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    Non-Mammalian Vertebrates: Distinct Models to Assess the Role of Ion Gradients in Energy Expenditure.
    Front Endocrinol (Lausanne) 2017 1;8:224. Epub 2017 Sep 1.
    School of Animal and Comparative Biomedical Science, University of Arizona, Tucson, AZ, United States.
    Animals store metabolic energy as electrochemical gradients. At least 50% of mammalian energy is expended to maintain electrochemical gradients across the inner mitochondrial membrane (H(+)), the sarcoplasmic reticulum (Ca(++)), and the plasma membrane (Na(+)/K(+)). The potential energy of these gradients can be used to perform work (e. Read More

    Enzymatic testing sensitivity, variability and practical diagnostic algorithm for pyruvate dehydrogenase complex (PDC) deficiency.
    Mol Genet Metab 2017 Sep 8. Epub 2017 Sep 8.
    Center for Inherited Disorders of Energy Metabolism (CIDEM), University Hospitals Cleveland Medical Center (UHCMC), Cleveland, OH, USA; Department of Genetics and Genome Sciences, CWRU, Cleveland, OH, USA; Center for Human Genetics, UHCMC, Cleveland, OH, USA. Electronic address:
    Pyruvate dehydrogenase complex (PDC) deficiency is a major cause of primary lactic acidemia in children. Prompt and correct diagnosis of PDC deficiency and differentiating between specific vs generalized, or secondary deficiencies has important implications for clinical management and therapeutic interventions. Both genetic and enzymatic testing approaches are being used in the diagnosis of PDC deficiency. Read More

    Model Informed Dose Optimization of Dichloroacetate for the Treatment of Congenital Lactic Acidosis in Children.
    J Clin Pharmacol 2017 Sep 15. Epub 2017 Sep 15.
    Center for Pharmacometrics & Systems Pharmacology, College of Pharmacy, University of Florida, Orlando, FL, USA.
    Dichloroacetate (DCA) is an investigational drug used to treat congenital lactic acidosis and other mitochondrial disorders. Response to DCA therapy in young children may be suboptimal following body weight-based dosing. This is because of autoinhibition of its metabolism, age-dependent changes in pharmacokinetics, and polymorphisms in glutathione transferase zeta 1 (GSTZ1), its primary metabolizing enzyme. Read More

    Risk factor analysis for nonocclusive mesenteric ischemia following cardiac surgery: A case-control study.
    Medicine (Baltimore) 2017 Sep;96(37):e8029
    Departments of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
    Although rare, postcardiac surgery nonocclusive mesenteric ischemia (NOMI) is a life-threatening condition. Identifying the risk factors for NOMI during immediate postoperative period may help early detection and intervention, which leads to improved clinical outcomes. The objective of this study was to identify the clinical features and risk factors of NOMI for prognosis identification after cardiac surgery, focusing on immediate postoperative parameters. Read More

    Biallelic variants in WARS2 encoding mitochondrial tryptophanyl-tRNA synthase in six individuals with mitochondrial encephalopathy.
    Hum Mutat 2017 Sep 14. Epub 2017 Sep 14.
    Radboud Center for Mitochondrial Medicine, Department of Pediatrics, Radboud University Medical Center, Nijmegen, The Netherlands.
    Mitochondrial protein synthesis involves an intricate interplay between mitochondrial DNA encoded RNAs and nuclear DNA encoded proteins, such as ribosomal proteins and aminoacyl-tRNA synthases. Eukaryotic cells contain 17 mitochondria-specific aminoacyl-tRNA synthases. WARS2 encodes mitochondrial tryptophanyl-tRNA synthase (mtTrpRS), a homodimeric class Ic enzyme (mitochondrial tryptophan-tRNA ligase; EC 6. Read More

