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https://www.readbyqxmd.com/read/27922496/metabolic-myopathies
#1
Mark A Tarnopolsky
PURPOSE OF REVIEW: Metabolic myopathies are genetic disorders that impair intermediary metabolism in skeletal muscle. Impairments in glycolysis/glycogenolysis (glycogen-storage disease), fatty acid transport and oxidation (fatty acid oxidation defects), and the mitochondrial respiratory chain (mitochondrial myopathies) represent the majority of known defects. The purpose of this review is to develop a diagnostic and treatment algorithm for the metabolic myopathies. RECENT FINDINGS: The metabolic myopathies can present in the neonatal and infant period as part of more systemic involvement with hypotonia, hypoglycemia, and encephalopathy; however, most cases present in childhood or in adulthood with exercise intolerance (often with rhabdomyolysis) and weakness...
December 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27922494/approach-to-the-patient-with-hyperckemia
#2
Shannon L Venance
PURPOSE OF REVIEW: Neurologists commonly receive consultation requests regarding the evaluation of patients with an elevated serum creatine kinase (CK), a condition known as hyperCKemia. This article outlines an approach to the history and examination of patients with hyperCKemia in order to narrow the localization and differential of an elevated CK and guide possible next steps. This article aims to help clinicians identify treatable or reversible etiologies as well as those that will change management...
December 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27920825/evaluation-of-cardioprotective-effect-of-aqueous-extract-of-allium-cepa-linn-bulb-on-isoprenaline-induced-myocardial-injury-in-wistar-albino-rats
#3
Geeta B Kharadi, Kaksha J Patel, Bhargav M Purohit, Seema N Baxi, C B Tripathi
To investigate the cardioprotective potential of the aqueous extract of Allium cepa Linn. bulb in isoprenaline-induced myocardial injury in Wistar albino rats. In vitro total phenolic, total flavonoid content and 2, 2'-diphenyl-1-picrylhydrazyl hydrate radical scavenging activity was measured. Isoprenaline-induced myocardial injury model was used to evaluate in vivo effect of aqueous extract of A. cepa in Wistar albino rats. Seventy two rats were randomly divided in 6 groups. Rats were treated with A. cepa 400 mg/kg and 800 mg/kg doses for 30 days and myocardial injury was produced by subcutaneous injection of isoprenaline (ISO) 85 mg/kg on day 28 and 29...
October 2016: Research in Pharmaceutical Sciences
https://www.readbyqxmd.com/read/27920658/isolated-bilateral-gastrocnemius-myositis-in-crohn-disease-successfully-treated-with-adalimumab
#4
Salvatore Vadala di Prampero, Marco Marino, Francesco Toso, Claudio Avellini, Vu Nguyen, Dario Sorrentino
Extraintestinal manifestations are common in inflammatory bowel disease; however, muscular involvement in Crohn disease is rarely reported. We present a case of a 26-year-old male with ileocolonic Crohn disease who developed sudden tenderness in both calves. Doppler ultrasound was negative for deep vein thrombosis. Magnetic resonance imaging of the gastrocnemius muscle showed high intensity signal in the muscle fibers, and muscle biopsy demonstrated nonspecific lymphocytic myositis. Other relevant laboratory results included normal antineutrophil cytoplasmic antibodies and creatine kinase as well as elevated C-reactive protein, erythrocyte sedimentation rate, and anti-Saccharomyces cerevisiae IgG titer...
September 2016: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/27918857/new-approach-to-molsidomine-active-metabolites-coming-from-the-results-of-2-models-of-experimental-cardiology
#5
Michał Żorniak, Katarzyna A Mitręga, Maurycy Porc, Tadeusz F Krzemiński
Molsidomine is a well-known vasodilatating, antianginal drug. Despite earlier studies with its metabolites (3-morpholino-syndnonimine (SIN-1) and N-nitroso-N-morpholino-amino-acetonitrile (SIN-1A)), which indicated a potential favorable cardioprotective activity, a lot of controversy remains. The aim of our research was to compare molsidomine, SIN-1, SIN-1A, and lidocaine influence on arrhythmias and hemodynamic parameters in 2 experimental models in rats. In the Langendorff heart study, SIN-1A markedly elevated left ventricular systolic pressure, maximum rise and fall of the first pressure derivative, coronary flow, and myocardial oxygen consumption...
October 4, 2016: Canadian Journal of Physiology and Pharmacology
https://www.readbyqxmd.com/read/27917636/-immune-mediated-necrotizing-myopathy-associated-with-statin-treatment
#6
Jiří Vencovský
Immune mediated necrotizing myopathy (IMNM) is an acquired inflammatory myopathy. One form of the disease is associated with serum anti-HMGCR autoantibodies and significant proportion of patients has a history of statin treatment. The disease is rare with the incidence estimated around 23 cases/100 000 statin treated individuals. In contrast to direct statin toxicity, anti-HMGCR associated IMNM is an induced autoimmune disease.Patients suffer particularly from severe muscle weakness, which may be occasionally totally immobilizing...
