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Liver disease in children

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https://www.readbyqxmd.com/read/28430754/biomarkers-for-early-acute-kidney-injury-diagnosis-and-severity-prediction-a-pilot-multicenter-canadian-study-of-children-admitted-to-the-icu
#1
Jennifer Palermo, Allison B Dart, Alanna De Mello, Prasad Devarajan, Ronald Gottesman, Gonzalo Garcia Guerra, Greg Hansen, Ari R Joffe, Cherry Mammen, Nick Majesic, Catherine Morgan, Peter Skippen, Michael Pizzi, Ana Palijan, Michael Zappitelli
OBJECTIVE: Acute kidney injury occurs early in PICU admission and increases risks for poor outcomes. We evaluated the feasibility of a multicenter acute kidney injury biomarker urine collection protocol and measured diagnostic characteristics of urine neutrophil gelatinase-associated lipocalin, interleukin-18, and liver fatty acid binding protein to predict acute kidney injury and prolonged acute kidney injury. DESIGN: Prospective observational pilot cohort study...
April 20, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28427477/plasma-chitotriosidase-activity-versus-ccl18-level-for-assessing-type-i-gaucher-disease-severity-protocol-for-a-systematic-review-with-meta-analysis-of-individual-participant-data
#2
Tatiana Raskovalova, Patrick B Deegan, Ruby Yang, Elena Pavlova, Jérome Stirnemann, José Labarère, Ari Zimran, Pramod K Mistry, Marc Berger
BACKGROUND: Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by deficiency in acid beta-glucosidase. GD exhibits a wide clinical spectrum of disease severity with an unpredictable natural course. Plasma chitotriosidase activity and CC chemokine ligand 18 (CCL18) have been exchangeably used for monitoring GD activity and response to enzyme replacement therapy in conjunction with clinical assessment. Yet, a large-scale head-to-head comparison of these two biomarkers is currently lacking...
April 20, 2017: Systematic Reviews
https://www.readbyqxmd.com/read/28426902/hepatic-steatosis-after-pediatric-liver-transplant
#3
Emily R Perito, Tabitha Vase, Rageshree Ramachandran, Andrew Phelps, Kuang-Yu Jen, Robert H Lustig, Vickie A Feldstein, Philip Rosenthal
RATIONALE: Hepatic steatosis develops after liver transplant in 30% of adults, and non-alcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in non-transplanted children. However, post-transplant steatosis has been minimally studied in pediatric liver transplant recipients. We explored the prevalence, persistence, and association with chronic liver damage of hepatic steatosis in these children. RESULTS: In this single-center study of pediatric patients transplanted 1988-2015 (n=318), 31% of those with any post-transplant biopsy (n=271) had ≥1 biopsy with steatosis...
April 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28426447/measles-cases-in-children-requiring-hospital-access-in-an-academic-pediatric-hospital-in-italy-2008-2013
#4
Marta Ciofi Degli Atti, Antonietta Filia, Antonino Bella, Annamaria Sisto, Maria Antonietta Barbieri, Antonino Reale, Massimiliano Raponi
BACKGROUND: The Lazio Region is one of the Italian regions where sustained measles transmission continues to occur. We investigated measles cases reported by the Emergency Department (ED) of the largest pediatric hospital in Italy, located in Lazio. METHODS: We reviewed clinical records of all measles cases from 0 to 18 years of age evaluated in the ED in 2008-2013. We compared demographic and clinical characteristics of patients admitted to the inpatient setting with those of patients discharged home, to assess possible determinants of hospital admission...
