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Pediatric liver disease

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https://www.readbyqxmd.com/read/28632826/-pyogenic-liver-abscess-in-children-clinical-experience-in-a-pediatric-reference-center
#1
Carolina Carballo, Claudia Cazes, Mariana Matsuda, M Laura Praino, Noemí Rivas, Eduardo López
INTRODUCTION: Liver abscess is a serious and an uncommon pediatric disease. OBJECTIVE AND METHOD: Description of 20 pediatric patients with liver abscess admitted in "Ricardo Gutierrez" Children's Hospital, a Tertiary Reference Center of Buenos Aires, between 2009 and 2015. RESULTS: The hospitalization rate was 35/100.000 admissions. Median age was 5 years old, male/female ratio 4/1. Five patients (25%) had predisposing factors. Median days at diagnosis were 12...
April 2017: Revista Chilena de Infectología: órgano Oficial de la Sociedad Chilena de Infectología
https://www.readbyqxmd.com/read/28629152/the-association-between-pediatric-nafld-and-common-genetic-variants
#2
REVIEW
Giuseppina Rosaria Umano, Mariangela Martino, Nicola Santoro
Non-alcoholic fatty liver disease (NAFLD) is one of the most common complications of obesity. Several studies have shown that genetic predisposition probably plays an important role in its pathogenesis. In fact, in the last few years a large number of genetic studies have provided compelling evidence that some gene variants, especially those in genes encoding proteins regulating lipid metabolism, are associated with intra-hepatic fat accumulation. Here we provide a comprehensive review of the gene variants that have affected the natural history of the disease...
June 18, 2017: Children
https://www.readbyqxmd.com/read/28612751/fighting-mortality-in-the-waiting-list-liver-transplantation-in-north-america-europe-and-asia
#3
Daniel Zamora-Valdes, Pilar Leal-Leyte, Peter T W Kim, Giuliano Testa
Liver disease is a major cause of mortality worldwide. Liver transplantation (LT) is the most effective treatment for end stage liver disease. Available resources and social circumstances have led to different ways of implementing LT around the world. The experience with pediatric LT corroborates the hypothesis that a combination of surgical strategies can be beneficial. The goal of this manuscript is to describe the strategies used by LT centers in North America, Europe and Asia and how these strategies can be applied to reduce waitlist mortality and increase access to LT...
August 1, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28606575/good-adherence-to-the-mediterranean-diet-reduces-the-risk-for-nash-and-diabetes-in-pediatric-patients-with-obesity-the-results-of-an-italian-study
#4
Claudia Della Corte, Antonella Mosca, Andrea Vania, Arianna Alterio, Salvatore Iasevoli, Valerio Nobili
OBJECTIVE: In the last decade, it was demonstrated that the Mediterranean diet (MD) represents an ideal diet for all age groups and has an important role in the prevention of metabolic and cardiovascular diseases, as well as nonalcoholic fatty liver disease (NAFLD). The aim of the present study was to analyze the association between adherence to the MD and NAFLD, with laboratory and histologic evaluation, in a group of children and adolescents with obesity. METHODS: We enrolled 243 patients with obesity referred to our department from March 2014 to November 2015...
July 2017: Nutrition
https://www.readbyqxmd.com/read/28604513/severe-sarcopenia-and-increased-fat-stores-in-pediatric-patients-with-liver-kidney-or-intestine-failure
#5
Richard S Mangus, Weston J Bush, Chandrashekhar A Kubal, Christina Miller
OBJECTIVES: Malnutrition and wasting predict clinical outcomes in children with severe chronic illness. Objectively calculated malnutrition in children with end-stage organ failure has not been well studied. This analysis compares children with kidney, liver or intestine failure to healthy controls to quantitate the disparity in muscle and fat stores. METHODS: Children younger than age 19 with end stage liver, kidney or intestine failure and with pre-transplant computed tomography (CT) imaging were selected from the transplant database...
