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Pediatric Liver

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https://www.readbyqxmd.com/read/28735109/design-and-rationale-for-a-real-world-observational-cohort-of-patients-with-nonalcoholic-fatty-liver-disease-the-target-nash-study
#1
A Sidney Barritt, Norman Gitlin, Samuel Klein, Anna S Lok, Rohit Loomba, Laura Malahias, Margaret Powell, Miriam B Vos, L Michael Weiss, Kenneth Cusi, Brent A Neuschwander-Tetri, Arun Sanyal
Nonalcoholic fatty liver disease (NAFLD) is highly prevalent and can lead to cirrhosis, hepatocellular carcinoma, and end-stage liver disease. NAFLD comprises the spectrum from simple steatosis (nonalcoholic fatty liver, NAFL), to steatosis with inflammation (nonalcoholic steatohepatitis, NASH). Current primary therapy recommended for NAFLD is weight loss induced by lifestyle modification. The difficulty in achieving this has led to robust pharmacological therapy development. While new drugs may show efficacy in selected phase II/III clinical trial populations, their real-world effectiveness is unknown...
July 19, 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/28731841/survey-of-nutrition-management-practices-in-centers-for-pediatric-intestinal-rehabilitation
#2
Anita M Nucci, Kipp Ellsworth, Austin Michalski, Emily Nagel, Jackie Wessel
BACKGROUND: Nutrition management of pediatric intestinal failure (IF) requires interdisciplinary coordination of parenteral nutrition (PN) and enteral nutrition (EN) support. Nutrition strategies used by specialists in pediatric intestinal rehabilitation to promote gut adaptation and manage complications have not been previously summarized. METHODS: A practice survey was distributed to members of the dietitian subgroup of the American Society for Parenteral and Enteral Nutrition Pediatric Intestinal Failure Section...
July 1, 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28731654/validation-of-the-hematopoietic-cell-transplantation-specific-comorbidity-index-in-a-retrospective-cohort-of-children-and-adolescents-who-received-an-allogeneic-transplantation-in-argentina
#3
Carlos M Figueroa Turienzo, Carolina Cernadas, Mariana Roizen, Silvia Pizzi, Raquel Staciuk
INTRODUCTION: Hematopoietic cell transplantationis a therapy with a risk of transplant-related mortality (TRM), which may vary depending on prior comorbidities. The Hematopoietic Cell Transplantation-Specific Comorbidity Index (HCT-CI) is an instrument developed to measure this risk. There are very few reports on its use in pediatrics. The objective of this study was to validate the HCT-CI in a pediatric cohort of allogeneic hematopoietic-cell transplantation recipients in Argentina. POPULATION AND METHODS: Retrospective cohort made up of 140 transplant patients at Hospital J...
August 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28730729/traffic-related-air-pollution-associations-with-cytokeratin-18-a-marker-of-hepatocellular-apoptosis-in-an-overweight-and-obese-paediatric-population
#4
S Hsieh, B P Leaderer, A E Feldstein, N Santoro, L A McKay, S Caprio, R McConnell
INTRODUCTION: Traffic-related air pollution causes fatty liver, inflammation and fibrosis in animal models, but there have been few studies in humans. OBJECTIVES: To test the hypothesis that traffic-related air pollution causes non-alcoholic fatty liver disease (NAFLD) and increased markers for non-alcoholic steatohepatitis (NASH); and that NAFLD increases liver susceptibility to increased NASH risk. METHODS: Data collected prospectively from 74 overweight or obese children were obtained from the Yale Pediatric Obesity Clinic...
July 20, 2017: Pediatric Obesity
https://www.readbyqxmd.com/read/28729907/current-status-of-pediatric-transplantation-in-japan
#5
REVIEW
Nao Nishimura, Mureo Kasahara, Kenji Ishikura, Satoshi Nakagawa
Brain-dead donor organ transplantation has been available to children in Japan since the 2010 revision of the Organ Transplant Law. Of the 50-60 brain-dead donor organ transplants performed annually in Japan, however, very few (0-4 per year) are performed in children. Again, while those receiving liver, heart, and kidney transplants are reported to fare better than their counterparts in the rest of the world, organ shortage is becoming a matter of great concern. Very few organs become available from brain-dead donors or are transplanted to adults if made available at all, with some children dying while on the brain-dead organ waiting list...
