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Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis
Author(s) -
Wang Kasper S.,
Tiao Greg,
Bass Lee M.,
Hertel Paula M.,
Mogul Douglas,
Kerkar Nanda,
Clifton Matthew,
Azen Colleen,
Bull Laura,
Rosenthal Philip,
Stewart Dylan,
Superina Riccardo,
Ar Ronen,
Bozic Molly,
Brandt Mary L.,
Dillon Patrick A.,
Fecteau Annie,
Iyer Kishore,
Kamath Binita,
Karpen Saul,
Karrer Frederick,
Loomes Kathleen M.,
Mack Cara,
Mattei Peter,
Miethke Alexander,
Soltys Kyle,
Turmelle Yumirle P.,
West Karen,
Zagory Jessica,
Goodhue Cat,
Shneider Benjamin L.
Publication year - 2017
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.29019
Subject(s) - enterohepatic circulation , cholestasis , alagille syndrome , medicine , gastroenterology , progressive familial intrahepatic cholestasis , bile salt export pump , bile acid , liver transplantation , chemistry , biochemistry , transplantation , transporter , gene
To evaluate the efficacy of nontransplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 with Alagille syndrome (ALGS), 16 with familial intrahepatic cholestasis‐1 (FIC1), 18 with bile salt export pump (BSEP) disease, and 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 (PEBDs), 11 ileal exclusions (IEs), and seven gallbladder‐to‐colon (GBC) diversions. Serum total bilirubin decreased after PEBD in FIC1 (8.1 ± 4.0 vs. 2.9 ± 4.1 mg/dL, preoperatively vs. 12‐24 months postoperatively, 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, preoperatively vs. 12‐24 months postoperatively, respectively; P = 0.0001). Alanine aminotransferase levels increased in ALGS after PEBD (182 ± 70 vs. 260 ± 73 IU/L, preoperatively vs. 24 months; 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%, preoperatively vs. >24 months postoperatively, 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. Twelve patients (3 ALGS, 3 FIC1, and 6 BSEP) subsequently underwent liver transplantation. Conclusion : This was a multicenter analysis of nontransplant surgical approaches to intrahepatic cholestasis. Approaches vary, are well tolerated, and generally, although not uniformly, result in improvement of pruritus and cholestasis. (H epatology 2017;65:1645‐1654).