April 18, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28420301/seizures-in-pediatric-patients-with-liver-transplant-and-efficacy-of-levetiracetam
#5
Betül Kılıç, Serdal Güngör, Müjgan Arslan, Mukadder Ayşe Selimoğlu, Sezai Yılmaz
The aim of this study was to evaluate the risk factors, clinical implications, and prognosis of new-onset seizures that occurred after pediatric liver transplantation, and to assess the efficacy of levetiracetam treatment. The clinical and laboratory data of liver transplanted 28 children who had seizures after liver transplantation and specifically of 18 children who received levetiracetam were analyzed retrospectively. Sixteen patients (88.9%) remained seizure-free and in 2 (11.1%), more than 50% reduction in seizures were detected with levetiracetam treatment...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28411361/liver-transplant-in-children-with-hepatoblastoma
#6
Mehmet Hanifi Okur, Yücel Yankol, Bayındır Cimşit, Nesimi Mecit, Gökhan Ertuğrul, Turan Kanmaz, Ceyhun Bozkurt, Koray Acarlı, Münci Kalayoğlu
OBJECTIVES: In this paper, the results of liver transplant due to hepatoblastoma in 10 pediatric patients at Istanbul Şişli Memorial Hospital Transplantation Center are presented. MATERIALS AND METHODS: We retrospectively evaluated medical records of pediatric patients diagnosed with hepatoblastoma and who underwent liver transplant at our clinic between January 2009 and March 2014. We examined age, weight, chemotherapy regimen, graft type for liver transplant, duration of hospital stay, complications, follow-up duration, and survival information...
April 14, 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28411336/fdg-pet-for-therapy-monitoring-in-hodgkin-and-non-hodgkin-lymphomas
#7
REVIEW
Sally F Barrington, Regine Kluge
PET using (18)F-FDG for treatment monitoring in patients with lymphoma is one of the most well-developed clinical applications. PET/CT is nowadays used during treatment to assess chemosensitivity, with response-adapted therapy given according to 'interim' PET in clinical practice to adults and children with Hodgkin lymphoma. PET is also used to assess remission from disease and to predict prognosis in the pretransplant setting. Mature data have been reported for the common subtypes of aggressive B-cell lymphomas, with more recent data also supporting the use of PET for response assessment in T-cell lymphomas...
April 14, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28411097/-acute-liver-failure-related-to-inherited-metabolic-diseases-in-young-children
#8
Filipa Dias Costa, Rita Moinho, Sandra Ferreira, Paula Garcia, Luísa Diogo, Isabel Gonçalves, Carla Pinto
INTRODUCTION: Pediatric acute liver failure (ALF) due to inherited metabolic diseases (IMD) is a rare life-threatening condition with a poor prognosis. Early intervention may be lifesaving. OBJECTIVE: To describe clinical presentation, investigation and outcomes of ALF related to IMD in young children. MATERIAL AND METHODS: Retrospective review of the medical records of children aged up to 24 months, admitted to a tertiary pediatric and neonatal Intensive Care Unit during a 27-year period, fulfilling the ALF criteria, with documented metabolic etiology...
April 11, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28399870/the-role-of-traditional-healers-in-the-diagnosis-and-management-of-burkitt-lymphoma-in-cameroon-understanding-the-challenges-and-moving-forward
#9
Glenn M Afungchwi, Peter B Hesseling, Elena J Ladas
BACKGROUND: Burkittlymphoma(BL) is the most common childhood cancer in Cameroon with a reported incidence of 3 per 100,000 children under 15 years in the Northwest region. Treatment at three Baptist mission hospitals has a recorded cure rate of over 50%. Traditional medicine(TM) is recognized by the national health system, but its scope is undefined and entraps children with BL. The aim of this study was to investigate the attitudes and practices of parents and traditional healers (TH) towards TM in children with BL in order to develop recommendations for an integrative approach and improved access to life-saving treatment for children with BL...
April 11, 2017: BMC Complementary and Alternative Medicine
https://www.readbyqxmd.com/read/28397655/-liver-failure-in-neonatal-children-is-a-diagnostic-challenge
#10
Heidi Smedegaard, Vibeke Brix Christensen, Morten Hanefeld Dziegiel, Marianne Hørby Jørgensen
This case report presents a newborn boy with hypoglycaemia, anaemia, jaundice and severe coagulopathy during the first day of his life, imitating sepsis and disseminated intravascular coagulation. One week after the birth he was diagnosed with acute liver failure due to gestational alloimmune liver disease (GALD). Despite the fact that GALD is rare, it must be suspected in all unexplained stillborn children and infants with severe liver disease. If diagnosed, it is possible to prevent death and severe liver failure in future newborns by treating the affected women with immunoglobulin during pregnancy...
