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Encephalopathy

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https://www.readbyqxmd.com/read/29232283/levocarnitine-for-the-treatment-of-valproic-acid-induced-hyperammonemic-encephalopathy-in-children-the-experience-of-large-tertiary-care-pediatric-hospital-and-a-poison-center
#1
Miguel Glatstein, Pedro Bonifacio Rino, Silvina de Pinho, Dennis Scolnik, Dikla Pivko-Levi, Christopher Hoyte
BACKGROUND: Although rare, symptomatic hyperammonemia is sometimes associated with valproic acid (VPA), especially in children. L-carnitine (levocarnitine), sometimes classified as an essential amino acid, is vital to mitochondrial utilization of fatty acids and can be helpful in treating this condition. The data supporting this, however, are limited. STUDY QUESTION: The aim of the study was to illustrate the role of L-carnitine in the treatment of patients with VPA-induced hyperammonemic encephalopathy (VPE) at 2 different institutions...
November 22, 2017: American Journal of Therapeutics
https://www.readbyqxmd.com/read/29232196/mechanisms-of-action-of-intravenous-immunoglobulin-in-septic-encephalopathy
#2
Figen Esen, Perihan Ergin Ozcan, Erdem Tuzun, M Dustin Boone
Acute brain dysfunction associated with sepsis is a serious complication that results in morbidity and mortality. Intravenous immunoglobulin (IVIg) treatment is known to alleviate behavioral deficits in the experimentally induced model of sepsis. To delineate the mechanisms by which IVIg treatment prevents neuronal dysfunction, an array of immunological and apoptosis markers was investigated. Our results suggest that IVIgG and IgGAM administration ameliorates neuronal dysfunction and behavioral deficits by reducing apoptotic cell death and glial cell proliferation...
December 12, 2017: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/29231188/major-hepatic-complications-in-ursodeoxycholic-acid-treated-patients-with-primary-biliary-cholangitis-risk-factors-and-time-trends-in-incidence-and-outcome
#3
Maren H Harms, Willem J Lammers, Douglas Thorburn, Christophe Corpechot, Pietro Invernizzi, Harry L A Janssen, Pier M Battezzati, Frederik Nevens, Keith D Lindor, Annarosa Floreani, Cyriel Y Ponsioen, Marlyn J Mayo, George N Dalekos, Tony Bruns, Albert Parés, Andrew L Mason, Xavier Verhelst, Kris V Kowdley, Jorn C Goet, Gideon M Hirschfield, Bettina E Hansen, Henk R van Buuren
OBJECTIVES: In this era of near universal ursodeoxycholic acid (UDCA) treatment for primary biliary cholangitis (PBC), progression to cirrhosis still occurs in an important proportion of patients. The aim of this study was to describe the incidence of cirrhosis-associated complications in patients with PBC and assess risk factors and impact on survival. METHODS: Cohorts of UDCA-treated patients from 16 European and North-American liver centers were included. We used Cox proportional hazards assumptions and Kaplan-Meier estimates...
December 12, 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29230145/practical-issues-in-the-management-of-overt-hepatic-encephalopathy
#4
Ganesh Pantham, Kevin D Mullen
Hepatic encephalopathy (HE) is a condition that encompasses a range of neuropsychiatric abnormalities in patients with significant liver disease. Overt HE occurs in approximately 30% to 45% of patients with cirrhosis. This article discusses practical issues in the management of patients with overt HE and cirrhosis, including a recently developed 4-pronged approach that consists of identifying and correcting precipitating factors, recognizing and treating concomitant medical conditions, commencing empiric treatment, and caring for the unconscious patient...
November 2017: Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29230112/clinical-images-transjugular-intrahepatic-portosystemic-shunt-reduction-for-management-of-recurrent-hepatic-encephalopathy
#5
Hafez Khalili, Daryl Goldman, Sarah Frischhertz, David Kirsch
No abstract text is available yet for this article.
2017: Ochsner Journal
https://www.readbyqxmd.com/read/29228911/intrapartum-factors-associated-with-neonatal-hypoxic-ischemic-encephalopathy-a-case-controlled-study
#6
Vanessa E Torbenson, Mary Catherine Tolcher, Kate M Nesbitt, Christopher E Colby, Sherif A El-Nashar, Bobbie S Gostout, Amy L Weaver, Michaela E Mc Gree, Abimbola O Famuyide
BACKGROUND: Neonatal encephalopathy (NE) affects 2-4/1000 live births with outcomes ranging from negligible neurological deficits to severe neuromuscular dysfunction, cerebral palsy and death. Hypoxic ischemic encephalopathy (HIE) is the sub cohort of NE that appears to be driven by intrapartum events. Our objective was to identify antepartum and intrapartum factors associated with the development of neonatal HIE. METHODS: Hospital databases were searched using relevant diagnosis codes to identify infants with neonatal encephalopathy...
