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Pediatric Liver

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https://www.readbyqxmd.com/read/28816795/voriconazole-antifungal-prophylaxis-in-children-with-malignancies-a-nationwide-study
#1
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
https://www.readbyqxmd.com/read/28816151/quantification-of-pediatric-abdominal-organ-motion-with-a-4-dimensional-magnetic-resonance-imaging-method
#2
Jinsoo Uh, Matthew J Krasin, Yimei Li, Xingyu Li, Christopher Tinkle, John T Lucas, Thomas E Merchant, Chiaho Hua
PURPOSE: To characterize respiration-induced abdominal organ motion in children receiving radiation treatment with a 4-dimensional (4D) magnetic resonance imaging (MRI) method. METHODS AND MATERIALS: We analyzed free-breathing coronal 4D MRI datasets acquired from 35 patients (aged 1-20 years) with abdominal tumors. A deformable image registration of the 4D MRI datasets was performed to derive motion trajectories of selected anatomic landmarks, from which organ motions were quantified...
September 1, 2017: International Journal of Radiation Oncology, Biology, Physics
https://www.readbyqxmd.com/read/28809725/clinical-characteristics-and-complications-of-pediatric-liver-biopsy-a-single-centre-experience
#3
Patricia Almeida, Richard A Schreiber, Jennifer Liang, Quais Mujawar, Orlee R Guttman
INTRODUCTION: Percutaneous liver biopsy (LB) is the gold standard method for evaluation and management of patients with liver disease. The purpose of this study was to characterize pediatric patients undergoing LB at British Columbia Children's Hospital, and to determine the rate and timing of complications following the procedure. MATERIAL AND METHODS: The medical records of all pediatric patients who underwent LB during a six-year retrospective study were reviewed to collect demographic and procedure-related data...
August 8, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28807197/perioperative-blood-transfusion-and-complications-in-children-undergoing-surgery-for-solid-tumors
#4
Dani O Gonzalez, Jennifer N Cooper, Erica Mantell, Peter C Minneci, Katherine J Deans, Jennifer H Aldrink
BACKGROUND: The objective was to assess whether perioperative blood transfusion (PBT) is associated with postoperative complications in children undergoing surgery for a solid tumor. METHODS: Using 2012-2014 National Surgical Quality Improvement Program Pediatric data, we identified patients aged 0-18 years who underwent surgery (biopsy or resection) for solid tumors. We compared demographic, clinical, and 30-day outcome characteristics between children who did and did not receive a PBT within 72 hours after surgery...
August 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28805745/insulin-resistance-and-nafld-a-dangerous-liaison-beyond-the-genetics
#5
REVIEW
Melania Manco
Over the last decade, the understanding of the association between insulin resistance (IR) and non-alcoholic fatty liver disease (NAFLD) has dramatically evolved. There is clear understanding that carriers of some common genetic variants, i.e., the patatin-like phospholipase domain-containing 3 (PNPLA3) or the transmembrane 6 superfamily member 2 (TM6SF2) are at risk of developing severe forms of NAFLD even in the presence of reduced or absent IR. In contrast, there are obese patients with "metabolic" (non-genetically driven) NAFLD who present severe IR...
August 14, 2017: Children
https://www.readbyqxmd.com/read/28803487/a-mechanism-based-approach-to-management-of-children-with-end-stage-liver-disease
#6
Allah B Haafiz
Due to parallel advances in surgical and acute care disciplines, liver transplantation (LT) has revolutionized the outlook for children with end-stage liver disease (ESLD). Contrary to advances in technical aspects of LT and the peri-operative care, pre-transplant management of ESLD remains quite a formidable challenge. Areas covered: This review provides mechanisms based management strategies to address common complications of ESLD including malnutrition, amended metabolic pathways, gastrointestinal dysfunction, and development of ascites...
August 14, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28801937/in-situ-split-liver-splitting-under-extra-corporeal-membrane-oxygenation-in-brain-dead-donor
#7
Assalino Michela, Pietro Majno, Christian Toso, Thierry Berney, Raphaël Giraud, Philipp Dutkowski, Axel Andres, Barbara Wildhaber, Laure Elkrief
Hemodynamic instability is generally considered as a contraindication to liver splitting, in particular when using an in-situ technique. We describe the cases of two young donors with brain death in whom refractory cardiac arrest and hemodynamic instability were supported by veno-arterial extracorporeal membrane oxygenation (VA-ECMO), allowing uneventful in situ splitting. Two adult and two pediatric liver recipients were successfully transplanted with immediate graft function. Favorable outcomes were also observed for the other transplanted organs, including one heart, two lungs and four kidneys...
August 12, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28795234/demographic-and-clinical-characteristics-of-patients-with-serum-sickness-like-reaction
#8
Alaaddin Yorulmaz, Fatih Akın, Ahmet Sert, Mehmet Akif Ağır, Rıza Yılmaz, Şükrü Arslan
In this study, we aimed to review the demographic, clinical, and laboratory characteristics of patients who were followed up and treated with the diagnosis of serum sickness-like reactions (SSLR) in our pediatric rheumatology clinic retrospectively and emphasize the importance of early diagnosis and treatment. The files of 29 patients who were hospitalized in the pediatric rheumatology clinic between September 2016 and March 2017 with the diagnosis of type 3 hypersensitivity reaction were reviewed retrospectively...
