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    Consideration of alternative causes of lactic acidosis: Thiamine deficiency in malignancy.
    Am J Emerg Med 2017 May 15. Epub 2017 May 15.
    Department of Medicine, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, United States.
    Lactic acidosis is a common metabolic acidosis characterized by increased serum lactate and is usually associated with a decreased blood pH. Lactic acidosis has many different causes but has been differentiated into type A, hypoxic causes, and type B, non-hypoxic causes. Tissue hypoxia, type A, is the most common cause, usually secondary to processes such as sepsis and multi-organ failure. Read More

    Linezolid-induced lactic acidosis: the thin line between bacterial and mitochondrial ribosomes.
    Expert Opin Drug Saf 2017 May 24. Epub 2017 May 24.
    a Dipartimento di Anestesia, Rianimazione ed Emergenza-Urgenza , Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico , via F. Sforza 35, 20100 Milan , Italy.
    Introduction - Linezolid inhibits bacterial growth by targeting bacterial ribosomes and by interfering with bacterial protein synthesis. Lactic acidosis is a rare, but potentially lethal, side effect of linezolid. Areas covered - The pathogenesis of linezolid-induced lactic acidosis is reviewed with special emphasis on aspects relevant to the recognition, prevention and treatment of the syndrome. Read More

    Diabetes Update: New Pharmacotherapy for Type 2 Diabetes.
    FP Essent 2017 May;456:27-35
    University of Tennessee Health Science Center Department of Medical Education, 920 Madison Building 7th floor, Memphis, TN 3816-30000.
    Multiple new drugs for managing type 2 diabetes have entered the market in the past 5 years. Guidelines from the American Diabetes Association recommend metformin for initial therapy, followed by a second drug if A1c goals are not met or initially for patients with A1c levels greater than 9%. Conversely, the American Association of Clinical Endocrinologists recommends initial management with two drugs if the A1c level is greater than 7. Read More

    Risk of acute kidney injury and survival in patients treated with Metformin: an observational cohort study.
    BMC Nephrol 2017 May 19;18(1):163. Epub 2017 May 19.
    Division of Population Health Sciences, School of Medicine, University of Dundee, Dundee, UK.
    Background: Whether metformin precipitates lactic acidosis in patients with chronic kidney disease (CKD) remains under debate. We examined whether metformin use was associated with an increased risk of acute kidney injury (AKI) as a proxy for lactic acidosis and whether survival among those with AKI varied by metformin exposure.

    Methods: All individuals with type 2 diabetes and available prescribing data between 2004 and 2013 in Tayside, Scotland were included. Read More

    Case report: a novel frameshift mutation in the mitochondrial cytochrome c oxidase II gene causing mitochondrial disorder.
    BMC Neurol 2017 May 18;17(1):96. Epub 2017 May 18.
    Research Unit of Clinical Neuroscience, University of Oulu, P.O. Box 5000, FI-90014, Oulu, Finland.
    Background: Mitochondrial cytochrome c oxidase 2, MT-CO2, encodes one of the three subunits, which form the catalytic core of cytochrome c oxidase (COX), complex IV. Mutations in MT-CO2 are rare and the associated phenotypes are variable including nonsyndromic and syndromic forms of mitochondrial diseases.

    Case Presentation: We describe a 30-year-old man with cognitive decline, epilepsy, psychosis, exercise intolerance, sensorineural hearing impairment, retinitis pigmentosa, cataract and lactic acidosis. Read More

    [Importance of stopping some medications in case of gastrointestinal disturbances leading to dehydration].
    Rev Med Liege 2017 May;72(5):266-271
    Service de Diabétologie, Nutrition et Maladies métaboliques et Unité de Pharmacologie clinique, CHU de Liège, Site Sart Tilman, Liège, Belgique.
    This clinical case draws attention to the importance of stopping some medications in case of gastrointestinal disturbances that could induce dehydration. The latter can provoke an acute renal failure, which may lead to a toxic accumulation of these pharmacological agents and/or increase their nephrotoxicity. This may induce a vicious circle that is potentially dangerous and even fatal. Read More

