keyword
MENU ▼
Read by QxMD icon Read
search

Hyperammonemia

keyword
https://www.readbyqxmd.com/read/29334534/symptomatic-hyperammonemia-with-erwinia-chrysanthemi-derived-asparaginase-in-pediatric-leukemia-patients
#1
Nathan Gossai, Michael Richards, Lara Boman, Yoav Messinger, Sara Gernbacher, Joanna Perkins, Bruce Bostrom
Erwinia chrysanthemi-derived asparaginase is increasingly integral to acute lymphoblastic leukemia therapy. In our series, 16% of patients developed symptomatic hyperammonemia following Erwinia administration with symptoms including refractory nausea, vomiting, profound fatigue, malaise, and coma. This series of patients receiving Erwinia indicates higher than expected incidence of hyperammonemia, correlation between ammonia and asparaginase levels and therapeutic asparaginase activity levels despite dose reduction...
January 12, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29326055/a-retrospective-biochemical-molecular-and-neurocognitive-review-of-saudi-patients-with-argininosuccinic-aciduria
#2
Ruqaiah AlTassan, Dalal Bubshait, Faiqa Imtiaz, Zuhair Rahbeeni
A retrospective review was compiled of 54 patients with argininosuccinic aciduria who were either identified through the Saudi National Newborn Screening Program or diagnosed clinically from January 2000 to December 2015. The duration of follow-up is from 2 to 19 years. The majority of patients (65%) originated from the central province of Saudi Arabia. The mean patient age at review was 10 years (2-19 years), 92% received an early diagnosis (<28 days of age) and most were symptomatic at the time of the diagnosis (n = 34)...
January 8, 2018: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29314318/two-novel-cps1-mutations-in-a-case-of-carbamoyl-phosphate-synthetase-1-deficiency-causing-hyperammonemia-and-leukodystrophy
#3
Xihui Chen, Lijuan Yuan, Mao Sun, Qingbo Liu, Yuanming Wu
BACKGROUND: Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is a rare autosomal recessive disorder of the urea cycle, mostly characterized by hyperammonemia and the concomitant leukodystrophy. The onset of CPS1D can be at any age, and the clinical manifestations are variable and atypical. Genetic tests are indispensable for accurate diagnosis of CPS1D on the basis of biochemical tests. METHODS: Blood tandem mass spectrometric analysis and urea organic acidemia screening were performed on a Chinese neonatal patient with low activity, recurrent seizures, and hyperammonemia...
January 4, 2018: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/29307995/rifaximin-ameliorates-hepatic-encephalopathy-and-endotoxemia-without-affecting-the-gut-microbiome-diversity
#4
Kosuke Kaji, Hiroaki Takaya, Soichiro Saikawa, Masanori Furukawa, Shinya Sato, Hideto Kawaratani, Mitsuteru Kitade, Kei Moriya, Tadashi Namisaki, Takemi Akahane, Akira Mitoro, Hitoshi Yoshiji
AIM: To determine the efficacy of rifaximin for hepatic encephalopathy (HE) with the linkage of gut microbiome in decompensated cirrhotic patients. METHODS: Twenty patients (12 men and 8 women; median age, 66.8 years; range, 46-81 years) with decompensated cirrhosis (Child-pugh score > 7) underwent cognitive neuropsychological testing, endotoxin analysis, and fecal microbiome assessment at baseline and after 4 wk of treatment with rifaximin 400 mg thrice a day...
December 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29301138/the-effect-of-cisplatin-on-blood-ammonia-elevation-by-alanyl-glutamine-supplementation
#5
Yoko Obayashi, Kenta Kajiwara, Eiji Nakamura
BACKGROUND: Although there are many clinical studies in which the beneficial effect of glutamine formulation on mucositis induced by chemo/radiotherapy was evaluated, the results are sometimes conflicting with the report of clinical deterioration. Then, we hypothesized that chemotherapy may increase the incidence of hyperammonemia without comparable change of major parameters of hepatic/renal disorder. METHODS: To verify our hypothesis, we examined the increase in blood ammonia level with 1-h intravenous infusion of alanyl-glutamine on day 1-4 after cisplatin (CDDP) administration in rats and assessed the correlation with hepatic/renal parameters...
