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https://www.readbyqxmd.com/read/28229311/prevalence-of-frailty-in-end-stage-renal-disease-a-systematic-review-and-meta-analysis
#1
REVIEW
Gotaro Kojima
PURPOSE: The purpose of this study is to perform a systematic review and meta-analysis on prevalence of frailty among patients with end-stage renal disease (ESRD). ESRD is associated with malnutrition, chronic inflammation, acidemia, impaired hormonal changes, and low physical activity, all of which can directly and indirectly contribute to the development of frailty. Coexistence of ESRD and frailty has been shown to increase risks of adverse health outcomes. Given potential reversibility of frailty, it is important to examine frailty status in this high-risk population...
February 22, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28223158/aminoguanidine-alleviated-mma-induced-impairment-of-cognitive-ability-in-rats-by-downregulating-oxidative-stress-and-inflammatory-reaction
#2
Qiliang Li, Wenqi Song, Ze Tian, Peichang Wang
Methylmalonic acidemia (MMA) is the most common organic acidemia in childhood. Many "treated" patients continued to display various degrees of mental retardation and psychomotor delay, which could be caused by brain damage from elevated oxidative stress. Aminoguanidine (AG), a synthetic antioxidant, was tested in a MMA rat model for its potential therapeutic effects on memory impairment. The effects of AG on MMA-induced cognitive impairment in Wistar rats were evaluated with Morris Water Maze. The levels of nerve cell apoptosis and microglial activation were investigated to illustrate the mechanisms of the improvement of cognition with AG treatment in MMA rats...
February 13, 2017: Neurotoxicology
https://www.readbyqxmd.com/read/28206825/letter-in-response-to-efficiency-of-acidemia-correction-on-intermittent-versus-continuous-hemodialysis-in-acute-methanol-poisoning
#3
Lindsay M Fox, Mark K Su, Lewis R Goldfrank
No abstract text is available yet for this article.
April 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28206824/reply-to-letter-in-response-to-efficiency-of-acidemia-correction-on-intermittent-versus-continuous-hemodialysis-in-acute-methanol-poisoning
#4
Sergey Zakharov, Darren M Roberts, Knut Erik Hovda
No abstract text is available yet for this article.
April 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28194286/central-venous-catheter-related-bloodstream-infection-with-kocuria-kristinae-in-a-patient-with-propionic-acidemia
#5
Masato Kimura, Eichiro Kawai, Hisao Yaoita, Natsuko Ichinoi, Osamu Sakamoto, Shigeo Kure
Kocuria kristinae is a catalase-positive, coagulase-negative, Gram-positive coccus found in the environment and in normal skin and mucosa in humans; however, it is rarely isolated from clinical specimens and is considered a nonpathogenic bacterium. We describe a case of catheter-related bacteremia due to K. kristinae in a young adult with propionic acidemia undergoing periodic hemodialysis. The patient had a central venous catheter implanted for total parenteral nutrition approximately 6 months prior to the onset of symptoms because of repeated acute pancreatitis...
2017: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/28189105/determination-of-methylmalonyl-coenzyme-a-by-ultra-high-performance-liquid-chromatography-tandem-mass-spectrometry-for-measuring-propionyl-coenzyme-a-carboxylase-activity-in-patients-with-propionic-acidemia
#6
Kana Gotoh, Yoko Nakajima, Go Tajima, Yoriko Watanabe, Yuji Hotta, Tomoya Kataoka, Yoshihiro Kawade, Naruji Sugiyama, Tetsuya Ito, Kazunori Kimura, Yasuhiro Maeda
Propionic acidemia (PA) is an inherited metabolic disease caused by low activity of propionyl coenzyme A (CoA) carboxylase (PCC), which metabolizes propionyl-CoA into methylmalonyl-CoA. Although many patients with PA have been identified by tandem mass spectrometry since the test was first included in neonatal mass screening in the 1990s, the disease severity varies. Thus, determining the specific level of PCC activity is considered to be helpful to grasp the severity of PA. We developed a new PCC assay method by the determination of methylmalonyl-CoA, which is formed by an enzyme reaction using peripheral lymphocytes, based on ultra high-performance liquid chromatography tandem mass spectrometry (UPLC-MS/MS)...