    Renal manifestations of primary mitochondrial disorders.
    Biomed Rep 2017 05 12;6(5):487-494. Epub 2017 Apr 12.
    Paulista de Medicina School, Federal University of São Paulo, Primeiro Andar CEP, São Paulo 04039-032, SP, Brazil.
    The aim of the present review was to summarize and discuss previous findings concerning renal manifestations of primary mitochondrial disorders (MIDs). A literature review was performed using frequently used databases. The study identified that primary MIDs frequently present as mitochondrial multiorgan disorder syndrome (MIMODS) at onset or in the later course of the MID. Read More

    Assessment of Nitric Oxide Production in Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes Syndrome with the Use of a Stable Isotope Tracer Infusion Technique.
    J Nutr 2017 May 17. Epub 2017 May 17.
    US Department of Agriculture/Agricultural Research Service, Children's Nutrition Research Center, Department of Pediatrics, Baylor College of Medicine, Houston, TX
    Mitochondrial disorders result from dysfunctional mitochondria that are unable to generate sufficient energy to meet the needs of various organs. Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is one of the most frequent maternally inherited mitochondrial disorders. There is growing evidence that nitric oxide (NO) deficiency occurs in MELAS syndrome and results in impaired blood perfusion that contributes significantly to several complications in this disease. Read More

    Metformin Does Not Induce Hyperlactatemia in Patients Admitted to Internal Medicine Ward.
    Isr Med Assoc J 2017 May;19(5):300-3
    Department of Internal Medicine A, Assaf Harofeh Medical Center, Zerifin, affiliated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
    Background: Concerns about metformin-associated lactic acidosis (MALA) prohibit the use of metformin in a large subset of diabetic patients, mostly in patients with chronic kidney disease. Increasing evidence suggests that the current safety regulations may be overly restrictive.

    Objectives: To examine the association between chronic metformin treatment and lactate level in acute illness on the first day of admission to an internal medicine ward. Read More

    Transient dilutional acidosis but no lactic acidosis upon cardiopulmonary bypass in patients undergoing coronary artery bypass grafting.
    Arch Med Sci 2017 Apr 7;13(3):585-590. Epub 2016 Apr 7.
    Institute of Physiological Chemistry, University Hospital Essen, University of Duisburg-Essen, Essen, Germany.
    Introduction: Dilutional acidosis may result from the introduction of a large fluid volume into the patients' systemic circulation, resulting in a considerable dilution of endogenous bicarbonate in the presence of a constant carbon dioxide partial pressure. Its significance or even existence, however, has been strongly questioned. Blood gas samples of patients operated on with standard cardiopulmonary bypass (CPB) were analyzed in order to provide further evidence for the existence of dilutional acidosis. Read More

    Identification of FASTKD2 compound heterozygous mutations as the underlying cause of autosomal recessive MELAS-like syndrome.
    Mitochondrion 2017 May 9. Epub 2017 May 9.
    Department of Biological Sciences, Kongju National University, Gongju, Republic of Korea. Electronic address:
    Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a condition that affects many parts of the body, particularly the brain and muscles. This study examined a Korean MELAS-like syndrome patient with seizure, stroke-like episode, and optic atrophy. Target sequencing of whole mtDNA and 73 nuclear genes identified compound heterozygous mutations p. Read More

    Deoxyguanosine kinase deficiency: a report of four patients.
    J Pediatr Endocrinol Metab 2017 May 11. Epub 2017 May 11.
    .
    Background: Hepatic involvement is a common feature in childhood mitochondrial disorders. Deoxyguanosine kinase (DGUOK) deficiency is one of the mitochondrial DNA depletion syndromes associated with hepatocerebral syndrome. Hepatic disease and neurologic dysfunction occurs within weeks after birth. Read More

