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Pediatric acute liver failure

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https://www.readbyqxmd.com/read/27900388/data-driven-modeling-for-precision-medicine-in-pediatric-acute-liver-failure
#1
Ruben Zamora, Yoram Vodovotz, Qi Mi, Derek Barclay, Jinling Yin, Simon Horslen, David Rudnick, Kathleen M Loomes, Robert H Squires
Absence of early outcome biomarkers for Pediatric Acute Liver Failure (PALF) hinders medical and liver transplant decisions. We sought to define dynamic interactions among circulating inflammatory mediators to gain insights into PALF outcome sub-groups. Serum samples from 101 participants in the PALF study, collected over the first 7 days following enrollment, were assayed for 27 inflammatory mediators. Outcomes (Spontaneous survivors [S, n=61], Non-survivors [NS, n=12], and liver transplant patients [LTx, n=28]) were assessed at 21 days post-enrollment...
November 23, 2016: Molecular Medicine
https://www.readbyqxmd.com/read/27882234/dysgerminoma-developing-from-an-ectopic-ovary-in-a-patient-with-wagr-syndrome-a-case-report
#2
Rie Miura, Yoshihito Yokoyama, Tatsuhiko Shigeto, Masayuki Futagami, Hideki Mizunuma, Akira Kurose, Kazushi Tsuruga, Shinya Sasaki, Kiminori Terui, Etsuro Ito
WAGR syndrome is caused by an 11p13 deletion and includes Wilms' tumor, aniridia, genitourinary anomalies and mental retardation. We encountered a case of a dysgerminoma originating in an ectopic ovary in a woman with WAGR syndrome. Our patient was a 24-year-old nulliparous woman who was diagnosed with WAGR syndrome. The patient had undergone left nephrectomy for a Wilms' tumor and postoperative chemotherapy at the age of 7 months. She also had a history of glaucoma surgery in both eyes, and was followed up at the Department of Pediatrics for diabetes mellitus, hypertension, liver dysfunction and hyperuricemia...
November 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27862115/pediatric-acute-liver-failure-of-undetermined-cause-a-research-workshop
#3
Estella M Alonso, Simon P Horslen, Edward M Behrens, Edward Doo
: Pediatric Acute liver failure (PALF) is a potentially devastating condition which occurs in previously healthy children of all ages and frequently leads to a rapid clinical deterioration. An identified cause for liver injury is lacking in approximately 30% of cases. Children with undetermined diagnosis have lower spontaneous survival and higher rates of transplantation and death than other diagnostic groups. A single day workshop sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases brought together clinicians and basic scientists to integrate aligned research findings and develop a foundation for new mechanistic studies and future treatment trials...
November 14, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27830542/hepatocyte-transplantation-in-special-populations-clinical-use-in-children
#4
Zahida Khan, Stephen C Strom
Orthotopic liver transplantation remains the only proven cure for end-stage liver failure. Despite significant advances in the field, the clinical demand for donor organs far outweighs the supply. Hepatocyte transplantation has been proposed as an alternative approach to whole liver transplant in select diseases. Several international centers have reported experimental trials of human hepatocyte transplantation in acute liver failure and liver-based metabolic disorders. This chapter provides an introduction to hepatocyte transplantation from both a technical and clinical perspective...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/27811533/spectral-electroencephalogram-analysis-for-the-evaluation-of-encephalopathy-grade-in-children-with-acute-liver-failure
#5
Craig A Press, Lindsey Morgan, Michele Mills, Cynthia V Stack, Joshua L Goldstein, Estella M Alonso, Mark S Wainwright
OBJECTIVE: Spectral electroencephalogram analysis is a method for automated analysis of electroencephalogram patterns, which can be performed at the bedside. We sought to determine the utility of spectral electroencephalogram for grading hepatic encephalopathy in children with acute liver failure. DESIGN: Retrospective cohort study. SETTING: Tertiary care pediatric hospital. PATIENTS: Patients between 0 and 18 years old who presented with acute liver failure and were admitted to the PICU...