February 6, 2017: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/28165820/leukotriene-mediated-neuroinflammation-toxic-brain-damage-and-neurodegeneration-in-acute-methanol-poisoning
#7
Sergey Zakharov, Katerina Kotikova, Olga Nurieva, Jiri Hlusicka, Petr Kacer, Pavel Urban, Manuela Vaneckova, Zdenek Seidl, Pavel Diblik, Pavel Kuthan, Tomas Navratil, Daniela Pelclova
CONTEXT: The role of neuroinflammation in methanol-induced toxic brain damage has not been studied. OBJECTIVE: We studied acute concentrations and the dynamics of leukotrienes (LT) in serum in hospitalized patients with acute methanol poisoning and in survivors. METHODS: Series of acute cysteinyl-LT and LTB4 concentration measurements were performed in 28/101 hospitalized patients (mean observation time: 88 ± 20 h). In 36 survivors, control LT measurements were performed 2 years after discharge...
February 6, 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28153297/detection-of-inborn-errors-of-metabolism-utilizing-gc-ms-urinary-metabolomics-coupled-with-a-modified-orthogonal-partial-least-squares-discriminant-analysis
#8
Qin Yang, Shan-Shan Lin, Jiang-Tao Yang, Li-Juan Tang, Ru-Qin Yu
GC-MS urinary metabolomic analysis coupled with chemometrics is used to detect inborn errors of metabolism (IEMs), which are genetic disorders causing severe mental and physical debility and even sudden infant death. Orthogonal partial least squares discriminant analysis (OPLS-DA) is an efficient multivariate statistical method that conducts data analysis of metabolite profiling. However, performance degradation is often observed for OPLS-DA due to increasing size and complexity of metabolomic datasets. In this study, hybrid particle swarm optimization (HPSO) is employed to modify OPLS-DA by simultaneously selecting the optimal variable subset, associated weights and the appropriate number of orthogonal components, constructing a new algorithm called HPSO-OPLSDA...
April 1, 2017: Talanta
https://www.readbyqxmd.com/read/28141776/acute-illness-protocol-for-organic-acidemias-methylmalonic-acidemia-and-propionic-acidemia
#9
Saud H Aldubayan, Lance H Rodan, Gerard T Berry, Harvey L Levy
Inborn errors of metabolism (IEM) are genetic disorders that disrupt enzyme activity, cellular transport, or energy production. They are individually rare, but collectively have an incidence of 1:1000. Most patients with IEMs are followed by a physician with expertise in Biochemical Genetics (Metabolism), but may present outside of this setting. Because IEMs can present acutely with life-threatening crises that require specific interventions, it is critical for the emergency medicine physicians, pediatricians, internists, and critical care physicians as well as biochemical geneticists to be familiar with the initial assessment and management of patients with these disorders...
February 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28118413/a-double-blinded-randomized-comparison-of-medetomidine-tiletamine-zolazepam-and-dexmedetomidine-tiletamine-zolazepam-anesthesia-in-free-ranging-brown-bears-ursus-arctos
#10
Núria Fandos Esteruelas, Marc Cattet, Andreas Zedrosser, Gordon B Stenhouse, Susanne Küker, Alina L Evans, Jon M Arnemo
We compared anesthetic features, blood parameters, and physiological responses to either medetomidine-tiletamine-zolazepam or dexmedetomidine-tiletamine-zolazepam using a double-blinded, randomized experimental design during 40 anesthetic events of free-ranging brown bears (Ursus arctos) either captured by helicopter in Sweden or by culvert trap in Canada. Induction was smooth and predictable with both anesthetic protocols. Induction time, the need for supplemental drugs to sustain anesthesia, and capture-related stress were analyzed using generalized linear models, but anesthetic protocol did not differentially affect these variables...