    Novel RRM2B Mutation and Severe Mitochondrial DNA Depletion: Report of 2 Cases and Review of the Literature.
    Neuropediatrics 2017 May 8. Epub 2017 May 8.
    Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
    Purpose To describe the clinical presentation and implications of mitochondrial DNA depletion disorder of two siblings with early fatal encephalomyopathy and a novel mutation in the RRM2B gene. The relevant literature is reviewed. Methods We describe two brothers aged 2. Read More

    Preliminary Study of Neurodevelopmental Outcomes and Parenting Stress in Pediatric Mitochondrial Disease.
    Pediatr Neurol 2017 Jun 2;71:43-49.e1. Epub 2017 Feb 2.
    Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea. Electronic address:
    Background: Little is known regarding the neuropsychological profiles of pediatric patients with mitochondrial diseases or their parents, information that is crucial for improving the quality of life (QOL) for both patients and parents. We aimed to delineate neurodevelopment and psychological comorbidity in children with mitochondrial diseases in the preliminary investigation of adequate intervention methods, better prognoses, and improved QOL for both patients and parents.

    Methods: Seventy children diagnosed with mitochondrial diseases were neuropsychologically evaluated. Read More

    Metformin-associated lactic acidosis (MALA): moving towards a new paradigm?
    Diabetes Obes Metab 2017 May 4. Epub 2017 May 4.
    Elcelyx Therapeutics, Inc., San Diego, CA, USA.
    The history of Metformin-Associated Lactic Acidosis (MALA) is rooted in the earlier experience with phenformin, another biguanide treatment for type 2 diabetes that was removed from the market owing to a clear causal association with cases of lactic acidosis (LA). Although metformin is less problematic in this regard, metformin can and does induce lactic acidosis, and under the right circumstances it continues to present a potential risk to patients. Recent liberalization of the prescribing guidelines in the US and EU specifically regarding use in more advanced stages of Chronic Kidney Disease (CKD) have led to renewed interest in the topic of MALA, and this issue of the Journal presents two timely articles on the topic by Lalau et al. Read More

    Familial Pernicious Chronic Intestinal Pseudo-obstruction with a Mitochondrial DNA A3243G Mutation.
    Intern Med 2017 1;56(9):1089-1093. Epub 2017 May 1.
    Department of Neurology, TOYOTA Memorial Hospital, Japan.
    We report the case of a mother and two children who shared a mitochondrial DNA A3243G mutation. The mother had diabetes mellitus, neurogenic bladder, bradykinesia, dystonia, and slowly progressive cerebellar ataxia. Her two daughters were diagnosed with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes at adolescence. Read More

    SIRT5 Binds to Cardiolipin and Regulates the Electron Transport Chain.
    J Biol Chem 2017 Apr 30. Epub 2017 Apr 30.
    Children's Hospital of Pittsburgh, United States;
    SIRT5 is a lysine desuccinylase known to regulate mitochondrial fatty acid oxidation and the urea cycle. Here, SIRT5 was observed to bind to cardiolipin via an amphipathic helix on its amino terminus. In vitro, succinyl-CoA was used to succinylate liver mitochondrial membrane proteins. Read More

    Idiosyncratic recognition of UUG/UUA codons by modified nucleoside 5-taurinomethyluridine, τm5U present at 'wobble' position in anticodon loop of tRNALeu: A molecular modeling approach.
    PLoS One 2017 28;12(4):e0176756. Epub 2017 Apr 28.
    Structural Bioinformatics Unit, Department of Biochemistry, Shivaji University, Kolhapur, (M.S.), India.
    Lack of naturally occurring modified nucleoside 5-taurinomethyluridine (τm5U) at the 'wobble' 34th position in tRNALeu causes mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). The τm5U34 specifically recognizes UUG and UUA codons. Structural consequences of τm5U34 to read cognate codons have not been studied so far in detail at the atomic level. Read More

