Event Videos

https://pubmed.ncbi.nlm.nih.gov/34046333/ NAFLD

Pediatr Gastroenterol Hepatol Nutr. 2021 May;24(3):295-305.
doi: 10.5223/pghn.2021.24.3.295.Epub 2021 May 4.

Hepcidin Levels and Pathological Characteristics in Children with Fatty Liver Disease

Norito Tsutsumi 1, Shigeo Nishimata 1, Masaru Shimura 1 2, Yasuyo Kashiwagi 1, Hisashi Kawashima 1

Abstract

Purpose: Hepcidin levels have previously been reported to be correlated with liver damage. However, the association between hepcidin levels and liver fibrosis in children with fatty liver disease remains unclear. This study therefore aimed to investigate the pathophysiology of fibrosis in children with fatty liver disease and its association with hepcidin levels.

Published on: 
May-2021

https://pubmed.ncbi.nlm.nih.gov/34046462/ PFIC

Case Reports

World J Clin Cases. 2021 May 26;9(15):3631-3636.
doi: 10.12998/wjcc.v9.i15.3631.

Progressive familial intrahepatic cholestasis - farnesoid X receptor deficiency due to NR1H4mutation: A case report

Piotr Czubkowski 1, Richard J Thompson 2, Irena Jankowska 3, A S Knisely 4, Milton Finegold 5, Pamela Parsons 5, Joanna Cielecka-Kuszyk 6, Sandra Strautnieks 2, Joanna Pawłowska 3, Laura N Bull 7

Abstract

Background: Functioning farnesoid X receptor (FXR; encoded by NR1H4) is key to normal bile acid homeostasis. Biallelic mutations in NR1H4 are reported in a few children with intrahepatic cholestasis. We describe a boy with progressive familial intrahepatic cholestasis and homozygous mutation in NR1H4.

Published on: 
May-2021

https://pubmed.ncbi.nlm.nih.gov/34058057/ Liver Transplant
Liver Transpl. 2021 May 31.

Liver Transplantation in Children with Urea Cycle Disorders: The Importance of Minimizing Waiting Time

Ioannis A Ziogas 1, W Kelly Wu 1, Lea K Matsuoka 1, Anita K Pai 2, Einar T Hafberg 2, Lynette A Gillis 2, Thomas M Morgan 3, Sophoclis P Alexopoulos 1

Abstract

Background: Liver transplantation (LT) for children with urea cycle disorders (UCD) is capable of correcting the enzymatic defect and preventing progressive neurologic injury. We describe the characteristics and outcomes of pediatric LT recipients with UCD.

Published on: 
May-2021

https://pubmed.ncbi.nlm.nih.gov/33908738/ Wilson disease
J Pediatr Gastroenterol Nutr. 2021 Mar 17.
doi: 10.1097/MPG.0000000000003128.Online ahead of print.

Fatty Acid-Binding Protein 1 as a Potential New Serological Marker of Liver Status in Children with Wilson Disease

Joanna Beata Bierła 1, Wojciech Jańczyk, Ewa Konopka, Aldona Wierzbicka-Rucińska, Sebastian Więckowski, Łukasz Obrycki, Jędrzej Sarnecki, Ewelina Kanarek, Bożena Cukrowska, Piotr Socha

Abstract

Published on: 
Apr-2021

https://pubmed.ncbi.nlm.nih.gov/33908737/ liver transplantation

J Pediatr Gastroenterol Nutr. 2021 Mar 17.
doi: 10.1097/MPG.0000000000003127.Online ahead of print.

Current Evidence on the Clinical Relevance of Donor-Specific Antibodies in Paediatric Liver Transplantation

Juliane K Götz 1, Hella Kiene, Imeke Goldschmidt, Norman Junge, Eva-Doreen Pfister, Christoph Leiskau, Rachel M Brown, Stephan Immenschuh, Ulrich Baumann

Abstract

Published on: 
Apr-2021

CLF Intro movie

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