8,502 results match your criteria Lactic Acidosis


Thiamine and Difficulties in Differentiating Type A From B Lactic Acidosis.

Crit Care Med 2019 May;47(5):e434-e435

Division of Pulmonary & Critical Care Medicine, Department of Medicine, Medical College of Wisconsin, Milwaukee, WI.

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http://dx.doi.org/10.1097/CCM.0000000000003684DOI Listing

Understanding Lactatemia in Human Sepsis: Potential Impact for Early Management.

Am J Respir Crit Care Med 2019 Apr 15. Epub 2019 Apr 15.

Regions Hospital, St. Paul, Minnesota, United States.

Rationale: Hyperlactatemia in sepsis may derive from a prevalent impairment of oxygen supply/demand and/or oxygen utilization. Discriminating between these two mechanisms may be relevant for the early fluid resuscitation strategy.

Objectives: Understanding the relationship between central venous oxygen saturation (ScvO2), lactate and base excess to better determine the origin of lactate. Read More

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http://dx.doi.org/10.1164/rccm.201812-2342OCDOI Listing

Flavin adenine dinucleotide synthase deficiency due to FLAD1 mutation presenting as multiple acyl-CoA dehydrogenation deficiency-like disease: A case report.

Brain Dev 2019 Apr 11. Epub 2019 Apr 11.

Department of Pediatrics, Shimane University, Faculty of Medicine, Izumo, Shimane, Japan.

Multiple acyl-CoA dehydrogenase deficiency (MADD), also known as glutaric acidemia type II, is classically caused by a congenital defect in electron transfer flavoprotein (ETF) or ETF dehydrogenase (ETFDH). Flavin adenine dinucleotide synthase (FADS) deficiency caused by mutations in FLAD1 was recently reported as a novel riboflavin metabolism disorder resembling MADD. Here, we describe a Japanese boy with FADS deficiency due to a novel mutation (p. Read More

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http://dx.doi.org/10.1016/j.braindev.2019.04.002DOI Listing

Autopsied case with MERRF/MELAS overlap syndrome accompanied by stroke-like episodes localized to the precentral gyrus.

Neuropathology 2019 Apr 10. Epub 2019 Apr 10.

Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan.

We present an autopsied case with A8344G-mutated myoclonus epilepsy with ragged red fibers (MERRF)/mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) overlap syndrome accompanied by stroke-like episodes localized to the precentral gyrus. A 16-year-old Japanese woman suddenly experienced repetitive consciousness disturbances with increased serum lactate and creatine kinase levels. Magnetic resonance imaging showed abnormal intensity of bilateral precentral gyrus. Read More

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http://dx.doi.org/10.1111/neup.12551DOI Listing
April 2019
1 Read

Incidence and risk factors for hyperlactatemia in ED patients with acute metformin overdose.

Am J Emerg Med 2019 Mar 23. Epub 2019 Mar 23.

Division of Medical Toxicology, Department of Emergency Medicine, The Icahn School of Medicine at Mount Sinai, Elmhurst Hospital Center, NY, New York, USA. Electronic address:

Introduction: The goals of this study are to describe clinical characteristics and risk factors for metabolic acidosis with hyperlactatemia in emergency department (ED) patients with acute metformin overdose.

Methods: This was a secondary analysis of data from a retrospective observational cohort of adult ED patients presenting with acute drug overdose at two tertiary care hospitals over 5 years. The primary outcomes were: (1) hyperlactatemia, defined as a lactate concentration ≥ 2 mmol/L at any point during hospital admission and, (2) metformin associated lactic acidosis (MALA), defined as a lactate concentration ≥ 5 mmol/L and pH <7. Read More

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http://dx.doi.org/10.1016/j.ajem.2019.03.033DOI Listing
March 2019
1 Read

Lactic acidosis due to metformin in type 2 diabetes mellitus and chronic kidney disease stage 3-5: is it significant?

Int Urol Nephrol 2019 Apr 8. Epub 2019 Apr 8.

Department of Statistics, Prasanna School of Public Health, MAHE, Udupi, India.

Purpose: To study the incidence of lactic acidosis due to metformin in patients with type 2 diabetes mellitus (T2DM) and chronic kidney disease (CKD) stage 3-5.

Methods: We estimated plasma lactate in patients of CKD stage 3 and worse who were continuing metformin on their own prior to stopping the drug.

