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acute liver failure in children

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https://www.readbyqxmd.com/read/29749699/histological-findings-in-protocol-biopsies-following-pediatric-liver-transplant-low-incidence-of-abnormalities-at-5-years
#1
Amin Sheikh, Kai Y Chau, Helen M Evans
Histological abnormalities, including chronic hepatitis, fibrosis, and steatosis, are increasingly reported in liver biopsies of children after LT. These changes may be progressive and represent a form of rejection. Liver biochemistry is often initially normal. Our LT program began in 2002, utilizing tacrolimus and low-dose steroids for the first year post-LT. Patients undergo a protocol biopsy at 1 year post-LT prior to stopping steroids, then at 5 years and every 5 years thereafter. Target tacrolimus levels are 5-8 μg/L and 3-5 μg/L after 3 and 12 months, respectively...
May 11, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29743795/prevalence-and-impact-of-bacterial-infections-in-children-with-liver-disease-a-prospective-study
#2
Rishi Bolia, Anshu Srivastava, Rungmei Marak, Surender K Yachha, Ujjal Poddar
Background and aims: Risk of infections is increased in patients with Acute Liver Failure (ALF) and Decompensated Chronic Liver Disease (DCLD). We evaluated the frequency, site, type and risk-factors for bacterial infections in children with ALF and DCLD and its effect on outcome. Methods: ALF or DCLD children were enrolled prospectively. Clinical and laboratory details were recorded. Cultures (blood, urine and ascites) and chest X-ray were done at admission followed by weekly surveillance cultures...
March 2018: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29735782/an-overview-of-liver-transplant-pathology-data-from-a-tertiary-referral-centre-in-western-india
#3
Fiona Fonseca, Bijal Kulkarni, Meenal Hastak, Vinay Kumaran, Vibha Varma, Sorabh Kapoor
INTRODUCTION AND AIM: 1. Study of liver explants - Etiologic types of end-stage chronic liver disease (ESCLD) and acute liver failure (ALF) in adults and children. 2. Assessment of donor steatosis and incidental granulomas. 3. Post-transplant liver biopsies. MATERIAL AND METHODS: Specimens of 180 explant hepatectomies, 173 donor wedge and 30 core liver biopsies, and 58 post transplant liver biopsies received in our department from April 2013 to March 2017. RESULTS: 1...
April 9, 2018: Annals of Hepatology
https://www.readbyqxmd.com/read/29735580/natural-history-clinical-manifestations-and-pathogenesis-of-hepatitis-e-virus-genotype-1-and-2-infections
#4
Rakesh Aggarwal, Amit Goel
Infection with genotype 1 or 2 hepatitis E virus (HEV) results primarily from human-to-human transmission through the fecal-oral route in low-resource countries. It presents primarily as "acute viral hepatitis" syndrome, usually a self-limiting illness. A few cases progress to acute liver failure, a serious illness with high fatality. Clinical disease is infrequent among children. Infection during pregnancy is associated with a higher risk of symptomatic disease, severe liver injury, and mortality...
May 7, 2018: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/29723692/a-learning-collaborative-approach-increases-specificity-of-diagnosis-of-acute-liver-failure-in-pediatric-patients
#5
Michael R Narkewicz, Simon Horslen, Regina M Hardison, Benjamin L Shneider, Norberto Rodriguez-Baez, Estella M Alonso, Vicky L Ng, Mike A Leonis, Kathleen M Loomes, David A Rudnick, Philip Rosenthal, Rene Romero, Girish C Subbarao, Ruosha Li, Steven H Belle, Robert H Squires
BACKGROUND & AIMS: Many pediatric patients with acute liver failure (PALF) do not receive a specific diagnosis (such as herpes simplex virus or Wilson disease or fatty acid oxidation defects)-they are left with an indeterminate diagnosis and are more likely to undergo liver transplantation, which is contraindicated for some disorders. Strategies to facilitate complete diagnostic testing should increase identification of specific liver diseases and might reduce liver transplantation...
April 30, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29668570/value-of-serum-zinc-in-diagnosing-and-assessing-severity-of-liver-disease-in-children-with-wilson-disease
#6
Palittiya Sintusek, Eirini Kyrana, Anil Dhawan
Supplemental Digital Content is available in the textABSTRACT OBJECTIVES:: Wilson disease (WD) is a rare inborn error of copper metabolism with diverse manifestations. There has been no study of zinc (Zn), the copper's antagonist, in WD diagnosis and severity so far. Our aims were to evaluate serum Zn in WD and its correlation with the disease severity score (revised WD index). Although the ATP7B mutation analysis is highly accurate for WD diagnosis, it may not be readily available in a resource-limiting setting...
