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Low-GGT intrahepatic cholestasis associated with biallelic USP53 variants: clinical, histological, and ultrastructural characterization. https://www.ncbi.nlm.nih.gov/pubmed/32124521 Cholestasis Liver Int. 2020 Mar 2. doi: 10.1111/liv.14422. [Epub ahead of print] Low-GGT intrahepatic cholestasis associated with biallelic USP53 variants: clinical, histological, and ultrastructural characterization. Zhang J1,2, Yang Y1,2, Gong JY1, Li LT2, Li JQ1, Zhang MH1, Lu Y2, Xie XB2, Hong YR3, Zhang YU3, Knisely AS4, Wang JS2. Abstract BACKGROUND AND AIMS: In about 20% of children with cholestasis and normal or low serum gamma-glutamyltransferase (GGT) activity, no etiology is identified. We sought new genes implicated in pediatric hepatobiliary disease. METHODS: Published on: Feb-2020 Read more	Evaluation of frequency of autoimmune hepatitis autoantibodies in children with type 1 diabetes https://www.ncbi.nlm.nih.gov/pubmed/32097394 Autoimmune hepatitis Rev Gastroenterol Peru. 2019 Oct-Dec;39(4):344-347. Evaluation of frequency of autoimmune hepatitis autoantibodies in children with type 1 diabetes. Honar N1, Karamizadeh Z2, Vazirian MV3, Jahanshahi K4, Manouchehri A5, Dehghani SM1, Javaherizadeh H6. Abstract BACKGROUND: Diabetes mellitus type 1 (T1DM) is one of the childhood diseases with growing prevalence. Various accompanying autoimmune diseases were seen with type 1 diabetes. The most common autoimmune diseases with T1DM are autoimmune thyroiditis and celiac disease. In some reports, autoimmune hepatitis has been reported in association with DM-1. OBJECTIVES: Published on: Feb-2020 Read more
Exome Sequencing in Individuals with Isolated Biliary Atresia https://www.ncbi.nlm.nih.gov/pubmed/32066793 Biliary atresia Sci Rep. 2020 Feb 17;10(1):2709. doi: 10.1038/s41598-020-59379-4. Exome Sequencing in Individuals with Isolated Biliary Atresia. Rajagopalan R1, Tsai EA1,2,3, Grochowski CM1,4, Kelly SM5, Loomes KM6,7, Spinner NB1,8, Devoto M9,10,11. Abstract Published on: Feb-2020 Read more	Cystic echinococcosis in children: high frequency of multiple organ involvement in North of Iran. https://www.ncbi.nlm.nih.gov/pubmed/32108011 Infect Disord Drug Targets. 2020 Feb 27. doi: 10.2174/1871526520666200228104316. [Epub ahead of print] Cystic echinococcosis in children: high frequency of multiple organ involvement in North of Iran. Mohammadi M1, Mamishi S2, Pourakbari B2, Faraz Z1, Khodabandeh M3, Mahmoudi S2. Abstract INTRODUCTION: Echinococcosis as an important zoonotic infection has a major worldwide distribution. Pediatric echinococcosis has still become a health concerning issue in developing countries specifically in the Middle East and Iran. The aim of this study was to investigate the human echinococcosis in children in the northern Iran. MATERIAL AND METHODS: Published on: Feb-2020 Read more
Hepatic Failure following Metronidazole in Children with Cockayne Syndrome. https://www.ncbi.nlm.nih.gov/pubmed/32082677 Drug induced Case Rep Pediatr. 2020 Jan 29;2020:9634196. doi: 10.1155/2020/9634196. eCollection 2020. Hepatic Failure following Metronidazole in Children with Cockayne Syndrome. Ataee P1, Karimi A2, Eftekhari K3. Author information Abstract Published on: Feb-2020 Read more	Conservative Management of Pneumatosis Intestinalis and Portal Venous Gas After Pediatric Liver Transplantation. https://www.ncbi.nlm.nih.gov/pubmed/32122661 liver transplant Transplant Proc. 2020 Feb 28. pii: S0041-1345(19)30964-9. doi: 10.1016/j.transproceed.2020.01.010. [Epub ahead of print] Conservative Management of Pneumatosis Intestinalis and Portal Venous Gas After Pediatric Liver Transplantation. Ryan JL1, Dandridge LM1, Andrews WS2, Daniel JF3, Fischer RT3, Rivard DC4, Wieser AB2, Kane BJ5, Hendrickson RJ6. Abstract BACKGROUND: Published on: Feb-2020 Read more
Thrombin dynamics in children with liver disease or extrahepatic portal vein obstruction or shunt. https://www.ncbi.nlm.nih.gov/pubmed/32087412 Thromb Res. 2020 Feb 13;188:65-73. doi: 10.1016/j.thromres.2020.02.008. [Epub ahead of print] Thrombin dynamics in children with liver disease or extrahepatic portal vein obstruction or shunt. Beattie W1, Kremers R2, Magnusson M3, Peters T2, de Laat B2, Hardikar W4, Monagle P5, Ignjatovic V6. Abstract INTRODUCTION: Published on: Feb-2020 Read more	Characteristics and Outcomes of Autoimmune Hepatitis from a Tertiary Paediatric Centre, Cape Town, South Africa. https://www.ncbi.nlm.nih.gov/pubmed/31943108 Autoimmune hepatitis J Trop Pediatr. 2020 Jan 14. pii: fmz088. doi: 10.1093/tropej/fmz088. [Epub ahead of print] Characteristics and Outcomes of Autoimmune Hepatitis from a Tertiary Paediatric Centre, Cape Town, South Africa. Yassin S1, De Lacy R1, Pillay K2, Goddard E1. Abstract OBJECTIVES: To describe the clinical characteristics, biochemical and histological features, outcomes and predictors of prognosis of children with autoimmune hepatitis (AIH) from a paediatric centre in South Africa. METHODS: Published on: Jan-2020 Read more
NR1H4-related Progressive Familial Intrahepatic Cholestasis 5: Further Evidence for Rapidly Progressive Liver Failure. https://www.ncbi.nlm.nih.gov/pubmed/32097368 PFIC J Pediatr Gastroenterol Nutr. 2020 Feb 24. doi: 10.1097/MPG.0000000000002670. [Epub ahead of print] NR1H4-related Progressive Familial Intrahepatic Cholestasis 5: Further Evidence for Rapidly Progressive Liver Failure. Himes RW1, Mojarrad M2,3, Eslahi A2,3, Finegold MJ4, Maroofian R5, Moore DD6. Author information Abstract Published on: Feb-2020 Read more	Etiology of liver dysfunction after liver transplantation in children with metabolic disorders https://www.ncbi.nlm.nih.gov/pubmed/31957215 Hepatol Res. 2020 Jan 19. doi: 10.1111/hepr.13487. [Epub ahead of print] Etiology of liver dysfunction after liver transplantation in children with metabolic disorders. Irie R1,2, Nakazawa A3, Sakamoto S4, Takeda M4, Yanagi Y4, Shimizu S4, Uchida H4, Fukuda A4, Horikawa R5, Kasahara M4. Abstract AIM: It has been reported that the long-term outcome for children with metabolic disorders after liver transplantation (LT) is excellent. However, there are several reports citing LT patients with metabolic disorders developing liver dysfunction early after LT. We examined the pathogenesis of liver dysfunction observed after LT in recipients with metabolic disorders at the National Center for Child Health and Development. METHODS: Published on: Jan-2020 Read more

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