    Hemodialysis for Lactic Acidosis.
    Indian J Crit Care Med 2017 Aug;21(8):537-538
    Department of Critical Care Medicine, Apollo First Med Hospital, Chennai, Tamil Nadu, India.
    Lactic acidosis (Type A) is common in critically ill patients and usually treated by correcting the underlying etiology. We present the case of a young female who presented with life-threatening lactic acidosis secondary to hematological malignancy. Timely initiation of hemodialysis was lifesaving. Read More

    The pathophysiological basis of the protective effects of metformin in heart failure.
    Postepy Hig Med Dosw (Online) 2017 Aug;71(1):773-787
    Katedra i Zakład Patofizjologii, Uniwersytet Medyczny w Lublinie.
    Metformin, currently recommended as the drug of first choice in type 2 diabetes mellitus (T2DM), is one of the few antihiperglycemic drugs to reduce cardiovascular risk. Nonetheless, due to the risk of lactic acidosis during metformin therapy, its usage in patients with diabetes and heart failure (HF) is still a matter of debate. The aim of this review is to present data supporting the possibility of using metformin in the treatment of diabetic patients with concomitant heart failure. Read More

    MELAS syndrome associated with a new mitochondrial tRNA-Val gene mutation (m.1616A>G).
    BMJ Case Rep 2017 Sep 11;2017. Epub 2017 Sep 11.
    Department of Neurology, Gifu Municipal Hospital, Gifu City, Japan.
    We describe the case of a 40-year-old-man with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome, with cardiomyopathy and severe heart failure. He had a mitochondrial transfer RNA (tRNA) mutation (m.1616A>G) of the (tRNA-Val) gene, and it was not found in MELAS syndrome ever before. Read More

    Role of cathepsin K in the development of chronic subdural hematoma.
    J Clin Neurosci 2017 Sep 5. Epub 2017 Sep 5.
    Department of Neurological Surgery, Juntendo University School of Medicine, Japan.
    Despite extensive investigations, the process of development of chronic subdural hematoma (CSDH) is not known. The present study aims to investigate CSDH by measuring biomarkers in it, gas analysis, and immunohistochemical examination. A total of 42 patients with symptomatic CSDH who underwent burr-hole drainage were enrolled. Read More

    Out-of-hospital cardiac arrest: always coronary angiography?
    Expert Rev Cardiovasc Ther 2017 Sep 18:1-5. Epub 2017 Sep 18.
    c Division of Cardiology , A.S.L. VCO , Omegna , Italy.
    Introduction: Out-of-hospital cardiac arrest (OHCA) remains one of the principle challenges in the setting of critical care medicine and emergency cardiology. Areas covered: Long-term survival rates even after successful resuscitation are variable but increasing in the recent years; due to the improvement of base and advanced cardiac life support techniques an increasing number of resuscitated patients are admitted to the hospital. Recent data suggested that patients surviving to hospital discharge after OHCA presented long-term outcome similar to patients with ST-elevation myocardial infarction. Read More

    Peptide-mediated delivery of donor mitochondria improves mitochondrial function and cell viability in human cybrid cells with the MELAS A3243G mutation.
    Sci Rep 2017 Sep 6;7(1):10710. Epub 2017 Sep 6.
    Vascular and Genomic Center, Changhua Christian Hospital, Changhua, Taiwan.
    The cell penetrating peptide, Pep-1, has been shown to facilitate cellular uptake of foreign mitochondria but further research is required to evaluate the use of Pep-1-mediated mitochondrial delivery (PMD) in treating mitochondrial defects. Presently, we sought to determine whether mitochondrial transplantation rescue mitochondrial function in a cybrid cell model of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) disease. Following PMD, recipient cells had internalized donor mitochondria after 1 h, and expressed higher levels of normal mitochondrial DNA, particularly at the end of the treatment and 11 days later. Read More