August 9, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28795147/impact-of-the-trough-level-of-calcineurin-inhibitor-on-the-prevalence-of-donor-specific-human-leukocyte-antigen-antibodies-during-long-term-follow-up-after-pediatric-liver-transplantation-antibody-strength-and-complement-binding-ability
#9
Kazuaki Tokodai, Shigehito Miyagi, Chikashi Nakanishi, Yasuyuki Hara, Wataru Nakanishi, Masafumi Goto, Michiaki Unno, Takashi Kamei
BACKGROUND: In pediatric patients, long-term immunosuppression after liver transplantation (LT) is typically minimal. However, posttransplant donor-specific HLA antibodies (DSAs) may be prevalent under these conditions. Here, we evaluated the effects of minimized calcineurin inhibitor (CNI) on DSA development to assess the validity of minimized/withdrawn immunosuppression. METHODS: We retrospectively examined 66 patients who underwent pediatric LT at our institution between July 1991 and October 2013...
August 2017: Transplantation Direct
https://www.readbyqxmd.com/read/28792509/regional-citrate-anticoagulation-for-continuous-renal-replacement-therapy-in-pediatric-patients-with-liver-failure
#10
Keila Rodriguez, Poyyapakkam R Srivaths, Leyat Tal, Mary N Watson, Alyssa A Riley, Ryan W Himes, Moreshwar S Desai, Michael C Braun, Ayse Akcan Arikan
Pediatric liver failure patients frequently develop multiple organ failure and require continuous renal replacement therapy (CRRT) as part of supportive therapy in the pediatric intensive care unit. While many centers employ no anticoagulation for fear of bleeding complications, balanced coagulation disturbance predisposes these patients to clotting as well as bleeding, making maintenance of longer circuit life to deliver adequate dialysis clearance challenging. Regional citrate anticoagulation (RCA) is an attractive option as it avoids systemic anticoagulation, but since citrate metabolism is impaired in liver failure, concerns about toxicity has limited its use...
2017: PloS One
https://www.readbyqxmd.com/read/28782692/deleterious-impact-of-c3d-binding-donor-specific-anti-hla-antibodies-after-pediatric-liver-transplantation
#11
Eduardo Couchonnal, Christine Rivet, Stéphanie Ducreux, Jérôme Dumortier, Alexie Bosch, Olivier Boillot, Sophie Collardeau-Frachon, Rémi Dubois, Valérie Hervieu, Patrice André, Jean-Yves Scoazec, Alain Lachaux, Valérie Dubois, Olivier Guillaud
BACKGROUND: The prevalence and clinical impact of anti-HLA donor-specific antibodies (DSA) after liver transplantation (LT) have not been extensively studied, especially in pediatric population. METHODS: The present cross-sectional study included 100 patients who underwent a first LT in childhood. Anti HLA immunization study was performed at a single time point during routine follow-up using Luminex® single antigen tests with classical anti-IgG conjugate and anti-C3d conjugate...
August 3, 2017: Transplant Immunology
https://www.readbyqxmd.com/read/28781878/bilateral-diaphragmatic-paresis-following-pediatric-liver-transplantation
#12
Jillian Olmscheid, Helena Molero, William Gershan, Nadir Demirel
BACKGROUND: Although diaphragm paresis or paralysis is fairly common following cardiac procedures; it is a less common complication following liver transplantation. Unilateral diaphragm paresis, usually right sided, has been described following liver transplantation in adults and has been rarely described in children. PURPOSE: Diaphragmatic injury following LT is often unrecognized and is typically unilateral, involving the right hemidiaphragm. Bilateral diaphragm dysfunction following liver transplantation in children is a rare complication...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28776909/pediatric-liver-transplant-patients-transition-to-adulthood-patient-and-parent-experiences
#13
Yaprak Sarigol Ordin, Özgül Karayurt, Tarkan Ünek, İbrahim Astarcıoğlu
This qualitative research study describes the experiences of child and adolescent liver transplant recipients and their parents during the patients' transition to adulthood. Data were collected from pediatric liver transplant recipients and their parents during individual interviews, and these were later analyzed using conventional content analysis. Seven main themes emerged: coping, self-management, body image, social relationships, academic life, work life, and live donors. Study results revealed that the patients who received liver transplants during their childhood and adolescence used both effective and ineffective strategies to cope with the difficulties they faced during the transition period into adulthood...
August 4, 2017: Nursing & Health Sciences
https://www.readbyqxmd.com/read/28776642/diagnosis-of-monogenic-liver-diseases-in-childhood-by-next-generation-sequencing
#14
Amelie Stalke, Britta Skawran, Bernd Auber, Thomas Illig, Brigitte Schlegelberger, Norman Junge, Imeke Goldschmidt, Christoph Leiskau, Nils von Neuhoff, Ulrich Baumann, Eva-Doreen Pfister
Next-Generation Sequencing (NGS) has opened up novel diagnostic opportunities for children with unidentified, but suspected inherited diseases. We describe our single-center experience with NGS diagnostics in standard clinical scenarios in pediatric hepatology. We investigated 135 children with suspected inherited hepatopathies, where initially no causative pathogenic variant had been identified, with an amplicon-based NGS panel of 21 genes associated with acute and chronic hepatopathies. In 23 of these patients we detected pathogenic or likely pathogenic variants in ten different genes...
