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Pediatric acute liver failure

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https://www.readbyqxmd.com/read/29149103/human-hepatocyte-transplantation-for-liver-disease-current-status-and-future-perspectives
#1
REVIEW
Valeria Iansante, Ragai R Mitry, Céline Filippi, Emer Fitzpatrick, Anil Dhawan
Liver transplantation is the accepted treatment for patients with acute liver failure and liver-based metabolic disorders. However, donor organ shortage and lifelong need for immunosuppression are the main limitations to liver transplantation. In addition, loss of the native liver as target organ for future gene therapy for metabolic disorders limits the futuristic treatment options, resulting in the need for alternative therapeutic strategies. A potential alternative to liver transplantation is allogeneic hepatocyte transplantation...
November 14, 2017: Pediatric Research
https://www.readbyqxmd.com/read/29080241/next-generation-sequencing-in-pediatric-hepatology-and-liver-transplantation
#2
REVIEW
Emanuele Nicastro, Lorenzo D'Antiga
Next Generation Sequencing (NGS) has revolutionized the analysis of human genetic variations, offering a highly cost-effective way to diagnose monogenic diseases (MDs). Since nearly half of children with chronic liver disorders have a genetic cause and approximately 20% of pediatric liver transplants are performed in children with MDs, NGS offers the opportunity to significantly improve the diagnostic yield in this field. Among the NGS strategies, the use of targeted gene panels has proven useful to rapidly and reliably confirm a clinical suspicion, whereas the whole exome sequencing (WES) with variants filtering has been adopted to assist the diagnostic work up in unclear clinical scenarios...
October 28, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29080238/-the-role-of-molecular-adsorbent-recirculating-system-in-pediatric-acute-liver-failure
#3
Jesús Quintero Bernabeu, Juan Ortega López, Javier Juampérez Goñi, Ecaterina Julio Tatis, Maria Mercadal, Itxarone Bilbao Aguirre, Carlos Rodrigo Gonzalo de Liria, Ramon Charco Torra
No abstract text is available yet for this article.
October 28, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28992518/comprehensive-detection-of-viruses-in-pediatric-patients-with-acute-liver-failure-using-next-generation-sequencing
#4
Takako Suzuki, Jun-Ichi Kawada, Yusuke Okuno, Satoshi Hayano, Kazuhiro Horiba, Yuka Torii, Yoshiyuki Takahashi, Syuichiro Umetsu, Tsuyoshi Sogo, Ayano Inui, Yoshinori Ito
BACKGROUND: Pediatric acute liver failure (PALF) is a rare and severe syndrome that frequently requires liver transplantation. Viruses are one of the most frequent causes of this disease, however, pathogenic viruses are not determined in many patients. Recently next-generation sequencing (NGS) has been applied to comprehensively detect pathogens of infectious diseases of unknown etiology. OBJECTIVES: To evaluate an NGS-based approach for detecting pathogenic viruses in patients with PALF or acute hepatitis of unknown etiology...
October 3, 2017: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/28946922/nbas-mutations-cause-acute-liver-failure-when-acetaminophen-is-not-a-culprit
#5
Pier Luigi Calvo, Francesco Tandoi, Tobias B Haak, Andrea Brunati, Michele Pinon, Dominic Dell Olio, Renato Romagnoli, Marco Spada
BACKGROUND: Pediatric acute-liver-failure due to acetaminophen (APAP) administration at therapeutic dosage is rare, while viral infections and metabolic defects are the prevalent causes. Yet, as acetaminophen is routinely used in febrile illnesses, it may be mistakenly held responsible for the acute liver damage. CASE PRESENTATION: An 11 month old boy had been on acetaminophen for 10 days (total dose 720 mg = 72 mg/kg) when he developed acute-liver-failure with encephalopathy...
September 25, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28928955/pediatric-liver-failure-we-came-we-saw-but-have-we-conquered
#6
REVIEW
Sara Kathryn Smith, Philip Rosenthal
Although there have been advances made in the diagnosis and management of pediatric acute liver failure, there is still no consensus regarding the definition or standardized evaluation, and an inability to predict outcomes, specifically irreversible brain injury, in many patients exists. Much of the research surrounding pediatric acute liver failure in the last several years has centered on the development of predictive scoring systems to enhance diagnosis and treatment decisions. In this article, we will discuss our current understanding of liver failure and updated management strategies in children with acute liver failure...