March 27, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28397375/elevation-of-circulating-microrna-levels-in-obese-children-compared-to-healthy-controls
#11
M D Thompson, M J Cismowski, M Serpico, A Pusateri, D R Brigstock
As childhood obesity increases, it is becoming important to understand the complications of obesity in children and develop novel biomarkers. Evidence indicates that microRNAs (miRNA) are dys-regulated in obesity and may serve as sensitive and specific circulating biomarkers. Non-alcoholic fatty liver disease (NAFLD) is a complication of obesity that ultimately requires a liver biopsy to determine disease severity. While studies have been conducted in adults, no study to date has examined circulating miRNAs in children with obesity and NAFLD...
April 10, 2017: Clinical Obesity
https://www.readbyqxmd.com/read/28396861/non-invasive-investigations-for-the-diagnosis-of-fontan-associated-liver-disease-in-pediatric-and-adult-fontan-patients
#12
Amyna Fidai, Frederic Dallaire, Nanette Alvarez, Yvonne Balon, Robin Clegg, Michael Connelly, Frank Dicke, Deborah Fruitman, Joyce Harder, Kimberley Myers, David J Patton, Tim Prieur, Erika Vorhies, Robert P Myers, Steven R Martin, Steven C Greenway
Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8...
2017: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28395968/-aetiology-outcomes-and-prognostic-indicators-of-paediatric-acute-liver-failure
#13
Juan José Gilbert Pérez, Belén Jordano Moreno, Mónica Rodríguez Salas
INTRODUCTION: Acute liver failure (ALF) is a multisystem disease with severe impairment of liver function of acute onset. The Paediatric End-stage Liver Disease (PELD) score is used as a predictor of mortality in chronic liver disease, however experience is limited in ALF. OBJECTIVES: To evaluate the aetiology and outcomes of children with ALF in a Children's Liver Transplant Centre, and to investigate the validity of PELD as a prognostic indicator. PATIENTS AND METHODS: A retrospective study was conducted on patients diagnosed with ALF in our hospital from 2000 to 2013 using the criteria of the Paediatric ALF Study Group...
April 7, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28393504/-growth-in-children-with-choledochal-cyst
#14
A Sánchez Galán, A Vilanova Sánchez, L Martínez Martínez, M Dore Reyes, M Romo Muñoz, V Núñez Cerezo, F Hernández Oliveros, M López Santamaría
AIM: In patients with a choledochal cyst, the malformation and subsequent biliodigestive reconstruction lead to a non-physiological state in the digestive tract and biliary excretion that could affect the child's growth. The effect of this malformation and its surgical treatment are reviewed here in a large patient cohort. MATERIAL AND METHODS: Retrospective review of patients undergoing surgery for choledochal cyst in the last 20 years. Nutrition and liver function parameters, weight and height data were collected from medical records...
July 10, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28393434/multivisceral-transplantation-for-abdominal-tumors-in-children-a-single-center-experience-and-review-of-the-literature
#15
Eliza Lee, Nicole Hodgkinson, Rima Fawaz, Khashayar Vakili, Heung Bae Kim
Standard management of intra-abdominal pediatric solid tumors requires complete resection. However, tumors with multiple organ and vascular involvement present a unique surgical challenge. We conducted a retrospective chart review of four patients, aged 2-14 years, undergoing MVT for intra-abdominal tumors with significant involvement of the visceral arteries and/or portomesenteric venous system at our institution. Indications for MVT included hepatocellular carcinoma, inflammatory myofibroblastic tumor, and two cases of hepatoblastoma...
