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    1 OF 157

    Treatment of Metformin Intoxication Complicated by Lactic Acidosis and Acute Kidney Injury: The Role of Prolonged Intermittent Hemodialysis.
    Am J Kidney Dis 2017 Feb 17. Epub 2017 Feb 17.
    Acute and Chronic Renal Failure Unit, Department of Clinical and Experimental Medicine, University of Parma, Parma, Italy.
    Metformin intoxication with lactic acidosis, a potentially lethal condition, may develop in diabetic patients when the drug dose is inappropriate and/or its clearance is reduced. Diagnosis and therapy may be delayed due to nonspecific symptoms at presentation, with severe anion gap metabolic acidosis and elevated serum creatinine values being the most prominent laboratory findings. Confirmation requires measurement of serum metformin by high-performance liquid chromatography-tandem mass spectrometry, but this technique is available only at specialized institutions and cannot be relied on as a guide to immediate treatment. Read More

    Lymphoma and Lactic Acidosis.
    Clin Nucl Med 2017 Feb 17. Epub 2017 Feb 17.
    From the Department of Radiology and Radiological Sciences, Vanderbilt University Medical Center, Nashville, TN.
    A 39-year-old man presented with new onset of sinus congestion, shortness of breath, and diaphoresis. His laboratory tests were notable for hypercalcemia and lactic acidosis. A CT scan of the head demonstrated mild paranasal disease. Read More

    The Dramatic Recovery of a Patient with Biguanide-associated Severe Lactic Acidosis Following Thiamine Supplementation.
    Intern Med 2017 15;56(4):455-459. Epub 2017 Feb 15.
    Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Japan.
    Biguanides are a drug of choice for the treatment of type 2 diabetes mellitus. Although they can cause lactic acidosis in susceptible patients with predisposing risk factors, the incidence of lactic acidosis is reported to be very low when they are used properly. We herein present a case of biguanide-associated severe lactic acidosis complicated with thiamine deficiency that was provoked without predisposing factors for thiamine deficiency. Read More

    [MELAS: Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-Like Episodes].
    Brain Nerve 2017 Feb;69(2):111-117
    Department of Neurology, Showa University School of Medicine.
    Mitochondrial disease is caused by a deficiency in the energy supply to cells due to mitochondrial dysfunction. Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) is a mitochondrial disease that presents with stroke-like episodes such as acute onset of neurological deficits and characteristic imaging findings. Stroke-like episodes in MELAS have the following features: 1) neurological deficits due to localization of lesions in the brain, 2) episodes often accompany epilepsy, 3) lesions do not follow the vascular supply area, 4) lesions are more often seen in the posterior brain than in the anterior brain, 5) lesions spread to an adjacent area in the brain, and 6) neurological symptoms often disappear together with imaging findings, but later relapse. Read More

    Lethal neonatal case and review of primary short-chain enoyl-CoA hydratase (SCEH) deficiency associated with secondary lymphocyte pyruvate dehydrogenase complex (PDC) deficiency.
    Mol Genet Metab 2017 Feb 2. Epub 2017 Feb 2.
    Departments of Clinical Chemistry and Pediatrics, Laboratory Genetic Metabolic Diseases, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands.
    Mutations in ECHS1 result in short-chain enoyl-CoA hydratase (SCEH) deficiency which mainly affects the catabolism of various amino acids, particularly valine. We describe a case compound heterozygous for ECHS1 mutations c.836T>C (novel) and c. Read More

    Antiretroviral therapy related adverse effects: Can sub-Saharan Africa cope with the new "test and treat" policy of the World Health Organization?
    Infect Dis Poverty 2017 Feb 15;6(1):24. Epub 2017 Feb 15.
    Department of Epidemiology and Public Health, Centre Pasteur of Cameroon, Yaoundé, Cameroon.
    Background: Recent studies have shown that early antiretroviral therapy (ART) initiation results in significant HIV transmission reduction. This is the rationale behind the "test and treat" policy of the World Health Organization (WHO). Implementation of this policy will lead to an increased incidence of ART-related adverse effects, especially in sub-Saharan Africa (SSA). Read More