November 1, 2016: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/27782894/etiologies-outcomes-and-prognostic-factors-of-pediatric-acute-liver-failure-a-single-center-s-experience-in-turkey
#6
Figen Özçay, Eda Karadağ Öncel, Zeren Barış, Oğuz Canan, Gökhan Moray, Mehmet Haberal
BACKGROUND/AIMS: Our aim was to determine the etiologies, outcomes, and prognostic indicators in children with acute liver failure. MATERIALS AND METHODS: Ninety-one patients who were followed for pediatric acute liver failure (PALF) over a 15-year period were included. Patients who survived with supportive therapy were designated as Group 1, while those who died or underwent liver transplantation were designated as Group 2. RESULTS: There were 37 (40...
September 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27755342/liver-transplantation-for-children-with-primary-sclerosing-cholangitis-and-autoimmune-hepatitis-unos-database-analysis
#7
Jacqueline Jossen, Rachel Annunziato, Hee-Sung Kim, Jaime Chu, Ronen Arnon
OBJECTIVES: Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are poorly studied in the Pediatric End Stage Liver Disease (PELD) era. We aimed to characterize the outcome of LT in children with AIH and PSC. METHODS: Children ≤18 years with PSC or AIH who had a first, isolated LT from 2002-2012 were identified from the UNOS database...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27752034/follow-up-of-pediatric-liver-transplant-patients-after-reaching-adulthood
#8
Justyna Rajchert, Monika Rosa, Joanna Pawłowska, Milosz Parczewski, Marta Wawrzynowicz-Syczewska
BACKGROUND Despite excellent transplantation results, there is a vulnerable period of time in the post-transplant management of pediatric liver recipients, which is the time when a child becomes an adolescent. This period of psychological distress influences compliance and may result in premature transplant failure and graft loss. We performed a retrospective review of pediatric liver transplant recipients, transferred to our service for adults, in order to identify key issues associated with transition into the adult care...
October 18, 2016: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/27749626/acute-liver-failure-outcome-and-value-of-pediatric-end-stage-liver-disease-score-in-pediatric-cases
#9
Raquel Núñez-Ramos, Soledad Montoro, Marcello Bellusci, María Rosa Del Fresno-Valencia, Marta Germán-Díaz, Pedro Urruzuno, Enrique Medina, Javier Manzanares
PURPOSE: The aims of this study were to analyze the characteristics of patients with acute liver failure (ALF) in our center and evaluate the prognostic value of the Pediatric End-Stage Liver Disease (PELD) score calculated at admission. PATIENTS AND METHODS: A retrospective analysis of patients with ALF younger than 15 years between 2005 and 2013 was performed. Information collected included age, sex, etiology of ALF, laboratory tests, PELD score, stage of encephalopathy, and need for liver support devices such as MARS and/or liver transplant (LT) and survival...
September 30, 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27741059/intensive-care-management-of-pediatric-acute-liver-failure
#10
Riad Lutfi, Kamal Abulebda, Mara E Nitu, Jean P Molleston, Molly A Bozic, Girish Subbarao
Pediatric acute liver failure is rare but life-threatening illness that occurs in children without preexisting liver disease. The rarity of the disease, along with its severity and heterogeneity, presents unique clinical challenges to the physicians providing care for pediatric patients with acute liver failure. In this review, practical clinical approaches to the care of critically ill children with acute liver failure are discussed with an organ system specific approach. The underlying pathophysiological processes, major areas of uncertainty, and approaches to the critical care management of pediatric acute liver failure are also reviewed...
