Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis.
Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis.
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DOI：
10.1002/hep.29019
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发表时间：
2017-05
期刊：
影响因子：
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通讯作者：
Childhood Liver Disease Research Network (ChiLDReN)
中科院分区：
文献类型：
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作者：
Wang KS;Tiao G;Bass LM;Hertel PM;Mogul D;Kerkar N;Clifton M;Azen C;Bull L;Rosenthal P;Stewart D;Superina R;Arnon R;Bozic M;Brandt ML;Dillon PA;Fecteau A;Iyer K;Kamath B;Karpen S;Karrer F;Loomes KM;Mack C;Mattei P;Miethke A;Soltys K;Turmelle YP;West K;Zagory J;Goodhue C;Shneider BL;Childhood Liver Disease Research Network (ChiLDReN)
为评价非移植手术治疗小儿胆汁淤积症的疗效，对58例临床诊断的小儿胆汁淤积症，包括20例Alagille综合征（ALGS）、16例家族性肝内胆汁淤积症-1（FIC-1）、18例胆盐输出泵（BSEP）病和4例其他低γ-谷氨酰转肽酶病，（水平<100 U/L），在14个儿童肝病研究网络（ChiLDReN）中心进行了鉴定。回顾性收集了39例部分外胆管分流术（PEBD）、11例回肠排除术（IE）和7例胆囊-结肠分流术（GBC）的患者的数据。FIC 1中PEBD后血清总胆红素降低（术前与术后12-24个月分别为8.1±4.0与2.9±4.1 mg/dL，p=0.02），但ALGS或BSEP中未降低。ALGS患者PEBD后血清总胆固醇降低（分别为695±465和457±319 mg/dL;术前和术后12-24个月; p=0.0001）。PEBD后ALGS的丙氨酸氨基转移酶水平升高（182±70 vs. 260±73 IU/L，术前vs. 24个月）。p=0.03），但在FIC 1或BSEP中没有。ALGS、FIC 1和BSEP患者在PEBD后瘙痒评分较低（ALGS：100%与9%重度，FIC 1：64%与10%; BSEP：50%与20%，术前与>24个月）。术后分别为p<0.001）。ALGS患者在PEBD后经历了更大的无黄瘤的趋势。IE和GBC后，FIC 1中瘙痒有减少的趋势。PEBD后ALGS中维生素K补充增加（33% vs. 77%，p=0.03）。术后共发生15例严重并发症。12例患者（ALGS 3例，FIC 1 3例，BSEP 6例）随后接受了肝移植。这是第一次对肝内胆汁淤积症的非移植手术方法进行多中心分析。方法各不相同，耐受性良好，并且通常但不一致地导致瘙痒和胆汁淤积的改善。
To evaluate the efficacy of non-transplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 Alagille syndrome (ALGS), 16 Familial Intrahepatic Cholestasis-1 (FIC1), 18 Bile Salt Export Pump (BSEP) disease, & 4 others with low γ-glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBD), 11 ileal exclusions (IE) and 7 gallbladder-to-colon diversion (GBC). Serum total bilirubin decreased after PEBD in FIC1 (8.1±4.0 vs. 2.9±4.1 mg/dL, preop vs. 12–24 months postop respectively, p=0.02) but not in ALGS or BSEP. Total serum cholesterol decreased after PEBD in ALGS patients (695±465 vs. 457±319 mg/dL; preop vs. 12–24 months postop respectively; p=0.0001). Alanine aminotransferase levels increased in ALGS after PEBD (182±70 vs. 260±73 IU/L, preop vs. 24 mos. p=0.03) but not in FIC1 or BSEP. ALGS, FIC1, and BSEP patients experienced less severely scored pruritus after PEBD (ALGS: 100% vs. 9% severe, FIC1: 64% vs. 10%; BSEP: 50% vs. 20%, preop vs. >24 mos. postop respectively, p<0.001). ALGS patients experienced a trend toward greater freedom from xanthomata after PEBD. There was a trend toward decreased pruritus in FIC1 after IE and GBC. Vitamin K supplementation increased in ALGS after PEBD (33% vs. 77%, p=0.03. Overall, there were 15 major complications after surgery. 12 patients (3 ALGS, 3 FIC1, 6 BSEP) subsequently underwent liver transplantation. This is the first multi-center analysis of non-transplant surgical approaches to intrahepatic cholestasis. Approaches vary, are well tolerated, and generally although not uniformly result in improvement of pruritus and cholestasis.