    Defects in RNA metabolism in mitochondrial disease.
    Int J Biochem Cell Biol 2017 Feb 8. Epub 2017 Feb 8.
    Harry Perkins Institute of Medical Research and Centre for Medical Research, Level 7 QQ Block, QEII Medical Centre, 6 Verdun Street, Nedlands, WA 6009, Australia; School of Chemistry and Biochemistry, The University of Western Australia, Nedlands, Western Australia 6009, Australia. Electronic address:
    The expression of mitochondrially-encoded genes requires the efficient processing of long precursor RNAs at the 5' and 3' ends of tRNAs, a process which, when disrupted, results in disease. Two such mutations reside within mt-tRNA(Leu(UUR)); a m.3243A > G transition, which is the most common cause of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes), and m. Read More

    Metformin-associated lactic acidosis treated with continuous renal replacement therapy.
    BMJ Case Rep 2017 Feb 10;2017. Epub 2017 Feb 10.
    Department of Hematology and Oncology, Mie University Graduate School of Medicine, Tsu, Japan.
    Metformin-associated lactic acidosis (MALA) is a rare but life-threatening complication. We report a case of MALA in a man aged 71 years who was treated with continuous renal replacement therapy (CRRT). The patient was brought to the hospital for prolonged and gradual worsening gastrointestinal symptoms. Read More

    Lactic Acidosis with Chloramphenicol Treatment in a Child with Cystic Fibrosis.
    J Popul Ther Clin Pharmacol 2017 Jan 30;24(1):40-45. Epub 2017 Jan 30.
    Department of Pediatrics, University of Montreal.
    Children with cystic fibrosis are commonly colonized with multi-resistant bacteria. In such patients, infectious exacerbation may require salvage therapy with uncommonly used antimicrobials, including chloramphenicol. Chloramphenicol is rarely used nowadays because of the associated severe adverse events. Read More

    [Hemorrhagic shock : General principles].
    Internist (Berl) 2017 Feb 9. Epub 2017 Feb 9.
    Klinik für Intensivmedizin, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland.
    Bleeding associated with hemorrhagic shock is often seen in emergency medical services or in the intensive care unit. Identifying the origin of the bleeding and additional disorders helps to determine the degree of the hemorrhagic shock. In order to be effective, the initial therapy until blood products are available needs to be differentiated to be effective in terms of hemodynamic stabilization and coagulation. Read More

    A 66-Year-Old Woman With Sudden Onset of Disseminated Intravascular Coagulation, Lactic Acidosis, and Hypoglycemia.
    Chest 2017 Feb;151(2):e41-e44
    Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, Department of Medicine, University of Minnesota, Minneapolis, MN.
    A 66-year-old woman presented to an urgent care clinic for 2 to 3 weeks of general malaise, nausea/vomiting, night sweats, and dyspnea. On examination, she was tachycardic, and her laboratory evaluation was normal except for a lactate level of 4.4 mmol/L and platelet count of 118 × 10(9)/L. Read More

    A Mechanism-based Pharmacokinetic Enzyme Turnover Model for Dichloroacetic Acid Auto-Inhibition in Rats.
    J Pharm Sci 2017 Feb 2. Epub 2017 Feb 2.
    Division of Pharmaceutics and Translational Therapeutics, College of Pharmacy, University of Iowa, IA. Electronic address:
    Dichloroacetic acid (DCA), a halogenated organic acid, is a pyruvate dehydrogenase kinase (PDK) inhibitor that has been used to treat congenital or acquired lactic acidosis and is currently in early phase clinical trials for cancer treatment. DCA was found to inhibit its own metabolism by irreversibly inactivating glutathione transferase zeta 1 (GSTZ1-1), resulting in non-linear kinetics and abnormally high accumulation ratio after repeated dosing. In this analysis, a semi-mechanistic pharmacokinetic-enzyme turn over model was developed for the first time to capture DCA auto-inhibition, gastrointestinal region-dependent absorption, and time dependent change in bioavailability in rats. Read More

