Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis

on behalf of the Childhood Liver Disease Research Network (ChiLDReN)

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

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. (Hepatology 2017;65:1645-1654).

Original languageEnglish (US)
Pages (from-to)1645-1654
Number of pages10
JournalHepatology
Volume65
Issue number5
DOIs
StatePublished - May 1 2017

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Alagille Syndrome
Enterohepatic Circulation
Intrahepatic Cholestasis
Cholestasis
Pediatrics
Pruritus
Bile Acids and Salts
Xanthomatosis
gamma-Glutamyltransferase
Vitamin K
Gastroenterology
Therapeutics
Liver Transplantation

ASJC Scopus subject areas

  • Hepatology

Cite this

on behalf of the Childhood Liver Disease Research Network (ChiLDReN). / Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis. In: Hepatology. 2017 ; Vol. 65, No. 5. pp. 1645-1654.
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title = "Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis",
abstract = "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. (Hepatology 2017;65:1645-1654).",
author = "{on behalf of the Childhood Liver Disease Research Network (ChiLDReN)} and Wang, {Kasper S.} and Greg Tiao and Bass, {Lee M.} and Hertel, {Paula M.} and Douglas Mogul and Nanda Kerkar and Matthew Clifton and Colleen Azen and Laura Bull and Philip Rosenthal and Dylan Stewart and Riccardo Superina and Ronen Arnon and Molly Bozic and Brandt, {Mary L.} and Dillon, {Patrick A.} and Annie Fecteau and Kishore Iyer and Binita Kamath and Saul Karpen and Frederick Karrer and Loomes, {Kathleen M.} and Cara Mack and Peter Mattei and Alexander Miethke and Kyle Soltys and Turmelle, {Yumirle P.} and Karen West and Jessica Zagory and Cat Goodhue and Shneider, {Benjamin L.}",
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on behalf of the Childhood Liver Disease Research Network (ChiLDReN) 2017, 'Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis', Hepatology, vol. 65, no. 5, pp. 1645-1654. https://doi.org/10.1002/hep.29019

Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis. / on behalf of the Childhood Liver Disease Research Network (ChiLDReN).

In: Hepatology, Vol. 65, No. 5, 01.05.2017, p. 1645-1654.

Research output: Contribution to journalArticle

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T1 - Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis

AU - on behalf of the Childhood Liver Disease Research Network (ChiLDReN)

AU - Wang, Kasper S.

AU - Tiao, Greg

AU - Bass, Lee M.

AU - Hertel, Paula M.

AU - Mogul, Douglas

AU - Kerkar, Nanda

AU - Clifton, Matthew

AU - Azen, Colleen

AU - Bull, Laura

AU - Rosenthal, Philip

AU - Stewart, Dylan

AU - Superina, Riccardo

AU - Arnon, Ronen

AU - Bozic, Molly

AU - Brandt, Mary L.

AU - Dillon, Patrick A.

AU - Fecteau, Annie

AU - Iyer, Kishore

AU - Kamath, Binita

AU - Karpen, Saul

AU - Karrer, Frederick

AU - Loomes, Kathleen M.

AU - Mack, Cara

AU - Mattei, Peter

AU - Miethke, Alexander

AU - Soltys, Kyle

AU - Turmelle, Yumirle P.

AU - West, Karen

AU - Zagory, Jessica

AU - Goodhue, Cat

AU - Shneider, Benjamin L.

PY - 2017/5/1

Y1 - 2017/5/1

N2 - 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. (Hepatology 2017;65:1645-1654).

AB - 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. (Hepatology 2017;65:1645-1654).

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on behalf of the Childhood Liver Disease Research Network (ChiLDReN). Analysis of surgical interruption of the enterohepatic circulation as a treatment for pediatric cholestasis. Hepatology. 2017 May 1;65(5):1645-1654. https://doi.org/10.1002/hep.29019