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Hyperammonemia

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https://www.readbyqxmd.com/read/29150106/reactions-related-to-asparaginase-infusion-in-a-10-year-retrospective-cohort
#1
Amanda Cabral Dos Santos, Marcelo Gerardin Poirot Land, Nathalia Peroni da Silva, Kelly Oliveira Santos, Elisangela da Costa Lima-Dellamora
INTRODUCTION: Although it is an essential component of the treatment of acute lymphoid leukemia in children, asparaginase causes adverse reactions that sometimes make it impossible to use it fully. Hypersensitivity reactions are the most frequent and may lead to early discontinuation of treatment. The present study aimed to investigate suspicions of adverse reactions during the infusion of asparaginase in a pediatric cohort. METHODS: A retrospective observational study was carried out at a university pediatric institute in the state of Rio de Janeiro...
October 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/29137068/carnitine-acylcarnitine-translocase-deficiency-with-c-199-10-t-g-and-novel-c-1a-g-mutation-two-case-reports-and-brief-literature-review
#2
Hui-Ming Yan, Hao Hu, Aisha Ahmed, Bing-Bing Feng, Jing Liu, Zheng-Jun Jia, Hua Wang
RATIONALE: Carnitine-acylcarnitine translocate deficiency (CACTD) is a rare and life-threatening, autosomal recessive disorder of fatty acid β-oxidation characterized by hypoketotic hypoglycemia, hyperammonemia, cardiomyopathy, liver dysfunction, and muscle weakness; culminating in early death. To date, CACTD cases screened from the Chinese mainland population, especially patient with compound heterozygote with c.199-10T>G and a novel c.1A>G mutation in the SLC25A20 gene has never been described...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29134448/hyperammonemia-associated-with-distal-renal-tubular-acidosis-or-urinary-tract-infection-a-systematic-review
#3
Caterina M Clericetti, Gregorio P Milani, Sebastiano A G Lava, Mario G Bianchetti, Giacomo D Simonetti, Olivier Giannini
BACKGROUND: Hyperammonemia usually results from an inborn error of metabolism or from an advanced liver disease. Individual case reports suggest that both distal renal tubular acidosis and urinary tract infection may also result in hyperammonemia. METHODS: A systematic review of the literature on hyperammonemia secondary to distal renal tubular acidosis and urinary tract infection was conducted. RESULTS: We identified 39 reports on distal renal tubular acidosis or urinary tract infections in association with hyperammonemia published between 1980 and 2017...
November 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29118705/oxaliplatin-induced-hyperammonemic-encephalopathy-in-a-patient-with-metastatic-pancreatic-cancer-a-case-report
#4
Takatsugu Ogata, Hironaga Satake, Misato Ogata, Yukimasa Hatachi, Hisateru Yasui
Oxaliplatin-based chemotherapy is widely used to treat advanced cancer. Oxaliplatin-induced hyperammonemic encephalopathy is rarely reported. Here, we report a case of oxaliplatin-induced hyperammonemic encephalopathy occurring after gemcitabine plus oxaliplatin (GEMOX) chemotherapy in a patient with pancreatic cancer. A 76-year-old man received GEMOX regimen as first-line treatment for pancreatic adenocarcinoma with peritoneal dissemination. GEMOX consists of gemcitabine (1,000 mg/m(2)) and oxaliplatin (100 mg/m(2)) on day 1, repeated every 2 weeks...
September 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/29107806/chronic-hyperammonemia-alters-in-opposite-ways-membrane-expression-of-glua1-and-glua2-ampa-receptor-subunits-in-cerebellum-molecular-mechanisms-involved
#5
Andrea Cabrera-Pastor, Lucas Taoro-González, Esperanza López-Merino, Ferran Celma, Marta Llansola, Vicente Felipo
Hyperammonemia contributes to altered neurotransmission and cognition in patients with hepatic encephalopathy. Hyperammonemia in rats affects differently high- and low-affinity AMPA receptors (AMPARs) in cerebellum. We hypothesized that hyperammonemia would alter differently membrane expression of AMPARs GluA1 and GluA2 subunits by altering its phosphorylation. This work aims were: 1) assess if hyperammonemia alters GluA1 and GluA2 subunits membrane expression in cerebellum and 2) analyze the underlying mechanisms...