    Factors Associated With Mortality in Continuous Renal Replacement Therapy for Pediatric Patients With Acute Kidney Injury.
    Pediatr Crit Care Med 2017 Feb;18(2):e56-e61
    All authors: Division of Pediatric Critical Care Medicine, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Songpa-gu, Seoul, Republic of Korea.
    Objectives: To analyze the epidemiology of pediatric acute kidney injury requiring continuous renal replacement therapy and identify prognostic factors affecting mortality rates.

    Design: Retrospective analysis.

    Setting: PICU of a tertiary medical center. Read More

    Further delineation of a rare recessive encephalomyopathy linked to mutations in GFER thanks to Data Sharing of whole exome sequencing data.
    Clin Genet 2017 Feb 2. Epub 2017 Feb 2.
    Centre de Génétique et Centre de référence «Anomalies du Développement et Syndromes Malformatifs», Hôpital d'Enfants, Centre Hospitalier Universitaire de Dijon, Dijon, France.
    Alterations in GFER gene have been associated with progressive mitochondrial myopathy, congenital cataracts, hearing loss, developmental delay, lactic acidosis and respiratory chain deficiency in 3 siblings born to consanguineous Moroccan parents by homozygosity mapping and candidate gene approach (OMIM#613076). Next generation sequencing recently confirmed this association by the finding of compound heterozygous variants in 19-year-old girl with a strikingly similar phenotype, but this ultra-rare entity remains however unknown from most of the scientific community. Whole exome sequencing was performed as part of a "diagnostic odyssey" for suspected mitochondrial condition in two patients, presenting congenital cataracts, progressive encephalomyopathy and hypotrophy and detected unreported compound heterozygous variants in GFER. Read More

    Metformin-SGLT2, Dehydration, and Acidosis Potential.
    J Am Geriatr Soc 2017 Feb 2. Epub 2017 Feb 2.
    Kidney and Hypertension Section, Joslin Diabetes Center, Harvard Medical School, Boston, Massachusetts.
    Clinicians are aware of the risks and benefits of metformin in type 2 diabetes. In geriatric populations lactic acidosis due to diminishing kidney function is an issue. The recent addition of a group of agents to control diabetes through increased glycosuria may increase the risk of dehydration and acidosis. Read More

    A case of rapid progression of postoperative hyperthermia: Dantrolene or not dilemma?
    Int J Crit Illn Inj Sci 2016 Oct-Dec;6(4):203-205
    Department of Anesthesiology and Pain Medicine, Seattle Children's Hospital, Seattle, WA 98105, USA.
    Malignant hyperthermia (MH) is an extremely rare and life-threatening differential diagnosis of postoperative fever. We present an 8-month-old child scheduled for elective outpatient procedure who rapidly developed high fever, tachycardia, and respiratory acidosis shortly after transfer to the postanesthesia care unit. MH hotline expert recommended administering dantrolene, but there was no evidence of hypermetabolism or lactic acidosis. Read More

    A profile of metabolic acidosis in patients with sepsis in an Intensive Care Unit setting.
    Int J Crit Illn Inj Sci 2016 Oct-Dec;6(4):178-181
    Department of Internal Medicine, Amrita Institute of Medical Sciences, Kochi, Kerala, India.
    Context: Metabolic acidosis is frequently found in patients with severe sepsis. An understanding of types of acidosis in sepsis and their evolution over the course of treatment may give us insight into the behavior of acid-base balance in these patients.