    Drug-induced hyperlactatemia.
    Clin Toxicol (Phila) 2017 Apr 27:1-10. Epub 2017 Apr 27.
    a Department of Emergency Medicine, Division of Toxicology , University of Massachusetts Medical Center , Worcester , MA , USA.
    Background: Hyperlactatemia is common in critically ill patients and has a variety of etiologies. Medication toxicity remains an uncommon cause that providers often fail to recognize. In this article, we review several medications that cause hyperlactatemia in either therapeutic or supratherapeutic dosing. Read More

    Case 13-2017. A 41-Year-Old Man with Hearing Loss, Seizures, Weakness, and Cognitive Decline.
    N Engl J Med 2017 04;376(17):1668-1678
    From the Departments of Neurology (H.M.R.), Radiology (W.A.C.), Pediatrics (A.K.), and Pathology (D.H.O.), Massachusetts General Hospital, and the Departments of Neurology (H.M.R.), Radiology (W.A.C.), Pediatrics (A.K.), and Pathology (D.H.O.), Harvard Medical School - both in Boston.

    Regulation of organelle function by metformin.
    IUBMB Life 2017 Apr 26. Epub 2017 Apr 26.
    Nagoya Research Center for Brain and Neural Circuits, Graduate School of Science, Nagoya University, Nagoya, Japan.
    Metformin ameliorates hyperglycemia without the side effects of lactic acidosis or hypoglycemia. Metformin lowers the blood glucose level by decreasing hepatic glucose production in the liver and by increasing glucose uptake in the muscle. Recent studies show that metformin induces cell death in certain cancer cell lines by interfering with the metabolism of the cancer cells. Read More

    Hemodynamics and gas exchange during chest compressions in neonatal resuscitation.
    PLoS One 2017 25;12(4):e0176478. Epub 2017 Apr 25.
    Pediatrics, SUNY University at Buffalo, Buffalo, New York, United States of America.
    Purpose: Current knowledge about pulmonary/systemic hemodynamics and gas exchange during neonatal resuscitation in a model of transitioning fetal circulation with fetal shunts and fluid-filled alveoli is limited. Using a fetal lamb asphyxia model, we sought to determine whether hemodynamic or gas-exchange parameters predicted successful return of spontaneous circulation (ROSC).

    Methods: The umbilical cord was occluded in 22 lambs to induce asphyxial cardiac arrest. Read More

    A Systematic Review of the Clinical Presentation, Diagnosis, and Treatment of Small Bowel Obstruction.
    Curr Gastroenterol Rep 2017 Jun;19(6):28
    Division of Gastroenterology and Hepatology, Department of Medicine, William Beaumont Hospital, 3535 West Thirteen Mile Road, Royal Oak, MI, 48073, USA.
    Purpose Of Review: This study aimed to systematically review small bowel obstruction (SBO), focusing on recent changes in diagnosis/therapy.

    Recent Findings: SBO incidence is about 350,000/annum in the USA. Etiologies include adhesions (65%), hernias (10%), neoplasms (5%), Crohn's disease (5%), and other (15%). Read More

    Vitamin B1 in critically ill patients: needs and challenges.
    Clin Chem Lab Med 2017 Apr 22. Epub 2017 Apr 22.
    Department of Intensive Care, Austin Health, Heidelberg.
    Background: Thiamine has a crucial role in energy production, and consequently thiamine deficiency (TD) has been associated with cardiac failure, neurological disorders, oxidative stress (lactic acidosis and sepsis) and refeeding syndrome (RFS). This review aims to explore analytical methodologies of thiamine compound quantification and highlight similarities, variances and limitations of current techniques and how they may be relevant to patients.