    Parameters for Ordering Blood Cultures in Major Burn Injury Patients: Improving Clinical Assessment.
    J Burn Care Res 2017 Aug 31. Epub 2017 Aug 31.
    From the *Department of Plastic Surgery, University of Kansas Medical Center, Kansas City, KS; †Department of Surgery, University of Illinois College of Medicine at Urbana-Champaign, Urbana, IL; ‡University of Kansas School of Medicine, Kansas City, KS; §Department of Preventative Medicine and Public Health, University of Kansas Medical Center, Kansas City, KS.
    Hypermetabolic and catabolic states in large TBSA burns lead to higher basal body temperature and tachycardia. These metabolic changes complicate the diagnosis of bacteremia and sepsis. Current indications for obtaining blood cultures (BCs) in this population are poorly described and nonstandardized. Read More

    [Clinical effect of terminal branches portal vein embolization combined with transcatheter arterial chemoembolization on liver neoplasms].
    Zhonghua Wai Ke Za Zhi 2017 Sep;55(9):655-660
    Department of Surgery, the Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou 310009, China.
    Objective: To analyze the efficacy of branches portal vein embolization (TBPVE) combined with transcatheter arterial chemoembolization (TACE) on liver neoplasms. Methods: From August 2016 to May 2017, there were 13 patients including 11 males and 2 females with primary hepatocellular carcinoma who underwent TBPVE+ TACE , among whom there were 11 cases with a history of HBV infection.Average age of the 13 patients was (60. Read More

    Mutation in the AGK gene in two siblings with unusual Sengers syndrome.
    Metab Brain Dis 2017 Sep 3. Epub 2017 Sep 3.
    Service de Génétique Médicale, Hôpital Estaing, Centre Hospitalier Universitaire de Clermont-Ferrand, Clermont-Ferrand, France.
    Sengers syndrome is a rare autosomal recessive metabolic disorder caused by lack of acylglycerol kinase due to mutations in the AGK gene. It is characterized by congenital cataract, hypertrophic cardiomyopathy, myopathy and lactic acidosis. Two clinical forms have been described: a severe neonatal form, and a more benign form displaying exercise intolerance. Read More

    Still sour about lactic acidosis years later: role of metformin in heart failure.
    Heart Fail Rev 2017 Sep 4. Epub 2017 Sep 4.
    Division of Cardiovascular Medicine, Gill Heart & Vascular Institute, University of Kentucky Albert B. Chandler Hospital, 800 Rose Street, Lexington, KY, 40536, USA.
    Metformin remains a widely-used, first-line pharmacotherapy agent for patients with type 2 diabetes mellitus because of its efficacy, mild side effects, and affordability.However, use of this medication has traditionally been shunned by clinicians in patient populations that are considered at risk of lactic acidosis, such as those with heart failure. The underutilization of metformin can largely be attributed to the historical stigma of its biguanide predecessor, phenformin, and its association with lactic acidosis. Read More

    Peripheral venoarterial extracorporeal membrane oxygenation for severe hyperlactataemia after cardiac surgery: a pilot study.
    Crit Care Resusc 2017 Sep;19(3):274-279
    Warringal Private Hospital, Melbourne, VIC, Australia.
    Background: Severe hyperlactataemia in patients after cardiac surgery is associated with poor prognosis and implies possible splanchnic hypoperfusion. Peripheral venoarterial extracorporeal membrane oxygenation (splanchnic ECMO) may be more effective at reducing lactic acidosis for these patients.

    Objective: To investigate whether splanchnic ECMO attenuates hyperlactataemia and liver enzyme release in these patients, despite them having a cardiac index > 2 L/min/m(2) and a mixed venous oxygen saturation > 55%. Read More