January 4, 2018: Pharmacology
https://www.readbyqxmd.com/read/29290677/clinical-relevance-and-cost-savings-of-levocarnitine-versus-ammonul-in-the-management-of-hyperammonemia-in-a-cancer-patient-the-impact-of-a-clinical-pharmacist
#6
Chukwuma Anyanwu, Chinonso Ezeudu, Hoa Le, Oliver Egwim
Background: Hyperammonemia, a relatively uncommon condition characterized by elevated ammonia levels in the blood, presents with varied physiological etiologies that may send patients to the intensive care unit (ICU) with encephalopathy. An immediate decrease in ammonia levels is necessary to avert neurological damage. However, due to the multifaceted nature of hyperammonemia, a definite determination of etiology is not always possible. Objective: This case report examines the clinical and economic impact of a pharmacist in managing acute hyperammonemia of unknown etiology in a 62-year-old Hispanic man who had recently been diagnosed with metastatic medullary thyroid cancer and associated hypercalcemia...
January 2018: P & T: a Peer-reviewed Journal for Formulary Management
https://www.readbyqxmd.com/read/29288802/increasing-extracellular-cgmp-in-cerebellum-in-vivo-reduces-neuroinflammation-gabaergic-tone-and-motor-in-coordination-in-hyperammonemic-rats
#7
Andrea Cabrera-Pastor, Tiziano Balzano, Vicente Hernández-Rabaza, Michele Malaguarnera, Marta Llansola, Vicente Felipo
Hyperammonemia is a main contributor to cognitive impairment and motor in-coordination in patients with hepatic encephalopathy. Hyperammonemia-induced neuroinflammation mediates the neurological alterations in hepatic encephalopathy. Intracerebral administration of extracellular cGMP restores some but not all types of cognitive impairment. Motor in-coordination, is mainly due to increased GABAergic tone in cerebellum. We hypothesized that extracellular cGMP would restore motor coordination in hyperammonemic rats by normalizing GABAergic tone in cerebellum and that this would be mediated by reduction of neuroinflammation...
December 27, 2017: Brain, Behavior, and Immunity
https://www.readbyqxmd.com/read/29285339/medium-chain-acyl-coa-dehydrogenase-deficiency-in-a-premature-infant
#8
Steven F Dobrowolski, Lina Ghaloul-Gonzalez, Jerry Vockley
Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is identified by newborn screening (NBS). The natural history of MCADD includes metabolic decompensation with hypoglycemia, hyperammonemia, seizures, coma, and death. NBS enables expectant management thus severe symptoms are rare in managed patients. We report premature birth of an MCADD affected infant and resultant management challenges. Nutritional support advanced from parenteral nutrition at 24 hours to enteral feeds. A NBS sample was collected day 2, positive results for MCADD was reported day six, and diagnostic tests were performed day seven...
November 21, 2017: Pediatric Reports
https://www.readbyqxmd.com/read/29279777/ornithine-transcarbamylase-deficiency-if-at-first-you-do-not-diagnose-try-and-try-again
#9
Christan D Santos, Robert A Ratzlaff, Jennifer C Meder, Paldeep S Atwal, Nicole E Joyce
Ornithine transcarbamylase (OTC) deficiency is well known for its diagnosis in the neonatal period. Presentation often occurs after protein feeding and manifests as poor oral intake, vomiting, lethargy progressing to seizure, respiratory difficulty, and eventually coma. Presentation at adulthood is rare (and likely underdiagnosed); however, OTC deficiency can be life-threatening and requires prompt investigation and treatment. Reports and guidelines are scarce due to its rarity. Here, we present a 59-year-old woman with a past history of irritable bowel syndrome who underwent a reparative operation for rectal prolapse and enterocele...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/29279371/enhancement-of-hepatic-autophagy-increases-ureagenesis-and-protects-against-hyperammonemia
#10
Leandro R Soria, Gabriella Allegri, Dominique Melck, Nunzia Pastore, Patrizia Annunziata, Debora Paris, Elena Polishchuk, Edoardo Nusco, Beat Thöny, Andrea Motta, Johannes Häberle, Andrea Ballabio, Nicola Brunetti-Pierri
Ammonia is a potent neurotoxin that is detoxified mainly by the urea cycle in the liver. Hyperammonemia is a common complication of a wide variety of both inherited and acquired liver diseases. If not treated early and thoroughly, it results in encephalopathy and death. Here, we found that hepatic autophagy is critically involved in systemic ammonia homeostasis by providing key urea-cycle intermediates and ATP. Hepatic autophagy is triggered in vivo by hyperammonemia through an α-ketoglutarate-dependent inhibition of the mammalian target of rapamycin complex 1, and deficiency of autophagy impairs ammonia detoxification...
