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https://www.readbyqxmd.com/read/28318185/neonatal-acute-liver-failure-a-diagnosis-challenge
#1
Mirta Ciocca, Fernando Álvarez
Neonatal acute liver failure is a rare, very severe disease with a high rate of mortality. It is clinically and etiologically different from acute liver failure seen in older children and adults. Coagulopathy with an international normalized ratio ≥ 3 is the critical parameter that defines it. The most common causes are fetal alloimmune hepatitis, previously called neonatal hemochromatosis, viral infections, metabolic disorders, and hemophagocytic lymphohistiocytosis. There is a group of treatable diseases that require a very early diagnosis for the prescription of an adequate treatment...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28248208/extracorporeal-liver-support-systems-in-paediatric-liver-failure
#2
Vandana Jain, Anil Dhawan
Extracorporeal liver support systems (ELSS), encompassing artificial and bio-artificial devices, have been used for decades, with the aim of supporting patients with acute liver failure (ALF) and acute-on chronic liver failure (AoCLF), as a bridge to recovery (ALF only) or liver transplantation (LT), in an era of organ donation shortage. Although biochemical efficacy has been consistently demonstrated by these devices, translation into clinical and survival benefits has been unclear, due to study limitations and lack of reliable prognostic scoring in liver failure...
December 21, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28220684/living-donor-liver-transplantation-during-the-first-three-months-of-life
#3
Mureo Kasahara, Seisuke Sakamoto, Kengo Sasaki, Hajime Uchida, Toshihiro Kitajima, Takanobu Shigeta, Soichi Narumoto, Yoshihiro Hirata, Akinari Fukuda
BACKGROUND: Living donor liver transplantation is now an established technique for treating children with end-stage liver disease. Few data exist about liver transplantation for exclusively young infants, especially infants of <3 months of age. We report our single-center experience with 12 cases in which living donor liver transplantation (LT) was performed during the first 3 months of life and compare the results with those of older infants who underwent LT. All of the patients were treated at the National Center of Child Health and Development, Tokyo, Japan...
February 21, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28111909/profile-and-outcome-of-first-109-cases-of-pediatric-acute-liver-failure-at-a-specialized-pediatric-liver-unit-in-india
#4
Seema Alam, Rajeev Khanna, Vikrant Sood, Bikrant Bihari Lal, Dinesh Rawat
BACKGROUND AND AIMS: The outcome of Pediatric acute liver failure largely depends on age and etiology. The aim of this work was to study the etiological spectrum and outcome of the pediatric acute liver failure cases. METHODS: This prospective observational study included all children (< 18 years age) fulfilling pediatric acute liver failure study group definition. Etiological evaluation was done and predictive factors for poor outcome (death or liver transplantation) were analyzed...
January 23, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28088395/lactate-and-lactate-pyruvate-ratio-in-the-diagnosis-and-outcomes-of-pediatric-acute-liver-failure
#5
Amy G Feldman, Ronald J Sokol, Regina M Hardison, Estella M Alonso, Robert H Squires, Michael R Narkewicz
OBJECTIVES: To assess the accuracy of blood lactate and lactate: pyruvate molar ratio (L:P) as a screen for mitochondrial, respiratory chain, or fatty acid oxidation disorders in children with pediatric acute liver failure (PALF); to determine whether serum lactate ≥ 2.5 mmol/L or L:P  ≥ 25 correlated with biochemical variables of clinical severity; and to determine whether lactate or L:P is associated with clinical outcome at 21 days. STUDY DESIGN: Retrospective review of demographic, clinical, laboratory, and outcome data for PALF study group participants who had lactate and pyruvate levels collected on the same day...
March 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28060158/identifying-brain-dysfunction-among-children-with-acute-liver-failure-can-spectral-electroencephalography-help
#6
Eric T Payne, Robert J Kahoud
No abstract text is available yet for this article.
January 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28045774/study-of-carnitine-acylcarnitine-and-aminoacid-profile-in-children-and-adults-with-acute-liver-failure
#7
Vikrant Sood, Dinesh Rawat, Rajeev Khanna, Shvetank Sharma, Prem K Gupta, Seema Alam, Shiv Kumar Sarin
OBJECTIVES: Fatty acid oxidation defects (FAODs) may underlie or modify the course of acute liver failure (ALF). Overall significance of carnitine/acylcarnitine and aminoacid profile in ALF is similarly undetermined. Thus, this study was undertaken to study the abnormalities in carnitine/acylcarnitine and aminoacid profile in ALF. PATIENTS AND METHODS: A prospective study was performed including all cases of ALF and detailed evaluation including metabolic testing was done...