April 17, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29652753/hybrid-extracorporeal-therapies-as-a-bridge-to-pediatric-liver-transplantation
#7
Ayse Akcan Arikan, Poyyapakkam Srivaths, Ryan W Himes, Naile Tufan Pekkucuksen, Fong Lam, Trung Nguyen, Tamir Miloh, Michael Braun, John Goss, Moreshwar S Desai
OBJECTIVES: Standard intensive care treatment is inadequate to keep children with liver failure alive without catastrophic complications to ensure successful transplant, as accumulation of endogenous protein-bound toxins often lead to hepatic encephalopathy, hepatorenal syndrome, cardiovascular instability, and multiple organ failure. Given paucity of proven treatment modalities for liver failure, blood purification using different extracorporeal treatments as a bridge to transplantation is used, but studies evaluating the safety and efficacy of combination of these therapies, especially in pediatric liver failure, are lacking...
April 12, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29563973/down-syndrome-associated-childhood-myeloid-leukemia-with-yet-unreported-acquired-chromosomal-abnormalities-and-a-new-potential-adverse-marker-dup-1-q25q44
#8
Faten Moassass, Abdulsamad Wafa, Thomas Liehr, Ayman Al-Ablog, Walid Al Achkar
Background: Children with constitutional trisomy 21, i.e. Down syndrome (DS, OMIM #190685) have a 10 to 20-fold increased risk for a hematopoietic malignancy. They may suffer from acute lymphoblastic leukemia or acute myeloid leukemia (AML). AML referred to as myeloid leukemia of Down syndrome (ML-DS) is observed especially after birth at an early gestational age and characterized by enhanced white blood cell count, failure of spontaneous remission, liver fibrosis or liver dysfunction, and is significantly associated with early death...
2018: Molecular Cytogenetics
https://www.readbyqxmd.com/read/29551316/health-related-quality-of-life-and-neurocognitive-outcomes-in-the-first-year-after-pediatric-acute-liver-failure
#9
Lisa G Sorensen, Katie Neighbors, Regina M Hardison, Kathleen M Loomes, James W Varni, Vicky L Ng, Robert H Squires, Estella M Alonso
OBJECTIVE: To determine health-related quality of life (HRQoL) and neurocognitive impairment in survivors of pediatric acute liver failure (PALF). STUDY DESIGN: A longitudinal prospective study was conducted. At 6 and 12 months after PALF presentation, surveys of HRQoL were completed for 2- to 19-year-olds and executive functioning for ages 2-16 years. At 12 months, patients 3-16 years of age completed neurocognitive testing. HRQoL scores were compared with a healthy, matched sample...
May 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29538264/ingestion-of-fireworks-rare-cause-of-poisoning-in-children
#10
Hasan Yuksekkaya, Meltem Gumus, Aylin Yucel, Meltem Energin, Serafettin Demirci
BACKGROUND: Mistaken ingestion of all manner of toxic matter is common in childhood, but poisoning with fireworks and matchsticks is rare. Fireworks usually contain 10% yellow phosphorus and 50% potassium chlorate. Potassium chlorate is an extremely reactive and toxic agent that is used in fireworks and matchstick heads. METHODS: Eleven cases (7 females and 5 males; median age, 36 months [ranging from 24 to 48 months]) of poisoning after ingestion of fireworks and matchstick(s), between February 2008 and June 2014, were reviewed...
March 12, 2018: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29521119/soft-tissue-calcifications-secondary-to-hymenoptera-stings-a-potential-prognostic-ct-imaging-sign-in-pediatric-patients
#11
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
https://www.readbyqxmd.com/read/29497824/acute-kidney-injury-in-children-with-chronic-liver-disease
#12
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
https://www.readbyqxmd.com/read/29474400/acute-liver-failure-in-children-is-living-donor-liver-transplantation-justified
#13
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
https://www.readbyqxmd.com/read/29473717/interventions-for-paracetamol-acetaminophen-overdose
#14
REVIEW
Angela L Chiew, Christian Gluud, Jesper Brok, Nick A Buckley
BACKGROUND: Paracetamol (acetaminophen) is the most widely used non-prescription analgesic in the world. Paracetamol is commonly taken in overdose either deliberately or unintentionally. In high-income countries, paracetamol toxicity is a common cause of acute liver injury. There are various interventions to treat paracetamol poisoning, depending on the clinical status of the person. These interventions include inhibiting the absorption of paracetamol from the gastrointestinal tract (decontamination), removal of paracetamol from the vascular system, and antidotes to prevent the formation of, or to detoxify, metabolites...