April 9, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28392706/pancreatic-solitary-fibrous-tumor-in-a-toddler-managed-by-pancreaticoduodenectomy-a-case-report-and-review-of-the-literature
#16
Qingfeng Sheng, Weijue Xu, Jiangbin Liu, Baiyong Shen, Xiaxing Deng, Yibo Wu, Wei Wu, Shenghua Yu, Xueli Wang, Zhibao Lv
Solitary fibrous tumor (SFT) of the pancreas is rare, with 15 adult cases reported in the English literature. We described a 14-month-old boy who presented with obstructive jaundice. Dominantly elevated serum CA19-9 was detected. Imaging studies revealed a well-circumscribed, solid mass in the pancreatic head. A pancreaticoduodenectomy (child procedure) was performed using Shen's anastomosis technique. After resection of the tumor, liver function and serum tumor markers normalized and clinical signs receded...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28391883/childhood-adult-onset-lysosomal-acid-lipase-deficiency-a-serious-metabolic-and-vascular-phenotype-beyond-liver-disease-four-new-pediatric-cases
#17
Pierre Poinsot, Sophie Collardeau Frachon, Lioara Restier, André Sérusclat, Mathilde Di Filippo, Sybil Charrière, Philippe Moulin, Alain Lachaux, Noel Peretti
BACKGROUND: The childhood/adult-onset lysosomal acid lipase deficiency (LALD; late-onset LALD) is a rare genetic disease. Children present severe fatty liver disease with early cirrhosis. Before enzyme replacement therapy, statins were the standard treatment to improve the severe dyslipidemia. However, late-onset LALD should be considered as a systemic metabolic disease: chronic hyper-low-density lipoprotein and hypo-high-density lipoprotein cholesterolemia induces early atherosclerosis in addition to the liver morbidity...
January 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28390159/the-natural-history-of-primary-sclerosing-cholangitis-in-781-children-a-multicenter-international-collaboration
#18
Mark R Deneau, Wael El-Matary, Pamela L Valentino, Reham Abdou, Khaled Alqoaer, Mansi Amin, Achiya Z Amir, Marcus Auth, Fateh Bazerbachi, Annemarie Broderick, Albert Chan, Jillian Cotter, Sylvia Doan, Mounif El-Youssef, Federica Ferrari, Katryn N Furuya, Madeleine Gottrand, Frederic Gottrand, Nitika Gupta, Matjaz Homan, M K Jensen, Binita M Kamath, Kyung Mo Kim, Kaija-Leena Kolho, Anastasia Konidari, Bart Koot, Raffaele Iorio, Oren Ledder, Cara Mack, Mercedes Martinez, Tamir Miloh, Parvathi Mohan, Niamh O'Cathain, Alexandra Papadopoulou, Amanda Ricciuto, Lawrence Saubermann, Pushpa Sathya, Eyal Shteyer, Vratislav Smolka, Atushi Tanaka, Raghu Varier, Veena Venkat, Bernadette Vitola, Miriam B Vos, Marek Woynarowski, Jason Yap
BACKGROUND: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. METHODS: We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death...
April 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28386718/globus-pallidus-mr-signal-abnormalities-in-children-with-chronic-liver-disease-and-or-porto-systemic-shunting
#19
Sylviane Hanquinet, Claire Morice, Delphine S Courvoisier, Vladimir Cousin, Mehrak Anooshiravani, Laura Merlini, Valérie A McLin
OBJECTIVES: Detection of subclinical hepatic encephalopathy in children is difficult. We aimed to assess the changes in imaging of the central nervous system in children with chronic liver disease using MR imaging, diffusion, and (1)H -spectroscopy. METHODS: Forty three children with chronic liver disease and/or porto-systemic shunting (111.4±56.9 months) and 24 controls (72.0±51.8 months) underwent brain MRI/spectroscopy on a 1.5T to examine T1, T2, ADC, Cho/Cr, ml/Cr, Glx/Cr ratio spectroscopy in the globus pallidus...
April 6, 2017: European Radiology
https://www.readbyqxmd.com/read/28377061/respiratory-gated-bilateral-pulmonary-radiotherapy-for-ewing-s-sarcoma-and-nephroblastoma-in-children-and-young-adults-dosimetric-and-clinical-feasibility-studies
#20
C Demoor-Goldschmidt, S Chiavassa, S Josset, M-A Mahé, S Supiot
PURPOSE: Bilateral pulmonary radiotherapy in children and young adults aims to reduce the recurrence of lung metastases. The radiation field includes liver tissue, which is sensitive to even low radiation doses. We investigated the feasibility of respiratory gating radiotherapy using voluntary deep inspiration breath hold and its toxicity in these patients. PATIENTS AND METHOD: A retrospective clinical review was conducted for all patients who had undergone bilateral pulmonary radiotherapy, with or without deep inspiration breath hold, treated in our institution between October 1999 and May 2012...
April 1, 2017: Cancer Radiothérapie: Journal de la Société Française de Radiothérapie Oncologique
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