December 11, 2017: BMC Pregnancy and Childbirth
https://www.readbyqxmd.com/read/29227269/reye-ray-s-syndrome-a-problem-everyone-should-remember
#7
I Chornomydz, O Boyarchuk, A Chornomydz
Reye syndrome is a rare but a very dangerous emergency that children and teenagers suffer. This threatening condition occurs during the treatment of fever in the clinical course of viral diseases with drugs containing acetylsalicylic acid and other salicylates. The high mortality rate from this disease is associated with the development of a rapidly progressing toxic encephalopathy and hepatic insufficiency. The etiology and pathogenesis of the Reye syndrome, despite the large number of investigations, is not clear enough...
November 2017: Georgian Medical News
https://www.readbyqxmd.com/read/29226766/a-case-report-of-living-donor-liver-transplantation-for-fulminant-hepatitis-related-to-hepatitis-e-virus-infection
#8
Keita Shimata, Yasuhiko Sugawara, Hidekazu Yamamoto, Hiroaki Okamoto, Koushi Uchida, Seiichi Kawabata, Kaori Isono, Shintaro Hayashida, Yukihiro Inomata
Hepatitis E virus (HEV) infection which may become fulminant, especially in elderly people is more common than previously recognized in develop countries. Here we report successful living-donor liver transplantation (LDLT) in a case of acute liver failure due to HEV. A 63-year-old Japanese man with no previous history of liver disease was admitted for severe acute hepatitis. Detection of anti-HEV immunoglobulin A established a diagnosis of this virus-related liver failure. The patient suffered from hepatic encephalopathy 10 days after symptom onset and underwent LDLT...
January 1, 2017: Progress in Transplantation
https://www.readbyqxmd.com/read/29226764/chimeric-antigen-receptor-t-cells-in-refractory-b-cell-lymphomas
#9
Stephen J Schuster, Jakub Svoboda, Elise A Chong, Sunita D Nasta, Anthony R Mato, Özlem Anak, Jennifer L Brogdon, Iulian Pruteanu-Malinici, Vijay Bhoj, Daniel Landsburg, Mariusz Wasik, Bruce L Levine, Simon F Lacey, Jan J Melenhorst, David L Porter, Carl H June
Background Patients with diffuse large B-cell lymphoma or follicular lymphoma that is refractory to or that relapses after immunochemotherapy and transplantation have a poor prognosis. High response rates have been reported with the use of T cells modified by chimeric antigen receptor (CAR) that target CD19 in B-cell cancers, although data regarding B-cell lymphomas are limited. Methods We used autologous T cells that express a CD19-directed CAR (CTL019) to treat patients with diffuse large B-cell lymphoma or follicular lymphoma that had relapsed or was refractory to previous treatments...
December 10, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29226520/a-novel-homozygous-slc25a1-mutation-with-impaired-mitochondrial-complex-v-possible-phenotypic-expansion
#10
Idan Cohen, Orna Staretz-Chacham, Ohad Wormser, Yonatan Perez, Ann Saada, Rotem Kadir, Ohad S Birk
SLC25A1 mutations are associated with combined D,L-2-hydroxyglutaric aciduria (DL- 2HGA; OMIM #615182), characterized by muscular hypotonia, severe neurodevelopmental dysfunction and intractable seizures. SLC25A1 encodes the mitochondrial citrate carrier (CIC), which mediates efflux of the mitochondrial tricarboxylic acid (TCA) cycle intermediates citrate and isocitrate in exchange for cytosolic malate. Only a single family with an SLC25A1 mutation has been described in which mitochondrial respiratory chain dysfunction was documented, specifically in complex IV...
December 11, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29225908/an-unusual-presentation-of-tyrosine-hydroxylase-deficiency
#11
Linn E Katus, Steven J Frucht
Background: Dopa-responsive dystonia (DRD) has largely been associated with autosomal dominant mutations in the GCH1 gene leading to GTP cyclohydrolase 1 deficiency. More recently, a deficiency in tyrosine hydroxylase (TH) has been recognized to cause DRD. This is a rare disorder resulting from genetic mutations in the TH gene on chromosome 11. The phenotype ranges from DRD with complete resolution on levodopa to infantile parkinsonism and encephalopathy only partially responsive to levodopa...