    Aims: To describe at Intensive Care Unit (ICU) admission and over the first 5 days the composition of metabolic acidosis in patients with sepsis and to evaluate and compare acidosis patterns in survivors and nonsurvivors. Read More

    Differences in Physiological Responses to Interval Training in Cyclists With and Without Interval Training Experience.
    J Hum Kinet 2016 Apr 13;50:93-101. Epub 2016 Apr 13.
    University School of Physical Education in Wroclaw, Department of Physiology and Biochemistry, Poland.
    The aim of this study was to determine differences in glycolytic metabolite concentrations and work output in response to an all-out interval training session in 23 cyclists with at least 2 years of interval training experience (E) and those inexperienced (IE) in this form of training. The intervention involved subsequent sets of maximal intensity exercise on a cycle ergometer. Each set comprised four 30 s repetitions interspersed with 90 s recovery periods; sets were repeated when blood pH returned to 7. Read More

    [Lactic acidosis due to metformin accumulation complicating acute gastroenteritis].
    Klin Mikrobiol Infekc Lek 2016 Dec;22(4):144-147
    Department of infectious diseases, 3rd faculty of medicine Charles University, Na Bulovce Hospital, Prague, Czech Republic, e-mail:
    Lactic acidosis is the most severe adverse effect associated with metformin therapy of type 2 diabetes mellitus. The risk increases in patients with impaired renal function, most commonly due to diabetic nephropathy, and may be augmented when concurrent medication with a negative impact on renal function is used. The authors present a series of three patients who were admitted to a department of infectious diseases for acute gastroenteritis and within a few hours developed shock syndrome caused by severe lactic acidosis due to accumulation of metformin. Read More

    Metformin-Associated Lactic Acidosis: An Atypical Presentation.
    Adv Emerg Nurs J 2017 Jan/Mar;39(1):26-30
    Department of Emergency Medi-cine, North Shore University Health Systems, Evanston, Illinois.
    Many Type 2 diabetic patients take metformin for its safety profile and lack of hypoglycemia. Although this drug is safe in those without renal dysfunction, lactic acidosis may rarely occur. Metformin-associated lactic acidosis is a potentially fatal yet rare diagnosis. Read More

    Identification of miRNA, lncRNA and mRNA-associated ceRNA networks and potential biomarker for MELAS with mitochondrial DNA A3243G mutation.
    Sci Rep 2017 Jan 31;7:41639. Epub 2017 Jan 31.
    Laboratory of Neuromuscular Disorders and Department of Neurology, Qilu Hospital, Shandong University, Jinan, China.
    Researchers in the field of mitochondrial biology are increasingly unveiling of the complex mechanisms between mitochondrial dysfunction and noncoding RNAs (ncRNAs). However, roles of ncRNAs underlying mitochondrial myopathy remain unexplored. The aim of this study was to elucidate the regulating networks of dysregulated ncRNAs in Mitochondrial myopathy, Encephalopathy, Lactic Acidosis, and Stroke-like episodes (MELAS) with mitochondrial DNA (mtDNA) A3243G mutation, which might make contributions to the unveiling of the complex mechanisms underlying mitochondrial myopathy and, possibly, new tools applicable to clinical practice. Read More

    Bilateral thigh methicillin-resistant Staphylococcus aureus necrotising fasciitis in a man with newly diagnosed Human Immunodeficiency Virus (HIV).
    BMJ Case Rep 2017 Jan 30;2017. Epub 2017 Jan 30.
    Department of Pulmonary, Critical Care, and Sleep Disorders Medicine, University of Louisville School of Medicine, Louisville, Kentucky, USA.
    Methicillin-resistant Staphylococcus aureus (MRSA) necrotising fasciitis (NF) is rare and constitutes a medical and surgical emergency. We report a case of a 53-year-old man with type-2 diabetes mellitus and newly diagnosed HIV infection who presented with 1 week of progressively worsening bilateral sharp thigh pain. On presentation, he was febrile, tachycardic and had bilateral thigh erythema and mild tenderness without open wounds. Read More

    [Metformin-associated lactic acidosis and acute kidney injury].
    G Ital Nefrol 2016 Nov-Dec;33(6)
    Metformin is recommended as the treatment of choice in patients with type 2 diabetes mellitus because of its efficacy, general tolerability and low cost. Recent guidelines have extended the use of metformin to patients with Chronic Kidney Disease (CKD) up to stage III. However, in the recent literature, cases of MALA (metformin-associated lactic acidosis) are increasingly reported. Read More

