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The role of sebelipase alfa in the treatment of lysosomal acid lipase deficiency https://www.ncbi.nlm.nih.gov/pubmed/28804516 Therap Adv Gastroenterol. 2017 Jul;10(7):553-562. doi: 10.1177/1756283X17705775. Epub 2017 Apr 26. Erwin AL1. Abstract Published on: Jul-2017 Read more	Management of iron overload in hemoglobinopathies https://www.ncbi.nlm.nih.gov/pubmed/2867350 Allali S1, de Montalembert M2, Brousse V1, Chalumeau M3, Karim Z4. ABSTRACT Published on: Jun-2017 Read more
Outcomes in Pediatric Autoimmune Hepatitis and Significance of Azathioprine Metabolites https://www.ncbi.nlm.nih.gov/pubmed/28272159 Sheiko, Melissa A.,†; Sundaram, Shikha S.,†; Capocelli, Kelley E.‡,§; Pan, Zhaoxing\|\|; McCoy, Annette M.,†; Mack, Cara L.,† Journal of Pediatric Gastroenterology & Nutrition: July 2017 - Volume 65 - Issue 1 - p 80–85 Objectives: Autoimmune hepatitis (AIH) is a common pediatric liver disease and long-term remission is usually maintained with azathioprine (AZA). There is no consensus on the target range for AZA active metabolite 6-thioguanine (6-TGN) levels in pediatric AIH. The aim of the present study was to characterize the outcomes of pediatric patients with AIH and determine correlations between AZA dosing or 6-TGN metabolite levels and biochemical remission. Published on: Jun-2017 Read more	A multidisciplinary approach to laparoscopic sleeve gastrectomy among multiethnic adolescents in the United States. https://www.ncbi.nlm.nih.gov/pubmed/28697852 Jaramillo JD1, Snyder E1, Farrales S2, Stevens M2, Wall JK2, Chao S2, Morton J1, Pratt JS2, Hammer L3, Shepard WE3, Bruzoni M4. ABSTRACT BACKGROUND: Childhood obesity has become a serious public health problem in our country with a prevalence that is disproportionately higher among minority groups. Laparoscopic sleeve gastrectomy (LSG) is gaining attention as a safe bariatric alternative for severely obese adolescents. STUDY DESIGN: A retrospective study on morbidly obese adolescents that underwent LSG at our institution from 2009 to 2017. Primary outcomes were weight loss as measured by change in BMI and percent excess weight loss (%EWL) at 1 year after surgery, resolution of comorbidities and occurrence of complications. Published on: Jun-2017 Read more
Low Hepatic Tissue Copper in Pediatric Nonalcoholic Fatty Liver Disease https://www.ncbi.nlm.nih.gov/pubmed/28644356 Mendoza, Michael; Caltharp, Shelley†,‡; Song, Ming§; Collin, Lindsay; Konomi, Juna V.; McClain, Craig J.§; Vos, Miriam B.,‡ Journal of Pediatric Gastroenterology & Nutrition: July 2017 - Volume 65 - Issue 1 - p 89–92 ABSTRACT Objective: Animal models and studies in adults have demonstrated that copper restriction increases severity of liver injury in nonalcoholic fatty liver disease (NAFLD). This has not been studied in children. We aimed to determine if lower tissue copper is associated with increased NAFLD severity in children. Methods: Published on: Jun-2017 Read more	Pediatric autoimmune hepatitis shows a disproportionate decline of regulatory T cells in the liver and of IL-2 in the blood of patients undergoing therapy. https://www.ncbi.nlm.nih.gov/pubmed/28700730 Diestelhorst J1,2, Junge N2, Schlue J3, Falk CS4, Manns MP1, Baumann U2, Jaeckel E1, Taubert R1. ABSTRACT BACKGROUND & AIMS: The autoimmune hepatitis (AIH) is a chronic hepatitis driven by the adaptive immunity that affects all age groups. A functional and numerical regulatory T cell (Treg) defect has been reported in pediatric AIH (pAIH), while an intrahepatic increase in adult AIH (aAIH) patients has been detected in current research findings. METHODS: Therefore, we quantified the intrahepatic numbers of Treg, T and B cells, as well as serum cytokine levels before and during therapy in pAIH. RESULTS: Published on: Jun-2017 Read more
Randomised clinical trial: sofosbuvir and ledipasvir in patients with transfusion-dependent thalassaemia and HCV genotype 1 or 4 infection https://www.ncbi.nlm.nih.gov/pubmed/28660640 Mangia A1, Sarli R2, Gamberini R3, Piga A4, Cenderello G5, Piazzolla V1, Santoro R1, Caruso V6, Quarta A7, Ganga R8, Copetti M9, Forni G10. ABSTRACT BACKGROUND: Patients with thalassaemia major depend on blood transfusions. In Italy, up to 80% of thalassaemia patients bear HCV antibodies due to HCV contaminated transfusions before 1990. Thalassaemia patients with HCV infection have high risk of developing HCC. Treatment based on Pegylated-IFN (Peg-IFN) and Ribavirin (RBV) was limited by relevant side effects. AIM: To evaluate the impact of Sofosbuvir/Ledipasvir (SOF/LDV) fixed dose combination for 12 weeks without RBV, in patients with thalassaemia major and HCV Genotype 1 or 4 (GT1/4). METHODS: Published on: Jun-2017 Read more	Low Birthweight Increases the Likelihood of Severe Steatosis in Pediatric Non-Alcoholic Fatty Liver Disease https://www.ncbi.nlm.nih.gov/pubmed/28555633 Elisabetta Bugianesi, Carla Bizzarri, Chiara Rosso, Antonella Mosca, Nadia Panera, Silvio Veraldi, Andrea Dotta, Germana Giannone, Massimiliano Raponi, Marco Cappa, Anna Alisi and Valerio Nobili ABSTRACT Objectives: Small for gestational age (SGA) is associated with an increased risk of non-alcoholic fatty liver disease (NAFLD). Our aim was to investigate the correlation of birthweight with the severity of liver damage in a large cohort of children with NAFLD. Methods: Published on: Jun-2017 Read more
Successful management of acute liver failure in Italian children: A 16-year experience at a referral centre for paediatric liver transplantation https://www.ncbi.nlm.nih.gov/pubmed/28663066 Di Giorgio A1, Sonzogni A2, Piccichè A3, Alessio G4, Bonanomi E5, Colledan M6, D'Antiga L7. ABSTRACT BACKGROUND: Identifying the causes of acute liver failure (ALF) and predictors of death or liver transplantation (LTX) is crucial to decide its management. We aimed to describe features and outcome of ALF in Italian children. METHODS: Retrospective review of cases presenting between 1996-2012. ALF was defined by high transaminases, INR ≥2.0 regardless of hepatic encephalopathy (HE), no evidence of underlying chronic liver disease. RESULTS: Published on: Jun-2017 Read more	Scrub typhus causing neonatal hepatitis with acute liver failure—A case series https://www.ncbi.nlm.nih.gov/pubmed/28612320 Shailja Vajpayee, R K Gupta, M L Gupta Abstract Neonatal hepatitis with acute liver failure due to varied etiology including various infections is reported in the past. Scrub typhus as a cause of neonatal hepatitis has rarely been reported in literature. A high index of clinical suspicion is required for early diagnosis and timely treatment. Severity and prognosis of the disease varies widely because several different strains of Orientia tsutsugamushi exist with different virulence. Delayed diagnosis can result in complication and significant morbidity and mortality. Here, we report three cases of neonatal hepatitis with acute liver failure caused by scrub typhus to increase awareness. KEYWORDS: Published on: Jun-2017 Read more

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