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Hepatic Parenchymal Injury in Crigler-Najjar Type I https://www.ncbi.nlm.nih.gov/pubmed/29176474 Neonatal Jaundice J Pediatr Gastroenterol Nutr. 2017 Nov 22. doi: 10.1097/MPG.0000000000001843. [Epub ahead of print] Mitchell E, Ranganathan S, McKiernan P, Squires RH, Strauss K, Soltys K, Mazariegos G, Squires JE. Abstract BACKGROUND: Crigler-Najjar syndrome type I (CNI) arises from biallelic variants of UGT1A1 that abrogate UGT1A1 activity resulting in unconjugated hyperbilirubinemia. Historically, liver parenchyma in CNI was considered structurally and histologically normal. Recent review of CNI liver explants revealed fibrosis. Our aim was to investigate the association between hepatic histology and disease phenotype in CNI. METHODS: Published on: Nov-2017 Read more	Non-alcoholic fatty liver disease and its associations among adolescents in an urban, Sri Lankan community https://www.ncbi.nlm.nih.gov/pubmed/29187144 NAFLD BMC Gastroenterol. 2017 Nov 29;17(1):135. doi: 10.1186/s12876-017-0677-7. Rajindrajith S, Pathmeswaran A, Jayasinghe C, Kottahachchi D, Kasturiratne A, de Silva ST, Niriella MA, Dassanayake AS, de Silva AP, de Silva HJ. Abstract BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is a common problem across the world. We aimed to determine the prevalence of NAFLD and its associations in Sri Lankan adolescents living in an urban Sri Lankan community. METHOD: Published on: Nov-2017 Read more
Noninvasive Methods of Predicting Large Esophageal Varices in Children With Intrahepatic Portal Hypertension https://www.ncbi.nlm.nih.gov/pubmed/29176477 Portal Hypertension J Pediatr Gastroenterol Nutr. 2017 Nov 22. doi: 10.1097/MPG.0000000000001841. [Epub ahead of print] Adami MR, Kieling CO, Schwengber FP, Hirakata VN, Vieira SMG. Abstract OBJECTIVE: Esophageal variceal bleeding is a severe complication of portal hypertension. The standard diagnostic screening test and therapeutic procedure for esophageal varices is endoscopy, which is invasive in pediatric patients. This study aimed to evaluate the role of noninvasive parameters as predictors of large varices in children with intrahepatic portal hypertension. METHODS: Published on: Nov-2017 Read more	Quality of Life in Adolescents with Hepatitis C Treated with Sofosbuvir and Ribavirin. https://www.ncbi.nlm.nih.gov/pubmed/29193603 J Viral Hepat. 2017 Nov 28. doi: 10.1111/jvh.12830. [Epub ahead of print] Younossi ZM, Stepanova M, Schwarz KB, Wirth S, Rosenthal P, Gonzalez-Peralta R, Murray K, Henry L, Hunt S. Abstract Published on: Nov-2017 Read more
ATP7B Mutation Detection and Pathogenicity Analysis: One Atypical Case of Wilson's Disease with Adrenocortical Insufficiency. https://www.ncbi.nlm.nih.gov/pubmed/29181760 Wilson disease J Mol Neurosci. 2017 Nov 28. doi: 10.1007/s12031-017-0997-7. [Epub ahead of print] Liu M, Jin M, Chen X, Wan B, Guo Y, Sheng M, Chen L, Zhao L, Huang D, Li Y. Abstract Published on: Nov-2017 Read more	Type 1 primary hyperoxaluria: From childhood to adult, how to manage adequately medical therapy compliance? https://www.ncbi.nlm.nih.gov/pubmed/29198962 Nephrol Ther. 2017 Nov 29. pii: S1769-7255(17)30583-7. doi: 10.1016/j.nephro.2017.06.004. [Epub ahead of print] Leflot M, Krzesinski JM, Collard L, Thomas A, Ghuysen MS. Abstract Published on: Nov-2017 Read more
Congenital vascular anomalies of the liver https://www.ncbi.nlm.nih.gov/pubmed/29183422 S Afr Med J. 2017 Nov 6;107(10):12130. Tyraskis A, Durkin N, Davenport M. Abstract Congenital vascular anomalies of the liver include a range of malformations of the portal venous, hepatic arterial and venous systems. Congenital portosystemic shunts and arteriovenous malformations make up the two most frequent such malformations. While infantile haemangiomas of the liver, endothelial tumours characterised by vascular proliferation should also be considered, as a proportion of them form prenatally. Evidence to support treatment strategies for these infants and children has been mainly based on small case series. In this review, we explore classification, clinical presentation, investigation and treatment strategies. Published on: Nov-2017	Neurological Crises after Discontinuation of Nitisinone (NTBC) Treatment in Tyrosinemia. https://www.ncbi.nlm.nih.gov/pubmed/29201126 Iran J Child Neurol. 2017 Fall;11(4):66-70. Honar N, Shakibazad N, Serati Shirazi Z, Dehghani SM, Inaloo S. Abstract OBJECTIVE: Tyrosinemia type 1 is a hereditary disorder with liver, kidney and nervous system involvement. Neurological crises can occur in tyrosinemic patients without treatment or when treatment stops. Here we report three children that developed diaphragmatic paralysis after discontinuation of nitisinone. In patients with tyrosinemia type 1, combined treatment with nitisinone and a low-tyrosine diet have prevented neurological crises. The purpose of this article was to express the importance of taking nitisinone (NTBC) for tyrosinemia diseases and risks of inadvertent discontinuation. MATERIALS & METHODS: Published on: Nov-2017 Read more
Outcomes of Kasai hepatoportoenterostomy in children with biliary atresia in Johannesburg, South Africa https://www.ncbi.nlm.nih.gov/pubmed/29183423 Biliary atresia S Afr Med J. 2017 Nov 6;107(10):12131. De Maayer T, Lala SG, Loveland J, Okudo G, Mohanlal R, Hajinicolaou C. Abstract BACKGROUND: Without timely surgical intervention, most children with biliary atresia (BA) are not expected to live beyond 2 years of age. The initial intervention, the Kasai hepatoportoenterostomy (KPE), aims to achieve biliary drainage. Liver transplantation (LT) is performed if jaundice fails to clear or when biliary cirrhosis occurs. In under-resourced South African (SA) academic state hospitals, KPE procedures are the standard of care for the majority of children with BA, but LT is becoming more routinely available. OBJECTIVES: Published on: Nov-2017 Read more	Effects of activated carbon N-acetylcysteine sustained-release microcapsule on dipeptidyl peptidase IV expression in young rats with non-alcoholic fatty liver disease https://www.ncbi.nlm.nih.gov/pubmed/29201174 NAFLD Exp Ther Med. 2017 Nov;14(5):4737-4744. doi: 10.3892/etm.2017.5128. Epub 2017 Sep 19. Zhou H, Shi T, Yan J, Chen X, Liao L, Zhao S, Fang H, Zhuang R. Abstract Published on: Nov-2017 Read more

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