2017: Journal of Intensive Care
https://www.readbyqxmd.com/read/28727970/severe-fat-accumulation-in-multiple-organs-in-pediatric-autopsies-an-uncommon-but-significant-finding
#6
Jeannette C Bleeker, Gepke Visser, Frits A Wijburg, Sacha Ferdinandusse, Hans R Waterham, Peter G J Nikkels
Background The observation of fat accumulation in different organs at pediatric autopsy may help determine the cause of death. However, a comprehensive study on fat accumulation and related etiologies is still lacking. Aim To investigate the incidence of fat accumulation in different organs in pediatric autopsies and their relevance to the cause of death. Methods From February 2004 until March 2015, fat accumulation was assessed in 561 routinely performed pediatric autopsies in our center. Age at death ranged from 14 weeks of gestation for fetuses to 16 years...
July 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28719006/relative-exchangeable-copper-a-valuable-tool-for-the-diagnosis-of-wilson-disease
#7
Olivier Guillaud, Anne-Sophie Brunet, Isabelle Mallet, Jérôme Dumortier, Martine Pelosse, Sophie Heissat, Christine Rivet, Alain Lachaux, Muriel Bost
BACKGROUND & AIMS: Measuring of the relative exchangeable copper seems to be a promising tool for the diagnosis of Wilson disease. The aim of our study is to determine the performance of REC for the diagnosis of Wilson disease in a population of patients with chronic liver diseases. METHODS: Measuring of exchangeable serum copper levels and relative exchangeable copper was performed in a group of Wilson disease patients at diagnosis or at clinical deterioration because of non-compliance (group 1, n=9), a group of stable WD patients (group 2, n=40), and 2 groups of patients (adult and pediatric) followed for non-Wilsonian liver diseases (group 3, n=103 and group 4, n=49, respectively)...
July 18, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28718192/cardiometabolic-risks-vary-by-weight-status-in-pediatric-kidney-and-liver-transplant-recipients-a-cross-sectional-single-center-study-in-the-usa
#8
S He, N A Le, J K Frediani, P D Winterberg, R Jin, R Liverman, A Hernandez, R L Cleeton, M B Vos
There is an increasing need to understand long-term metabolic changes and resultant comorbidities because life expectancy is increasing after pediatric kidney and liver transplants. We evaluated differences in classic and novel cardiometabolic biomarkers among obese and normal weight adolescent transplant recipients. We enrolled a total of 80 adolescent (mean±SD, 14.8 years ±3.0) transplant recipients (63 kidney, 17 liver) with mean duration from transplantation of 6.0 (±4.1) years. Among kidney transplant recipients, overweight and obese individuals had higher leptin (16...
July 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28718042/contrast-enhanced-ultrasound-for-focal-liver-lesions-how-accurate-is-it
#9
Richard G Barr
With the recent FDA approval for characterization of focal liver lesions (FLL) in both pediatric and adult patients using Lumason (sulfur hexafluoride microbubbles), increased use of ultrasound contrast for routine clinical use is expected. This agent has been available for many years in Europe and Asia, and a large body of literature is available regarding the sensitivity and specificity of this agent. In addition, a few studies have directly compared CEUS to CECT and CEMRI for the characterization of focal liver lesions...
July 17, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28717867/totally-laparoscopic-living-donor-left-hepatectomy-for-liver-transplantation-in-a-child
#10
Helayel Almodhaiberi, Seok-Hwan Kim, Ki-Hun Kim
BACKGROUND: Minimally invasive surgery has been validated to be a new standard in living donor hepatectomy for adult-to-pediatric transplantation with less morbidity [1]. Laparoscopic donor hepatectomy can reduce the major concerns about pain and morbidity associated with open surgery and a slow return to daily activities of donors [2]. Herein, we present one case of totally laparoscopic living donor left hepatectomy including the middle hepatic vein (MHV). DONOR AND METHOD: A 37-year-old mother volunteered to donate to her 3-year-old son with biliary atresia (PELD score 7)...
