivariate analysis, age-adjusted small bowel length (ss = -0.533; P = 0.001), portal inflammation (ss = 0.291; P = 0.030), and absence of an ileocecal valve (ss = 0.267; P = 0.048) were predictive for fibrosis stage. Conclusion: Despite resolution of cholestasis and portal inflammation, significant liver fibrosis and steatosis persist after weaning off PN. Extensive small intestinal resection was the major predictor for liver fibrosis stage. (Hepatology 2013;58:729-738)