    [Metabolic acidosis].
    G Ital Nefrol 2016 Nov-Dec;33(6)
    Metabolic acidosis is frequently observed in clinical practice, especially among critically ill patients and/or in the course of renal failure. Complex mechanisms are involved, in most cases identifiable by medical history, pathophysiology-based diagnostic reasoning and measure of some key acid-base parameters that are easily available or calculable. On this basis the bedside differential diagnosis of metabolic acidosis should be started from the identification of the two main subtypes of metabolic acidosis: the high anion gap metabolic acidosis and the normal anion gap (or hyperchloremic) metabolic acidosis. Read More

    Heat stroke leading to acute liver injury & failure: A case series from the Acute Liver Failure Study Group.
    Liver Int 2017 Jan 27. Epub 2017 Jan 27.
    Division of Digestive and Liver Diseases, UT Southwestern Medical Center, Dallas, TX, USA.
    Background & Aims: In the United States, nearly 1000 annual cases of heat stroke are reported but the frequency and outcome of severe liver injury in such patients is not well described. The aim of this study was to describe cases of acute liver injury (ALI) or failure (ALF) caused by heat stroke in a large ALF registry.

    Methods: Amongst 2675 consecutive subjects enrolled in a prospective observational cohort of patients with ALI or ALF between January 1998 and April 2015, there were eight subjects with heat stroke. Read More

    B-cell lymphoma, thiamine deficiency, and lactic acidosis.
    Proc (Bayl Univ Med Cent) 2017 Jan;30(1):69-70
    Department of Internal Medicine, State University of New York Upstate Medical University, Syracuse, New York.
    Type B lactic acidosis is found in the absence of tissue hypoperfusion, can be associated with malignancies, and can be caused by thiamine deficiency. We present a patient who presented with an abdominal mass that biopsy disclosed to be a diffuse large B-cell lymphoma. Because thiamine deficiency is a rare cause of lactic acidosis in cancer, the patient was treated with intravenous thiamine with rapid normalization of the lactic acid level. Read More

    MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis, Stroke) - a Diagnosis Not to be Missed.
    Ir Med J 2016 Sep 9;109(8):455. Epub 2016 Sep 9.
    Departments of Paediatrics, Neurology and Radiology, Childrens University Hospital, Temple St, Dublin 1.
    MELAS is a rare mitochondrial disorder. We report two cases in Irish males where the characteristics were evident, but the diagnosis not made for a considerable period of time. In one of the cases the symptoms were presumed secondary to prematurity. Read More

    Association between Metformin Use and Risk of Lactic Acidosis or Elevated Lactate Concentration in Type 2 Diabetes.
    Yonsei Med J 2017 Mar;58(2):312-318
    Division of Endocrinology and Metabolism, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
    Purpose: Metformin can reduce diabetes-related complications and mortality. However, its use is limited because of potential lactic acidosis-associated adverse effects, particularly in renal impairment patients. We aimed to investigate the association of metformin use with lactic acidosis and hyperlactatemia in patients with type 2 diabetes. Read More

    [A regenerative anemia in infants: 2 cases of Pearson´s syndrome].
    Arch Argent Pediatr 2017 02;115(1):e24-e27
    Unidad de Oncohematología Pediátrica, Servicio de Pediatría, Hospital Universitario Miguel Servet, Zaragoza, España.
    Anemia is very common in infants. Although its causes are usually not severe and treatable, proper etiologic diagnosis should be established. When anemia is non-regenerative, it can be caused by aplastic anemia, myelodysplastic syndrome, bone marrow infiltration or hematopoietic factors deficiencies. Read More

    The midpoint between ventilatory thresholds approaches maximal lactate steady state intensity in amateur cyclists.
    Biol Sport 2016 Dec 10;33(4):373-380. Epub 2016 Oct 10.
    Department of Health and Human Performance, Technical University of Madrid, Madrid, Spain.
    The aim was to determine whether the midpoint between ventilatory thresholds (MPVT) corresponds to maximal lactate steady state (MLSS). Twelve amateur cyclists (21.0 ± 2. Read More

