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Pediatric liver disease

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https://www.readbyqxmd.com/read/28921939/extreme-hepatic-resections-for-the-treatment-of-advanced-hepatoblastoma-are-planned-close-margins-an-acceptable-approach
#1
Adriana Fonseca, Abha Gupta, Furqan Shaikh, Raveena Ramphal, Vicky Ng, Ian McGilvray, J Ted Gerstle
BACKGROUND: Orthotopic liver transplantation (OLT) is considered the standard for children with hepatoblastoma (HB) in whom complete surgical resection is not possible. However, OLT is not always available or feasible. OBJECTIVE: To describe the outcome of children with HB who were initially deemed unresectable and underwent complex hepatectomy with planned close margins, and ultimately avoided OLT. METHODS: Demographic data, surgical and pathologic details, and survival information were collected from children treated for HB between January 2010 to December 2015...
September 17, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28917237/screening-for-nutritional-risk-in-hospitalized-children-with-liver-disease
#2
Tiantian Song, Ying Mu, Xue Gong, Wenyan Ma, Li Li
BACKGROUND AND OBJECTIVES: Malnutrition is a major contributor to morbidity and mortality from pediatric liver disease. We investigated the prevalence of both malnutrition and high nutritional risk in hospitalized children with liver disease as well as the rate of in-hospital nutritional support. METHODS AND STUDY DESIGN: A total of 2,874 hospitalized children and adolescents with liver disease aged 1 to 17 years (inclusive) were enrolled. Malnutrition was screened by anthropometric measures (height-for-age, weight-for-height, weight-for-age, and BMI- for-age z-scores)...
2017: Asia Pacific Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28914695/autoimmune-acute-liver-failure-and-seronegative-autoimmune-liver-disease-in-children-are-they-different-from-classical-disease
#3
Vikas Jain, Anshu Srivastava, Surender K Yachha, Niraj Kumari, Rachana Kathuria, Moinak S Sarma, Ujjal Poddar, Narendra Krishnani
OBJECTIVES: Presentation as autoimmune acute liver failure (AI-ALF) and seronegative autoimmune liver disease (SN-AILD) represents two uncommon variants of AILD. We compared the clinical profile and outcome of AI-ALF with autoimmune-non-acute liver failure (AI-non-ALF) and also SN-AILD with seropositive autoimmune liver disease (SP-AILD). MATERIALS AND METHODS: Children managed as AI-ALF and AI-non-ALF including SN-AILD and SP-AILD were enrolled and compared. AI-non-ALF was diagnosed by simplified diagnostic criteria and AI-ALF by Pediatric Acute Liver Failure Study Group criteria with positive autoantibody, exclusion of other etiologies, elevated immunoglobulin G and histology when available...
September 13, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28902846/downregulation-of-microrna-145-may-contribute-to-liver-fibrosis-in-biliary-atresia-by-targeting-add3
#4
Yongqin Ye, Zhihan Li, Qi Feng, Zimin Chen, Zhouguang Wu, Jianyao Wang, Xiaoshuo Ye, Dahao Zhang, Lei Liu, Wei Gao, Lihui Zhang, Bin Wang
BACKGROUND AND OBJECTIVES: Biliary atresia (BA) is a pediatric liver disease characterized by fibro-obliteration and obstruction of the extrahepatic biliary system, that invariably leads to cirrhosis and even death, if left untreated for extended time. However, its pathology and etiology still remained unknown. In this study, we tested the expression of adducin 3 (ADD3), the gene identified as a susceptibility gene in BA by GWAS, and uncovered its upstream regulatory microRNA in the pathogenesis of BA...
2017: PloS One
https://www.readbyqxmd.com/read/28884036/severe-hepatopulmonary-syndrome-in-a-child-with-caroli-syndrome
#5
W De Jesus-Rojas, K McBeth, A Yadav, J M Stark, R A Mosquera, C Jon
Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28870776/consensus-report-by-palisi-and-pbmtc-joint-working-committees-supportive-care-guidelines-for-management-of-vod-in-children-and-adolescents-part-3-focus-on-cardio-respiratory-dysfunction-infections-liver-dysfunction-and-delirium
#6
Nadia Ovchinsky, Warren Frazier, Jeffery J Auletta, Christopher C Dvorak, Monica Ardura, Enkyung Song, Jennifer McArthur, Asumthia Jeyapalan, Robert Tamburro, Kris M Mahadeo, Chani Traube, Christine N Duncan, Rajinder P S Bajwa
Some patients with veno-occlusive disease (VOD) have multi-organ dysfunction (MOD) and multiple teams are involved in their daily care in the pediatric intensive care units (PICU). Cardio-respiratory dysfunction is critical in these patients as it needs immediate action. Whether to use non-invasive or invasive ventilation strategy may be a difficult decision in the setting of mucositis or other comorbidities in patients with VOD. Similarly monitoring of their organ functions could be very challenging; they may have fulminant hepatic failure with or without hepatic encephalopathy complicated by delirium and/or infections...