October 28, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29095003/-spanish-multicenter-study-hyperammonemia-not-associated-with-inborn-errors-of-metabolism-in-children
#6
Mercedes Gil Campos, Javier Blasco-Alonso, Concepción Sierra Córcoles, José Luis Cuevas Cervera, Luisa Arrabal Fernández, Luis Aldámiz Echevarría, Amaya Belanger
INTRODUCTION: The hyperammonemic encephalopathy induced by causes different from inborn errors of metabolism is a relatively uncommon but severe complication. OBJETIVES: To study the characteristics of a secondary hyperammonemia episode to discern the triggering causes to get to the diagnosis, and the development in the therapeutic intervention. METHODS: A multicenter retrospective study of children with hyperammonemia unrelated to inborn errors of metabolism, conducted in Spanish hospitals...
July 28, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/29094226/aminoacidopathies-prevalence-etiology-screening-and-treatment-options
#7
REVIEW
Muhammad Wasim, Fazli Rabbi Awan, Haq Nawaz Khan, Abdul Tawab, Mazhar Iqbal, Hina Ayesha
Inborn errors of metabolism (IEMs) are a group of inherited metabolic disorders which are caused by mutations in the specific genes that lead to impaired proteins or enzymes production. Different metabolic pathways are perturbed due to the deficiency or lack of enzymes. To date, more than 500 IEMs have been reported with most of them being untreatable. However, fortunately 91 such disorders are potentially treatable, if diagnosed at an earlier stage of life. IEMs have been classified into different categories and one class of IEMs, characterized by the physiological disturbances of amino acids is called as aminoacidopathies...
November 1, 2017: Biochemical Genetics
https://www.readbyqxmd.com/read/29080224/safety-tolerability-and-pharmacokinetics-of-l-ornithine-phenylacetate-in-patients-with-acute-liver-injury-failure-and-hyperammonemia
#8
R Todd Stravitz, Michelle Gottfried, Valerie Durkalski, Robert J Fontana, A James Hanje, David Koch, Bilal Hameed, Daniel Ganger, Ram M Subramanian, Stan Bukofzer, William R Ravis, Kristen Clasen, Averell Sherker, Lanna Little, William M Lee
Cerebral edema remains a significant cause of morbidity and mortality in patients with acute liver failure (ALF) and has been linked to elevated blood ammonia levels. L-ornithine phenylacetate (OPA) may decrease ammonia by promoting its renal excretion as phenylacetylglutamine (PAGN), decreasing the risk of cerebral edema. We evaluated the safety, tolerability, and pharmacokinetics of OPA in patients with ALF and acute liver injury (ALI), including those with renal failure. Forty-seven patients with ALI/ALF and ammonia ≥60µM were enrolled...
October 28, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29072450/ebselen-mechanisms-of-glutamate-dehydrogenase-and-glutaminase-enzyme-inhibition
#9
Yan Yu, Yanhong Jin, Jie Zhou, Haoqiang Ruan, Han Zhao, Shiying Lu, Yue Zhang, Di Li, Xiaoyun Ji, Benfang Helen Ruan
Ebselen modulates target proteins through redox reactions with selenocysteine/cysteine residues, or through binding to the zinc finger domains. However, a recent contradiction in ebselen inhibition of kidney type glutaminase (KGA) stimulated our interest in investigating its inhibition mechanism with glutamate dehydrogenase (GDH), KGA, thioredoxin reductase (TrxR), and glutathione S-transferase. Fluorescein- or biotin-labeled ebselen derivatives were synthesized for mechanistic analyses. Biomolecular interaction analyses showed that only GDH, KGA, and TrxR proteins can bind to the ebselen derivative, and the binding to GDH and KGA could be competed off by glutamine or glutamate...
November 7, 2017: ACS Chemical Biology
https://www.readbyqxmd.com/read/29062904/ammonia-induced-mitochondrial-dysfunction-and-energy-metabolism-disturbances-in-isolated-brain-and-liver-mitochondria-and-the-effect-of-taurine-administration-relevance-to-hepatic-encephalopathy-treatment
#10
Hossein Niknahad, Akram Jamshidzadeh, Reza Heidari, Mahdi Zarei, Mohammad Mehdi Ommati
INTRODUCTION: Ammonia-induced oxidative stress, mitochondrial dysfunction, and energy crisis are known as some the major mechanisms of brain injury in hepatic encephalopathy (HE). Hyperammonemia also affects the liver and hepatocytes. Therefore, targeting mitochondria seems to be a therapeutic point of intervention in the treatment of HE. Taurine is an abundant amino acid in the human body. Several biological functions including the mitochondrial protective properties are attributed to this amino acid...