    Multi-modality imaging to assess metabolic response to dichloroacetate treatment in tumor models.
    Oncotarget 2016 Dec;7(49):81741-81749
    Biomedical Magnetic Resonance Research Group, Louvain Drug Research Institute, Université catholique de Louvain, Brussels, Belgium.
    Reverting glycolytic metabolism is an attractive strategy for cancer therapy as upregulated glycolysis is a hallmark in various cancers. Dichloroacetate (DCA), long used to treat lactic acidosis in various pathologies, has emerged as a promising anti-cancer drug. By inhibiting the pyruvate dehydrogenase kinase, DCA reactivates the mitochondrial function and decreases the glycolytic flux in tumor cells resulting in cell cycle arrest and apoptosis. Read More

    Gut Microbes manuscript type: Commentary and views submission probiotics, D-Lactic acidosis, oxidative stress and strain specificity.
    Gut Microbes 2017 Jan 12:1-12. Epub 2017 Jan 12.
    b Medlab Clinical Ltd , Sydney , New South Wales , Australia.
    The existence of an implicit living microscopic world, composed primarily of bacteria, has been known for centuries. The exact mechanisms that govern the contribution of bacteria to human health and disease have only recently become the subject of intense research efforts. Within this very evident shift in paradigms, the rational design of probiotic formulations has led to the creation of an industry that seeks to progress the engineering of probiotic bacteria that produce metabolites that may enhance human host health and prevent disease. Read More

    The role of bicarbonate precursors in balanced fluids during haemorrhagic shock with and without compromised liver function.
    Br J Anaesth 2016 Oct;117(4):521-528
    Department of Translational Physiology, Academic Medical Centre, Amsterdam, The Netherlands
    Background: Lactate, acetate, and gluconate are anions used in balanced resuscitation fluids, of which lactate and acetate are considered bicarbonate precursors. This study investigated the role of the liver in the ability of balanced and unbalanced solutions to correct acid-base alterations and renal haemodynamics and microvascular oxygenation in a rat model of resuscitated haemorrhagic shock.

    Methods: Ringer's lactate, Ringer's acetate, PlasmaLyte, or normal saline were administered following haemorrhagic shock in the presence or absence of a 70% partial liver resection. Read More

    Use of Metformin for Cardiometabolic Risks in Psychiatric Practice: Need-to-Know Safety Issues.
    J Clin Psychiatry 2016 Nov;77(11):e1491-e1494
    Department of Psychopharmacology, National Institute of Mental Health and Neurosciences, Bangalore, India.
    Metformin, a biguanide drug, is emerging as an important treatment option for the prevention or treatment of weight gain, type 2 diabetes mellitus, and the metabolic syndrome in psychiatric patients, especially those who require or receive antipsychotic drugs. Metformin treatment is commonly associated with gastrointestinal adverse effects; the risk of these is reduced by gradual dose uptitration, administration of the drug with meals, and use of a time-release formulation. Lactic acidosis, a potentially fatal complication of biguanide therapy, is very rare with metformin; the risk can be reduced by avoidance of its prescription in patients with impaired renal function, impaired liver function, cardiac failure, and certain other conditions. Read More

    Barth Syndrome: Connecting Cardiolipin to Cardiomyopathy.
    Lipids 2017 Feb 9;52(2):99-108. Epub 2017 Jan 9.
    Children's Hospital Oakland Research Institute, 5700 Martin Luther King Jr. Way, Oakland, CA, 94609, USA.
    The Barth syndrome (BTHS) is caused by an inborn error of metabolism that manifests characteristic phenotypic features including altered mitochondrial membrane phospholipids, lactic acidosis, organic acid-uria, skeletal muscle weakness and cardiomyopathy. The underlying cause of BTHS has been definitively traced to mutations in the tafazzin (TAZ) gene locus on chromosome X. TAZ encodes a phospholipid transacylase that promotes cardiolipin acyl chain remodeling. Read More

