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Pediatric acute liver failure

Wen-Jun Liu, Wei Li, Yang Tang, Si-Jie Gao, Fang Fang, Feng Xu, Ye Xu
CONTEXT: Soft tissue calcifications (STCs) were incidentally found in some of the Hymenoptera-stung (HS) children when they underwent computed tomography (CT) scans for evaluating complications of vital organs. Afterwards, a predilection of STCs to the children with severe complications was clinically noticed. A hypothesis was then developed that STCs secondary to HS may correlate with poor outcomes. OBJECTIVE: This study aims to firstly characterize the CT findings of STCs in HS children and to confirm our hypothesis that the occurrence of STCs may act as an indicator of poor outcomes in HS children...
March 9, 2018: Clinical Toxicology
Anilawan Smitthimedhin, Angela Suarez, Ryan L Webb, Hansel J Otero
Intestinal malrotation is a continuum of congenital anomalies due to lack of rotation or incomplete rotation of the fetal intestine around the superior mesenteric artery axis. The abnormal bowel fixation (by mesenteric bands) or absence of fixation of portions of the bowel increases the risk of bowel obstruction, acute or chronic volvulus, and bowel necrosis. The clinical presentation of patients with malrotation without, with intermittent, or with chronic volvulus can be problematic, with an important minority presenting late or having atypical or chronic symptoms, such as intermittent vomiting, abdominal pain, duodenal obstruction, or failure to thrive...
March 3, 2018: Abdominal Radiology
Akash Deep, Romit Saxena, Bipin Jose
Acute kidney injury (AKI) is a common accompaniment in patients with liver disease. The causes, risk factors, manifestations and management of AKI in these patients vary according to the liver disease in question (acute liver failure, acute-on-chronic liver failure, post-liver transplantation or metabolic liver disease). There are multiple causes of AKI in patients with liver disease-pre-renal, acute tubular necrosis, post-renal, drug-induced renal failure and hepatorenal syndrome (HRS). Definitions of AKI in liver failure are periodically revised and updated, but pediatric definitions have still to see the light of the day...
March 1, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Elizabeth A Feldman, Christopher D Miller, Sarabeth Wojnowicz, Robert Seabury
Despite a boxed warning, postmarketing reports of deferasirox-associated hepatic injury in patients with chronic transfusions are not well described. Hepatic impairment, including failure, has been reported to occur more frequently in patients older than 55 years and in those with significant comorbidities, including liver cirrhosis and multiorgan failure. In this case report, we describe significant hyperbilirubinemia and acute hepatocellular jaundice related to deferasirox in a 7-year-old female being treated for iron overload secondary to chronic transfusions...
January 2018: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
Marek Szymczak, Piotr Kaliciński, Grzegorz Kowalewski, Dorota Broniszczak, Małgorzata Markiewicz-Kijewska, Hor Ismail, Marek Stefanowicz, Adam Kowalski, Joanna Teisseyre, Irena Jankowska, Waldemar Patkowski
OBJECTIVES: Living donor liver transplantation (LDLT) in patients with acute liver failure (ALF) has become an acceptable alternative to transplantation from deceased donors (DDLT). The aim of this study was to analyze outcomes of LDLT in pediatric patients with ALF based on our center's experience. MATERIAL AND METHODS: We enrolled 63 children (at our institution) with ALF who underwent liver transplantation between 1997 and 2016. Among them 24 (38%) underwent a LDLT and 39 (62%) received a DDLT...
2018: PloS One
Robert Hegarty, Maesha Deheragoda, Emer Fitzpatrick, Anil Dhawan
The recognition of a pattern of steatotic liver injury where histology mimicked alcoholic liver disease but alcohol consumption was denied, led to the identification of non-alcoholic fatty liver disease (NAFLD). Non-alcoholic fatty liver disease has since become the most common chronic liver disease in adults owing to the global epidemic of obesity. However, in pediatrics, the term NAFLD seems incongruous: alcohol consumption is largely not a factor and inherited metabolic disorders (IMD) can mimic or co-exist with a diagnosis of NAFLD...
February 19, 2018: Journal of Hepatology
Suk Kyun Hong, Kyung-Suk Suh, Hyo-Sin Kim, Kyung Chul Yoon, Sung-Woo Ahn, Hyeyoung Kim, Nam-Joon Yi, Kwang-Woong Lee
BACKGROUND: Improvements in laparoscopic imaging systems and instruments have increased the performance of pure laparoscopic living donor hepatectomy. This operation is no longer limited to left lateral sectionectomy but is used for left hepatectomy and right hepatectomy.1-5 This report describes a donor who underwent pure laparoscopic left lateral sectionectomy and in situ reduction using 3D laparoscopy and indocyanine green (ICG) near-infrared fluorescence cholangiography to obtain a monosegment...