2017: PloS One
https://www.readbyqxmd.com/read/28101778/eight-novel-mut-loss-of-function-missense-mutations-in-chinese-patients-with-isolated-methylmalonic-academia
#11
Lian-Shu Han, Zhuo Huang, Feng Han, Yu Wang, Zhu-Wen Gong, Xue-Fan Gu
BACKGROUND: Isolated methylmalonic acidemia is a rare autosomal recessive metabolic disorder mostly caused by mutations in the methylmalonyl coenzyme A mutase (MCM) gene (MUT). This study aimed to verify whether missense mutations in MUT in Chinese patients affect the stability and enzymatic activity of MCM. METHODS: Eight Chinese patients were identified with novel mutations. Plasmids carrying the wild-type and mutated MUT cDNA were constructed and transfected into HEK293T cells for functional analyses...
January 19, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28093112/clinical-indicators-of-blood-gas-disturbances-elevated-l-lactate-concentration-and-other-abnormal-blood-parameters-in-newborn-beef-calves
#12
E R Homerosky, N A Caulkett, E Timsit, E A Pajor, J P Kastelic, M C Windeyer
Blood gas disturbances, commonly resulting from dystocia, are associated with failed transfer of passive immunity, morbidity and mortality in newborn calves. Modified APGAR scores intended to identify compromised calves are not widely adopted due to lack of practicality and inconsistent associations with blood parameters. The objective of this study was to determine clinical indicators of acidemia in newborn beef calves. Blood parameters at 10 min and 24 h after birth were compared to at-birth clinical examination parameters and calving characteristics in 77 commercial beef calves...
January 2017: Veterinary Journal
https://www.readbyqxmd.com/read/28070528/methylmalonic-acidemia-diagnosis-by-laboratory-methods
#13
Keyfi Fatemeh, Talebi Saeed, Varasteh Abdol-Reza
Methylmalonic acidemia (MMA) is usually caused by a deficiency of the enzyme methylmalonyl-CoA mutase (MCM), a defect in the transport or synthesis of its cofactor, adenosyl-cobalamin (cblA, cblB, cblC, cblF, cblD, and cblX), or deficiency of the enzyme methylmalonyl-CoA epimerase. A comprehensive diagnostic approach involves investigations of metabolites with tandem mass spectrometry, organic acid analysis with gas chromatography, enzymatic studies with fibroblast cell culture, and finally, mutation analysis...
October 2016: Reports of Biochemistry & Molecular Biology
https://www.readbyqxmd.com/read/28064509/acute-salicylate-poisoning-risk-factors-for-severe-outcome
#14
Rachel M Shively, Robert S Hoffman, Alex F Manini
CONTEXT: Salicylate poisoning remains a significant public health threat with more than 20,000 exposures reported annually in the United States. OBJECTIVE: We aimed to establish early predictors of severe in-hospital outcomes in Emergency Department patients presenting with acute salicylate poisoning. METHODS: This was a secondary data analysis of adult salicylate overdoses from a prospective cohort study of acute drug overdoses at two urban university teaching hospitals from 2009 to 2013...
March 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28027700/management-of-persistent-occiput-posterior-position-a-substantial-role-of-instrumental-rotation-in-the-setting-of-failed-manual-rotation
#15
Paul Guerby, Mickael Allouche, Caroline Simon-Toulza, Christophe Vayssiere, Olivier Parant, Fabien Vidal
INTRODUCTION: To compare the maternal and neonatal outcomes associated with Instrumental Rotation (IR) to operative vaginal delivery in occiput posterior (OP) position with Thierry's spatulas (TS), in the setting of failed manual rotation (MR). STUDY DESIGN: We led a prospective observational cohort study in a tertiary referral hospital in Toulouse, France. All women presenting in labour with persistent OP position at full cervical dilatation and who delivered vaginally after failed MR and with IR or OP assisted delivery were included from January 2014 to December 2015...