    Combined metformin-associated lactic acidosis and euglycemic ketoacidosis.
    Wien Klin Wochenschr 2017 Sep 1. Epub 2017 Sep 1.
    Department of Internal Medicine, Intensive Care Unit, Medical University of Graz, Graz, Austria.
    Background: In renal failure metformin can lead to lactic acidosis. Additional inhibition of hepatic gluconeogenesis by accumulation of the drug may aggravate fasting-induced ketoacidosis. We report the occurrence of metformin-associated lactic acidosis (MALA) with concurrent euglycemic ketoacidosis (MALKA) in three patients with renal failure. Read More

    d(-) Lactic Acid-Induced Adhesion of Bovine Neutrophils onto Endothelial Cells Is Dependent on Neutrophils Extracellular Traps Formation and CD11b Expression.
    Front Immunol 2017 15;8:975. Epub 2017 Aug 15.
    Laboratory of Inflammation Pharmacology, Faculty of Veterinary Science, Institute of Pharmacology and Morphophysiology, Universidad Austral de Chile, Valdivia, Chile.
    Bovine ruminal acidosis is of economic importance as it contributes to reduced milk and meat production. This phenomenon is mainly attributed to an overload of highly fermentable carbohydrate, resulting in increased d(-) lactic acid levels in serum and plasma. Ruminal acidosis correlates with elevated acute phase proteins in blood, along with neutrophil activation and infiltration into various tissues leading to laminitis and aseptic polysynovitis. Read More

    Effect of Propionibacterium acidipropionici P169 on the rumen and faecal microbiota of beef cattle fed a maize-based finishing diet.
    Benef Microbes 2017 Aug 31:1-16. Epub 2017 Aug 31.
    1 Department of Animal Science, University of Manitoba, 12 Dafoe road, Winnipeg, MB R3T 2N2, Canada.
    Direct fed microbial supplementation with lactic acid utilising bacteria (i.e. Propionibacterium acidipropionici P169) has been shown to alleviate the severity of subacute ruminal acidosis in high-grain fed beef cattle. Read More

    Use of high b value diffusion-weighted magnetic resonance imaging in acute encephalopathy/encephalitis during childhood.
    Brain Dev 2017 Aug 21. Epub 2017 Aug 21.
    Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
    Aim: To determine the use of high b value diffusion-weighted imaging (DWI) in the diagnosis and assessment of acute febrile encephalopathy/encephalitis in childhood.

    Subjects And Methods: We enrolled 22 children, for whom we examined DWI with b=1000s/mm(2), DWI with b=3000s/mm(2), and apparent diffusion coefficient (ADC) map with b=1000 during the acute phase of febrile encephalopathy/encephalitis. Clinical diagnoses included acute encephalopathy with biphasic seizures and late reduced diffusion (AESD; n=6), clinically mild encephalopathy/encephalitis with a reversible splenial lesion (MERS; n=6), and herpes simplex virus encephalitis (HSE; n=3), unclassified acute encephalopathy/acute encephalitis (n=2); acute encephalitis with refractory, repetitive partial seizures (AERRPS; n=1); other encephalopathy (n=1); infarction (n=1); head injury (n=1); or mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (n=1). Read More

    Lactate Levels with Chronic Metformin Use: A Narrative Review.
    Clin Drug Investig 2017 Aug 23. Epub 2017 Aug 23.
    Faculty of Health, University of Tasmania, Hobart, Australia.
    Metformin has been associated with lactic acidosis. Lactate levels are not commonly tested in clinical practice, and it is unclear to what extent metformin would typically increase lactate levels with chronic use. The aim of this review was to determine whether regular monitoring of the plasma lactate level would be beneficial in avoiding lactate accumulation and, ultimately, minimising the incidence of lactic acidosis in metformin-treated patients. Read More

    Monocarboxylate transporter MCT1 promotes tumor metastasis independently of its activity as a lactate transporter.
    Cancer Res 2017 Aug 21. Epub 2017 Aug 21.
    Institute of Experimental and Clinical Research (IREC), Pole of Pharmacology and Therapeutics, Université catholique de Louvain
    Extracellular acidosis resulting from intense metabolic activities in tumors promotes cancer cell migration, invasion and metastasis. While host cells die at low extracellular pH, cancer cells resist, as they are well equipped with transporters and enzymes to regulate intracellular pH homeostasis. A low extracellular pH further activates proteolytic enzymes that remodel the extracellular matrix to facilitate cell migration and invasion. Read More