December 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28031453/acute-liver-failure-meets-soph-syndrome-a-case-report-on-an-intermediate-phenotype
#8
Fanny Kortüm, Iris Marquardt, Malik Alawi, Georg Christoph Korenke, Stephanie Spranger, Peter Meinecke, Kerstin Kutsche
Acute liver failure (ALF) is a life-threatening condition in the absence of preexisting liver disease in children. The main clinical presentation comprises hepatic dysfunction, elevated liver biochemical values, and coagulopathy. The etiology of ALF remains unclear in most affected children; however, the recent identification of mutations in the neuroblastoma amplified sequence (NBAS) gene in autosomal recessively inherited ALF has shed light on the cause of a subgroup of fever-triggered pediatric ALF episodes...
January 2017: Pediatrics
https://www.readbyqxmd.com/read/28027602/is-size-the-only-determinant-of-delayed-abdominal-closure-in-pediatric-liver-transplant
#9
Shirin Elizabeth Khorsandi, Arthur William Raven Day, Miriam Cortes, Akash Deep, Anil Dhawan, Hector Vilca-Melendez, Nigel Heaton
The aim was to determine the factors associated with the use of delayed abdominal closure in pediatric liver transplantation (LT) and whether this affected outcome. From a prospectively maintained database, transplants performed in children (≤18 years) were identified (October 2010 to March 2015). Primary abdominal closure was defined as mass closure performed at time of transplant. Delayed abdominal closure was defined as mass closure not initially performed at the same time as transplant; 230 children underwent LT...
March 2017: Liver Transplantation
https://www.readbyqxmd.com/read/27941191/diagnostic-and-therapeutic-management-of-children-with-lysosomal-acid-lipase-deficiency-lal-d-review-of-the-literature-and-own-experience
#10
Aldona Wierzbicka-Rucińska, Wojciech Jańczyk, Agnieszka Ługowska, Dariusz Lebensztejn, Piotr Socha
Lysosomal acid lipase deficiency may present at any age (in infants, children and adults). Its presenting features commonly include elevated serum transaminase activity levels, hypercholesterolemia, fatty liver, progressive liver fibrosis, and cirrhosis. Nonspecific clinical manifestations can lead to a delay in the diagnosis of both children and adults. The early development of fibrosis and cirrhosis suggests that the lysosomal accumulation of cholesterol esters and triglycerides in the liver is a potent inducer of fibrosis...
2016: Developmental Period Medicine
https://www.readbyqxmd.com/read/27862115/pediatric-acute-liver-failure-of-undetermined-cause-a-research-workshop
#11
Estella M Alonso, Simon P Horslen, Edward M Behrens, Edward Doo
Pediatric acute liver failure (PALF) is a potentially devastating condition that occurs in previously healthy children of all ages and frequently leads to a rapid clinical deterioration. An identified cause for liver injury is lacking in approximately 30% of cases. Children with undetermined diagnosis have lower spontaneous survival and higher rates of transplantation and death than other diagnostic groups. A single-day workshop sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases brought together clinicians and basic scientists to integrate aligned research findings and develop a foundation for new mechanistic studies and future treatment trials...
March 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27830542/hepatocyte-transplantation-in-special-populations-clinical-use-in-children
#12
Zahida Khan, Stephen C Strom
Orthotopic liver transplantation remains the only proven cure for end-stage liver failure. Despite significant advances in the field, the clinical demand for donor organs far outweighs the supply. Hepatocyte transplantation has been proposed as an alternative approach to whole liver transplant in select diseases. Several international centers have reported experimental trials of human hepatocyte transplantation in acute liver failure and liver-based metabolic disorders. This chapter provides an introduction to hepatocyte transplantation from both a technical and clinical perspective...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/27811533/spectral-electroencephalogram-analysis-for-the-evaluation-of-encephalopathy-grade-in-children-with-acute-liver-failure
#13
Craig A Press, Lindsey Morgan, Michele Mills, Cynthia V Stack, Joshua L Goldstein, Estella M Alonso, Mark S Wainwright
OBJECTIVE: Spectral electroencephalogram analysis is a method for automated analysis of electroencephalogram patterns, which can be performed at the bedside. We sought to determine the utility of spectral electroencephalogram for grading hepatic encephalopathy in children with acute liver failure. DESIGN: Retrospective cohort study. SETTING: Tertiary care pediatric hospital. PATIENTS: Patients between 0 and 18 years old who presented with acute liver failure and were admitted to the PICU...