2017: Journal of Clinical Movement Disorders
https://www.readbyqxmd.com/read/29225466/korsakoff-s-syndrome-a-critical-review
#12
REVIEW
Nicolaas Jm Arts, Serge Jw Walvoort, Roy Pc Kessels
In this review, we present a survey on Korsakoff's syndrome (KS), a residual syndrome in patients who suffered from a Wernicke encephalopathy (WE) that is predominantly characterized by global amnesia, and in more severe cases also by cognitive and behavioral dysfunction. We describe the history of KS and its definition, its epidemiology, and the lack of consensus criteria for its diagnosis. The cognitive and behavioral symptoms of KS, which include anterograde and retrograde amnesia, executive dysfunction, confabulation, apathy, as well as affective and social-cognitive impairments, are discussed...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/29225269/late-relapse-after-a-sustained-virologic-response-at-24-weeks-after-treatment-with-daclatasvir-and-asunaprevir-combination-therapy-for-chronic-hepatitis-c-virus-genotype-1b-infection-with-liver-cirrhosis
#13
Haruki Uojima, Shuko Murakami, Seigo Nakatani, Hisashi Hidaka, Atsuko Takeuchi, Yoshiaki Tanaka, Tomoyoshi Inoue, Keiko Yamane, Kousuke Kubota, Takahide Nakazawa, Akitaka Shibuya, Yasuhito Tanaka, Wasaburo Koizumi
There have been few studies on relapse after a sustained virological response in hepatitis C virus (HCV) patients treated with interferon-free regimens. Thus, the risk of late relapse in patients treated with interferon-free therapy remains unclear. A 67-year-old woman with HCV genotype 1b and liver cirrhosis received oral daclatasvir and asunaprevir. Combination therapy was stopped after 4 weeks because of an episode of encephalopathy. Nonetheless, an HCV polymerase chain reaction at 24 weeks posttreatment was negative...
December 8, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29225265/posterior-reversible-encephalopathy-syndrome-after-lenvatinib-therapy-in-a-patient-with-anaplastic-thyroid-carcinoma
#14
Yoko Osawa, Rikako Gozawa, Keisuke Koyama, Takeo Nakayama, Tadashi Sagoh, Hiroshi Sunaga
Posterior reversible encephalopathy syndrome (PRES) is a rare reversible neurological syndrome that causes subcortical vasogenic brain edema and which is associated with the use of target-specific agents. Lenvatinib is a target-specific agent that was recently approved for inoperable thyroid cancer. We herein describe the case of a 66-year-old woman with anaplastic thyroid cancer (ATC) who was treated with lenvatinib and who subsequently developed PRES. The clinical and radiological findings improved after suspending therapy for 1 week, and there was no recurrence with intermittent lower-dose lenvatinib treatment...
December 8, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29223591/mild-hypothermia-protects-hippocampal-neurons-from-oxygen-glucose-deprivation-injury-through-inhibiting-caspase-3-activation
#15
Tianen Zhou, Hui Lin, Longyuan Jiang, Tao Yu, Chaotao Zeng, Juanhua Liu, Zhengfei Yang
Mild hypothermia (MH) is thought to be one of the most effective therapeutic methods to treat hypoxic-ischemic encephalopathy (HIE) after cardiac arrest (CA). However, its precise mechanisms remain unclear. In this research, hippocampal neurons were cultured and treated with mild hypothermia and Ac-DEVD-CHO after oxygen-glucose deprivation (OGD). The activity of caspase-3 was detected, in order to find the precise concentration of Ac-DEVD-CHO with the same protective role in OGD injury as MH treatment. Western blot and immunofluorescence staining were conducted to analyze the effects of MH and Ac-DEVD-CHO on the expressions of caspase-3, caspase-8, and PARP...
December 6, 2017: Cryobiology
https://www.readbyqxmd.com/read/29223473/-cryptogenic-west-syndrome-clinical-profile-response-to-treatment-and-prognostic-factors
#16
María Calderón Romero, Elena Arce Portillo, Mercedes López Lobato, Beatriz Muñoz Cabello, Bárbara Blanco Martínez, Marcos Madruga Garrido, Olga Alonso Luego
INTRODUCTION: West syndrome (WS) is an age-dependent epileptic encephalopathy in which the prognosis varies according to the, not always identified, underlying origin. OBJECTIVES: To define the profile of cryptogenic (a least studied isolated sub-group) WS, in Spain. To study its outcome, response to different treatments, and to establish prognostic factors. PATIENTS AND METHODS: The study included a review of the medical records of 16 patients diagnosed with cryptogenic WS during the period, 2000-2015...