December 26, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29249740/a-case-of-infectious-enterocolitis-with-hyperammonemia
#11
Ken Otsuji, Satoko Simizu, Takeru Endo, Ayako Kanazawa, Hideaki Arai, Keiji Nagata, Nobuya Harayama, Shunichi Nihei, Keiji Aibara, Mitsumasa Saito, Masayuki Kamochi
Case reports of hyperammonemia due to urease-producing bacteria are found occasionally, but most of them are associated with urinary tract infections. We experienced a case of infectious enterocolitis with hyperammonemia in which the causative bacteria was speculated to be urease-producing bacteria. A Japanese woman in her 70s had been diagnosed with microscopic polyangiitis in a nearby hospital and was transferred to our hospital. Although the microscopic polyangiitis was relatively under control after treatment with steroids and rituximab, frequent diarrhea with hyperammonemia (324 µg/dl) appeared and she became comatose...
2017: Journal of UOEH
https://www.readbyqxmd.com/read/29235218/efficacy-and-safety-of-rifaximin-in-japanese-patients-with-hepatic-encephalopathy-a-phase-2-3-multicenter-randomized-evaluator-blinded-active-controlled-trial-and-a-phase-3-multicenter-open-trial
#12
Kazuyuki Suzuki, Ryujin Endo, Yasuhiro Takikawa, Fuminori Moriyasu, Yutaka Aoyagi, Hisataka Moriwaki, Shuji Terai, Isao Sakaida, Yoshiyuki Sakai, Shuhei Nishiguchi, Toru Ishikawa, Hitoshi Takagi, Atsushi Naganuma, Takuya Genda, Takafumi Ichida, Koichi Takaguchi, Katsuhiko Miyazawa, Kiwamu Okita
AIM: The efficacy and safety of rifaximin in the treatment of hepatic encephalopathy (HE) are widely known, but they have not been confirmed in Japanese patients with HE. Thus, two prospective, randomized studies (phase 2/3 study and phase 3 study) were conducted. METHODS: Subjects with grade I or II HE and hyperammonemia were enrolled. The phase 2/3 study, which was a randomized, evaluator-blinded, active-comparator, parallel-group study, was conducted at 37 institutions in Japan...
December 13, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29234200/nutrition-and-muscle-in-cirrhosis
#13
REVIEW
Anil C Anand
As the cirrhosis progresses, development of complication like ascites, hepatic encephalopathy, variceal bleeding, kidney dysfunction, and hepatocellular carcinoma signify increasing risk of short term mortality. Malnutrition and muscle wasting (sarcopenia) is yet other complications that negatively impact survival, quality of life, and response to stressors, such as infection and surgery in patients with cirrhosis. Conventionally, these are not routinely looked for, because nutritional assessment can be a difficult especially if there is associated fluid retention and/or obesity...
December 2017: Journal of Clinical and Experimental Hepatology
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
#14
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/29212999/-a-case-of-hyperammonemia-resulting-from-urinary-tract-infection-caused-by-urease-producing-bacteria-in-a-parkinson-s-disease-patient-with-drug-induced-urinary-retention
#15
Masahiro Yasunishi, Akihiro Koumura, Yuichi Hayashi, Shohei Nishida, Takashi Inuzuka
A 71-year-old woman with a 9-year history of Parkinson's disease was admitted to our hospital emergently because of consciousness disturbance. Her consciousness level was 200 on the Japan coma scale (JCS), and she presented with tenderness and distension of the lower abdomen. Brain computed tomography showed normal findings. Blood tests showed an increased ammonia level (209 μg/dl) with normal AST and ALT levels. We catheterized the bladder for urinary retention. Five hours after admission, the blood ammonia level decreased to 38 μg/dl, and her consciousness level improved dramatically...
