Event Videos

https://pubmed.ncbi.nlm.nih.gov/36828986/ NAFLD

J Endocrinol Invest. 2023 Feb 24.
doi: 10.1007/s40618-023-02041-3. Online ahead of print.

Association between non-alcoholic fatty liver disease and subclinical hypothyroidism in children with obesity

A Di Sessa 1, N Cembalo Sambiase Sanseverino 2, R F De Simone 2, M M Marrapodi 2, G Cirillo 2, G R Umano 2, S Guarino 2, A Papparella 2, E Miraglia Del Giudice 2, P Marzuillo 2

Abstract

Published on: 
Feb-2023

https://pubmed.ncbi.nlm.nih.gov/36800295/

J Pediatr Gastroenterol Nutr. 2023 Feb 20;e003744.
doi: 10.1097/MPG.0000000000003744.Online ahead of print.

Accuracy and Precision of Point-of-Care International Normalized Ratio in Patients with Liver Disease

Tevyn Shadlyn 1, Mary Bauman 2, Puneeta Tandon 3, Jason Yap 4, Patricia Kawada 5
Affiliations expand

Abstract
Objective: To determine if the CoaguChek® XS-Pro Point-of-Care (POC) device can accurately and precisely measure the international normalized ratio (INR) compared with the gold standard laboratory INR in pediatric and adult patients with liver disease.

Published on: 
Feb-2023

https://pubmed.ncbi.nlm.nih.gov/36828986/ NAFLD

J Endocrinol Invest. 2023 Feb 24.
doi: 10.1007/s40618-023-02041-3. Online ahead of print.

Association between non-alcoholic fatty liver disease and subclinical hypothyroidism in children with obesity

A Di Sessa 1, N Cembalo Sambiase Sanseverino 2, R F De Simone 2, M M Marrapodi 2, G Cirillo 2, G R Umano 2, S Guarino 2, A Papparella 2, E Miraglia Del Giudice 2, P Marzuillo 2

Abstract

Published on: 
Feb-2023

https://pubmed.ncbi.nlm.nih.gov/36645229/ Biliary atresia

Hepatology. 2023 Jan 3.
doi: 10.1097/HEP.0000000000000029. Online ahead of print.

Liver-restricted deletion of the biliary atresia candidate gene Pkd1l1 causes bile duct dysmorphogenesis and ciliopathy.

Dominick J Hellen 1, Ashley Bennett, Sudarshan Malla, Caroline Klindt, Anuradha Rao, Paul A Dawson, Saul J Karpen

Abstract

Background and aims: A recent multicenter genetic exploration of the biliary atresia splenic malformation syndrome identified mutations in the ciliary gene PKD1L1 as candidate etiologic contributors. We hypothesized that deletion of Pkd1l1 in developing hepatoblasts would lead to cholangiopathy in mice.

Published on: 
Jan-2023

https://pubmed.ncbi.nlm.nih.gov/36822480/ Acute liver failure

J Hepatol. 2023 Feb 21;S0168-8278(23)00101-0.
doi: 10.1016/j.jhep.2023.02.013.Online ahead of print.

Outbreak of indeterminate acute liver failure in children with adenoviraemia - Not a new disease

Barath Jagadisan 1, Anita Verma 2, Maesha Deheragoda 3, Akash Deep 4, Tassos Grammatikopoulos 5, Malur Sudhanva 6, Sanjay Bansal 7, Nedim Hadzic 8, Sunitha Vimalesvaran 9, Nigel Heaton 10, Anil Dhawan 11

Abstract

Published on: 
Feb-2023

https://pubmed.ncbi.nlm.nih.gov/36816379/ Cholangitis

Front Pediatr. 2023 Feb 3;11:1094895.
doi: 10.3389/fped.2023.1094895. eCollection 2023.

Neonatal sclerosing cholangitis with novel mutations in DCDC2 (doublecortin domain-containing protein 2) in Chinese children

Xia Wei 1, Yuan Fang 1, Jian-She Wang 2, Yi-Zhen Wang 1, Yuan Zhang 3, Kuerbanjiang Abuduxikuer 2, Lian Chen 4

Abstract

Background: Neonatal sclerosing cholangitis (NSC) is a rare and severe autosomal recessive inherited liver disease with mutations in DCDC2, commonly requiring liver transplantation (LT) for decompensated biliary cirrhosis in childhood.

Published on: 
Feb-2023

https://pubmed.ncbi.nlm.nih.gov/36675485/ NAFLD

J Clin Med. 2023 Jan 10;12(2):557.
doi: 10.3390/jcm12020557.

The Burden of Non-Alcoholic Fatty Liver Disease in Adolescents with Polycystic Ovary Syndrome: A Case-Control Study

Aikaterini Giannouli 1, Vasiliki Efthymiou 1, Marianna Konidari 2, Iliana Mani 3, Leon Aravantinos 4, Spyridon P Dourakis 3, Aristeidis Antoniou 2, Efthymios Deligeoroglou 5, Flora Bacopoulou 1

Abstract

Published on: 
Jan-2023

https://pubmed.ncbi.nlm.nih.gov/36816376/ Metabolic liver disease

Front Pediatr. 2023 Feb 3;11:1084336.
doi: 10.3389/fped.2023.1084336. eCollection 2023.

Clinical features and enzyme replacement therapy in 10 children with Fabry disease

Qian Li 1 2, Jing Wang 1 2, Minle Tian 3, Zhenle Yang 1 2, Lichun Yu 1 2, Suwen Liu 1 2, Cong Wang 1 2, Xiaoyuan Wang 1 2, Shuzhen Sun 1 2

Abstract

Objective: To summarize the clinical features, diagnosis and enzyme replacement therapy(ERT) of Fabry disease (FD) in children.
Methods: The clinical data, laboratory tests, genetic variations and treatment of 10 FD children diagnosed in Shandong Provincial Hospital from September 2020 to June 2022 were retrospectively analyzed.

Published on: 
Feb-2023

https://pubmed.ncbi.nlm.nih.gov/36692476/ Biliary atresia

Hepatology. 2023 Jan 3.

doi: 10.1097/HEP.0000000000000048. Online ahead of print.

Serum FGF19 predicts outcomes of Kasai portoenterostomy in biliary atresia.

Iiris Nyholm 1 2, Maria Hukkinen 1 2, Marjut Pihlajoki 1, Joseph R Davidson 3 4, Athanasios Tyraskis 4, Jouko Lohi 5, Päivi Heikkilä 5, Satu Hänninen 5, Noora Andersson 5, Katja Eloranta 1, Olli Carpén 5, Markku Heikinheimo 1 6, Mark Davenport 4, Mikko P Pakarinen 1 2 7

Abstract

Background and aims: Outcomes after Kasai portoenterostomy (KPE) for biliary atresia remain highly variable for unclear reasons. As reliable early biomarkers predicting KPE outcomes are lacking, we studied the prognostic value of FGF19.

Published on: 
Jan-2023

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