    [Thyroid storm associated with multiorganic dysfunction].
    Medicina (B Aires) 2017 ;77(4):337-340
    Servicio de Endocrinología, Fundación Valle del Lili, Cali, Colombia. E-mail:
    Thyroid storm is a rare and potentially fatal condition. Unusual presentations in patients with thyroid storm have been described but multiorganic dysfunction is uncommonly seen. We describe the case of a 36-year-old woman with unknown underlying Graves's disease who developed thyroid storm. Read More

    [Clinical Analysis of 10 Cases of B Cell Lymphoma-Associated Hemophagocytic Syndrome].
    Zhongguo Shi Yan Xue Ye Xue Za Zhi 2017 Aug;25(4):1022-1029
    Department of Hematology, Peking University Shenzhen Hospital, Shenzhen 518036, Guangdong Province, China.
    Objective: To investigate the clinical and laboratory features of patient with B cell lymphoma associated hemophagocytic syndrome(B-LAHS).

    Methods: The clinical data of 10 cases of B-LAHS were retrospectively analysed and the relevant literatures were reviewed.

    Results: The median age of 10 cases diagnosed as B-LAHS was 55. Read More

    Black Toenail Sign in MELAS Syndrome.
    Pediatr Neurol 2017 Jul 12. Epub 2017 Jul 12.
    Department of Neurology, Children's National Medical Center, Washington, District of Columbia.
    Background: Mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome is a mitochondrial disorder often causing progressive brain injury that is not confined to large arterial territories. Severe insults ultimately lead to gyral necrosis affecting the cortex and juxtacortical white matter; the neuroimaging correlate is partial gyral signal suppression on T2/FLAIR sequences that resemble black toenails. We aimed to characterize the imaging features and the natural history of MELAS-related gyral necrosis. Read More

    The Liver Circadian Clock Modulates Biochemical and Physiological Responses to Metformin.
    J Biol Rhythms 2017 Aug 15;32(4):345-358. Epub 2017 Jun 15.
    Department of Molecular Medicine, The Scripps Research Institute, La Jolla, California, USA.
    Metformin is widely used in the treatment of type 2 diabetes to lower blood glucose. Although metformin is a relatively safe and effective drug, its clinical efficacy is variable and under certain circumstances it may contribute to life-threatening lactic acidosis. Thus, additional understanding of metformin pharmacokinetics and pharmacodynamics could provide important information regarding therapeutic use of this widely prescribed drug. Read More

    Importance of Distinguishing Between Mitochondrial Encephalomyopathy With Elderly Onset of Stroke-Like Episodes and Cerebral Infarction.
    J Clin Med Res 2017 Sep 27;9(9):812-819. Epub 2017 Jul 27.
    Department of Neurology, Hospital of International University of Health and Welfare, 537-3, Iguchi, Nasushiobara, Tochigi 329-2763, Japan.
    The most common disease-causing mitochondrial DNA (mtDNA) mutation in mitochondrial encephalomyopathy (ME) with lactic acidosis and stroke-like episodes (MELAS) is m.3243A>G. In the future, the incidence of patients with cerebral infarction and diabetes mellitus is expected to increase tremendously. Read More

    Combined Respiratory Chain Deficiency and UQCC2 Mutations in Neonatal Encephalomyopathy: Defective Supercomplex Assembly in Complex III Deficiencies.
    Oxid Med Cell Longev 2017 19;2017:7202589. Epub 2017 Jul 19.
    Department of Pediatrics, Salzburger Landeskliniken (SALK) and Paracelsus Medical University (PMU), 5020 Salzburg, Austria.
    Vertebrate respiratory chain complex III consists of eleven subunits. Mutations in five subunits either mitochondrial (MT-CYB) or nuclear (CYC1, UQCRC2, UQCRB, and UQCRQ) encoded have been reported. Defects in five further factors for assembly (TTC19, UQCC2, and UQCC3) or iron-sulphur cluster loading (BCS1L and LYRM7) cause complex III deficiency. Read More