2017: Nihon Ronen Igakkai Zasshi. Japanese Journal of Geriatrics
https://www.readbyqxmd.com/read/29203429/oxidative-stress-in-urea-cycle-disorders-findings-from-clinical-and-basic-research
#16
REVIEW
Belisa Parmeggiani, Carmen Regla Vargas
Inborn errors of metabolism (IEM) comprise a group of over 600 disorders, each with a specific metabolic impairment due to a genetic defect. Urea cycle disorders (UCD) are IEM that affect the nitrogen disposal system, leading to hyperammonemia and the accumulation of other toxic metabolites in tissues of affected patients. UCD arise from mutations in the genes coding any of the enzymes participating in the urea cycle, either directly or as regulators of this pathway, causing severe respiratory alkalosis. Considering that the exact mechanisms underlying the damage found in UCD, the purpose of this minireview is to obtain data and search for links between UCD and oxidative stress, a phenomenon common to several IEM...
December 1, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/29200567/the-menacing-side-of-valproate-a-case-series-of-valproate-induced-hyperammonemia
#17
Deeksha Elwadhi, Rashmi Prakash, Manushree Gupta
Valproate (VPA) is a well-tolerated and commonly used drug to treat variety of psychiatric and neurological disorders. VPA-induced hyperammonemic encephalopathy is a rare adverse effect which can commonly occur in the background of normal liver function and therapeutic serum levels. Any delay in treatment of VPA-induced hyperammonemic encephalopathy can lead to life-threatening coma thus a strong clinical suspicion, fair understanding of the pathophysiology, and management of this drug-related complication can prevent fatal outcome...
September 2017: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/29187686/-portal-systemic-encephalopathy-with-bilateral-thalamic-and-internal-capsule-lesions-using-diffusion-weighted-mri-in-a-super-aged-patient
#18
Michiaki Matsuda, Shinpei Takesako, Mitsuhiro Nakazaki, Toru Nandate, Fujio Umehara
We describe the case of a 90-year-old woman who was hospitalized in July 2016 and subsequently experienced a sudden decline in consciousness level resulting in a state of deep coma. Involuntary movements were not observed, and bilateral Babinski signs were inconclusive. Diffusion-weighted MRI (DWI) of the brain showed bilateral hyperintensity in the thalamus and internal capsule, laboratory testing detected high levels of plasma ammonia, and an electroencephalogram showed delta waves and triphasic waves predominantly in the frontal lobe...
November 28, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29171503/-prevention-and-treatment-of-hepatic-encephalopathy
#19
Yu P Sivolap
Hepatic encephalopathy is a common neuropsychiatric complication of serious liver diseases and serves as a marker for worsening of their course and increasing the risk of death. The principal pathogenesis factors of hepatic encephalopathy is the excessive formation of ammonia and its accumulation by astrocytes. A key role in the prevention and treatment of hepatic encephalopathy is played by drugs that prevent hyperammonemia and promote the removal of ammonia from the body, namely lactulose, rifaximin and L-ornithine-L-aspartate...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/29159461/severe-hyperammonemic-encephalopathy-requiring-dialysis-aggravated-by-prolonged-fasting-and-intermittent-high-fat-load-in-a-ramadan-fasting-month-in-a-patient-with-cptii-homozygous-mutation
#20
P Phowthongkum, C Ittiwut, V Shotelersuk
BACKGROUND: Carnitine palmitoyltransferase II (CPTII) deficiency is a mitochondrial fatty acid oxidation disorder that can present antenatally as congenital brain malformations, or postnatally with lethal neonatal, severe infantile, or the most common adult myopathic forms. No case of severe hyperammonemia without liver dysfunction has been reported. CASE PRESENTATION: We described a 23-year-old man who presented to the emergency department with seizures and was found to have markedly elevation of serum ammonia...
November 21, 2017: JIMD Reports
keyword
keyword
2581
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"