June 9, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28600116/features-of-nonalcoholic-steatohepatitis-in-severely-obese-children-and-adolescents-undergoing-sleeve-gastrectomy
#6
Aayed Alqahtani, Mohamed Elahmedi, Khalid Alswat, Maha Arafah, Mosa Fagih, Jaehoon Lee
BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is an underrecognized co-morbidity of obesity. The characteristic features and severity of NAFLD in severe childhood obesity remain unknown. OBJECTIVES: To investigate features of NAFLD in obese children and identify predictors of significant disease. SETTING: Academic center with a standardized pathway for pediatric bariatric surgery and a dedicated multidisciplinary team. DESIGN: This is a baseline cross-sectional analysis for a data set obtained from a prospective clinical outcome study that included severely obese children (≤14 yr of age) and adolescents (14-21 yr of age) who underwent laparoscopic sleeve gastrectomy between March 2008 and March 2015...
April 7, 2017: Surgery for Obesity and related Diseases: Official Journal of the American Society for Bariatric Surgery
https://www.readbyqxmd.com/read/28598585/epithelioid-hemangioendotheliomas-of-the-liver-and-lung-in-children-and-adolescents
#7
REVIEW
Simone Hettmer, Geoffroy Andrieux, Jochen Hochrein, Philipp Kurz, Jochen Rössler, Silke Lassmann, Martin Werner, Nikolas von Bubnoff, Christoph Peters, Ewa Koscielniak, Monika Sparber-Sauer, Charlotte Niemeyer, Thomas Mentzel, Hauke Busch, Melanie Boerries
Epithelioid hemangioendothelioma (EHE) is a rare, vascular sarcoma. Visceral forms arise in the liver/ lungs. We review the clinical and molecular phenotype of pediatric visceral EHE based on the case of a 9-year-old male child with EHE of the liver/lungs. His tumor expressed the EHE-specific fusion oncogene WWTR1-CAMTA1. Molecular characterization revealed a low somatic mutation rate and activated interferon signaling, angiogenesis regulation, and blood vessel remodeling. After polychemotherapy and resection of lung tumors, residual disease remained stable on oral lenalidomide...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28598410/pediatric-non-alcoholic-fatty-liver-disease
#8
REVIEW
Haley Bush, Pegah Golabi, Zobair M Younossi
Abstract: With the increase in the prevalence of obesity, non-alcoholic fatty liver disease (NAFLD) has become among the leading causes of chronic liver disease in the pediatric age group. Once believed to be a "two-hit process", it is now clear that the actual pathophysiology of NAFLD is complex and involves multiple pathways. Moreover, NAFLD is not always benign, and patients with non-alcoholic steatohepatitis (NASH) are at increased risk of developing advanced stages of liver disease. It has also been shown that NAFLD is not only a liver disease, but is also associated with multiple extrahepatic manifestations, including cardiovascular diseases, type 2 diabetes, and low bone mineral density...
June 9, 2017: Children
https://www.readbyqxmd.com/read/28590589/living-donor-liver-transplantation-for-post-kasai-biliary-atresia-analysis-of-pretransplant-predictors-of-outcomes-in-infants
#9
Toshihiro Kitajima, Seisuke Sakamoto, Kengo Sasaki, Hajime Uchida, Soichi Narumoto, Akinari Fukuda, Satoshi Teramukai, Shinji Uemoto, Mureo Kasahara
After decades of dramatic surgical innovations in pediatric living donor liver transplantation (LDLT), LDLT for biliary atresia (BA) still poses various challenges. This study reviewed our experience with LDLT for children with post-Kasai BA and evaluated outcomes and prognostic factors. From 2005 to 2016, 168 post-Kasai BA LDLT patients were enrolled and divided into three groups by age. Patient characteristics and perioperative data were compared. Predictors of morbidity and mortality following LDLT were analyzed in 93 infants...
June 7, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28588171/clinical-and-laboratory-predictors-of-esophageal-varices-in-children-with-chronic-liver-disease
#10
M I Hasan, M Rukunuzzaman, M Nurullah, F Sultana
Esophageal varices are a serious consequence of portal hypertension in patients with chronic liver diseases. Several studies have evaluated possible noninvasive markers of esophageal varices to reduce the number of unnecessary endoscopies in patients with chronic liver disease. Aim of this study was to identify clinical and laboratory predictors of esophageal varices in children with chronic liver disease. This cross sectional observational study was done at Pediatric Gastroenterology and Nutrition Department of Bangabandhu Sheikh Mujib Medical University, Dhaka, over a period of 6 months...