August 4, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28768535/developing-a-comprehensive-response-for-treatment-of-children-under-6%C3%A2-years-of-age-with-schistosomiasis-research-and-development-of-a-pediatric-formulation-of-praziquantel
#15
Jutta Reinhard-Rupp, Katharina Klohe
Schistosomiasis is a parasitic disease caused by blood flukes. The disease is caused by an inflammatory reaction to parasite eggs retained in the liver, bladder and reproductive organs. According to 2017 World Health Organization (WHO) estimates 220 million people are potentially infected, from which probably 10% are children under 6 years of age. The regular treatment approach of a single, oral dose of 40 mg/kg body weight with praziquantel however, is difficult for children under the age of 6, leaving them without a treatment option...
August 3, 2017: Infectious Diseases of Poverty
https://www.readbyqxmd.com/read/28767319/systematic-review-and-meta-analysis-of-the-utilization-of-ethanol-locks-in-pediatric-patients-with-intestinal-failure
#16
Riad Rahhal, Maisam A Abu-El-Haija, Lin Fei, Dawn Ebach, Sarah Orkin, Elizabeth Kiscaden, Conrad R Cole
BACKGROUND: Intestinal failure is a chronic condition related to loss of bowel length and/or function, resulting in dependence on central venous catheters for fluids and nutrition. Catheter use can be associated with significant complications, including catheter-related bloodstream infections (CRBSIs), which can lead to loss of vascular access, advancing intestinal failure associated-liver disease and death. Our objective was to evaluate the effectiveness and safety of ethanol locks as compared with standard heparin locks in pediatric intestinal failure...
August 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28767077/gut-liver-axis-derangement-in-non-alcoholic-fatty-liver-disease
#17
REVIEW
Marco Poeta, Luca Pierri, Pietro Vajro
Non-alcoholic fatty liver disease (NAFLD) is the most frequent type of chronic liver disease in the pediatric age group, paralleling an obesity pandemic. A "multiple-hit" hypothesis has been invoked to explain its pathogenesis. The "first hit" is liver lipid accumulation in obese children with insulin resistance. In the absence of significant lifestyle modifications leading to weight loss and increased physical activity, other factors may act as "second hits" implicated in liver damage progression leading to more severe forms of inflammation and hepatic fibrosis...
August 2, 2017: Children
https://www.readbyqxmd.com/read/28766021/acute-kidney-injury-after-pediatric-liver-transplantation-incidence-risk-factors-and-association-with-outcome
#18
Miho Hamada, Shino Matsukawa, Satoshi Shimizu, Shinichi Kai, Toshiyuki Mizota
PURPOSE: Data on the incidence of, risk factors for, and association with outcomes of acute kidney injury (AKI) after pediatric liver transplantation are scarce. We conducted a retrospective cohort study to determine the incidence of AKI after pediatric liver transplantation. In addition, we examined risk factors for AKI and association of AKI with outcomes. METHODS: This study included 156 children aged between 3 months and 18 years undergoing liver transplantation at Kyoto University Hospital...
August 1, 2017: Journal of Anesthesia
https://www.readbyqxmd.com/read/28765741/experience-with-mechanical-circulatory-support-for-medically-intractable-low-cardiac-output-in-a-pediatric-intensive-care-unit
#19
Jung Bin Park, Jae Gun Kwak, Hong-Gook Lim, Woong-Han Kim, Jeong Ryul Lee, Yong Jin Kim
BACKGROUND AND OBJECTIVES: Mechanical circulatory support with extracorporeal membrane oxygenation (ECMO) and ventricular assist device has always been the optimal choice for treating the majority of medically intractable low cardiac output case. We retrospectively investigated our institution's outcomes and variables associated with a high risk of mortality. SUBJECTS AND METHODS: From 1999 to 2014, 86 patients who were of pediatric age or had grown-up congenital heart disease underwent mechanical circulatory support for medically intractable low cardiac output in our pediatric intensive care unit...
July 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28760295/diagnostic-challenges-of-hemophagocytic-lymphohistiocytosis
#20
REVIEW
Zaher K Otrock, Naval Daver, Hagop M Kantarjian, Charles S Eby
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by excessive activation of the immune system, resulting in overproduction of inflammatory cytokines. Patients usually present with high fever, cytopenias, hyperferritinemia, and hepatosplenomegaly, and their disease process ranges from mild to fatal multiorgan failure. HLH is a heterogeneous group of disorders that can be triggered by infections, neoplasms, or autoimmune diseases. The HLH diagnosis can be difficult to confidently confirm in critically ill patients while waiting for pathology or reference laboratory results to return, delaying the diagnosis with significantly worsened outcomes...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
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