Ileal Exclusion in Children With Progressive Familial Intrahepatic Cholestasis

Jankowska, Irena; Czubkowski, Piotr; Kaliciński, Piotr; Ismail, Hor; Kowalski, Adam; Ryżko, Józef; Pawłowska, Joanna

doi:10.1097/mpg.0b013e3182a9097c

Cited by 29 publications

(26 citation statements)

References 13 publications

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“…The indications for and outcomes of nontransplant surgical interventions for intrahepatic cholestasis are not well delineated. Most of the published literature is single centered and subject to potential bias toward positive results . The current study was designed to be cross‐sectional, multicentered, and unbiased toward outcomes with an intent to give a real‐world view of this complicated area of surgical therapy.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2017

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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. Conclusion 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.

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Section: Discussionmentioning

confidence: 99%

“…Chief among these alternative procedures are an ileal exclusion (IE; Fig. B), which internally bypasses ∼15% of the terminal ileum, and an internal biliary diversion, which entails connecting the gallbladder either directly to the colon or utilizing a segment of jejunum as a conduit (gallbladder to colon [GBC]; Fig. C) .…”

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confidence: 99%

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

et al. 2017

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“…Surgical interventions to interrupt the enterohepatic circulation include PEBD, partial internal biliary diversion (PIBD) and ileal exclusion [7,11,12].…”

Section: Accepted Manuscriptmentioning

confidence: 99%

Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome

Woerd

Kokke

Zee

et al. 2015

Journal of Pediatric Surgery

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“…An external stoma can also be formed by using the appendix creating a cholecystoappendicectomy as reported by Rebhandl et al [18]. Another alternative is ileal exclusion (IE), which has also been performed in cases with previous cholecystectomy or as rescue treatment [2,15,19,20].…”

Section: Fic1 Deficiencymentioning

confidence: 94%

“…Partial external biliary diversion, ileal exclusion (IE) and LT are all recommended surgical treatment options [16,20,34,35]. Hepatocellular malignancy remains an indication for early LT in BSEP patients.…”

Section: Bsep Deficiencymentioning

confidence: 99%

Familial Intrahepatic Cholestasis

Grammatikopoulos

Thompson

2015

Textbook of Pediatric Gastroenterology, Hepatology and Nutrition

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Ileal Exclusion in Children With Progressive Familial Intrahepatic Cholestasis

Abstract: IE is an alternative rescue option to PEBD and should be offered cautiously, only to patients who cannot benefit from PEBD.

Cited by 29 publications

References 13 publications

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

Total biliary diversion as a treatment option for patients with progressive familial intrahepatic cholestasis and Alagille syndrome

Familial Intrahepatic Cholestasis

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