    The proton-sensing G protein-coupled receptor T-cell death-associated gene 8 (TDAG8) shows cardioprotective effects against myocardial infarction.
    Sci Rep 2017 Aug 10;7(1):7812. Epub 2017 Aug 10.
    Department of Pharmacology and Toxicology, Graduate School of Pharmaceutical Sciences, Kyushu University, Fukuoka, 812-8582, Japan.
    Myocardial infarction (MI) is an ischaemic heart condition caused by the occlusion of coronary arteries. Following MI, lactic acid from anaerobic glycolysis increases and infiltrating immune cells produce severe inflammation, which leads to acidosis in the ischaemic heart. However, the physiological implication of this pH reduction remains largely unknown. Read More

    Quantification of paracetamol and 5-oxoproline in serum by capillary electrophoresis: Implication for clinical toxicology.
    J Pharm Biomed Anal 2017 Oct 26;145:616-620. Epub 2017 Jul 26.
    Charles University and General University Hospital, First Faculty of Medicine, Institute of Forensic Medicine and Toxicology, Ke Karlovu 2, 121 08, Prague 2, Czech Republic; Charles University, Faculty of Science, Department of Analytical Chemistry, Albertov 6, 128 43, Prague 2, Czech Republic. Electronic address:
    High anion gap metabolic acidosis frequently complicates acute paracetamol overdose and is generally attributed to lactic acidosis or compromised hepatic function. However, metabolic acidosis can also be caused by organic acid 5-oxoproline (pyroglutamic acid). Paracetamol's toxic intermediate, N-acetyl-p-benzoquinoneimine irreversibly binds to glutathione and its depletion leads to subsequent disruption of the gamma glutamyl cycle and an excessive 5-oxoproline generation. Read More

    Oral pyridoxine can substitute for intravenous pyridoxine in managing patients with severe poisoning with isoniazid and rifampicin fixed dose combination tablets: a case report.
    BMC Res Notes 2017 Aug 8;10(1):370. Epub 2017 Aug 8.
    University Medical Unit, National Hospital of Sri Lanka, Colombo 10, Sri Lanka.
    Background: Fixed drug combination of isoniazid and rifampicin is a rare cause of poisoning even in endemic countries for tuberculosis infection. Severe poisoning can cause severe morbidity and mortality if not treated promptly. Though intravenous pyridoxine is the preferred antidote for severe standard isoniazid poisoning it is not freely available even in best of care centers. Read More

    Status Epilepticus due to Intraperitoneal Injection of Vehicle Containing Propylene Glycol in Sprague Dawley Rats.
    Vet Med Int 2017 12;2017:1757059. Epub 2017 Jul 12.
    Advanced Platform Technology Center, Louis Stokes Cleveland Department of Veterans Affairs Medical Center, 10701 East Blvd, 151 W/APT, Cleveland, OH 44106, USA.
    Published reports of status epilepticus due to intraperitoneal injection containing propylene glycol in rats are sparse. In fact, there are no reports specifying a maximum safe dose of propylene glycol through intraperitoneal administration. We report here a case of unexpected seizures in Sprague Dawley rats after receiving an intraperitoneal injection containing propylene glycol. Read More

    Novel mutations in SERAC1 gene in two Indian patients presenting with dystonia and intellectual disability.
    Eur J Med Genet 2017 Aug 1. Epub 2017 Aug 1.
    Paediatric Neurologist, Rainbow Children Hospital, Hyderabad, 500082, India.
    In this study we present the first two cases from India of a rare inborn error of metabolism manifesting as dystonia and 3-methylglutaconic aciduria and a Leigh like lesions in the brain MRI associated with SERAC1 gene mutation, a phenotype characteristic of MEGDEL syndrome. A four base pair duplication in exon 15 i.e. Read More