February 12, 2018: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
Y Hirata, Y Sanada, T Urahashi, Y Ihara, N Yamada, N Okada, T Katano, S Otomo, K Ushijima, K Mizuta
BACKGROUND: Antibody drugs have been used to treat steroid-resistant rejection (SRR) after liver transplantation. Although anti-thymocyte globulin has been used for SRR after liver transplantation in place of muromonab-CD3 since 2011 in Japan, the effectiveness of anti-thymocyte globulin after pediatric living-donor liver transplantation (LDLT) has not yet been reported. The aim of this study was to evaluate the effectiveness of antibody drug treatment for SRR after pediatric LDLT in our single center...
January 2018: Transplantation Proceedings
Bikrant B Lal, Seema Alam, Vikrant Sood, Dinesh Rawat, Rajeev Khanna
BACKGROUND & AIMS: There are no studies on acute kidney injury in paediatric acute-on-chronic liver failure. This study was planned with aim to describe the clinical presentation and outcome of acute kidney injury among paediatric acute-on-chronic liver failure patients. METHODS: Data of all children 1-18 years of age presenting with acute chronic liver failure (Asia pacific association for the study of the liver definition) was reviewed. Acute kidney injury was defined as per Kidney Diseases-Improving Global Outcomes guidelines...
January 11, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
Javier J Lasa, Daniel A Castellanos, Susan W Denfield, William J Dreyer, Sebastian C Tume, Henri Justino, Athar M Qureshi
There is a growing interest in the use of percutaneously delivered ventricular assist devices (PVAD) in the pediatric patient population. A 16 year old female and 18 year old male both status post heart transplantation presented with evidence of graft rejection and acute severe global systolic and diastolic heart failure necessitating hemodynamic catheterization and inotropic support. Both underwent percutaneous Impella CP LVAD (Abiomed, Danvers, MA) placement with close monitoring of right ventricular function...
December 11, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
Vikrant Sood, Bikrant B Lal, Suman Lata, Archana Rastogi, Seema Alam
Cholemic or bile cast nephropathy is an under-reported entity characterized by acute renal dysfunction in patients with hepatic insult. Limited literature is available regarding its clinical presentation, pathogenesis and prognosis. We hereby present a pediatric case who presented with acute on chronic liver failure with renal dysfunction secondary to cholemic nephropathy.
December 2017: Journal of Clinical and Experimental Hepatology
Yilun Sun, Rachael L Huskey, Li Tang, Hiroto Inaba, Aditya H Gaur, Raul Ribeiro, Jeffrey E Rubnitz, Joshua Wolf
Children and adolescents with acute myeloid leukemia (AML) are at risk of life-threatening bacterial infections, especially with viridans group streptococci. Primary antibacterial prophylaxis with vancomycin-based regimens reduces this risk but might increase the risks of renal or liver toxicity or Clostridium difficile infection (CDI). A retrospective review of data for patients treated for newly diagnosed AML at St. Jude Children's Research Hospital between 2002 and 2008 was conducted. Nephrotoxicity was classified according to pediatric risk, injury, failure, loss, and end-stage renal disease (pRIFLE) criteria and hepatotoxicity according to Common Terminology Criteria for Adverse Events (CTCAE) criteria...
March 2018: Antimicrobial Agents and Chemotherapy
Soichi Narumoto, Seisuke Sakamoto, Hajime Uchida, Kengo Sasaki, Takanobu Shigeta, Akinari Fukuda, Shunsuke Nosaka, Rie Irie, Takako Yoshioka, Mureo Kasahara
NEC is an idiopathic intestinal mucosal injury that may progress to transmural bowel necrosis without mesenteric ischemia. NEC usually affects 7- to 10-day-old neonates following enteral feeding. A 10-month-old girl with no history of laparotomy underwent LDLT for acute liver failure. After starting enteral feeding on postoperative day 5, she developed abdominal distention. Diffuse PVG and PI were detected by radiologic modalities. Exploratory laparotomy revealed patchy necrosis of the intestine without perforation...
February 2018: Pediatric Transplantation
F Chiusolo, V Fanelli, M L Ciofi Degli Atti, G Conti, F Tortora, R Pariante, L Ravà, C Grimaldi, J de Ville de Goyet, S Picardo
ARF after pediatric liver transplantation accounts for high rate of morbidity and mortality associated with this procedure. The role of CPAP in postoperative period is still unknown. The aim of the study was to describe current practice and risk factors associated with the application of helmet CPAP. In this retrospective observational cohort study, 119 recipients were divided into two groups based on indication to CPAP after extubation. Perioperative variables were studied, and determinants of CPAP application were analyzed in a multivariate logistic model...
February 2018: Pediatric Transplantation
V Iansante, R R Mitry, C Filippi, E Fitzpatrick, A 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 a 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...
December 6, 2017: Pediatric Research
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). Because nearly half of the children with chronic liver disorders have a genetic cause and approximately 20% of pediatric liver transplantations 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 workup in unclear clinical scenarios...
February 2018: Liver Transplantation
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
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
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
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
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