    An atypical presentation of ACAD9 deficiency: Diagnosis by whole exome sequencing broadens the phenotypic spectrum and alters treatment approach.
    Mol Genet Metab Rep 2017 Mar 29;10:38-44. Epub 2016 Dec 29.
    Phoenix Children's Hospital, Division of Genetics and Metabolism, United States; Phoenix Children's Hospital Rosenberg Children's Medical Building 1920 E. Cambridge Ave Ste 301 Phoenix, AZ 85006, United States.
    Acyl-CoA dehydrogenase 9 (ACAD9), linked to chromosome 3q21.3, is one of a family of multimeric mitochondrial flavoenzymes that catalyze the degradation of fatty acyl-CoA from the carnitine shuttle via β-oxidation (He et al. 2007). Read More

    Mitochondrial iron-sulfur cluster biogenesis from molecular understanding to clinical disease.
    Neurosciences (Riyadh) 2017 Jan;22(1):4-13
    Division of Genetics, Department of Pediatrics, King Saud bin Abdulaziz University for Health Sciences, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia.
    Iron_sulfur clusters (ISCs) are known to play a major role in various protein functions. Located in the mitochondria, cytosol, endoplasmic reticulum and nucleus, they contribute to various core cellular functions. Until recently, only a few human diseases related to mitochondrial ISC biogenesis defects have been described. Read More

    Enterolobium contortisiliquum is a cause of acute ruminal acidosis in sheep.
    Toxicon 2017 Feb 3;126:90-95. Epub 2017 Jan 3.
    FAMEZ, UFMS, Campo Grande, MS, Brazil. Electronic address:
    The ingestion of pods of Enterolobium contortisiliquum is associated with digestive disturbances, photosensitivity and abortion in domestic ruminants. This experiment was designed to test the hypothesis that digestive disturbances in this toxicosis are really caused by acute ruminal acidosis. Three sheep fed large doses (10-15 g/kg/body weight [bw]) of E. Read More

    Effect of biochanin A on corn grain (Zea mays) fermentation by bovine rumen amylolytic bacteria.
    J Appl Microbiol 2017 Jan 5. Epub 2017 Jan 5.
    Forage-Animal Production Research Unit, USDA, Agricultural Research Service, Lexington, KY, USA.
    Aims: The objective was to determine the effect of biochanin A (BCA), an isoflavone produced by red clover (Trifolium pratense L.), on corn fermentation by rumen micro-organisms.

    Methods And Results: When bovine rumen bacterial cell suspensions (n = 3) were incubated (24 h, 39°C) with ground corn, amylolytic bacteria including group D Gram-positive cocci (GPC; Streptococcus bovis; enterococci) proliferated, cellulolytic bacteria were inhibited, lactate accumulated and pH declined. Read More

    Adult-onset Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke (MELAS)-like Encephalopathy Diagnosed Based on the Complete Sequencing of Mitochondrial DNA Extracted from Biopsied Muscle without any Myopathic Changes.
    Intern Med 2017;56(1):95-99. Epub 2017 Jan 1.
    Department of Neurology, Tokai University School of Medicine, Japan.
    The clinical features of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) are not uniform. We herein report a male patient with unusual MELAS-like encephalopathy who had been experiencing isolated recurrent stroke-like episodes since he was 33 years old without any particular family history. Despite an extensive investigation, he had no other signs suggestive of MELAS. Read More

    Viekira Pak Induced Fatal Lactic Acidosis: A Case Report of an Unusual Side Effect.
    Case Reports Hepatol 2016 1;2016:8627139. Epub 2016 Dec 1.
    Department of Medicine, Division of Gastroenterology, Bronx Lebanon Hospital Center, 1650 Selwyn Avenue, Suite No. 10C, Bronx, NY 10457, USA.
    Viekira Pak is a new direct-acting antiviral agent that has an excellent efficacy in treating patients with chronic HCV. FDA released a safety warning that Viekira Pak can cause serious liver injury mostly in patients with underlying advanced liver disease. We report the first case of fatal lactic acidosis presenting 3 days after initiating therapy with Viekira Pak. Read More