October 12, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27736823/corticosteroid-use-and-growth-after-pediatric-solid-organ-transplantation-a-systematic-review-and-meta-analysis
#11
Anne Tsampalieros, Greg A Knoll, Amber O Molnar, Nicholas Fergusson, Dean A Fergusson
BACKGROUND: A number of corticosteroid minimization and avoidance protocols for post-solid organ transplant have been developed. The study objective was to examine the effect of corticosteroid withdrawal/avoidance on growth and safety parameters in pediatric solid organ transplant recipients. METHODS: A systematic review using Medline and Embase was performed. All randomized controlled trials (RCT) and observational studies comparing corticosteroid withdrawal/avoidance to controls receiving corticosteroids in pediatric transplant recipients which reported growth as change in height or final height were included...
July 12, 2016: Transplantation
https://www.readbyqxmd.com/read/27621562/clinically-profiling-pediatric-patients-with-dengue
#12
Sriram Pothapregada, Banupriya Kamalakannan, Mahalaskhmy Thulasingham, Srinivasan Sampath
OBJECTIVE: To study the clinical profile and outcome of dengue fever in children at a tertiary care hospital in Puducherry. MATERIALS AND METHODS: All children (0-12 years of age) diagnosed and confirmed as dengue fever from August 2012 to January 2015 were reviewed retrospectively from hospital case records as per the revised World Health Organization guidelines for dengue fever. The diagnosis was confirmed by NS1 antigen-based ELISA test or dengue serology for IgM and IgG antibodies, and the data were analyzed using SPSS 16...
July 2016: Journal of Global Infectious Diseases
https://www.readbyqxmd.com/read/27596079/-relationship-between-changes-of-increased-amylase-or-lipase-levels-and-pancreas-injury-in-critically-ill-children
#13
(no author information available yet)
OBJECTIVE: To observe the occurrence rate of elevated serum amylase and lipase in critically ill children, to explore the relationship between elevated pancreatic enzymes and pancreatic injury, and the effect of elevated pancreatic enzymes on state of illness and the prognosis. METHOD: The data of cases treated in pediatric intensive care unit(ICU) of 17 children's hospitals were collected prospectively from January 2012 to March 2014 according to the inclusion and exclusion criteria...
September 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27500264/outcomes-of-living-donor-liver-transplantation-alone-for-patients-on-maintenance-renal-replacement-therapy-in-japan-results-of-a-nationwide-survey
#14
Susumu Eguchi, Hiroyuki Furukawa, Shinji Uemoto, Koji Umeshita, Hajime Imamura, Akihiko Soyama, Tsuyoshi Shimamura, Shuji Isaji, Yasuhiro Ogura, Hiroto Egawa, Shigeyuki Kawachi, Mureo Kasahara, Hiroaki Nagano, Yonson Ku, Hideki Ohdan, Yoshihiko Maehara, Shuntaro Sato, Yukihiro Inomata
BACKGROUND: Because simultaneous liver and kidney transplantation has been limited as a standard practice because of a severe shortage of deceased donors in Japan, living donor (LD) liver transplantation alone (LTA) is indicated in most recipients with maintenance renal replacement therapy (MRRT). METHODS: A retrospective nationwide survey of LD LTA was performed for liver transplant patients on MRRT. The characteristics of donors and recipients, postoperative complications, survival rate, and causes of death were analyzed...
June 2016: Transplantation Direct
https://www.readbyqxmd.com/read/27498124/a-pharmacokinetic-and-pharmacodynamic-study-of-maraviroc-as-acute-graft-versus-host-disease-prophylaxis-in-pediatric-allogeneic-stem-cell-transplant-recipients-with-nonmalignant-diagnoses
#15
Pooja Khandelwal, Tsuyoshi Fukuda, Kana Mizuno, Ashley Teusink-Cross, Parinda A Mehta, Rebecca A Marsh, Angela D M Kashuba, Alexander A Vinks, Stella M Davies
Maraviroc is an allosteric small molecule antagonist of chemokine receptor type 5 (CCR5) and has been used in adult allogeneic hematopoietic stem cell transplant (HSCT) recipients to prevent acute graft-versus-host disease (GVHD) of the gastrointestinal (GI) tract and liver. The goal of this study was to establish feasibility and pharmacokinetic and pharmacodynamic profiles of maraviroc in pediatric HSCT recipients. Children ages 2 to 12 years were enrolled and maraviroc was added to standard GVHD prophylaxis, which included a calcineurin inhibitor and either steroids or mycophenolate mofetil...