    Content: An electronic search of Medline, PubMed and Embase databases for original articles published in peer-reviewed journals was conducted. Read More

    Acute kidney injury, plasma lactate concentrations and lactic acidosis in metformin users: A GoDarts study.
    Diabetes Obes Metab 2017 Apr 21. Epub 2017 Apr 21.
    Division of Molecular and Clinical Medicine, School of Medicine, University of Dundee.
    Aims: Metformin is renally excreted and has been associated with the development of lactic acidosis. Although current advice is to omit metformin during illnesses that may increase risk of acute kidney injury (AKI), the evidence supporting this is lacking. We investigated the relationship between AKI, lactate concentrations and the risk of lactic acidosis in those exposed to metformin. Read More

    Experience of carrier couples identified through a population-based carrier screening pilot program for four founder autosomal recessive diseases in Saguenay-Lac-Saint-Jean.
    Prenat Diagn 2017 Apr 18. Epub 2017 Apr 18.
    Medical Genetics, Department of Pediatrics, CHU Sainte-Justine and Department of Pediatrics, Université de Montréal, Montreal, Quebec, Canada.
    A pilot population-based carrier screening program started in 2010 in the Saguenay-Lac-Saint-Jean region of Quebec, Canada, for four recessive diseases with local founder effects (tyrosinemia type I, autosomal recessive spastic ataxia of Charlevoix-Saguenay, congenital lactic acidosis, and Andermann syndrome).

    Objectives: The objective of this study was to describe the experience of carrier couples identified through this program.

    Methods: Semi-structured interviews were performed with carrier couples. Read More

    Metformin-associated lactic acidosis (MALA): moving towards a new paradigm.
    Diabetes Obes Metab 2017 Apr 17. Epub 2017 Apr 17.
    Association REMEDES, Ville-sur-Jarnioux, France.
    Although metformin has been used for over 60 years, the balance between the drug's beneficial and adverse effects is still subject to debate. Following an analysis of how cases of so-called "metformin-associated lactic acidosis" (MALA) are reported in the literature, the present article reviews the pitfalls to be avoided when assessing the purported association between metformin and lactic acidosis. By starting from pathophysiological considerations, we propose a new paradigm for lactic acidosis in metformin-treated patients. Read More

    Effects of dystocia on blood gas parameters, acid-base balance and serum lactate concentration in heavy draft newborn foals.
    J Equine Sci 2017 28;28(1):27-30. Epub 2017 Mar 28.
    Department of Applied Veterinary Medicine, Obihiro University of Agriculture and Veterinary Medicine, Hokkaido 080-8555, Japan; United Graduate School of Veterinary Sciences, Gifu University, Gifu 501-1193, Japan; Research Center for Global Agromedicine, Obihiro University of Agriculture and Veterinary Medicine, Hokkaido 080-8555, Japan.
    Dystocia is often lethal for neonatal foals; however, its clinicopathological features remain largely unknown. We investigated the effect of dystocia on the foal blood profile. Venous blood samples were collected from 35 foals (5 Percheron and 30 crossbreds between Percheron, Belgian, and Breton heavy draft horses) at 0 hr, 1 hr, 12 hr and 1 day after birth. Read More

    Anesthetic Management of Mitochondrial Encephalopathy With Lactic Acidosis and Stroke-Like Episodes (MELAS Syndrome) in a High-Risk Pregnancy: A Case Report.
    A A Case Rep 2017 Apr 10. Epub 2017 Apr 10.
    From the *Department of Anesthesiology, University of Toronto; and †Department of Anesthesia and Pain Management, Mount Sinai Hospital, Toronto, Ontario, Canada.
    MELAS syndrome (mitochondrial encephalopathy, lactic acidosis, and stroke-like symptoms) is a rare and complex mitochondrial disorder. We present the in-hospital course of a 36-year-old gravida 2, para 0 with MELAS syndrome and severe preeclampsia, complicated by hyponatremia, hyperkalemia, and diabetes. A retained placenta with postpartum hemorrhage required urgent instrumental delivery under spinal anesthesia, transfusion, and intensive care unit admission for pulmonary edema, effusions, and atelectasis. Read More

    Clinical Features, Molecular Heterogeneity, and Prognostic Implications in YARS2-Related Mitochondrial Myopathy.
    JAMA Neurol 2017 Apr 10. Epub 2017 Apr 10.
    Wellcome Trust Centre for Mitochondrial Research, Institute of Neuroscience, The Medical School, Newcastle University, Newcastle upon Tyne, England.
    Importance: YARS2 mutations have been associated with a clinical triad of myopathy, lactic acidosis, and sideroblastic anemia in predominantly Middle Eastern populations. However, the identification of new patients expands the clinical and molecular spectrum of mitochondrial disorders.