December 27, 2016: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/27974893/recoverable-record-high-lactic-acidosis-in-a-patient-with-glycogen-storage-disease-type-1-a-mixed-type-a-and-type-b-lactate-disorder
#16
Yonatan Oster, Isaiah D Wexler, Samuel N Heyman, Elchanan Fried
A 17-year-old patient with GSD type 1a (von Gierke disease) was hospitalized with an extremely elevated serum lactate following an intercurrent infection and interruption of his frequent intake of carbohydrates. The patient developed shock, oliguric renal failure, and cardiorespiratory failure requiring mechanical ventilation and inotropes. At the peak of metabolic decompensation and clinical instability, serum lactate reached a level of 47.6 mmol/L which was accompanied by a severe anion gap metabolic acidosis with a pH of 6...
2016: Case Reports in Medicine
https://www.readbyqxmd.com/read/27928776/the-risk-of-fatty-acid-oxidation-disorders-and-organic-acidemias-in-children-with-normal-newborn-screening
#17
Callum Wilson, Detlef Knoll, Mark de Hora, Campbell Kyle, Emma Glamuzina, Dianne Webster
New Zealand has undertaken expanded newborn screening since 2006. During that period there have been no reported cases of fatty acid oxidation disorders or organic acidemias that have been diagnosed clinically that the screening programme missed. However there may have been patients that presented clinically that were not diagnosed correctly or notified.In order to investigate the false-negative screening rate a case-control study was undertaken whereby the clinical coding data and relevant medical records were reviewed for 150 controls and 525 cases...
December 8, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27904153/genotype-and-phenotype-characterization-in-a-spanish-cohort-with-isovaleric-acidemia
#18
María L Couce, Luís Aldamiz-Echevarría, María A Bueno, Patricia Barros, Amaya Belanger-Quintana, Javier Blasco, María-Teresa García-Silva, Ana M Márquez-Armenteros, Isidro Vitoria, Inmaculada Vives, Rosa Navarrete, Ana Fernández-Marmiesse, Belén Pérez, Celia Pérez-Cerdá
Isovaleric acidemia (IVA) is a rare disorder of leucine metabolism. We carried out a multicenter study of IVA patients diagnosed by newborn screening (NBS) or symptoms clinics over a period of 28 years in Spain. Evaluated at diagnosis, data included age, detection method, levels of C5 and IVG, enzymatic studies, clinical presentation parameters and genotype in 16 patients. Follow-up data included C5 levels, intellectual quotient and correlation genotype-phenotype. IVA was detected by NBS in 8 patients (prevalence of 1/326 629)...
March 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/27900673/expansion-of-the-phenotypic-spectrum-of-propionic-acidemia-with-isolated-elevated-propionylcarnitine
#19
Gerarda Cappuccio, Paldeep S Atwal, Taraka R Donti, Kiki Ugarte, Nadia Merchant, William J Craigen, V Reid Sutton, Sarah H Elsea
We report three patients with elevations of propionylcarnitine (C3), one without elevations of 2-methylcitrate and 3-hydroxypropionate in urine organic acid analysis, and the other two showing only mild elevations, all of whom were subsequently confirmed to have propionic acidemia by molecular analysis of PCCA and PCCB genes. To date, they have had a mild clinical course. These cases illustrate the importance of considering high C3 as the only biochemical abnormality in a diagnosis of propionic acidemia. Since mild C3 elevations may be overlooked and considered non-diagnostic in isolation, we advise considering a diagnosis of propionic acidemia even in the absence of significant elevations 2-methylcitrate or 3-hydroxypropionate in urine organic acid analysis...
November 30, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27899470/i-trach-validating-a-tool-for-predicting-prolonged-mechanical-ventilation
#20
Paul A Clark, Ryan C Inocencio, Christopher J Lettieri
PURPOSE: We previously developed a bedside model (I-TRACH), which used commonly obtained data at the time of intubation to predict the duration of mechanical ventilation (MV). We now sought to validate this in a prospective trial. METHODS: A prospective, observational study of 225 consecutive adult medical intensive care unit patients requiring MV. Utilizing the original 6 variables used in the I-TRACH model (Intubation in the ICU, Tachycardia [heart rate > 110], Renal dysfunction [blood urea nitrogen > 25], Acidemia [pH < 7...
November 28, 2016: Journal of Intensive Care Medicine
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