November 1, 2016: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/27782894/etiologies-outcomes-and-prognostic-factors-of-pediatric-acute-liver-failure-a-single-center-s-experience-in-turkey
#14
Figen Özçay, Eda Karadağ Öncel, Zeren Barış, Oğuz Canan, Gökhan Moray, Mehmet Haberal
BACKGROUND/AIMS: Our aim was to determine the etiologies, outcomes, and prognostic indicators in children with acute liver failure. MATERIALS AND METHODS: Ninety-one patients who were followed for pediatric acute liver failure (PALF) over a 15-year period were included. Patients who survived with supportive therapy were designated as Group 1, while those who died or underwent liver transplantation were designated as Group 2. RESULTS: There were 37 (40...
September 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27755342/liver-transplantation-for-children-with-primary-sclerosing-cholangitis-and-autoimmune-hepatitis-unos-database-analysis
#15
Jacqueline Jossen, Rachel Annunziato, Hee-Sung Kim, Jaime Chu, Ronen Arnon
OBJECTIVES: Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are poorly studied in the Pediatric End Stage Liver Disease (PELD) era. We aimed to characterize the outcome of LT in children with AIH and PSC. METHODS: Children ≤18 years with PSC or AIH who had a first, isolated LT from 2002-2012 were identified from the UNOS database...
October 13, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27741059/intensive-care-management-of-pediatric-acute-liver-failure
#16
Riad Lutfi, Kamal Abulebda, Mara E Nitu, Jean P Molleston, Molly A Bozic, Girish Subbarao
Pediatric acute liver failure is rare but life-threatening illness that occurs in children without preexisting liver disease. The rarity of the disease, along with its severity and heterogeneity, presents unique clinical challenges to the physicians providing care for pediatric patients with acute liver failure. In this review, practical clinical approaches to the care of critically ill children with acute liver failure are discussed with an organ system specific approach. The underlying pathophysiological processes, major areas of uncertainty, and approaches to the critical care management of pediatric acute liver failure are also reviewed...
October 12, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27736823/corticosteroid-use-and-growth-after-pediatric-solid-organ-transplantation-a-systematic-review-and-meta-analysis
#17
Anne Tsampalieros, Greg A Knoll, Amber O Molnar, Nicholas Fergusson, Dean A Fergusson
BACKGROUND: A number of corticosteroid minimization and avoidance protocols for post-solid organ transplant have been developed. The study objective was to examine the effect of corticosteroid withdrawal/avoidance on growth and safety parameters in pediatric solid organ transplant recipients. METHODS: A systematic review using Medline and Embase was performed. All randomized controlled trials (RCT) and observational studies comparing corticosteroid withdrawal/avoidance to controls receiving corticosteroids in pediatric transplant recipients which reported growth as change in height or final height were included...
April 2017: Transplantation
https://www.readbyqxmd.com/read/27621562/clinically-profiling-pediatric-patients-with-dengue
#18
Sriram Pothapregada, Banupriya Kamalakannan, Mahalaskhmy Thulasingham, Srinivasan Sampath
OBJECTIVE: To study the clinical profile and outcome of dengue fever in children at a tertiary care hospital in Puducherry. MATERIALS AND METHODS: All children (0-12 years of age) diagnosed and confirmed as dengue fever from August 2012 to January 2015 were reviewed retrospectively from hospital case records as per the revised World Health Organization guidelines for dengue fever. The diagnosis was confirmed by NS1 antigen-based ELISA test or dengue serology for IgM and IgG antibodies, and the data were analyzed using SPSS 16...
July 2016: Journal of Global Infectious Diseases
https://www.readbyqxmd.com/read/27612896/aetiology-and-outcome-of-acute-liver-failure-in-children-experience-at-a-tertiary-care-hospital-of-bangladesh
#19
M W Mazumder, A B Karim, M Rukunuzzaman, M A Rahman
Acute liver failure (ALF) is a rapidly progressive, potentially fatal syndrome resulting from rapid death or injury to a large proportion of hepatocytes, caused by a variety of insult, leaving insufficient hepatic paranchymal mass to sustain liver function. The aetiology of ALF varies according to the age of patient and development of the country. The outcome of ALF also varies according to aetiology: survival is better in paracetamol poisoning whereas it is poor in metabolic diseases. The present study was undertaken to observe the underlying aetiology and outcome of ALF in children under 18 years of age admitted at the department of Paediatric Gastroenterology & Nutrition, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh...
July 2016: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/27596079/-relationship-between-changes-of-increased-amylase-or-lipase-levels-and-pancreas-injury-in-critically-ill-children
#20
(no author information available yet)
OBJECTIVE: To observe the occurrence rate of elevated serum amylase and lipase in critically ill children, to explore the relationship between elevated pancreatic enzymes and pancreatic injury, and the effect of elevated pancreatic enzymes on state of illness and the prognosis. METHOD: The data of cases treated in pediatric intensive care unit(ICU) of 17 children's hospitals were collected prospectively from January 2012 to March 2014 according to the inclusion and exclusion criteria...
September 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
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