    Objectives: To review the clinical, molecular, and genetic features of YARS2-related mitochondrial disease and to demonstrate a new Scottish founder variant. Read More

    Inborn Errors of Fructose Metabolism. What Can We Learn from Them?
    Nutrients 2017 Apr 3;9(4). Epub 2017 Apr 3.
    Center for Molecular Diseases, Division of Genetic Medicine, Lausanne University Hospital (CHUV), Beaumont-02/248, Lausanne CH-1011, Switzerland.
    Fructose is one of the main sweetening agents in the human diet and its ingestion is increasing globally. Dietary sugar has particular effects on those whose capacity to metabolize fructose is limited. If intolerance to carbohydrates is a frequent finding in children, inborn errors of carbohydrate metabolism are rare conditions. Read More

    Homozygous p.(Glu87Lys) variant in ISCA1 is associated with a multiple mitochondrial dysfunctions syndrome.
    J Hum Genet 2017 Mar 30. Epub 2017 Mar 30.
    Department of Medical Genetics, Kasturba Medical College, Manipal University, Manipal, India.
    The iron-sulfur (Fe-S) cluster (ISC) biogenesis pathway is indispensable for many fundamental biological processes and pathogenic variations in genes encoding several components of the Fe-S biogenesis machinery, such as NFU1, BOLA3, IBA57 and ISCA2 are already implicated in causing four types of multiple mitochondrial dysfunctions syndromes (MMDS). We report on two unrelated families, with two affected children each with early onset neurological deterioration, seizures, extensive white matter abnormalities, cortical migrational abnormalities, lactic acidosis and early demise. Exome sequencing of two affected individuals, one from each family, revealed a homozygous c. Read More

    Risk factors of post-operative severe hyperlactatemia and lactic acidosis following laparoscopic resection for pheochromocytoma.
    Sci Rep 2017 Mar 24;7(1):403. Epub 2017 Mar 24.
    Department of Anesthesiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, P.R. China.
    Severe hyperlactatemia (SH)/lactic acidosis (LA) after laparoscopic resection of pheochromocytoma is an infrequently reported complication. The study aims to investigate the incidence of this complication and to determine the clinical risk factors. Patients who underwent laparoscopic resection for pheochromocytoma between 2011 and 2014 at Peking Union Medical College Hospital were enrolled. Read More

    Refining metformin prescribing in New Zealand.
    N Z Med J 2017 Mar 24;130(1452):49-53. Epub 2017 Mar 24.
    Women's and Children's Health, University of Otago, Dunedin.
    Metformin is the mainstay of treatment of type 2 diabetes. However, there has been significant concern on prescribing metformin in patients with renal impairment as a result of metformin-associated lactic acidosis (MALA). Recent studies have cast doubt on the existence of MALA purely related to metformin use. Read More

    Normothermic ex-situ liver preservation: the new gold standard.
    Curr Opin Organ Transplant 2017 Jun;22(3):274-280
    aLiver Unit, Queen Elizabeth Hospital, University Hospitals Birmingham NHS Foundation Trust bNational Institute for Health Research, Birmingham Liver Biomedical Research Unit and Centre for Liver Research, Institute of Immunology and Immunotherapy, Institute for Biomedical Research, College of Medical and Dental Sciences, University of Birmingham, Birmingham, United Kingdom cLiver and HPB Unit, Apollo Navi Mumbai Hospital, Mumbai, India.
    Purpose Of Review: Normothermic machine perfusion of the liver (NMP-L) is a novel technology recently introduced into the practice of liver transplantation. This review recapitulates benefits of normothermic perfusion over conventional static cold storage and summarizes recent publications in this area.

