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https://www.readbyqxmd.com/read/27933339/efficacy-and-safety-of-osteoporosis-medications-in-a-rat-model-of-late-stage-chronic-kidney-disease-accompanied-by-secondary-hyperparathyroidism-and-hyperphosphatemia
#1
M Ota, M Takahata, T Shimizu, Y Kanehira, H Kimura-Suda, Y Kameda, H Hamano, S Hiratsuka, D Sato, N Iwasaki
: This study showed that bisphosphonate was safe and effective for the treatment of bone disorders in stage 4 chronic kidney disease (CKD) rats. Intermittent teriparatide therapy showed an anabolic action on bone even under secondary hyperparathyroidism conditions without having an adverse effect on mineral metabolism in late-stage CKD. INTRODUCTION: Patients with late-stage CKD are at high risk for fragility fractures. However, there are no consensus on the efficacy and safety of osteoporosis medications for patients with late-stage CKD...
December 8, 2016: Osteoporosis International
https://www.readbyqxmd.com/read/27932386/aberrant-expression-of-fbxo2-disrupts-glucose-homeostasis-through-ubiquitin-mediated-degradation-of-insulin-receptor-in-obese-mice
#2
Bin Liu, Han Lu, Duanzhuo Li, Xuelian Xiong, Lu Gao, Zhixiang Wu, Yan Lu
Insulin resistance is a critical factor in the development of metabolic disorders, including type 2 diabetes (T2DM). However, its molecular mechanisms remain incompletely understood. In the present study, we found that F-box only protein 2 (FBXO2), a substrate recognition component of SKP1-Cullin1-F-box protein (SCF) E3 ubiquitin ligase complex, were up-regulated in livers of obese mice. Furthermore, using a protein purification approach combined with high performance liquid chromatography/tandem mass spectrometry (HPLC/MS/MS), we carried out a system-wide screening of FBXO2 substrates, in which insulin receptor (IR) was identified as a substrate for FBXO2...
December 8, 2016: Diabetes
https://www.readbyqxmd.com/read/27932282/serum-cardiac-troponin-i-in-canine-syncope-and-seizures
#3
E Dutton, J Dukes-McEwan, P J Cripps
OBJECTIVE: To determine if serum cardiac troponin I (cTnI) concentration distinguishes between cardiogenic syncope and collapsing dogs presenting with either generalized epileptic seizures (both with and without cardiac disease) or vasovagal syncope. ANIMALS: Seventy-nine prospectively recruited dogs, grouped according to aetiology of collapse: generalized epileptic seizures (group E), cardiogenic syncope (group C), dogs with both epileptic seizures and cardiac disease (group B), vasovagal syncope (group V) or unclassified (group U)...
December 5, 2016: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
https://www.readbyqxmd.com/read/27932276/the-why-and-how-of-amino-acid-analytics-in-cancer-diagnostics-and-therapy
#4
REVIEW
Friederike Manig, Konstantin Kuhne, Cläre von Neubeck, Uwe Schwarzenbolz, Zhanru Yu, Benedikt M Kessler, Jens Pietzsch, Leoni A Kunz-Schughart
Pathological alterations in cell functions are frequently accompanied by metabolic reprogramming including modifications in amino acid metabolism. Amino acid detection is thus integral to the diagnosis of many hereditary metabolic diseases. The development of malignant diseases as metabolic disorders comes along with a complex dysregulation of genetic and epigenetic factors affecting metabolic enzymes. Cancer cells might transiently or permanently become auxotrophic for non-essential or semi-essential amino acids such as asparagine or arginine...
December 5, 2016: Journal of Biotechnology
https://www.readbyqxmd.com/read/27931182/exposure-of-bovine-oocytes-and-embryos-to-elevated-non-esterified-fatty-acid-concentrations-integration-of-epigenetic-and-transcriptomic-signatures-in-resultant-blastocysts
#5
K L J Desmet, V Van Hoeck, D Gagné, E Fournier, A Thakur, A M O'Doherty, C P Walsh, M A Sirard, P E J Bols, J L M R Leroy
BACKGROUND: Metabolic stress associated with negative energy balance in high producing dairy cattle and obesity in women is a risk factor for decreased fertility. Non-esterified fatty acids (NEFA) are involved in this pathogenesis as they jeopardize oocyte and embryo development. Growing evidence indicates that maternal metabolic disorders can disturb epigenetic programming, such as DNA methylation, in the offspring. Oocyte maturation and early embryo development coincide with methylation changes and both are sensitive to adverse environments...