    Mutations in the accessory subunit NDUFB10 result in isolated complex I deficiency and illustrate the critical role of intermembrane space import for complex I holoenzyme assembly.
    Hum Mol Genet 2016 Dec 30. Epub 2016 Dec 30.
    Department of Chemistry, Institute of Biochemistry, University of Cologne, Cologne, Germany
    An infant presented with fatal infantile lactic acidosis and cardiomyopathy, and was found to have profoundly decreased activity of respiratory chain complex I in muscle, heart and liver. Exome sequencing revealed compound heterozygous mutations in NDUFB10, which encodes an accessory subunit located within the PD part of complex I. One mutation resulted in a premature stop codon and absent protein, while the second mutation replaced the highly conserved cysteine 107 with a serine residue. Read More

    A Quick Reference on High Anion Gap Metabolic Acidosis.
    Vet Clin North Am Small Anim Pract 2017 Mar 23;47(2):205-207. Epub 2016 Dec 23.
    Department of Clinical Sciences, College of Veterinary Medicine, Oregon State University, 700 Southwest 30th Street, Corvallis, OR 97331, USA. Electronic address:
    High anion gap (AG) metabolic acidoses can be identified by a decrease in pH, decrease in HCO3(-) or base excess, and an increased AG. The AG represents the difference between unmeasured cations and unmeasured anions; it increases secondary to the accumulation of anions other than bicarbonate and chloride. The most common causes of high AG acidosis are renal failure, diabetic ketoacidosis, and lactic acidosis. Read More

    Mitochondrial respiratory chain complex I deficiency causes intractable gastrointestinal symptoms.
    Pediatr Int 2016 Dec 6;58(12):1337-1340. Epub 2016 Nov 6.
    Division of Perinatology and Pediatrics, Faculty of Medicine, Tottori University, Yonago, Tottori, Japan.
    We report the case of a 13-month-old girl with frequent vomiting, intractable diarrhea, hyperlactatemia, and liver dysfunction. Although the symptoms were treatment resistant, enteral nutrition formula containing medium-chain triglycerides reduced the weight loss, vomiting, and diarrhea. Immunostaining of mitochondrial respiratory chain (MRC) complexes of the colonic mucosa confirmed the diagnosis of MRC complex I deficiency. Read More

    Graphene nanoflakes on transparent glass electrode sensor for electrochemical sensing of anti-diabetic drug.
    Bioprocess Biosyst Eng 2016 Dec 19. Epub 2016 Dec 19.
    Amity Institute of Nanotechnology (AINT), AMITY University, Noida, 201313, Uttar Pradesh, India.
    Metformin (Mf) plays a major role in controlling insulin level of individuals at risk of developing diabetes mellitus. Overdose of Mf can cause lactic acidosis, diarrhoea, cough, or hoarseness, etc. These particulars point out the identification for selective and sensitive methods of Mf determination. Read More

    Diagnosis of LCHAD/TFP deficiency in an at risk newborn using umbilical cord blood acylcarnitine analysis.
    Mol Genet Metab Rep 2017 Mar 9;10:8-10. Epub 2016 Dec 9.
    Genetics and Metabolism, Children's National Health System, Washington, DC,USA.
    Trifunctional protein deficiency/Long-chain hydroxyacyl-CoA dehydrogenase deficiency (LCHAD/TFP) deficiency is a disorder of fatty acid oxidation and ketogenesis. Severe neonatal lactic acidosis, cardiomyopathy, and hepatic dysfunction are caused by the accumulation of toxic long-chain acylcarnitines. The feasibility of umbilical cord blood use in screening for acylcarnitine analysis and free carnitine has been hypothesized but not reported in LCHAD/TFP neonates. Read More

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