2016: Casopís Lékar̆ů C̆eských
https://www.readbyqxmd.com/read/27917341/the-effect-of-n-acetyl-cysteine-nac-on-aluminum-phosphide-poisoning-inducing-cardiovascular-toxicity-a-case-control-study
#7
Fakhreddin Taghaddosinejad, Esmaeil Farzaneh, Mahdi Ghazanfari-Nasrabad, Nastaran Eizadi-Mood, Morteza Hajihosseini, Omid Mehrpour
BACKGROUND: Aluminum phosphide (AlP) is a very effective indoor and outdoor pesticide. We investigated the effects of N-acetyl cysteine (NAC) on the survival time, hemodynamics, and cardiac biochemical parameters at various time intervals in some cases of AlP poisoning. METHODS: This research was a case-control study to evaluate 63 AlP poisoned patients during 2010-2012. Patients with cardiovascular complications of AlP to be treated with intravenous NAC plus conventional treatment were considered as the case group and compared with patients who did not receive NAC...
2016: SpringerPlus
https://www.readbyqxmd.com/read/27917270/effect-of-creatine-supplementation-on-cognitive-performance-and-apoptosis-in-a-rat-model-of-amyloid-beta-induced-alzheimer-s-disease
#8
Malek Alimohammadi-Kamalabadi, Mohammadreza Eshraghian, Mohammad-Reza Zarindast, Abbas Aliaghaei, Hamideh Pishva
OBJECTIVES: Neuroprotective effect of creatine (Cr) against β-amyloid (Aβ) is reported in an in vitro study. This study investigated the effect of Cr supplementation on β-amyloid toxicity in vivo. MATERIALS AND METHODS: Thirty two, male Wistar rats were divided into 4 groups. During ten weeks of study, control group went through no surgical or dietary intervention. At the 4th week of study Sham group had a hippocampal normal saline injection, while Aβ and AβCr groups had an β-amyloid injection in the hippocampus...
November 2016: Iranian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/27916937/the-emergence-and-epidemiology-of-haff-disease-in-china
#9
REVIEW
Thomas Y K Chan
Haff disease is a rare syndrome of unexplained myalgia and rhabdomyolysis occurring within 24 h of consumption of certain types of cooked freshwater fish or crustacean. It is caused by a yet unidentified heat-stable toxin. In the present review of published case studies and official press releases, the main objective is to report the emergence and epidemiology of Haff disease in China. Haff disease first occurred in Beijing in 2000 and in Lianzhou and Liannan, Guangdong Province in 2009. Subsequent outbreaks mostly occurred in the Jiangsu Province-Nanjing, Yangzhou, Huai'an, and Yancheng...
December 1, 2016: Toxins
https://www.readbyqxmd.com/read/27916788/effects-of-dietary-oregano-essential-oil-and-vitamin-e-supplementation-on-meat-quality-stress-response-and-intestinal-morphology-in-pigs-following-transport-stress
#10
Yi Zou, Xiao Ming Hu, Ting Zhang, Hong Kui Wei, Yuan Fei Zhou, Zhong Xin Zhou, Jian Peng
This study investigates the effects of dietary oregano essential oil (OEO) and vitamin E (Vit E) supplementation on meat quality, stress response and intestinal morphology in pigs following transport stress. A total of 288 finishing pigs were randomly assigned to three groups: a basal diet or a basal diet supplemented either with 200 mg/kg Vit E or 25 mg/kg OEO. After a 28-day feeding trial, total of 132 finishing pigs according diet and transport stress were assigned to one of four treatment groups: 1) control treatment without transport stress (Control group), 2) control treatment with 5-hr transport stress (Negative group), 3) Vit E treatment with 5-hr transport stress and 4) OEO treatment with 5-hr transport stress...