September 1, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28846072/mice-expressing-krasg12d-in-hematopoietic-multipotent-progenitor-cells-develop-neonatal-myeloid-leukemia
#7
Stefan P Tarnawsky, Rebecca J Chan, Mervin C Yoder
Juvenile myelomonocytic leukemia (JMML) is a pediatric myeloproliferative neoplasm that bears distinct characteristics associated with abnormal fetal development. JMML has been extensively modeled in mice expressing the oncogenic KrasG12D mutation. However, these models have struggled to recapitulate the defining features of JMML due to in utero lethality, nonhematopoietic expression, and the pervasive emergence of T cell acute lymphoblastic leukemia. Here, we have developed a model of JMML using mice that express KrasG12D in multipotent progenitor cells (Flt3Cre+ KrasG12D mice)...
August 28, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28843663/risk-factors-and-outcomes-of-critically-ill-patients-with-acute-brain-failure-a-novel-end-point
#8
Tarun D Singh, John C O'Horo, Ognjen Gajic, Amra Sakusic, Courtney N Day, Jay Mandrekar, Rahul Kashyap, Dereddi Raja Shekar Reddy, Alejandro A Rabinstein
OBJECTIVE: To determine the incidence, risk factors and outcomes of acute brain failure (ABF) in a mixed medical and surgical cohort of critically ill patients and its effect on ICU & hospital mortality. DESIGN: Observational electronic medical record (EMR) based retrospective cohort study of critically ill patients admitted to the ICU between 2006 and 2013. SETTING: Tertiary academic medical center. PATIENTS: Consecutive adult (>18years) critically ill patients admitted to medical and surgical ICUs...
August 18, 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/28842757/successful-long-term-outcome-of-pediatric-liver-kidney-transplantation-a-single-center-study
#9
Jesús Quintero Bernabeu, Javier Juamperez, Marina Muñoz, Olalla Rodriguez, Ramon Vilalta, José A Molino, Marino Asensio, Itxarone Bilbao, Gema Ariceta, Carlos Rodrigo, Ramón Charco
INTRODUCTION: Liver-kidney transplantation is a rare procedure in children, with just ten to 30 cases performed annually worldwide. The main indications are autosomal recessive polycystic liver-kidney disease and primary hyperoxaluria. This study aimed to report outcomes of liver-kidney transplantation in a cohort of pediatric patients. METHODS: We retrospectively analyzed all pediatric liver-kidney transplantations performed in our center between September 2000 and August 2015...
August 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28839442/gut-barrier-failure-biomarkers-are-associated-with-poor-disease-outcome-in-patients-with-primary-sclerosing-cholangitis
#10
Tamas Tornai, Eszter Palyu, Zsuzsanna Vitalis, Istvan Tornai, David Tornai, Peter Antal-Szalmas, Gary L Norman, Zakera Shums, Gabor Veres, Antal Dezsofi, Gabriella Par, Alajos Par, Peter Orosz, Ferenc Szalay, Peter Laszlo Lakatos, Maria Papp
AIM: To assess the prevalence of a panel of serologic markers that reflect gut barrier dysfunction in a mixed cohort of pediatric and adult primary sclerosing cholangitis (PSC) patients. METHODS: Sera of 67 PSC patients [median age (range): 32 (5-79) years, concomitant IBD: 67% and cirrhosis: 20%] were assayed for the presence of antibodies against to F-actin (AAA IgA/IgG) and gliadin (AGA IgA/IgG)] and for serum level of intestinal fatty acid-binding protein (I-FABP) by ELISA...
