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CLINICAL, LABORATORIAL AND EVOLUTIONARY ASPECTS OF PEDIATRIC PATIENTS WITH LIVER DISEASE DUE TO ALPHA 1-ANTITRYPSIN DEFICIENCY https://pubmed.ncbi.nlm.nih.gov/38018549/ Alpha 1 antitripsin Observational Study Arq Gastroenterol. 2023 Oct-Dec;60(4):438-449. doi: 10.1590/S0004-2803.230402023-71. CLINICAL, LABORATORIAL AND EVOLUTIONARY ASPECTS OF PEDIATRIC PATIENTS WITH LIVER DISEASE DUE TO ALPHA 1-ANTITRYPSIN DEFICIENCY Mariana Pena Costa 1, Alexandre Rodrigues Ferreira 1, Adriana Teixeira Rodrigues 1, Eleonora Druve Tavares Fagundes 1, Thais Costa Nascentes Queiroz 2 Abstract Background: Alpha 1-antitrypsin deficiency (AATD) is a hereditary codominant autosomal disease. This liver disease ranges from asymptomatic cases to terminal illness, which makes early recognition and diagnosis challenging. It is the main cause of pediatric liver transplantation after biliary atresia. Published on: Nov-2023 Read more	Paediatric research sets new standards for therapy in paediatric and adult cholestasis https://pubmed.ncbi.nlm.nih.gov/38006895/ Cholestasis Review Lancet Child Adolesc Health. 2023 Nov 22:S2352-4642(23)00259-6. doi: 10.1016/S2352-4642(23)00259-6. Online ahead of print. Paediatric research sets new standards for therapy in paediatric and adult cholestasis Rebecca Jeyaraj 1, Eamonn R Maher 2, Deirdre Kelly 3 Abstract Published on: Nov-2023 Read more
Impact of preoperative bloodstream infection on outcomes of pediatric liver transplant recipients treated for acute liver failure https://pubmed.ncbi.nlm.nih.gov/38010711/ transplant Transpl Infect Dis. 2023 Nov 27:e14200. doi: 10.1111/tid.14200. Online ahead of print. Impact of preoperative bloodstream infection on outcomes of pediatric liver transplant recipients treated for acute liver failure Chiaki Baba 1 2, Takanori Funaki 3, Makoto Uranaka 1, Mai Hashiya 1, Jun Ninagawa 1, Seisuke Sakamoto 4, Mureo Kasahara 4, Yasuko Nagasaka 2, Yasuyuki Suzuki 1 2, Shugo Kasuya 1 Abstract Published on: Nov-2023 Read more	LRH-1 agonist DLPC through STAT6 promotes macrophage polarization and prevents parenteral nutrition associated cholestasis in mice https://pubmed.ncbi.nlm.nih.gov/37976384/ Cholestasis Hepatology. 2023 Nov 17. doi: 10.1097/HEP.0000000000000690. Online ahead of print. LRH-1 agonist DLPC through STAT6 promotes macrophage polarization and prevents parenteral nutrition associated cholestasis in mice Swati Ghosh 1, Michael W Devereaux 1, Cuining Liu 1, Ronald J Sokol 1 2 Abstract Background aims: Parenteral nutrition associated cholestasis (PNAC) is important complication in patients with intestinal failure with reduced LRH-1 expression. Here, we hypothesized that LRH-1 activation by its agonist, dilauroylphosphatidylcholine (DLPC), would trigger STAT6 signaling and hepatic macrophage polarization that would mediate hepatic protection in PNAC. Published on: Nov-2023 Read more
Danish children with ZZ-homozygous alpha-1 antitrypsin deficiency are more affected on liver parameters than children with heterozygosity https://pubmed.ncbi.nlm.nih.gov/38009616/ alpha 1 antitrypsin Acta Paediatr. 2023 Nov 27. doi: 10.1111/apa.17048. Online ahead of print. Danish children with ZZ-homozygous alpha-1 antitrypsin deficiency are more affected on liver parameters than children with heterozygosity Christina Louise Winther 1, Sofie Nyrann 1, Rasmus Gaardskaer Nielsen 2, Morten Duno 3, Klaus Birkelund Johansen 4, Thora Wesenberg Helt 5, Vibeke Brix Christensen 1 Abstract Aim: The longitudinal health status of Danish children with alpha-1 antitrypsin deficiency had never previously been characterised. This study aimed to assess the changes in growth, lung and liver function through childhood in these children. Published on: Nov-2023 Read more	Dietary and Nutritional Interventions in Nonalcoholic Fatty Liver Disease in Pediatrics https://pubmed.ncbi.nlm.nih.gov/38004223/ NAFLD Review Nutrients. 