February 23, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29471012/paediatric-fatty-liver-disease-pefld-all-is-not-nafld-pathophysiological-insights-and-approach-to-management
#15
REVIEW
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 paediatrics, the term NAFLD seems incongruous: alcohol consumption is largely not a factor and inherited metabolic disorders can mimic or co-exist with a diagnosis of NAFLD...
February 20, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29455437/live-donor-liver-transplantation-for-acute-liver-failure-a-single-center-experience
#16
Siddharth Mehrotra, Naimish Mehta, Prashantha S Rao, Shailendra Lalwani, Vivek Mangla, Samiran Nundy
INTRODUCTION: Acute liver failure (ALF) is an indication for emergency liver transplantation (LT). Although centers performing only deceased donor liver transplants (DDLT) have shown improved outcomes in this situation, they still have relatively long waiting lists. An alternative would be living donor liver transplantation (LDLT), which has shown equivalent outcomes in the elective situation but there is limited evidence of its results in ALF. AIM: The purpose of this study was to assess the outcomes in patients with ALF undergoing emergency LDLT in our center in Delhi, India...
January 2018: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/29417024/fulminant-hepatitis-e-in-an-infant
#17
Drishti Tolani, Ira Shah
Hepatitis E virus (HEV) is an enterically transmitted infection that is typically self-limited. It spreads by fecally contaminated water within endemic areas. Hepatitis E infection occurs in both sporadic and epidemic forms in developing countries. HEV infection is usually subclinical in children but in a pregnant woman, it manifests commonly as fulminant hepatic failure. A few cases of acute liver failure caused primarily by HEV infection in children have been reported. We present a case of fulminant hepatitis E in a 1-year-old child...
July 2017: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/29359844/hepatitis-a-cardiomyopathy-aplastic-anemia-and-acute-liver-failure-a-devastating-scenario
#18
Verónica Botero, Víctor H García, Ana M Aristizabal, Catalina Gomez, Paola Perez, Luis A Caicedo, Gabriel J Echeverri
Hepatitis A virus (HAV) causes an acute infection and is usually asymptomatic in children. When clinical manifestations appear, these include choluria, jaundice, and abdominal pain. Although infrequent, extra-hepatic manifestations related to HAV have been described, affecting the heart, bone marrow, blood vessels, and other tissues.A 10-year-old boy from a rural area presented with a 15-day history of malaise, fever, and jaundice; laboratory examinations were compatible with HAV infection. The patient turned encephalopathic and was remitted to our center, where laboratory examinations showed a medullary aplasia and fulminant hepatitis requiring a liver transplant that was performed 72 hours after admission...
April 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/29341979/wilson-s-disease-in-children-a-position-paper-by-the-hepatology-committee-of-the-european-society-for-paediatric-gastroenterology-hepatology-and-nutrition
#19
Piotr Socha, Wojciech Janczyk, Anil Dhawan, Ulrich Baumann, Lorenzo D'Antiga, Stuart Tanner, Raffaele Iorio, Pietro Vajro, Roderick Houwen, Björn Fischler, Antal Dezsofi, Nedim Hadzic, Loreto Hierro, Jörg Jahnel, Valérie McLin, Valerio Nobili, Francoise Smets, Henkjan J Verkade, Dominique Debray
BACKGROUND: Clinical presentations of Wilson's disease (WD) in childhood ranges from asymptomatic liver disease to cirrhosis or acute liver failure, whereas neurological and psychiatric symptoms are rare. The basic diagnostic approach includes serum ceruloplasmin and 24-hour urinary copper excretion. Final diagnosis of WD can be established using a diagnostic scoring system based on symptoms, biochemical tests assessing copper metabolism, and molecular analysis of mutations in the ATP7B gene...
February 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29325220/profile-risk-factors-and-outcome-of-acute-kidney-injury-in-paediatric-acute-on-chronic-liver-failure
#20
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
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