December 2, 2016: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/27916082/-effects-of-ammonium-pyrrolidine-dithiocarbamate-pdtc-on-osteopontin-expression-and-autophagy-in-tubular-cells-in-streptozotocin-induced-diabetic-nephropathy-rat
#11
S Gao, J Y Jia, T K Yan, Y M Yu, W Y Shang, L Wei, Z F Zheng, P Fang, B C Chang, S Lin
Objective: To investigate the effects of ammonium pyrrolidine dithiocarbamate (PDTC) on tubulointerstitial inflammatory molecules and autophagy in diabetic nephropathy (DN) rats. Methods: Twenty-four male Sprague-Dawley rats were assigned to DN group (n=6) and DN+ PDTC group (n=6, PDTC, ip, 100 mg·kg(-1)·d(-1)), all received streptozotocin (STZ) 60 mg/kg intraperitoneally, and the other 12 rats were randomly divided into control group (n=6) and PDTC group (n=6). At the end of 12 weeks, after serum creatine (Scr) and 24-hour urinary protein were determined, rats were sacrificed to determined the renal pathological damages and the changes of nuclear factor (NF)-κB p65, p62, osteopontin (OPN), microtubule associated protein 1 light chain 3 (LC3)-Ⅱ/LC3-Ⅰ, nuclear p-NF-κB p65 by immunohistological stainning and Western blot, and ultrastructural changes of autophagic process was observed by electron microscopy (EM)...
November 29, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27915162/high-fat-diet-induces-metabolic-changes-and-reduces-oxidative-stress-in-female-mouse-hearts
#12
Ignasi Barba, Elisabet Miró-Casas, José L Torrecilla, Eulàlia Pladevall, Sergi Tejedor, Rubén Sebastián-Pérez, Marisol Ruiz-Meana, José R Berrendero, Antonio Cuevas, David García-Dorado
After an acute myocardial infarction, obese patients generally have a better prognosis than their leaner counterparts, known as the "obesity paradox". In addition, female sex is associated with a lower risk of cardiac ischemic events and smaller infarct size compared to males. The objective of the present work was to study the metabolic phenotype and mitochondrial function associated to female sex and short-term high-fat diet. (1)H NMR spectra of mice heart extracts were analysed by mRMR variable selection and linear discriminant analysis was used to evaluate metabolic changes...
November 15, 2016: Journal of Nutritional Biochemistry
https://www.readbyqxmd.com/read/27913923/actn3-x-allele-carriers-had-greater-levels-of-muscle-damage-during-a-half-ironman
#13
Juan Del Coso, Juan José Salinero, Beatriz Lara, César Gallo-Salazar, Francisco Areces, Carlos Puente, David Herrero
PURPOSE: Alpha-actinin-3, encoded by the ACTN3 gene, is an actin-binding protein with an important role in myofibril contraction and muscle force output. In humans, there is a relatively common deficiency of the α-actinin-3 due to homozygosity in a polymorphism of the ACTN3 gene (R577X, rs1815739), that has been related to decreased resistance to strain during voluntary muscle contractions. The purpose of this study was to investigate the influence of the ACTN3 genotype on the level of exercise-induced muscle damage attained by 23 experienced triathletes during an official half-ironman competition...
December 2, 2016: European Journal of Applied Physiology
https://www.readbyqxmd.com/read/27911915/marathon-race-affects-neutrophil-surface-molecules-role-of-inflammatory-mediators
#14
Vinicius Coneglian Santos, Ana Paula Renno Sierra, Rodrigo Oliveira, Kim Guimarães Caçula, César Miguel Momesso, Fabio Takeo Sato, Maysa Braga Barros Silva, Heloisa Helena Oliveira, Maria Elizabeth Pereira Passos, Diego Ribeiro de Souza, Olivia Santos Gondim, Marino Benetti, Adriana Cristina Levada-Pires, Nabil Ghorayeb, Maria Augusta Peduti Dal Molin Kiss, Renata Gorjão, Tânia Cristina Pithon-Curi, Maria Fernanda Cury-Boaventura
The fatigue induced by marathon races was observed in terms of inflammatory and immunological outcomes. Neutrophil survival and activation are essential for inflammation resolution and contributes directly to the pathogenesis of many infectious and inflammatory conditions. The aim of this study was to investigate the effect of marathon races on surface molecules related to neutrophil adhesion and extrinsic apoptosis pathway and its association with inflammatory markers. We evaluated 23 trained male runners at the São Paulo International Marathon 2013...
2016: PloS One
https://www.readbyqxmd.com/read/27911900/clinical-and-pharmacological-investigation-of-myotoxicity-in-sri-lankan-russell-s-viper-daboia-russelii-envenoming
#15
Anjana Silva, Christopher Johnston, Sanjaya Kuruppu, Daniela Kneisz, Kalana Maduwage, Oded Kleifeld, A Ian Smith, Sisira Siribaddana, Nicholas A Buckley, Wayne C Hodgson, Geoffrey K Isbister
BACKGROUND: Sri Lankan Russell's viper (Daboia russelii) envenoming is reported to cause myotoxicity and neurotoxicity, which are different to the effects of envenoming by most other populations of Russell's vipers. This study aimed to investigate evidence of myotoxicity in Russell's viper envenoming, response to antivenom and the toxins responsible for myotoxicity. METHODOLOGY AND FINDINGS: Clinical features of myotoxicity were assessed in authenticated Russell's viper bite patients admitted to a Sri Lankan teaching hospital...