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28586157/role-of-point-shear-wave-elastography-in-the-determination-of-the-severity-of-fibrosis-in-pediatric-liver-diseases-with-pathologic-correlations
#11
Mehmet Burak Özkan, Meltem Ceyhan Bilgici, Esra Eren, Gonul Caltepe, Gulay Yilmaz, Cengiz Kara, Seda Gun
OBJECTIVES: Our aims in this study were as follows: (1) to determine the cutoff value that can distinguish between advanced liver fibrosis and normal liver tissue for two different elastographic techniques; (2) to determine the cutoff value that can distinguish mild liver fibrosis from normal liver tissue for the techniques; and (3) to assess tissue stiffness in nonalcoholic fatty liver disease (NAFLD). METHODS: Seventy-five patients assessed for liver biopsy on the same day were evaluated by point shear wave elastography...
June 6, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28580726/the-first-case-of-domino-split-liver-transplantation-in-maple-syrup-urine-disease
#12
Uta Herden, Jun Li, Lutz Fischer, Florian Brinkert, Martin Blohm, René Santer, Bjoern Nashan, Enke Grabhorn
The enzymatic defect in MSUD results in accumulation of neurotoxic metabolites of BCAAs. LTX has shown to be a feasible strategy in patients non-responsive to diet. Because of sufficient enzyme activity in extrahepatic tissues in healthy people, the MSUD liver graft is a suitable domino organ. We present the first case of a technical challenging ex situ split of a MSUD domino organ transplanted into two pediatric recipients. The domino graft donor was a 21-year-old female (58 kg) suffering from MSUD with recurrent metabolic decompensation despite strict diet...
June 5, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28575039/assessment-of-pathologic-increase-in-liver-stiffness-enables-earlier-diagnosis-of-cfld-results-from-a-prospective-longitudinal-cohort-study
#13
Victoria Klotter, Caroline Gunchick, Enno Siemers, Timo Rath, Helge Hudel, Lutz Naehrlich, Martin Roderfeld, Elke Roeb
About 30% of patients with Cystic Fibrosis (CF) develop CF-associated liver disease (CFLD). Recent studies have shown that transient elastography (TE), as a method to quantify liver stiffness, allows non-invasive diagnosis of CFLD in adults and children with CF. Within this study we aimed to prospectively identify patients at risk for development of CFLD by longitudinal analysis of liver stiffness and fibrosis scores in a 5-year follow-up. 36 pediatric and 16 adult patients with initial liver stiffness below the cut-off value indicative of CFLD (6...
2017: PloS One
https://www.readbyqxmd.com/read/28572090/gdf15-is-a-heart-derived-hormone-that-regulates-body-growth
#14
Ting Wang, Jian Liu, Caitlin McDonald, Katherine Lupino, Xiandun Zhai, Benjamin J Wilkins, Hakon Hakonarson, Liming Pei
The endocrine system is crucial for maintaining whole-body homeostasis. Little is known regarding endocrine hormones secreted by the heart other than atrial/brain natriuretic peptides discovered over 30 years ago. Here, we identify growth differentiation factor 15 (GDF15) as a heart-derived hormone that regulates body growth. We show that pediatric heart disease induces GDF15 synthesis and secretion by cardiomyocytes. Circulating GDF15 in turn acts on the liver to inhibit growth hormone (GH) signaling and body growth...
June 1, 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/28570120/characterization-of-the-coagulation-profile-in-children-with-liver-disease-and-extrahepatic-portal-vein-obstruction-or-shunt
#15
William Beattie, Maria Magnusson, Winita Hardikar, Paul Monagle, Vera Ignjatovic
INTRODUCTION: Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruction or shunt. This study aimed to describe the changes that occur in children with chronic liver disease and extrahepatic portal vein obstruction or shunt. MATERIALS AND METHODS: We measured the concentration and activity of key procoagulant and anticoagulant factors in children with liver disease, children with extrahepatic portal vein obstruction or shunt, and healthy children...