December 8, 2016: BMC Genomics
https://www.readbyqxmd.com/read/27931034/historical-perspective-on-clinical-trials-of-carnitine-in-children-and-adults
#6
Neil R M Buist
The metabolic roles of carnitine have been greatly clarified over the past 50 years, and it is now well established that carnitine is a key player in mitochondrial generation of energy and metabolism of acetyl coenzyme A. A therapeutic role for carnitine in treatment of nutritional deficiencies in infants and children was first demonstrated in 1958, and since that time it has been used to treat a number of inborn errors of metabolism. Carnitine was approved by the US Food and Drug Administration in 1985 for treatment of 'primary carnitine deficiency', and later in 1992 for treatment of 'secondary carnitine deficiency', a definition that included the majority of relevant metabolic disorders associated with low or abnormal plasma carnitine levels...
December 9, 2016: Annals of Nutrition & Metabolism
https://www.readbyqxmd.com/read/27931032/fatty-acid-oxidation-and-its-relation-with-insulin-resistance-and-associated-disorders
#7
Gary D Lopaschuk
Alterations in muscle fatty acid metabolism have been implicated in mediating the severity of insulin resistance. In the insulin resistant heart fatty acids are favored as an energy source over glucose, which is thus associated with increased fatty acid oxidation, and an overall decrease in glycolysis and glucose oxidation. In addition, excessive uptake and beta-oxidation of fatty acids in obesity and diabetes can compromise cardiac function. In animal studies, mice fed a high fat diet (HFD) show cardiac insulin resistance in which the accumulation of intra-myocardial diacylglycerol has been implicated, likely involving parallel signaling pathways...
December 9, 2016: Annals of Nutrition & Metabolism
https://www.readbyqxmd.com/read/27931018/primary-carnitine-deficiency-and-newborn-screening-for-disorders-of-the-carnitine-cycle
#8
Nicola Longo
Carnitine is needed for transfer of long-chain fatty acids across the inner mitochondrial membrane for subsequent β-oxidation. Carnitine can be synthesized by the body and is also obtained in the diet through consumption of meat and dairy products. Defects in carnitine transport such as those caused by defective activity of the OCTN2 transporter encoded by the SLC22A5 gene result in primary carnitine deficiency, and newborn screening programmes can identify patients at risk for this condition before irreversible damage...
December 9, 2016: Annals of Nutrition & Metabolism
https://www.readbyqxmd.com/read/27930821/assessment-of-asymmetrical-dimethylarginine-metabolism-in-patients-with-critical-illness
#9
Rawia A Ghashut, Scott Blackwell, Sylvia Ryan, Laura Willox, Donald C McMillan, John Kinsella, Dinesh Talwar
BACKGROUND: Critically ill patients experience metabolic disorders including hypercatabolic state and hyperglycaemia and these are associated with poor outcome. Hyperglycaemia and asymmetric dimethylarginine (ADMA) are reported to have significant influences on endothelial dysfunction. The aim of the present study was to examine the relationship between plasma asymmetric dimethylarginine (ADMA) and related arginine metabolism in patients with critical illness. MATERIALS AND METHODS: Two venous blood samples (EDTA) (104 patients), on admission and follow up sample in the last day in ICU (died or discharge sample median 7, IQR 6-8, range 5-15)...