    Metformin: historical overview.
    Diabetologia 2017 Aug 3. Epub 2017 Aug 3.
    School of Life and Health Sciences, Aston University, Gosta Green, Birmingham, B4 7ET, UK.
    Metformin (dimethylbiguanide) has become the preferred first-line oral blood glucose-lowering agent to manage type 2 diabetes. Its history is linked to Galega officinalis (also known as goat's rue), a traditional herbal medicine in Europe, found to be rich in guanidine, which, in 1918, was shown to lower blood glucose. Guanidine derivatives, including metformin, were synthesised and some (not metformin) were used to treat diabetes in the 1920s and 1930s but were discontinued due to toxicity and the increased availability of insulin. Read More

    Thrombotic microangiopathy associated with Valproic acid toxicity.
    BMC Nephrol 2017 Aug 3;18(1):262. Epub 2017 Aug 3.
    Department of Pediatrics, Division of Nephrology, McGovern Medical School at The University of Texas Health Science Center at Houston (UTHealth), Children's Memorial Hermann Hospital, 6431 Fannin Street, MSB 3. 121, Houston, TX, USA.
    Background: Thrombotic microangiopathy (TMA) is a serious, sometimes life-threatening disorder marked by the presence of endothelial injury and microvascular thrombi. Drug-induced thrombotic microangiopathy (DI-TMA) is one specific TMA syndrome that occurs following drug exposure via drug-dependent antibodies or direct tissue toxicity. Common examples include calcineurin inhibitors Tacrolimus and Cyclosporine and antineoplastics Gemcitabine and Mitomycin. Read More

    Migraine in mitochondrial disorders: Prevalence and characteristics.
    Cephalalgia 2017 Jan 1:333102417723568. Epub 2017 Jan 1.
    1 Unità di Neurofisiopatologia, Area Neuroscienze, Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Italy.
    Background Migraine is a well-known feature of mitochondrial disorders (MDs). However, no systematic epidemiological data are available in large populations of patients. Aims The aim of this cross-sectional cohort study was to describe the prevalence and migraine characteristics in a large cohort of patients with mitochondrial encephalomyopathies. Read More

    Metformin Use in Practice: Compliance With Guidelines for Patients With Diabetes and Preserved Renal Function.
    Clin Diabetes 2017 Jul;35(3):154-161
    Center on Aging, University of Connecticut Health Center, Farmington, CT.
    IN BRIEF Several contraindications limit the use of metformin, most notably the risk of lactic acidosis. This article reports on an examination of a population of patients with diabetes with preserved renal function to evaluate provider compliance with guidelines on metformin use and to identify factors that contributed when practice diverged from recommendations. It found that metformin was withheld from approximately one-third of these patients because of 1) an existent contraindication to metformin, 2) patient behavior or preference, or 3) provider preference or bias based on patient or personal factors. Read More

    High anion gap metabolic acidosis induced by cumulation of ketones, L- and D-lactate, 5-oxoproline and acute renal failure.
    Acta Clin Belg 2017 Jul 27:1-4. Epub 2017 Jul 27.
    a Department of Laboratory Medicine , Ziekenhuis Netwerk Antwerpen , Antwerp , Belgium.
    Introduction: Frequent causes of high anion gap metabolic acidosis (HAGMA) are lactic acidosis, ketoacidosis and impaired renal function. In this case report, a HAGMA caused by ketones, L- and D-lactate, acute renal failure as well as 5-oxoproline is discussed.