    Recent Findings: The first clinical trials have demonstrated both safety and feasibility of NMP-L. Read More

    [Accumulation of Metformin-associated Lactic Acidosis].
    Dtsch Med Wochenschr 2017 Mar 22;142(6):428-431. Epub 2017 Mar 22.
    Objective Lactic acidosis has been associated with the use of metformin since its introduction. The cause, however, is highly controversial. The incidence of metformin-associated lactic acidosis (MALA) is quoted at 3,0 to 16,7 cases per 100 000 patient-years according to current studies. Read More

    A non-fatal intoxication and seven deaths involving the dissociative drug 3-MeO-PCP.
    Forensic Sci Int 2017 Jun 7;275:76-82. Epub 2017 Mar 7.
    Department of Forensic Genetics and Forensic Toxicology, National Board of Forensic Medicine, Linköping, Sweden; Department of Medical and Health Sciences, Division of Drug Research, Linköping University, Linköping, Sweden.
    Introduction: 3-methoxyphencyclidine (3-MeO-PCP) appeared on the illicit drug market in 2011 and is an analogue of phencyclidine, which exhibits anesthetic, analgesic and hallucinogenic properties. In this paper, we report data from a non-fatal intoxication and seven deaths involving 3-MeO-PCP in Sweden during the period March 2014 until June 2016.

    Case Descriptions: The non-fatal intoxication case, a 19-year-old male with drug problems and a medical history of depression, was found awake but tachycardic, hypertensive, tachypnoeic and catatonic at home. Read More

    Late onset MELAS with m.3243A > G mutation and its association with aneurysm formation.
    Metab Brain Dis 2017 Mar 21. Epub 2017 Mar 21.
    Department of Neurology, First Affiliated Hospital of China Medical University, No. 155 Nanjing North Street, Heping District, Shenyang, Liaoning, 110001, China.
    We reported a 53-year-old with late-onset mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) accompanied by aneurysm and large vessel dilations. Most studies have focused on microangiopathy causing stroke-like episodes. We report a case to describe large vessel involvement in clinical considerations, and possible mechanisms of aneurysm formation. Read More

    Lactic Acidosis: A Rare Oncological Emergency in Solid Tumors at Presentation.
    Am J Med Sci 2017 Apr 6;353(4):402-406. Epub 2016 May 6.
    Department of Hematology and Oncology, Lehigh Valley Health Network, John and Dorothy Morgan Cancer Center, Allentown, Pennsylvania.
    Lactic acidosis is a potentially life-threatening complication characterized by accumulation of blood lactate resulting in low arterial pH. The majority of lactic acidosis in malignancies are reported in association with hematologic malignancies. It may result from an imbalance between lactate production and hepatic lactate utilization, but the exact pathophysiology is far more complex than what we can fathom from current micromolecular studies. Read More

    Serum osmolal gap in clinical practice: usefulness and limitations.
    Postgrad Med 2017 May 23;129(4):456-459. Epub 2017 Mar 23.
    a Department of Internal Medicine, School of Medicine , University of Ioannina , Ioannina , Greece.
    Background: Although serum osmolal gap can be a useful diagnostic tool, clinicians are not familiar with its use in clinical practice.

    Objectives: The review presents in a series of questions-answers and under a clinical point of view the current data regarding the use of osmolal gap.