June 1, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28556786/elevated-hemoglobin-level-is-associated-with-advanced-fibrosis-in-pediatric-non-alcoholic-fatty-liver-disease
#16
Valentina Giorgio, Antonella Mosca, Arianna Alterio, Anna Alisi, Antonio Grieco, Valerio Nobili, Luca Miele
OBJECTIVES: Hemoglobin (Hb) and red blood cell distribution width (RDW) have been reported to be a risk marker of metabolic syndrome (MS) and nonalcoholic fatty liver disease (NAFLD). No study exists on pediatric populations. We aimed to determine the association between hematological parameters, and the severity of disease in children with biopsy proven NAFLD. METHODS: 117 children (85 males, mean age 12 years) with ultrasound (US) evidence of NAFLD undergoing liver biopsy for diagnosis of non alcoholic steatohepatitis (NASH), were prospectively enrolled between January 2011 and May 2013 in the setting of a tertiary care centre...
April 27, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28556755/effects-of-exercise-training-alone-on-depot-specific-body-fat-stores-in-youth-review-of-recent-literature
#17
YoonMyung Kim
The prevalence of childhood obesity has increased at an alarming rate. The increased obesity rate in pediatrics parallels the increased risks for developing metabolic abnormalities such as insulin resistance, type 2 diabetes (T2DM), and non-alcoholic fatty liver disease (NAFLD). In particular, the strong relationship between obesity and such health consequences are well explained by the excessive accumulation of depot-specific body adiposity such as visceral adipose tissue (VAT), intrahepatic lipid content, intermuscular AT (IMAT), and/or intramyocellular lipid (IMCL) content...
May 30, 2017: Pediatric Exercise Science
https://www.readbyqxmd.com/read/28555633/low-birthweight-increases-the-likelihood-of-severe-steatosis-in-pediatric-non-alcoholic-fatty-liver-disease
#18
Elisabetta Bugianesi, Carla Bizzarri, Chiara Rosso, Antonella Mosca, Nadia Panera, Silvio Veraldi, Andrea Dotta, Germana Giannone, Massimiliano Raponi, Marco Cappa, Anna Alisi, Valerio Nobili
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: Two hundred and eighty-eight consecutive Caucasian Italian overweight/obese children with biopsy-proven NAFLD were included in the study. We examined the relative association of each histological feature of NAFLD with metabolic alterations, insulin-resistance, I148M polymorphism in the patatin-like phospholipase domain-containing protein 3 (PNPLA3) gene, and birthweight relative to gestational age...
May 30, 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/28555180/risk-factors-for-neurocognitive-functioning-in-children-with-autosomal-recessive-polycystic-kidney-disease
#19
REVIEW
Stephen R Hooper
This mini review provides an overview of the issues and challenges inherent in autosomal recessive polycystic kidney disease (ARPKD), with a particular focus on the neurological factors and neurocognitive functioning of this population. ARPKD typically is discovered at the end of pregnancy or during the neonatal developmental period and occurs in approximately 1 in 20,000 live births. During the neonatal period, there is a relatively high risk of death, with many infants dying from respiratory failure. As the child ages, they experience progressive kidney disease and become increasingly vulnerable to liver disease, with many individuals eventually requiring dual organ transplants...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28551940/-oxalate-stones-are-prevalent-among-druze-and-muslim-arabs-in-the-galilee
#20
Limor Kalfon, Irit Weissman, Miriam Hershkovits, Nadra Nasser Samra, Nurit Edri, Morad Khayat, Mary Tanus, Shihab Shihab, Hanna Mandel, Tzipora Falik-Zaccai
INTRODUCTION: Primary Hyperoxaluria type I (PH1) is a rare autosomal recessive disease caused by lack or dysfunction of the liver peroxisomal enzyme alanine: glyoxylate aminotransferase, AGT. AIMS: To conduct clinical and genetic characterization of Druze and Muslim Arab patients with PH1 in Northern Israel. METHODS: In the last 20 years, 36 children and families were diagnosed and treated in the Nephrology-Genetic Clinic at the Galilee Medical Center...
March 2017: Harefuah
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