August 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28838453/cholestasis-after-pediatric-liver-transplantation-recurrence-of-a-progressive-familial-intrahepatic-cholestasis-phenotype-as-a-rare-differential-diagnosis-a-case-report
#11
B Prusinskas, S Kathemann, D Pilic, B Hegen, P Küster, V Keitel, D Häussinger, R Büscher, H A Baba, P F Hoyer, E Lainka
INTRODUCTION: Nonobstructive cholestasis after pediatric liver transplantation is a common diagnostic and therapeutic dilemma. We describe a girl with neonatal cholestasis because of progressive familial intrahepatic cholestasis 2 (PFIC-2) and presence of a homozygous splice site mutation in the ABCB11 gene. Liver transplantation was performed because of end-stage liver disease at the age of 6. Cholestasis with normal gamma-glutamyl transferase (GGT) developed 8 years after liver transplantation...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28837506/coagulopathy-in-children-with-liver-disease
#12
Patricia Sachi Kawada, Aisha Bruce, Patti Massicotte, Mary Bauman, Jason Yap
It was thought that a high International Normalized Ratio (INR) predicted bleeding in patients with chronic liver disease (CLD) and patients were 'autoanticoagulated'. Contrary to this belief, while patients with CLD experienced bleeding, they also developed thromboses. In the last decade, the prevailing literature challenged the idea that an elevated INR increased bleeding risk. The global assays of coagulation such as: thromboelastography (TEG)/rotational thromboelastometry (ROTEM) and thrombin generation assays (TGA) provide additional insight into coagulation processes...
August 23, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28837442/diagnosis-follow-up-and-treatment-of-cystic-fibrosis-related-liver-disease
#13
Ivo P van de Peppel, Anna Bertolini, Johan W Jonker, Frank A J A Bodewes, Henkjan J Verkade
PURPOSE OF REVIEW: To provide an insight and overview of the challenges in the diagnosis, follow-up and treatment of cystic fibrosis-related liver disease (CFLD). RECENT FINDINGS: The variable pathophysiology of CFLD complicates its diagnosis and treatment. A 'gold standard' for CFLD diagnosis is lacking. Over the past years, new techniques to diagnose features of CFLD, such as transient elastography, have been investigated. Although most of these tests confirm cystic fibrosis-related liver involvement (CFLI), they are, however, not suitable to distinguish various phenotypical presentations or predict progression to clinically relevant cirrhosis or portal hypertension...
August 23, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28836933/intracerebral-hemorrhage-related-with-penicillium-species-following-deceased-donor-liver-transplant
#14
Huseyin Yonder, Sami Akbulut, Huseyin Kocaaslan, Volkan Ince, Nese Karadag, Gokhan Demirtas, Ayse Selimoglu, Sezai Yilmaz
Early or late posttransplant opportunistic infections are among the leading complications after liver transplant. The source of early posttransplant opportunistic infections is usually the patient, the implantation of an infected graft, contamination during a surgical procedure, or invasive interventions performed at the intensive care unit. A 10-year-old male patient with Wilson disease (Pediatric End-Stage Liver Disease Score of 42, Child-Pugh score of 12, total bilirubin 40 mg/dL, platelet count 55?000/mL, hemoglobin level 6...
August 24, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28834223/liver-and-systemic-hemodynamics-in-cirrhotic-children-impact-on-the-surgical-management-in-pediatric-living-donor-liver-transplantation
#15
Catherine de Magnée, Francis Veyckemans, Thierry Pirotte, Renaud Menten, Dana Dumitriu, Philippe Clapuyt, Karlien Carbonez, Catherine Barrea, Thierry Sluysmans, Christine Sempoux, Isabelle Leclercq, Francis Zech, Xavier Stephenne, Raymond Reding
BACKGROUND AND AIMS: Cirrhosis in adults is associated with modifications of systemic and liver hemodynamics, whereas little is known about the pediatric population. The aim of this work was to investigate whether alterations of hepatic and systemic hemodynamics were correlated with cirrhosis severity in children. The impact of hemodynamic findings on surgical management in pediatric living donor liver transplantation was evaluated. METHODS: Liver and systemic hemodynamics were studied prospectively in 52 children (median age= 1 year; 33 biliary atresia)...
August 17, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28834194/pediatric-liver-transplantation-for-hepatocellular-cancer-and-rare-liver-malignancies-us-multi-and-single-center-experience-1981-2015
#16
Rohan Vinayak, Ruy J Cruz, Sarangarajan Ranganathan, Ravi Mohanka, George Mazariegos, Kyle Soltys, Geoff Bond, Sameh Tadros, Abhinav Humar, J Wallis Marsh, Robert R Selby, Jorge Reyes, Kimberly Haberman, Rakesh Sindhi
Pediatric liver transplantation (LTx) is increasingly performed for rare unresectable liver malignancies other than hepatoblastoma (HBL). PURPOSE: We performed a retrospective review of outcomes after LTx for malignancy in the multi-center US Scientific Registry for Transplant Recipients (SRTR, n=677, 1987-2015). We then reviewed the Children's Hospital of Pittsburgh (CHP, n=74, 1981-2014) experience focusing on LTx for unresectable hepatocellular cancer (HCC), non-HBL embryonal tumors (EMB) and metastatic liver tumors (METS)...