    Discussion: The definition and the best formula used for the calculation of osmolal gap, the main causes of increased osmolal gap with or without increased anion gap metabolic acidosis, as well as the role of concurrent lactic acidosis or ketoacidosis are presented under a clinical point of view. Read More

    Severe Anaphylactic Reaction to Preoperative Aspiration Prophylaxis.
    J Coll Physicians Surg Pak 2017 03;27(3):S2-S3
    Deparment of Anaesthesia, The Aga Khan University Hospital, Karachi.
    It is a case of severe anaphylactic reaction in a young female who had received aspiration prophylaxis preoperatively. Patient required intensive care and developed severe lactic acidosis. She responded very well to fluid and phenylephrine boluses and later on norepinephrine infusion. Read More

    N-Acetylcysteine's Role in Sepsis and Potential Benefit in Patients With Microcirculatory Derangements.
    J Intensive Care Med 2017 Jan 1:885066617696850. Epub 2017 Jan 1.
    1 Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Florida College of Medicine, Gainesville, FL, USA.
    Objective: To review the data surrounding the utility of N-acetylcysteine (NAC) in sepsis and identify areas needed for additional research.

    Data Sources: A review of articles describing the mechanisms of action and clinical use of NAC in sepsis.

    Summary Of Review: Despite many advances in critical care medicine, still as many as 50% of patients with septic shock die. Read More

    AIFM1 mutation presenting with fatal encephalomyopathy and mitochondrial disease in an infant.
    Cold Spring Harb Mol Case Stud 2017 Mar;3(2):a001560
    Division of Newborn Medicine, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
    Apoptosis-inducing factor mitochondrion-associated 1 (AIFM1), encoded by the gene AIFM1, has roles in electron transport, apoptosis, ferredoxin metabolism, reactive oxygen species generation, and immune system regulation. Here we describe a patient with a novel AIFM1 variant presenting unusually early in life with mitochondrial disease, rapid deterioration, and death. Autopsy, at the age of 4 mo, revealed features of mitochondrial encephalopathy, myopathy, and involvement of peripheral nerves with axonal degeneration. Read More

    Multiple myeloma associated with an Evan's syndrome.
    Pan Afr Med J 2016 1;25:127. Epub 2016 Nov 1.
    Department of Hematology, Farhat Hached Hospital, Sousse Tunisia.
    Auto-immun events are rare in multiple myeloma (MM). Here, we report one MM case complicated by Evans syndrome (Autoimmun hemolytic anemia (AIHA) associated with thrombocytopenia). A 52-year-old man was admitted in nephrology department with severe anemia, renal insufficiency and hypergamma globulinemia. Read More

    Effectiveness of isosorbide dinitrate in cyanide poisoning as a function of the administration timing.
    BMC Pharmacol Toxicol 2017 Mar 14;18(1):13. Epub 2017 Mar 14.
    Institute for Research in Military Medicine, Faculty of Medicine, Hebrew University, Jerusalem, Israel.
    Background: Better and safer antidotes against cyanide poisoning are needed. Prior study has shown a favorable effect of isosorbide dinitrate (ISDN) on the survival of cyanide-poisoned rabbits when administered as early as 1 min after poisoning. The aim of the current study was to further evaluate the efficacy of intravenous ISDN administered in clinically relevant timing for first responders. Read More

    Kidney involvement in MELAS syndrome: Description of 2 cases.
    Med Clin (Barc) 2017 Apr 7;148(8):357-361. Epub 2017 Mar 7.
    Servei de Nefrologia i Trasplantament Renal, Hospital Clínic, Universidad de Barcelona, Barcelona, España. Electronic address:
    Introduction: MELAS syndrome -myopathy, encephalopathy, lactic acidosis and stroke-like episodes- is a maternally-inherited mitochondrial cytopathy related to several mitochondrial DNA mutations, with the A3243G mutation in tRNA(Leu) gene being the most frequent of them.

    Patients And Methods: Apart from its typical symptomatology, patients usually exhibit a maternally-inherited history of neurosensory deafness and insulin-dependent type 2 diabetes mellitus (T2DM). Recent studies have shown that few patients carrying a A3243G mutation also suffer from renal dysfunction, usually in form of focal segmental glomerulosclerosis (FSGS). Read More

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