2017: F1000Research
https://www.readbyqxmd.com/read/28914695/autoimmune-acute-liver-failure-and-seronegative-autoimmune-liver-disease-in-children-are-they-different-from-classical-disease
#7
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/28912999/tacrolimus-aggravated-tube-feeding-syndrome-with-acute-renal-failure-in-a-pediatric-liver-transplant-recipient
#8
R Kula, M Melter, J Kunkel, C Dörfler, S Alikadic, B Knoppke, R Zant
Acute renal failure can be caused by calcineurin inhibitors (CNIs), due to arteriolopathy and altered tubular function. Within this context, we present the case of a 14-month-old liver transplant recipient who suffered an acute polyuric renal failure during a short episode of hypercaloric feeding. In our case, CNI-induced distal RTA led to nephrocalcinosis and therefore to secondary nephrogenic diabetes insipidus. The diet with high renal solute load consequently resulted in an acute polyuric renal failure with severe hypernatremic dehydration...
2017: Case Reports in Transplantation
https://www.readbyqxmd.com/read/28859030/treatment-of-refractory-infantile-hemangiomas-and-pulmonary-hypertension-with-sirolimus-in-a-pediatric-patient
#9
Kelley K Hutchins, Robert D Ross, Daisuke Kobayashi, Alissa Martin, Madhvi Rajpurkar
Infantile hemangioma is a benign vascular neoplasm that spontaneously involutes over time. Management, when needed, consists of medications, laser treatment and surgical excision. We describe a 3-year-old girl who presented shortly after birth with diffuse cutaneous hemangiomas, hepatosplenomegaly with liver lesions, anemia, and acute heart failure. She was diagnosed with hepatic and cutaneous infantile hemangioma based on skin biopsy. She developed progressive pulmonary hypertension with numerous pulmonary nodules suspicious for pulmonary arteriovenous malformations...
October 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28843663/risk-factors-and-outcomes-of-critically-ill-patients-with-acute-brain-failure-a-novel-end-point
#10
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/28755194/hereditary-tyrosinemia-type-1-in-turkey
#11
Ayse Cigdem Aktuglu-Zeybek, Ertugrul Kiykim, M Serif Cansever
Hereditary tyrosinemia type 1 (HT1, OMIM 276700) is a rare autosomal recessively inherited inborn error of metabolism in the tyrosine catabolic pathway due to deficiency of the enzyme fumarylacetoacetate hydrolase. The clinical features of HT1 are widely heterogenous even within the same family members. Clinical features includes acute or chronic liver disease with increased risk of hepatocellular carcinoma, hypophosphatemic rickets due to renal tubular dysfunction, glomerulosclerosis, failure to thrive, neurological porphyria-like crisis, hypertrophic cardiomyopathy and hypoglycemia due to hyperinsulinism...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28708813/application-and-interpretation-of-histocompatibility-data-in-liver-transplantation
#12
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/28675359/the-romanian-national-program-for-liver-transplantation-852-procedures-in-815-patients-over-17-years-2000-2017-a-continuous-evolution-to-success
#13
Irinel Popescu, Mihnea Ionescu, Vladislav Braşoveanu, Doina Hrehoreţ, Narcis Copca, Cristian Lupaşcu, Florin Botea, Bogdan Dorobanţu, Sorin Alexandrescu, Mihai Grigorie, Emil Matei, Radu Zamfir, Vasile Lungu, Dana Tomescu, Gabriela Droc, Daniela Ungureanu, Ruxandra Fota, Graţiela Manga, Mihai Popescu, Laura Popa, Liana Gheorghe, Speranţa Iacob, Corina Pietrăreanu, Mariana Mihailă, Laurenţiu Mic, Sanda Constantinescu, Cristian Gheorghe, Bogdan Cotruta, Ioana Lupescu, Mugur Grasu, Mirela Boroş, Radu Dumitru, Mihai Toma, Liliana Paslaru, Laura Vlad, Ileana Constantinescu, Ileana Dima, Vlad Herlea, Gabriel Becheanu, Cătălin Pecheanu, Daniela Sasalovici
Background: Liver transplantation (LT) has become an established treatment for end-stage liver disease, with more than 20.000 procedures yearly worldwide. The aim of this study was to analyze the results of Romanian National Program of LT. Methods: Between April 2000 and April 2017, 817 pts received 852 LTs in Romania. Male/female ratio was 487/330, while adult/pediatric ratio was 753/64, with a mean age of 46 years (median 50 yrs; range 7 months - 68 yrs). Main LT indications were HBV cirrhosis (230 pts; 28...