December 2016: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/27910779/amelioration-of-doxorubicin-induced-cardiac-and-renal-toxicity-by-oxycarotenoid-lutein-and-its-mechanism-of-action
#16
Edakkadath Raghavan Sindhu, Thattaruparambil Raveendran Nithya, Ponnamparambil Purushothaman Binitha, Ramadasan Kuttan
We set out to determine the effect of oxycarotenoid lutein on reducing cardiac and renal toxicity induced by doxorubicin (DXR). We started with oral administration in rats of lutein for 15 d before administering DXR (30 mg/kg body weight, intraperitoneally, in a single dose). Animals in all groups were sacrificed 24 h after DXR administration. Serum markers of cardiac injury lactate dehydrogenase, creatine phosphokinase, serum glutamate oxaloacetate transaminase, and serum glutamate pyruvate transaminase increased drastically after DXR but decreased after lutein treatment (p < 0...
2016: Journal of Environmental Pathology, Toxicology and Oncology
https://www.readbyqxmd.com/read/27909856/metabolic-changes-in-the-bilateral-visual-cortex-of-the-monocular-blind-macaque-a-multi-voxel-proton-magnetic-resonance-spectroscopy-study
#17
Lingjie Wu, Zuohua Tang, Xiaoyuan Feng, Xinghuai Sun, Wen Qian, Jie Wang, Lixin Jin, Jingxuan Jiang, Yufeng Zhong
The metabolic changes accompanied with adaptive plasticity in the visual cortex after early monocular visual loss were unclear. In this study, we detected the metabolic changes in bilateral visual cortex of normal (group A) and monocular blind macaque (group B) for studying the adaptive plasticity using multi-voxel proton magnetic resonance spectroscopy ((1)H-MRS) at 32 months after right optic nerve transection. Then, we compared the N-Acetyl aspartate (NAA)/Creatine (Cr), myoinositol (Ins)/Cr, choline (Cho)/Cr and Glx (Glutamate + glutamine)/Cr ratios in the visual cortex between two groups, as well as between the left and right visual cortex of group A and B...
December 1, 2016: Neurochemical Research
https://www.readbyqxmd.com/read/27909746/brain-glutamate-in-anorexia-nervosa-a-magnetic-resonance-spectroscopy-case-control-study-at-7-tesla
#18
Beata R Godlewska, Alexandra Pike, Ann L Sharpley, Agnes Ayton, Rebecca J Park, Philip J Cowen, Uzay E Emir
RATIONALE: Anorexia nervosa (AN) is a serious psychiatric disorder with high morbidity and mortality. There are no established pharmacological treatments and the neurobiology of the condition is poorly understood. Previous studies using magnetic resonance spectroscopy (MRS) have shown that AN may be associated with reductions in indices of brain glutamate; however, at conventional field strengths (≤3 T), it is difficult to separate glutamate from its precursor and metabolite, glutamine...
December 1, 2016: Psychopharmacology
https://www.readbyqxmd.com/read/27909311/insights-into-the-phosphoryl-transfer-mechanism-of-human-ubiquitous-mitochondrial-creatine-kinase
#19
Quanjie Li, Shuai Fan, Xiaoyu Li, Yuanyuan Jin, Weiqing He, Jinming Zhou, Shan Cen, ZhaoYong Yang
Human ubiquitous mitochondrial creatine kinase (uMtCK) is responsible for the regulation of cellular energy metabolism. To investigate the phosphoryl-transfer mechanism catalyzed by human uMtCK, in this work, molecular dynamic simulations of uMtCK∙ATP-Mg(2+)∙creatine complex and quantum mechanism calculations were performed to make clear the puzzle. The theoretical studies hereof revealed that human uMtCK utilizes a two-step dissociative mechanism, in which the E227 residue of uMtCK acts as the catalytic base to accept the creatine guanidinium proton...
December 2, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27907819/giant-primary-malignant-mesothelioma-of-the-liver-a-case-report
#20
Ruba Haji Ali, Mohamad Khalife, Ghina El Nounou, Ruba Zuhri Yafi, Hussein Nassar, Zeinab Aidibe, Randa Raad, Rania Abou Eid, Walid Faraj
INTRODUCTION: Malignant mesothelioma is a rare neoplasm of mesothelial cells arising most frequently in the pleura or peritoneum and less frequently in the liver. CASE PRESENTATION: We present a case of primary hepatic mesothelioma of 41year old woman. She had no history of asbestos exposure or cancer. Abdominal computed tomography (CT) showed 21cm intrahepatic mass in the right lobe with many cystic lesions and few small calcifications. Pathology showed a biphasic cellular pattern...
November 11, 2016: International Journal of Surgery Case Reports
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