October 2016: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/27496798/prevalence-and-significance-of-autoantibodies-in-children-with-acute-liver-failure
#16
Michael R Narkewicz, Simon Horslen, Steven H Belle, David A Rudnick, Vicky L Ng, Philip Rosenthal, Rene Romero, Kathleen M Loomes, Song Zhang, Regina M Hardison, Robert H Squires
OBJECTIVES: The purpose of this study is to estimate autoantibody (autoAB) frequency, clinical characteristics and 21 day outcome of participants in the Pediatric Acute Liver Failure Study Group (PALFSG) by ANA, SMA and LKM antibody status. METHODS: AutoAB were determined at local and/or central laboratories. Subjects were assigned to autoimmune hepatitis (AIH), indeterminate and other diagnoses groups. RESULTS: Between 1999 and 2010, 986 subjects were enrolled in the PALFSG...
August 4, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27496402/liver-transplant-registry-in-poland-web-netted-quality-tool-in-liver-transplantation-from-deceased-and-living-donors
#17
J Czerwiński, D Lewandowska, P Małkowski, R Danielewicz
BACKGROUND: Poltransplant managed a national transplant registry with the use of the Web tool www.rejestry.net. It collects information about all organ transplantations in the country along with outcomes. This article presents a formal analysis of data collected in the registry for the years 1998 to 2014. MATERIALS AND METHODS: Results presented are actual, not extrapolated, numbers; these were calculated only for the events for which the observation was complete, meaning that a given term of follow-up had passed and the information on recipient's and graft survivals were available...
June 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27479018/fulminant-liver-failure-in-a-child-with-%C3%AE-thalassemia-on-deferasirox-a-case-report
#18
Archie Ramaswami, Danya J Rosen, Jaime Chu, Birte Wistinghausen, Ronen Arnon
Deferesirox (DFX), an oral chelating agent, is used to treat chronic iron overload in several hematological diseases such as β-thalassemia, sickle cell disease, and myelodysplastic anemia. DFX is generally well tolerated with the exception of gastrointestinal disturbances and rash, although cases of renal toxicity, as well as acute and chronic liver failure, have been reported in adults and children. Here we describe a 3-year-old girl with β-thalassemia undergoing treatment with DFX who presented with acute liver failure and Fanconi's syndrome...
July 29, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27471904/neuropsychological-functioning-and-health-related-quality-of-life-in-pediatric-liver-disease-the-sum-of-our-perspectives-is-greater-than-each-alone
#19
Lisa G Sorensen
PURPOSE OF REVIEW: The review will discuss current data on neuropsychological and health-related quality of life (HRQOL) outcomes in children with liver disease. Research has trickled in over the past 30 years, mostly in small, single-center studies with observational or retrospective design. As medical treatment has advanced, survival has improved dramatically, with the majority now living into adulthood. The focus is shifting toward more systematic exploration of functional outcomes...
October 2016: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/27457792/hepatotoxicity-induced-by-acute-and-chronic-paracetamol-overdose-in-children-where-do-we-stand
#20
Hoi Yan Tong, Nicolás Medrano, Alberto Manuel Borobia, José Antonio Ruiz, Ana María Martínez, Julia Martín, Manuel Quintana, Santos García, Antonio José Carcas, Elena Ramírez
BACKGROUND: There are few data on hepatotoxicity induced by acute or chronic paracetamol poisoning in the pediatric population. Paracetamol poisoning data can reveal the weaknesses of paracetamol poisoning management guidelines. METHODS: We retrospectively studied the patients of less than 18 years old with measurable paracetamol levels, who were brought to the emergency department (ED) of La Paz University Hospital, Madrid, Spain, for suspected paracetamol overdoses between 2005 and 2010...
July 23, 2016: World Journal of Pediatrics: WJP
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