Edorium Journal of

Hepatology

 
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Original Article
 
Metabolic profiles and liver steatosis in children with liver transplantation for progressive familial intrahepatic cholestasis type 1
Ana Catalina Arce-Clachar1, Jonathan Moses1, Gursimran Kochlar1, Peggy George1, Vera Hupertz1, Kadakkal Radhakrishnan1, Raed Dweik1, Naim Alkhouri1
1MD, Department of Pediatric Gastroenterology, Cleveland Clinic Children's Hospital.

Article ID: 100001H06AC2015
doi:10.5348/H06-2015-1-OA-1

Address correspondence to:
Naim Alkhouri
MD, Department of Pediatric Gastroenterology, Cleveland Clinic Foundation, 9500 Euclid Avenue
Cleveland, Ohio 44195
USA
Phone: 216-444-9000
Fax: 216-444-2974

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How to cite this article
Arce-Clachar AC, Moses J, Kochlar G, George P, Hupertz V, Radhakrishnan, Dweik R, Alkhouri N. Metabolic profiles and liver steatosis in children with liver transplantation for progressive familial intrahepatic cholestasis type 1. Edorium J Hepatol 2015;1:1–6.


Abstract
Aims: Severe liver steatosis is common complication post-transplantation in patient with progressive familial intrahepatic cholestasis type 1 (PFIC1). We aimed to evaluate metabolic profiles in children who received orthotopic liver transplantation (OLT) for PFIC1 and their correlation with the development of fatty liver disease (FLD).
Methods: Patients transplanted for PFIC1 disease were identified and matched in a 1:2 ratio with patients transplanted for other indications. Liver biopsy was used to determine the presence of FLD. Lipid profiles were compared in the 2 groups. To investigate mechanistic pathways leading to the development of FLD, volatile organic compounds (VOCs) were analyzed on prospectively collected samples using a selective ion flow tube mass spectrometry.
Results: Five children with PFIC1 disease and 10 children with other indications for liver transplantation were included. Liver biopsies demonstrated that all children with PFIC1 had moderate-to-severe steatosis. The PFIC1 group had significantly lower HDL (20.9±6.0 mg/dL versus 51.3±17.1 mg/dL, respectively, p < 0.001) and total cholesterol levels (72±8.0 versus 156±103, p < 0.001) post transplantation compared to the other group. VOCs, including acetaldehyde, ammonia and pentane were elevated in the PFIC1 group, while isoprene was lower.
Conclusion: Fatty liver disease was a consistent finding in the patients with PFIC1 post OLT. Lipid levels were uniformly low in PFIC1 patients. Metabolomic analysis suggested that pathways in oxidative stress, gut bacteria production and cholesterol synthesis were different in children with PFIC1.

Keywords: Liver transplantation, Steatosis, Progressive familial intrahepatic cholestasis, Children

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Author Contributions:
Ana Catalina Arce-Clachar – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Jonathan Moses – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Gursimran Kochlar – Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published
Peggy George – Substantial contributions to conception and design, Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published
Vera Hupertz – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Kadakkal Radhakrishnan – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Raed Dweik – Substantial contributions to conception and design, Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published
Naim Alkhouri – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2015 Catalina Arce-Clachar et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.



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