July 17, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28715116/delayed-transplantation-may-affect-intellectual-ability-in-children
#11
Jiwon M Lee, Yeon Kyung Jung, Jeong-Hoon Bae, Sun Ah Yoon, Ju Hee Kim, YoungRok Choi, Hyeyoung Kim, Kwang-Woong Lee, Hye Young Ahn, Jae Won Kim, Min-Sup Shin, Kyung-Suk Suh, Il-Soo Ha, Hae Il Cheong, Hee Gyung Kang, Nam-Joon Yi
BACKGROUND: Decline in neurocognitive function is a reported complication in children with chronic illness. Concerns have been increasing that exposure to a major surgery or trauma may negatively impact cognitive performance in children. This study has evaluated cognitive function in 43 Korean children who received organ transplantation, and sought to identify associated clinical factors. METHODS: Pediatric recipients of kidney (KT) or liver transplantation (LT) from years 1999 to 2011 were recruited for cognitive tests...
July 17, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28714558/everolimus-and-reduced-calcineurin-inhibitor-therapy-in-pediatric-liver-transplant-recipients-results-from-a-multicenter-prospective-study
#12
Rainer Ganschow, Bo-Goran Ericzon, Anil Dhawan, Khalid Sharif, El-Djouher Martzloff, Barbara Rauer, Jennifer Ng, Patricia Lopez
In a 24-month, multicenter, single-arm, prospective study, 56 pediatric liver transplant patients with or without basiliximab induction were converted at 1-6 months post-transplant from standard calcineurin inhibitor (CN) therapy (± mycophenolic acid), to everolimus with reduced exposure to CNI (tacrolimus n=50, cyclosporine n=6). Steroid therapy was optional. Recruitment was stopped prematurely due to high rates of PTLD, treatment-related serious infections leading to hospitalization and premature study drug discontinuation...
July 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28714114/split-liver-transplantation-for-retroperitoneal-immature-teratoma-masquerading-as-hepatoblastoma
#13
Dongkyu Oh, Nam-Joon Yi, Sanghee Song, Ok Kyung Kim, Suk Kyun Hong, Kyung Chul Yoon, Sung Woo Ahn, Hyo-Sin Kim, Hyeyoung Kim, Hyun-Young Kim, Hyoung Jin Kang, Myungsu Lee, Kyoung Bun Lee, Kwang-Woong Lee, Kyung Suk Suh
Extragonadal teratoma originating from the retroperitoneum represents less than 5% of all teratomas and accounts for less than 10% of all pediatric retroperitoneal neoplasms. To date, there has been no report of teratoma managed with LT. This study reports an infant aged 3 months with retroperitoneal immature teratoma involving the hepatic hilum, refractory to chemotherapy and treated with LT. The patient was referred to our hospital for management of a growing abdominal mass. Histopathology of a fine needle biopsy of the lesion suggested the possibility of a hepatoblastoma with teratoid features...
July 16, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28713797/health-related-quality-of-life-after-pediatric-liver-transplantation-a-qualitative-analysis-of-the-perspectives-of-health-care-providers
#14
Mar Miserachs, David B Nicholas, Anthony R Otley, Vicky Lee Ng
With improved survival outcomes after pediatric liver transplantation (LT), health-related quality of life (HRQoL) is an important outcome metric. Understanding the elements contributing to HRQoL after LT in children would enable more targeted strategies towards optimizing best outcomes. This qualitative study aimed to explore health care providers (HCP) perceptions about HRQoL after pediatric LT. Thirteen experienced HCP participated in two focus group discussions. Data analysis via a thematic analysis approach revealed 4 major themes: "LT as a facilitator of better HRQoL," "coping and adapting to LT," "living with a transplanted liver," and "the family context...