September 2017: Clin Exp Hepatol
https://www.readbyqxmd.com/read/29055942/interaction-of-excitatory-amino-acid-transporters-1-3-eaat1-eaat2-eaat3-with-n-carbamoylglutamate-and-n-acetylglutamate
#11
Birgitta C Burckhardt, Gerhard Burckhardt
BACKGROUND/AIMS: Inborn deficiency of the N-acetylglutamate synthase (NAGS) impairs the urea cycle and causes neurotoxic hyperammonemia. Oral administration of N-carbamoylglutamate (NCG), a synthetic analog of N-acetylglutamate (NAG), successfully decreases plasma ammonia levels in the affected children. Due to structural similarities to glutamate, NCG may be absorbed in the intestine and taken up into the liver by excitatory amino acid transporters (EAATs). METHODS: Using Xenopus laevis oocytes expressing either human EAAT1, 2, or 3, or human sodium-dependent dicarboxylate transporter 3 (NaDC3), transport-associated currents of NAG, NCG, and related dicarboxylates were assayed...
October 20, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29038875/sclerotherapy-for-rectal-varices-by-a-small-bore-needle-puncture-through-the-greater-sciatic-foramen
#12
Shuji Kariya, Miyuki Nakatani, Takuji Maruyama, Yasuyuki Ono, Yutaka Ueno, Hiroshi Shimizu, Atsushi Komemushi, Noboru Tanigawa
PURPOSE: To report a sclerotherapy technique for rectal varices consisting of direct puncture of the superior rectal vein with a small-bore sheathed needle via the greater sciatic foramen without insertion of a sheath or catheter. MATERIALS AND METHODS: The subjects of this retrospective study were three consecutive patients who underwent embolization of rectal varices, two for rupture of rectal varices and one for hepatic encephalopathy and hyperammonemia. A 5% solution of ethanolamine oleate with iodinated contrast agent (5% EOI) was injected through puncture of the superior rectal vein and carried in the blood flow, after which n-butyl cyanoacrylate mixed with lipiodol (NBCA-Lip) was immediately injected to stop the blood flow...
October 16, 2017: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/29035972/hyperammonemia-and-proteostasis-in-cirrhosis
#13
Srinivasan Dasarathy, Maria Hatzoglou
PURPOSE OF REVIEW: Skeletal muscle loss or sarcopenia is a frequent complication of cirrhosis that adversely affects clinical outcomes. As skeletal muscle is the largest store of proteins in the body, proteostasis or protein homeostasis is required for maintenance of muscle mass. This review will focus on disordered skeletal muscle proteostasis in liver disease. RECENT FINDINGS: Increased skeletal muscle uptake of ammonia initiates responses that result in disordered proteostasis including impaired protein synthesis and increased autophagy...
October 14, 2017: Current Opinion in Clinical Nutrition and Metabolic Care
https://www.readbyqxmd.com/read/29034137/valproic-induced-hyperammonemic-encephalopathy-in-a-known-case-of-epilepsy
#14
Syed F Imam, Omair Ul Haq Lodhi, Rizwan Zafar, Saneeya Nasim, Waseem T Malik
Valproic acid, a broad-spectrum anticonvulsant drug, commonly causes elevated ammonia levels, which is usually asymptomatic in most cases. On rare occasions, potentially fatal hyperammonemia-induced encephalopathy can occur. We present a case of a 24-year-old female who presented to the emergency department with status epilepticus that was being managed with valproic acid. Further workup was done because of prolonged postictal state, which revealed increased ammonia levels; she was eventually diagnosed with valproic-induced hyperammonemic encephalopathy...
August 10, 2017: Curēus
https://www.readbyqxmd.com/read/29033250/propionyl-coa-carboxylase-a-review
#15
REVIEW
Parith Wongkittichote, Nicholas Ah Mew, Kimberly A Chapman
Propionyl-CoA carboxylase (PCC) is the enzyme which catalyzes the carboxylation of propionyl-CoA to methylmalonyl-CoA and is encoded by the genes PCCA and PCCB to form a hetero-dodecamer. Dysfunction of PCC leads to the inherited metabolic disorder propionic acidemia, which can result in an affected individual presenting with metabolic acidosis, hyperammonemia, lethargy, vomiting and sometimes coma and death if not treated. Individuals with propionic acidemia also have a number of long term complications resulting from the dysfunction of the PCC enzyme...