December 8, 2016: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27930511/four-year-follow-up-of-a-wilson-disease-pedigree-complicated-with-epilepsy-and-hypopituitarism-case-report-with-a-literature-review
#10
Qi-Jie Zhang, Liu-Qing Xu, Chong Wang, Wei Hu, Ning Wang, Wan-Jin Chen
RATIONALE: Wilson's disease (WD) is an autosomal recessive inherited disorder of copper metabolism with excellent prognosis if treated timely. However, WD is usually prone to neglect and misdiagnosis at an early stage. We reported a rare WD pedigree, and the clinical features, laboratory tests, and gene mutations were analyzed in detail. PATIENT CONCERNS: The patient was a 17-year-old and 136-cm-tall girl who presented with limb weakness, combined with multi-organ disorders including blind eye, epilepsy, and hypopituitarism...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27929277/acute-monoarthritis-diagnosis-in-adults
#11
Jonathan A Becker, Jennifer P Daily, Katherine M Pohlgeers
Acute monoarthritis can be the initial manifestation of many joint disorders. The most common diagnoses in the primary care setting are osteoarthritis, gout, and trauma. It is important to understand the prevalence of specific etiologies and to use the appropriate diagnostic modalities. A delay in diagnosis and treatment, particularly in septic arthritis, can have catastrophic results including sepsis, bacteremia, joint destruction, or death. The history and physical examination can help guide the use of laboratory and imaging studies...
November 15, 2016: American Family Physician
https://www.readbyqxmd.com/read/27929082/a-retrospective-study-of-pyogenic-liver-abscess-focusing-on-klebsiella-pneumoniae-as-a-primary-pathogen-in-china-from-1994-to-2015
#12
Yun Qian, Chi Chun Wong, Sanchuan Lai, Huarong Chen, Xingkang He, Leimin Sun, Jiaguo Wu, Jiancang Zhou, Jun Yu, Weili Liu, Daoyang Zhou, Jianmin Si
Pyogenic liver abscess (PLA) is a common intra-abdominal infection in adults. In this study, we aim to explore demographic and clinical characteristics of PLA focusing on Klebsiella pneumoniae (K. pneumoniae) induced PLA (KP-PLA) in mainland China. A retrospective review of medical records from all patients with KP-PLA admitted to a tertiary teaching hospital over a 21-year period (1994-2015) was performed. Among 296 PLA cases with confirmed culture-positive data, K. pneumoniae was revealed as the predominant pathogen (n = 189, 63...
December 8, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27929056/gsk-3%C3%AE-controls-nf-kappab-activity-via-ikk%C3%AE-nemo
#13
Senad Medunjanin, Lisa Schleithoff, Christian Fiegehenn, Soenke Weinert, Werner Zuschratter, Ruediger C Braun-Dullaeus
The NF-κB signaling pathway is central for the innate immune response and its deregulation is found in multiple disorders such as autoimmune, chronic inflammatory and metabolic diseases. IKKγ/NEMO is essential for NF-κB activation and NEMO dysfunction in humans has been linked to so-called progeria syndromes, which are characterized by advanced ageing due to age-dependent inflammatory diseases. It has been suggested that glycogen synthase kinase-3β (GSK-3β) participates in NF-κB regulation but the exact mechanism remained incompletely understood...
December 8, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27928991/mortality-in-patients-with-diabetes-mellitus-and-addison-s-disease-a-nationwide-matched-observational-cohort-study
#14
Dimitrios Chantzichristos, Anders Persson, Björn Eliasson, Mervete Miftaraj, Stefan Franzén, Ragnhildur Bergthorsdottir, Soffia Gudbjörnsdottir, Ann-Marie Svensson, Gudmundur Johannsson
OBJECTIVE: Our hypothesis was that patients with diabetes mellitus obtain an additional risk of death if they develop Addison's disease (AD). DESIGN AND METHODS: Nationwide, matched, observational cohort study cross-referencing the Swedish National Diabetes Register with Inpatient, Cancer and Cause of Death Registers in patients with diabetes (type 1 and 2) and AD and matched controls with diabetes. Clinical characteristics at baseline, overall, and cause-specific mortality were assessed...