2017: Canadian Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28711630/analysis-of-liver-offers-to-pediatric-candidates-on-the-transplant-wait-list
#15
E K Hsu, M L Shaffer, L Gao, C Sonnenday, M L Volk, J Bucuvalas, J C Lai
BACKGROUND & AIMS: Approximately 10% of children on the liver transplant wait-list in the United States (US) die every year. We examined deceased donor liver offer acceptance patterns and their contribution to pediatric wait-list mortality. METHODS: We performed a retrospective cohort study of children on the US liver transplant wait-list from 2007 through 2014 using national transplant registry databases. We determined the frequency, patterns of acceptance, and donor and recipient characteristics associated with deceased donor liver organ offers for children who died or were delisted compared to those who underwent transplantation...
July 12, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28710878/6-mercaptopurine-modifies-cerebrospinal-fluid-t-cell-abnormalities-in-pediatric-opsoclonus-myoclonus-as-steroid-sparer
#16
Michael R Pranzatelli, Elizabeth D Tate, Tyler J Allison
The purpose was to evaluate the capacity of 6-mercaptopurine (6-MP), a known immunosuppressant, to normalize cerebrospinal fluid (CSF) lymphocyte frequencies in opsoclonus-myoclonus syndrome (OMS) and function as a steroid sparer. CSF and blood lymphocytes were immunophenotyped in 11 children with OMS (without CSF B cell expansion), using a comprehensive panel of cell surface adhesion, activation, and maturation markers by flow cytometry, and referenced to 18 pediatric controls. Drug metabolites, lymphocyte counts, and liver function tests were used clinically to monitoring therapeutic range and toxicity...
July 15, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28710680/development-of-a-novel-tool-to-assess-the-impact-of-itching-in-pediatric-cholestasis
#17
Binita M Kamath, Linda Abetz-Webb, Ciara Kennedy, Bonnie Hepburn, Martha Gauthier, Nathan Johnson, Sharon Medendorp, Alejandro Dorenbaum, Lora Todorova, Benjamin L Shneider
OBJECTIVES: The aim was to develop a clinical outcome assessment (COA) for itching in children with cholestatic pruritus. METHODS: This prospective study aimed to enroll patients aged 4-30 years with Alagille syndrome (ALGS) or progressive familial intrahepatic cholestasis type 1 and caregivers of patients aged 5 months to 14 years. Eligible patients experienced itching during ≥3 of the 7 days before enrollment and had not undergone liver transplant or surgical interruption of the enterohepatic circulation...
July 14, 2017: Patient
https://www.readbyqxmd.com/read/28708813/application-and-interpretation-of-histocompatibility-data-in-liver-transplantation
#18
Russell N Wesson, Eric W Etchill, Jacqueline Garonzik-Wang
PURPOSE OF REVIEW: There has been a resurgence of interest in histocompatibility as it applies to liver transplantation. The association of persistent and de-novo donor specific antibody (DSA) and outcomes after liver transplantation continues to be investigated. RECENT FINDINGS: Consensus continues to evolve regarding the existence of acute and chronic antibody-mediated rejection (AMR) and pathogenicity of DSA and associated pathologic findings after liver transplantation...
July 13, 2017: Current Opinion in Organ Transplantation
https://www.readbyqxmd.com/read/28708597/mri-based-evaluation-of-multiorgan-iron-overload-is-a-predictor-of-adverse-outcomes-in-pediatric-patients-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#19
Natalia Maximova, Massimo Gregori, Giulia Boz, Roberto Simeone, Davide Zanon, Giulia Schillani, Floriana Zennaro
The medical records of 44 pediatric patients who underwent allogeneic transplantation from 2011 to 2015 were retrospectively reviewed. Magnetic resonance imaging was used to measure iron concentrations in the liver, spleen, pancreas and bone. These patients were divided into two groups, 18 with non-elevated (< 100 μmol/g; Group 1) liver iron concentration before transplantation and 26 with elevated (> 100 μmol/g; Group 2) concentration . We compared transplant-related outcomes in the two groups. Iron overload was a negative prognostic risk factor for sinusoidal obstruction syndrome (OR = 17), osteoporosis (OR = 6...
July 5, 2017: Oncotarget
https://www.readbyqxmd.com/read/28700730/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
#20
Jana Diestelhorst, Norman Junge, Jerome Schlue, Christine S Falk, Michael P Manns, Ulrich Baumann, Elmar Jaeckel, Richard Taubert
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...
2017: PloS One
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