August 17, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28834054/herpesviruses-excretion-in-saliva-of-pediatric-transplant-recipients
#17
Consuelo Beatriz Correa Sierra, Vivian Kourí Cardellá, Lissette Pérez Santos, Cesar E Silverio, Norma Hondal, Jose Florin
BACKGROUND: Saliva samples could be used for follow-up of herpesviruses infection in the pediatric transplant recipients. OBJECTIVE: With the aim of determining the frequency of herpesviral infections in saliva samples after transplantation, and the association with viremia and complications, a pilot longitudinal follow-up of pediatric Cuban transplanted recipients (kidney and liver) was performed. METHODS: Quantitative real-time polymerase chain reaction of cytomegalovirus (CMV), Epstein-Barr virus, herpes simplex virus, human herpesevirus-6 (HHV6), varicella zoster virus, and human herpesvirus-8 were serially assayed in saliva and serum samples from 27 transplanted patients, during 32 weeks after the graft...
August 21, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28833992/successful-venoarterial-extracorporeal-membrane-oxygenation-for-prolonged-hepatopulmonary-syndrome-following-pediatric-liver-transplantation-a-case-report-and-review-of-the-literature
#18
Hamza Hassan Khan, Laura Schroeder, Megha S Fitzpatrick, Stuart S Kaufman, Nada A Yazigi, Gregory K Yurasek, David M Steinhorn, Thomas M Fishbein, Khalid M Khan
HPS is a major complicating feature of end-stage liver disease. Diagnosis is clinical, and LT is the only definitive treatment. While the general impression is that HPS improves quickly after transplantation, it may not always be the case. We describe the smallest reported child with HPS prior to LT and requiring prolonged venoarterial extracorporeal membrane oxygenation after LT; especially as it is a rare occurrence, physician managing such cases should be aware of the circumstances under which HPS may require specific treatment...
August 19, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28828531/quantitative-mri-of-fatty-liver-disease-in-a-large-pediatric-cohort-correlation-between-liver-fat-fraction-stiffness-volume-and-patient-specific-factors
#19
Madalsa Joshi, Jonathan R Dillman, Kamalpreet Singh, Suraj D Serai, Alexander J Towbin, Stavra Xanthakos, Bin Zhang, Weizhe Su, Andrew T Trout
PURPOSE: Magnetic resonance imaging (MRI) techniques are increasingly used to quantify and monitor liver tissue characteristics including fat fraction, stiffness, and liver volume. The purpose of this study was to assess the inter-relationships between multiple quantitative liver metrics and patient-specific factors in a large pediatric cohort with known or suspected fatty liver disease. MATERIALS AND METHODS: In this IRB-approved, HIPAA-compliant study, we retrospectively reviewed patient data and quantitative liver MRI results in children with known/suspected fatty liver disease...
August 21, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28816795/voriconazole-antifungal-prophylaxis-in-children-with-malignancies-a-nationwide-study
#20
Zoi Dorothea Pana, Maria Kourti, Katerina Vikelouda, Antonia Vlahou, Nikolaos Katzilakis, Maria Papageorgiou, Dimitrios Doganis, Loizos Petrikkos, Anna Paisiou, Dimitrios Koliouskas, Antonios Kattamis, Eftichia Stiakaki, Maria Chatzistilianou, Helen Vasilatou-Kosmidis, Sophia Polychronopoulou, Stelios Grafakos, Emmanuel Roilides
BACKGROUND: Antifungal prophylaxis (AFP) is recommended in at-risk hematology-oncology patients. We evaluated the safety of AFP with voriconazole (VRC) in pediatric hematology/oncology patients. MATERIALS AND METHODS: A retrospective study of VRC AFP in children with malignancies hospitalized in all 7 Greek pediatric hematology/oncology centers during 2008 to 2012 was conducted. Patients' demographics, outcome, and adverse event (AE) data were recorded. RESULTS: Four hundred twenty-nine VRC AFP courses in 249 patients (median age 6 y, 55% boys) were studied...
August 14, 2017: Journal of Pediatric Hematology/oncology
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