December 6, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/29222263/crisis-management-in-the-treatment-of-childhood-acute-lymphoblastic-leukemia-putting-right-what-can-go-wrong-emergency-complications-of-disease-and-treatment
#17
REVIEW
Rachael Hough, Ajay Vora
The improvement in overall survival in children with acute lymphoblastic leukemia (ALL) over the last 5 decades has been considerable, with around 90% now surviving long term. The risk of relapse has been reduced to such an extent that the risk of treatment-related mortality is now approaching that of mortality caused by relapse. Toxicities may also lead to the suboptimal delivery of chemotherapy (treatment delays, dose reductions, dose omissions), potentially increasing relapse risk, and short- and long-term morbidity, adding to the "burden of therapy" in an increasing number of survivors...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222087/dose-dependent-relationship-between-acidosis-at-birth-and-likelihood-of-death-or-cerebral-palsy
#18
Rod Kelly, S M Ramaiah, Helen Sheridan, Hilary Cruickshank, Magda Rudnicka, Chris Kissack, Julie-Clare Becher, Ben J Stenson
BACKGROUND: The acid-base status of infants around birth can provide information about their past, current and future condition. Although umbilical cord blood pH <7.0 or base deficit ≥12 mmol/L is associated with increased risk of adverse outcome, there is uncertainty about the prognostic value of degree of acidosis as previous studies have used different variables, thresholds, outcomes and populations. METHODS: Retrospective review of routinely collected clinical data in all live-born inborn infants of 35 weeks gestation or more delivered between January 2005 and December 2013 at the Simpson Centre for Reproductive Health, Edinburgh, UK...
December 8, 2017: Archives of Disease in Childhood. Fetal and Neonatal Edition
https://www.readbyqxmd.com/read/29221912/variable-clinical-phenotype-in-two-siblings-with-aicardi-gouti%C3%A3-res-syndrome-type-6-and-a-novel-mutation-in-the-adar-gene
#19
Lisa Schmelzer, Martin Smitka, Christine Wolf, Nadja Lucas, Victoria Tüngler, Gabriele Hahn, Andreas Tzschach, Nataliya Di Donato, Min Ae Lee-Kirsch, Maja von der Hagen
Aicardi-Goutières syndrome (AGS) is a hereditary inflammatory encephalopathy resulting in severe neurological damage in the majority of cases. We report on two siblings with AGS6 due to compound heterozygosity for a known and a novel mutation in the ADAR gene and a strikingly variable phenotype. The first sibling presented at 12 months of age with a subacute encephalopathy following a mild respiratory infection. The child developed a spastic tetraparesis, generalized dystonia and dysarthria. In contrast, the younger sibling presented with an acute episode of neurological impairment in his third year of life, from which he recovered without sequelae within a few weeks...
November 22, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29221886/a-model-including-sarcopenia-surpasses-the-meld-score-in-predicting-waiting-list-mortality-in-cirrhotic-liver-transplant-candidates
#20
Jeroen Laurens Ad van Vugt, Louise Johanna Maria Alferink, Stefan Buettner, Marcia Patricia Gaspersz, Daphne Bot, Sarwa Darwish Murad, Shirin Feshtali, Peter Martinus Adranius van Ooijen, Wojciech Grzegorz Polak, Robert Jan Porte, Bart van Hoek, Aad Pieter van den Berg, Herold Johnny Metselaar, Jan Nicolaas Maria IJzermans
BACKGROUND AND AIMS: Frail patients with low MELD scores may be underprioritised. Low skeletal muscle mass (i.e. sarcopenia) has been identified as risk factor for waiting list mortality and a recent study proposed to incorporate sarcopenia in the MELD score (i.e. MELD-Sarcopenia score). We aimed to investigate the association between sarcopenia and waiting list mortality, and to validate the MELD-Sarcopenia score (i.e. MELD+10.35∗Sarcopenia). METHODS: We identified consecutive patients with cirrhosis listed for liver transplantation in the Eurotransplant registry between 2007-2014 and measured skeletal muscle mass on computed tomography (CT)...
December 5, 2017: Journal of Hepatology
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