October 7, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29030642/saline-is-as-effective-as-nitrogen-scavengers-for-treatment-of-hyperammonemia
#16
G van Straten, M G M de Sain-van der Velden, I M van Geijlswijk, R P Favier, S J Mesu, N E Holwerda-Loof, M van der Ham, H Fieten, J Rothuizen, B Spee, N M Verhoeven-Duif
Urea cycle enzyme deficiency (UCED) patients with hyperammonemia are treated with sodium benzoate (SB) and sodium phenylacetate (SPA) to induce alternative pathways of nitrogen excretion. The suggested guidelines supporting their use in the management of hyperammonemia are primarily based on non-analytic studies such as case reports and case series. Canine congenital portosystemic shunting (CPSS) is a naturally occurring model for hyperammonemia. Here, we performed cross-over, randomized, placebo-controlled studies in healthy dogs to assess safety and pharmacokinetics of SB and SPA (phase I)...
October 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29027067/human-heterologous-liver-cells-transiently-improve-hyperammonemia-and-ureagenesis-in-individuals-with-severe-urea-cycle-disorders
#17
Jochen Meyburg, Thomas Opladen, Ute Spiekerkötter, Andrea Schlune, Jens-Peter Schenk, Jan Schmidt, Jürgen Weitz, Jürgen Okun, Friederike Bürger, Tawfeg Ben Omran, Ghassan Abdoh, Hilal Al Rifai, Ahmad Monavari, Vassiliki Konstantopoulou, Stefan Kölker, Marc Yudkoff, Georg F Hoffmann
BACKGROUND: Urea cycle disorders (UCDs) still have a poor prognosis despite several therapeutic advancements. As liver transplantation can provide a cure, liver cell therapy (LCT) might be a new therapeutic option in these patients. METHODS: Twelve patients with severe UCDs were included in this prospective clinical trial. Patients received up to six infusions of cryopreserved human heterologous liver cells via a surgically placed catheter in the portal vein. Portal vein pressure, portal vein flow, and vital signs were monitored continuously...
October 12, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28977297/initial-clinical-presentation-in-cases-of-inborn-errors-of-metabolism-in-a-reference-children-s-hospital-still-a-diagnostic-challenge
#18
Andressa Romão, Priscila Endlich Alves Simon, José Eduardo Coutinho Góes, Louise Lapagessede Camargo Pinto, Roberto Giugliani, Gisele Rozone de Luca, Francisca Ligia Cirilo Carvalho
OBJECTIVE: To assess the initial clinical presentation of confirmed cases of inborn errors of metabolism (IEM) at a reference facility for pediatric care. METHODS: Cross-sectional, observational and descriptive study with data collection of outpatients, from January 2009 to December 2013. Inclusion criterion: referral to IEM investigation. Exclusion criterion: prior diagnosis of IEM. Analyzed variables: identification data; status of diagnostic investigation; family history of IEM; initial clinical presentation, laboratory abnormalities related to the hypothesis of IEM...
July 2017: Revista Paulista de Pediatria: Orgão Oficial da Sociedade de Pediatria de São Paulo
https://www.readbyqxmd.com/read/28964916/portacaval-shunting-causes-differential-mitochondrial-superoxide-production-in-brain-regions
#19
Elena A Kosenko, Lyudmila A Tikhonova, Gubidat A Alilova, Carmina Montoliu, George E Barreto, Gjumrakch Aliev, Yury G Kaminsky
The portacaval shunting (PCS) prevents portal hypertension and recurrent bleeding of esophageal varices. On the other hand, it can induce chronic hyperammonemia and is considered to be the best model of mild hepatic encephalopathy (HE). Pathogenic mechanisms of HE and dysfunction of the brain in hyperammonemia are not fully elucidated, but it was originally suggested that the pathogenetic defect causes destruction of antioxidant defense which leads to an increase in the production of reactive oxygen species (ROS) and the occurrence of oxidative stress...
September 28, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28953637/sudden-valproate-induced-hyperammonemia-managed-with-l-carnitine-in-a-medically-healthy-bipolar-patient-essential-review-of-the-literature-and-case-report
#20
REVIEW
Carlo Ignazio Cattaneo, Francesca Ressico, Roberta Valsesia, Pierluigi D'Innella, Matteo Ballabio, Michele Fornaro
RATIONALE: Valproic Acid is a commonly used psychiatric drug primarily used as a mood stabilizer. Mild hyperammonemia is a Valproic Acid common adverse effect. This report presents an example of treated hyperammonemia on Valproic acid therapy managed with L-carnitine administration in BD patients characterized by sudden vulnerability. PATIENT CONCERNS: We report the case of a 29-year-old man suffering from bipolar disorder (BD) and substance use disorder who exhibited sudden altered mental status upon admittance to the inpatient unit...
September 2017: Medicine (Baltimore)
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