    Case Presentation: A 69-year-old woman was admitted to the emergency department with lowered consciousness, hyperventilation, diarrhoea and vomiting. Read More

    Acid-base and electrolyte disorders associated with the use of antidiabetic drugs.
    Expert Opin Drug Saf 2017 Oct 4;16(10):1121-1132. Epub 2017 Aug 4.
    a Department of Internal Medicine, School of Medicine , University of Ioannina , Ioannina , Greece.
    Introduction: The use of antidiabetic drugs is expected to substantially increase since diabetes mellitus incidence rises. Currently used antidiabetic drugs have a positive safety profile, but they are associated with certain acid-base and electrolyte abnormalities. The aim of the review is to present the current data regarding the antidiabetic drugs-associated acid-base and electrolyte abnormalities. Read More

    Evaluation of Mitochondrial Respiration in Cultured Rat Hepatocytes.
    Methods Mol Biol 2017 ;1641:297-308
    Investigative Toxicology, Preclinical Safety, Sanofi R&D, 13 quai Jules Guesde, BP14, Vitry-sur-Seine, 94403, France.
    Mitochondrial dysfunction is a major mechanism whereby drugs can induce liver injury and other serious side effects, such as lactic acidosis and rhabdomyolysis, in some patients. Several in vitro and in vivo investigations can be performed in order to determine if drugs can disturb mitochondrial fatty acid oxidation (FAO) and the oxidative phosphorylation (OXPHOS) process, deplete hepatic mitochondrial DNA (mtDNA), or trigger the opening of the mitochondrial permeability transition pore (MPT). Among these investigations, mitochondrial respiration is a relatively easy test to measure the potential toxicity of a drug. Read More

    Lactate serum concentrations during treatment with nucleos(t)ide analogues in hepatitis B with or without cirrhosis.
    Eur J Gastroenterol Hepatol 2017 Sep;29(9):998-1003
    Departments of aGastroenterology bBiochemistry cInternal Medicine dMicrobiology, University Hospital of Patras, Patras eDepartment of Hygiene, Epidemiology and Medical Statistics, Medical School University of Athens, Athens, Greece.
    Objective: The aim of this study is to evaluate the clinical implications of lactate concentrations in patients with hepatitis B with or without cirrhosis during treatment with nucleos(t)ide analogues.

    Patients And Methods: One hundred and seven consecutive patients with chronic hepatitis B and median age 57 (24-85) years were prospectively included. Lactate concentrations were measured at baseline and at 12, 24, 36, 48, and 60 months following the baseline measurements. Read More

    Epidemiology and Outcomes of Cardiac Arrest in Pediatric Cardiac ICUs.
    Pediatr Crit Care Med 2017 Jul 21. Epub 2017 Jul 21.
    1Department of Pediatric Cardiology, Section of Cardiac Critical Care Medicine, University of Alabama at Birmingham, Birmingham, AL. 2Departments of Pediatric Critical Care Medicine and Pediatric Cardiology, Children's National Health System, Washington, D.C. 3Department of Pediatric Cardiology, Medical City Children's Hospital, Dallas, TX. 4Department of Pediatric Cardiology, Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH. 5Department of Pediatrics and Communicable Diseases, Division of Cardiology, C. S. Mott Children's Hospital and University of Michigan Medical School, Ann Arbor, MI.
    Objectives: In-hospital cardiac arrest occurs in 2.6-6% of children with cardiac disease and is associated with significant morbidity and mortality. Much remains unknown about cardiac arrest in pediatric cardiac ICUs; therefore, we aimed to describe cardiac arrest epidemiology in a contemporary multicenter cardiac ICU cohort. Read More

    Arginine and citrulline for the treatment of MELAS syndrome.
    J Inborn Errors Metab Screen 2017 Jan 24;5. Epub 2017 Mar 24.
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA.
    MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) syndrome is a maternally inherited mitochondrial disease with a broad spectrum of manifestations. In addition to impaired energy production, nitric oxide (NO) deficiency occurs in MELAS syndrome and leads to impaired blood perfusion in microvasculature that can contribute to several complications including stroke-like episodes, myopathy, and lactic acidosis. The supplementation of NO precursors, L-arginine and L-citrulline, increases NO production and hence can potentially have therapeutic utility in MELAS syndrome. Read More

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