May 2017: Chirurgia
https://www.readbyqxmd.com/read/28643816/-budd-chiari-syndrome-and-ulcerative-colitis-in-an-adolescent
#14
Federico Marchetti, Martina Mainetti, Michela Giovannini, Cristina Morelli
In patients with inflammatory bowel disease (IBD) there is an increased incidence of thromboembolic events. We report a case of a female, age 14, with a 6 months history of diarrhea with occasional presence of red blood and with a very distended abdomen with evident ascites. The diagnosis was Budd-Chiari syndrome (BCS) in ulcerative colitis (UC). Therapy with subcutaneous low molecular weight heparin, methylprednisolone, mesalazine was started. Clinical and radiological features quickly improved. No thrombophilia abnormality nor other risk factor for thrombosis were detected...
May 2017: Recenti Progressi in Medicina
https://www.readbyqxmd.com/read/28621023/pediatric-liver-transplantation-for-fulminant-hepatic-failure-secondary-to-intentional-iron-overdose
#15
Joanne Lai, Jaime Chu, Ronen Arnon
Acute iron poisoning may lead to life-threatening hepatotoxicity. We present the cases of two pediatric patients with hepatotoxicity following intentional iron ingestion that progressed rapidly to fulminant hepatic failure despite treatment with deferoxamine. Liver transplantation was lifesaving in both patients. These cases emphasize the need for a high index of suspicion for iron ingestion, close monitoring for liver toxicity, and timely consideration for liver transplantation.
September 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28583557/in-situ-posterior-graft-segmentectomy-for-large-for-size-syndrome-in-deceased-donor-liver-transplantation-in-adults-a-case-report
#16
A Nagatsu, T Yoshizumi, T Ikegami, N Harimoto, N Harada, Y Soejima, A Taketomi, Y Maehara
Large-for-size syndrome (LFSS) is controversial in pediatric living donor liver transplantation patients and is associated with a poor graft outcome. Similar situations in deceased donor liver transplantation (DDLT) in adults have not been reported frequently, and there are no official guidelines worldwide. Deceased donation is extremely limited in Japan, and when a larger liver is allocated for a very sick small recipient in Japan, transplantation with a plan to address LFSS might be necessary. The patient is a 58-year-old female patient who had acute liver failure with coma...
June 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28524832/measles-outbreak-in-pediatric-hematology-and-oncology-patients-in-shanghai-2015
#17
Yan-Ling Ge, Xiao-Wen Zhai, Yan-Feng Zhu, Xiang-Shi Wang, Ai-Mei Xia, Yue-Fang Li, Mei Zeng
BACKGROUND: Despite substantial progress toward measles control are making in China, measles outbreaks in immunocompromised population still pose a challenge to interrupt endemic transmission. This study aimed to investigate the features of measles in pediatric hematology and oncology patients and explore the reasons behind the outbreak. METHODS: We collected demographic, epidemiological, and clinical data of immunocompromised measles children. All suspected measles cases were laboratory-confirmed based on the presence of measles IgM and/or identification of measles RNA...
June 5, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28497365/clinical-utility-of-soluble-interleukin-2-receptor-in-hemophagocytic-syndromes-a-systematic-scoping-review
#18
REVIEW
Molly Lin, Sujin Park, Anna Hayden, Dean Giustini, Martina Trinkaus, Morris Pudek, Andre Mattman, Marion Schneider, Luke Y C Chen
The serum-soluble interleukin-2 receptor (sIL-2r) level is considered an important diagnostic test and disease marker in hemophagocytic syndromes/hemophagocytic lymphohistiocytosis (HPS/HLH). However, this cytokine receptor is rarely measured in clinical practice and has been excluded from recent diagnostic/classification criteria such as the HScore and macrophage activation syndrome (MAS) 16. We performed a systematic scoping review of 64 articles (1975-2016) examining the clinical utility of sIL-2r in HPS/HLH...
August 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28437325/presenting-features-and-prognosis-of-ischemic-and-nonischemic-neonatal-liver-failure
#19
Carlos Zozaya Nieto, Beatriz Fernández Caamaño, Gema Muñoz Bartolo, Juan J Menéndez Suso, Esteban Frauca Remacha, Eva Valverde Núñez
OBJECTIVES: To describe the epidemiological features, clinical characteristics and outcomes of neonates diagnosed with liver failure, as well as determine prognostic factors. METHODS: Cohort study conducted at a single tertiary referral and university-affiliated pediatric center. Hospital records of all neonates diagnosed with liver failure between January 2003 and December 2015 were retrospectively reviewed, and data on clinical and laboratory findings, treatment, and outcomes were collected...
May 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28411097/-acute-liver-failure-related-to-inherited-metabolic-diseases-in-young-children
#20
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.)
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