2023 Nov 18;15(22):4829. doi: 10.3390/nu15224829. Dietary and Nutritional Interventions in Nonalcoholic Fatty Liver Disease in Pediatrics Camila Farías 1, Camila Cisternas 1, Juan Cristobal Gana 2, Gigliola Alberti 2, Francisca Echeverría 3, Luis A Videla 4, Lorena Mercado 1, Yasna Muñoz 1 5, Rodrigo Valenzuela 2 Abstract Published on: Nov-2023 Read more
Clinical profile of re-hospitalizations in pediatric kidney and liver transplant recipients https://pubmed.ncbi.nlm.nih.gov/38009427/ Transplant Pediatr Transplant. 2023 Nov 27:e14658. doi: 10.1111/petr.14658. Online ahead of print. Clinical profile of re-hospitalizations in pediatric kidney and liver transplant recipients Adi Shohet 1, Noa Ziv 1 2, Rachel Gavish 1 2, Orly Haskin 2 3, Hadas Alfandary 2 3, Orith Waisbourd-Zinman 2 4, Yael Mozer-Glassberg 2 4, Irit Krause 1 2 Abstract Background: Solid organ transplantation has evolved in recent decades, resulting in a rise in patient and graft survival. Frequent hospitalizations affect graft function, patients' health, and quality of life. This study characterizes the frequency and causes of post-transplant hospitalizations among pediatric recipients. Published on: Nov-2023 Read more	Multidisciplinary management of children with acute liver failure - Report on 104 children treated in single center https://pubmed.ncbi.nlm.nih.gov/37983943/ Acute liver failure Pediatr Transplant. 2023 Nov 20:e14654. doi: 10.1111/petr.14654. Online ahead of print. Multidisciplinary management of children with acute liver failure - Report on 104 children treated in single center Piotr Kaliciński 1, Ryszard Grenda 2, Marek Szymczak 1, Elżbieta Pietraszek 3, Joanna Pawłowska 4 Abstract Background: Pediatric acute liver failure (PALF) is one of the most demanding emergencies in hepatology, intensive care, and for transplant team. This report describes the clinical pattern, diagnostic and therapeutic modalities in children with ALF considered at risk of death without liver transplantation, basing on a long-term experience of the pediatric transplant center. Published on: Nov-2023 Read more
Demystifying the global outbreak of severe acute hepatitis of unknown aetiology in children: A systematic review and meta-analysis https://pubmed.ncbi.nlm.nih.gov/38007049/ Hepatitis J Infect. 2023 Nov 24:S0163-4453(23)00580-7. doi: 10.1016/j.jinf.2023.11.011.Online ahead of print. Demystifying the global outbreak of severe acute hepatitis of unknown aetiology in children: A systematic review and meta-analysis Julie Phan 1, Guy D Eslick 2, Elizabeth J Elliott 1 Abstract Published on: Nov-2023 Read more	Healthy Patients With AKR1D1 Mutation Not Requiring Primary Bile Acid Therapy: A Case Series https://pubmed.ncbi.nlm.nih.gov/38034430/ Cholestasis JPGN Rep. 2023 Oct 9;4(4):e372. doi: 10.1097/PG9.0000000000000372. eCollection 2023 Nov. Healthy Patients With AKR1D1 Mutation Not Requiring Primary Bile Acid Therapy: A Case Series Akihiko Kimura 1 2, Jun Mori 3, Anh-Hoa Nguyen Pham 4, Kim-Oanh Bui Thi 4, Hajime Takei 2, Tsuyoshi Murai 5, Hisamitsu Hayashi 6, Hiroshi Nittono 2 Abstract Published on: Nov-2023 Read more

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