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Liver-restricted deletion of the biliary atresia candidate gene Pkd1l1 causes bile duct dysmorphogenesis and ciliopathy. 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 Read more	Rising NAFLD and Metabolic Severity During the Sars-CoV-2 Pandemic among Children with Obesity in the United States https://pubmed.ncbi.nlm.nih.gov/36694381/ Covid-19 Obesity (Silver Spring). 2023 Jan 24. doi: 10.1002/oby.23728. Online ahead of print. Rising NAFLD and Metabolic Severity During the Sars-CoV-2 Pandemic among Children with Obesity in the United States Aaron L Slusher 1, Pamela Hu 1, Stephanie Samuels 1, Fuyuze Tokoglu 2, Jessica Lat 1, Zhongyao Li 1, Michele Alguard 1, Jordan Strober 3, Daniel Vatner 3, Veronika Shabanova 1, Sonia Caprio 1 Abstract Background: Non-alcoholic fatty liver disease (NAFLD), the most common liver disease among youth with obesity, precedes more severe metabolic and liver diseases. However, the impact of the Sars-CoV-2 global pandemic on the prevalence and severity of NAFLD and the associated metabolic phenotype among youth with obesity is unknown. Published on: Jan-2023 Read more
MAFLD and Celiac Disease in Children https://pubmed.ncbi.nlm.nih.gov/36675276/ Review Int J Mol Sci. 2023 Jan 16;24(2):1764. doi: 10.3390/ijms24021764. MAFLD and Celiac Disease in Children Serena Scapaticci 1, Annamaria Venanzi 1, Francesco Chiarelli 1, Cosimo Giannini 1 Abstract Published on: Jan-2023 Read more	Innate immune cell dysfunction and systemic inflammation in children with chronic liver diseases undergoing transplantation. https://pubmed.ncbi.nlm.nih.gov/36695617/ liver transplant Am J Transplant. 2023 Jan;23(1):26-36. doi: 10.1016/j.ajt.2022.09.004. Epub 2023 Jan 11. Innate immune cell dysfunction and systemic inflammation in children with chronic liver diseases undergoing transplantation. Krupa R Mysore 1, Sunil Kannanganat 2, Jeremy M Schraw 3, Philip J Lupo 3, John A Goss 4, Kenneth D R Setchell 5, Farrah Kheradmand 6, Xian C Li 7, Benjamin L Shneider 8 Abstract Published on: Jan-2023 Read more
The Burden of Non-Alcoholic Fatty Liver Disease in Adolescents with Polycystic Ovary Syndrome: A Case-Control Study 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 Read more	Protocol biopsies in pediatric liver transplantation recipients improve graft histology and personalize immunosuppression https://pubmed.ncbi.nlm.nih.gov/36705669/ liver transplant J Pediatr Gastroenterol Nutr. 2023 Jan 26. doi: 10.1097/MPG.0000000000003707. Online ahead of print. Protocol biopsies in pediatric liver transplantation recipients improve graft histology and personalize immunosuppression Eva Karbaum 1, Sören Weidemann 2, Enke Grabhorn 1, Lutz Fischer 3, Uta Herden 3, Jun Oh 1, Andrea Briem-Richter 1, Dorothee Krebs-Schmitt 1 Abstract Objectives: Protocol liver biopsies (PLB) are part of the follow-up program at many pediatric liver transplant centers, but the impact on clinical decision-making and allograft histology following adjustments of immunosuppression (IS) after PLB has not been thoroughly analyzed. Published on: Jan-2023 Read more
Serum FGF19 predicts outcomes of Kasai portoenterostomy in biliary atresia. 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 Read more	Long-term outcomes of patients with type 1 or 2 autoimmune hepatitis presenting in childhood https://pubmed.ncbi.nlm.nih.gov/36708813/ Autoimmune hepatitis J Hepatol. 2023 Jan 25;S0168-8278(23)00025-9. doi: 10.1016/j.jhep.2023.01.013. Online ahead of print. Long-term outcomes of patients with type 1 or 2 autoimmune hepatitis presenting in childhood Giuseppe Maggiore 1, Olivier Bernard 2, Antonella Mosca 3, Eric Ballot 4, Catherine Johanet 4, Emmanuel Jacquemin 5 Abstract Background & aims: In children with autoimmune hepatitis, issues include the particularities of type 2 hepatitis regarding outcome, the possibility of and criteria for attempting withdrawal of treatment and the long-term outcome. We report our experience on these issues. Published on: Jan-2023 Read more
Late airway complications following pediatric liver transplantation: A case series https://pubmed.ncbi.nlm.nih.gov/36694298/ Liver transplant Case Reports Pediatr Transplant. 2023 Jan 24;e14473. doi: 10.1111/petr.14473. Online ahead of print. Late airway complications following pediatric liver transplantation: A case series Vivek Rajasekaran 1 2, Craig McCaffer 3, Jonathan Bishop 1, Graeme Van Der Meer 3, Edward C Toll 3, Helen Evans 1 2 Abstract Background: Late airway complications, as consequence of immunosuppression following pediatric liver transplantation are uncommonly reported. Methods: In this retrospective case series, we describe two young children presenting with symptoms of airway obstruction, secondary to differing pathologies in the supraglottic airway, as a result of immunosuppression following liver transplantation. Published on: Jan-2023 Read more	Is Prolonged Copper Restriction Needed in Pediatric Wilson's Disease? https://pubmed.ncbi.nlm.nih.gov/36511610/ wilson disease Turk J Gastroenterol. 2022 Dec 13. doi: 10.5152/tjg.2022.22216. Online ahead of print. Is Prolonged Copper Restriction Needed in Pediatric Wilson's Disease? Hayriye Hizarcıoğlu-Gülşen 1, Pınar Şimşek Onat 1, Damla Yıldırım 2, Duygu Demirtaş 3, Meryem S Boyraz 4, Mehmet A Göktaş 4, Hülya Demir 1, Hasan Özen 1, İnci Nur Saltık Temizel 1 Abstract Published on: Dec-2022 Read more

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