January 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27928909/using-proteomics-to-discover-novel-biomarkers-for-fatty-liver-development-and-response-to-cb1r-antagonist-treatment-in-an-obese-mouse-model
#15
Chin-Chang Chen, Tzung-Yan Lee, Ching-Fai Kwok, Yung-Pei Hsu, Kuang-Chung Shih, Yan-Jie Lin, Low-Tone Ho
Over activity of cannabinoid receptor type 1 (CB1R) plays a key role in increasing the incidence of obesity-induced nonalcoholic fatty liver disease. Tissue proteome analysis has been applied to investigate the bioinformatics regarding the mode of action and therapeutic mechanism. The aim of this study was to explore the potential pathways altered with CB1R in obesity-induced fatty liver. Male C57BL/6 mice were fed either a standard chow diet (STD) or a high-fat diet (HFD) with or without 1-week treatment of CB1R inverse agonist AM251 at 5 mg/kg...
December 8, 2016: Proteomics
https://www.readbyqxmd.com/read/27928778/previously-unreported-biallelic-mutation-in-dnajc19-are-sensorineural-hearing-loss-and-basal-ganglia-lesions-additional-features-of-dilated-cardiomyopathy-and-ataxia-dcma-syndrome
#16
Sema Kalkan Ucar, Johannes A Mayr, René G Feichtinger, Ebru Canda, Mahmut Çoker, Saskia B Wortmann
BACKGROUND: Dilated cardiomyopathy (DCM), non-progressive cerebellar ataxia (A), testicular dysgenesis, growth failure, and 3-methylglutaconic aciduria are the hallmarks of DNAJC19 defect (or DCMA syndrome) due to biallelic mutations in DNAJC19. To date DCMA syndrome has been reported in 19 patients from Canada and in two Finnish siblings. The underlying pathomechanism is unknown; however, DNAJC19 is presumed to be involved in mitochondrial membrane related processes (e.g., protein import and cardiolipin remodeling)...
December 8, 2016: JIMD Reports
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/27928775/lysosomal-storage-disorders-in-nonimmune-hydrops-fetalis-nihf-an-indian-experience
#18
Jayesh Sheth, Mehul Mistri, Krati Shah, Mayank Chaudhary, Koumudi Godbole, Frenny Sheth
Lysosomal storage disorders (LSD) are rare inherited neurovisceral inborn errors of metabolism which may present as nonimmune hydrops fetalis (NIHF) during pregnancy. Although causes of NIHF are highly diverse, LSDs are one of the underlying causes of NIHF. The aim of this study was to elucidate most frequent causes of LSDs presenting as NIHF in Indian population. Several fetal tissues were investigated for enzymatic diagnosis of LSDs using modified fluorometric assays in the current prospective study carried out at our national tertiary center from 2006 through 2016...
December 8, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27928515/mitochondrial-dysfunction-in-autism-spectrum-disorders
#19
Maheen F Siddiqui, Clare Elwell, Mark H Johnson
Autism spectrum disorders (ASD) are classified as neurodevelopmental disorders characterised by diminished social communication and interaction. Recently, evidence has accrued that a significant proportion of individuals with autism have concomitant diseases such as mitochondrial disease and abnormalities of energy generation. This has therefore led to the hypothesis that autism may be linked to mitochondrial dysfunction. We review such studies reporting decreased activity of mitochondrial electron transport chain (ETC) complexes and reduced gene expression of mitochondrial genes, in particular genes of respiratory chain complexes, in individuals with autism...
September 27, 2016: Autism-open Access
https://www.readbyqxmd.com/read/27927815/pyroglutamic-acidosis-in-association-with-therapeutic-paracetamol-use
#20
Robert W Hunter, Cate Lawson, Evangelia Galitsiou, Fiona Gifford, John J Neary
Long-term use of paracetamol (at therapeutic doses) can cause the accumulation of endogenous organic pyroglutamate, resulting in metabolic acidosis with an elevated anion gap. This occurs in the presence of malnutrition, infection, antibiotic use, renal failure and pregnancy. Given the prevalence of these risk factors, this condition is thought to be relatively common in a hospitalised population but is probably significantly underdiagnosed. Prompt recognition is essential because the condition is entirely reversible if the causative agents are withdrawn...
December 2016: Clinical Medicine: Journal of the Royal College of Physicians of London
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