Result: Of 40 patients included, median duration of T2DM was 60 months (interquartile range IQR 24-120). Read More

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http://link.springer.com/10.1007/s11255-019-02136-y
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http://dx.doi.org/10.1007/s11255-019-02136-yDOI Listing
April 2019
3 Reads

Quantitative Variation in m.3243A > G Mutation Produce Discrete Changes in Energy Metabolism.

Sci Rep 2019 Apr 8;9(1):5752. Epub 2019 Apr 8.

Fralin Biomedical Research Institute at Virginia Tech Carilion, Roanoke, VA, 24016, USA.

Mitochondrial DNA (mtDNA) 3243A > G tRNALeu heteroplasmic mutation (m.3243A > G) exhibits clinically heterogeneous phenotypes. While the high mtDNA heteroplasmy exceeding a critical threshold causes mitochondrial encephalomyopathy, lactic acidosis with stroke-like episodes (MELAS) syndrome, the low mtDNA heteroplasmy causes maternally inherited diabetes with or without deafness (MIDD) syndrome. Read More

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http://dx.doi.org/10.1038/s41598-019-42262-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6453956PMC
April 2019
3 Reads

[Ultrastructural and clinical findings of mitochondrial encephalomyopathy:report of 27 cases].

Zhonghua Bing Li Xue Za Zhi 2019 Apr;48(4):298-302

Beijing Neurosurgical Institute, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China.

To investigate the ultrastructural features of muscle in patients with mitochondrial encephalomyopathy for its diagnosis and differential diagnosis. The clinical data of 27 mitochondrial encephalomyopathy patients who underwent left or right biceps brachii muscle biopsy at Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University from July 2006 to August 2017 were analyzed retrospectively. The muscle biopsy specimens were examined underlight microscope and transmission electron microscope. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0529-5807.2019.04.007DOI Listing
April 2019
2 Reads

New Variant of MELAS Syndrome With Executive Dysfunction, Heteroplasmic Point Mutation in the Gene (m.12015T>C; p.Leu419Pro) and Comorbid Polyglandular Autoimmune Syndrome Type 2.

Front Immunol 2019 21;10:412. Epub 2019 Mar 21.

Section for Experimental Neuropsychiatry, Department of Psychiatry and Psychotherapy, Medical Center-University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Mitochondrial diseases are caused by dysfunctions in mitochondrial metabolic pathways. MELAS syndrome is one of the most frequent mitochondrial disorders; it is characterized by encephalopathy, myopathy, lactic acidosis, and stroke-like episodes. Typically, it is associated with a point mutation with an adenine-to-guanine transition at position 3243 of the mitochondrial DNA (mtDNA; m. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00412DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6437310PMC
March 2019
2 Reads

Acute necrotizing esophagitis presenting with severe lactic acidosis and shock.

Proc (Bayl Univ Med Cent) 2018 Oct 28;31(4):457-459. Epub 2018 Sep 28.

Department of Internal Medicine, Texas Tech University Health Sciences CenterLubbockTexas.

Acute necrotizing esophagitis, also known as "black esophagus," is typically characterized by a circumferential, friable black mucosal surface and preferentially involves the distal esophagus. It predominantly affects elderly men and presents as an upper gastrointestinal bleed. We describe a 60-year-old man with an acute upper gastrointestinal bleed and sepsis and subsequently acute necrotizing esophagitis. Read More

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http://dx.doi.org/10.1080/08998280.2018.1488494DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6413971PMC
October 2018
2 Reads

Mitochondrial metabolic stroke: Phenotype and genetics of stroke-like episodes.

Authors:
Josef Finsterer

J Neurol Sci 2019 Mar 23;400:135-141. Epub 2019 Mar 23.

Krankenanstalt Rudolfstiftung, Messerli Institute, Vienna, Austria. Electronic address:

Stroke-like episodes (SLEs) are the hallmark of mitochondrial encephalopathy with lactic acidosis and stroke-like episode (MELAS) syndrome but rarely occur also in other specific or nonspecific mitochondrial disorders. Pathophysiologically, SLLs are most likely due to a regional disruption of the blood-brain barrier triggered by the underlying metabolic defect, epileptic activity, drugs, or other factors. SLEs manifest clinically with a plethora of cerebral manifestations, which not only include features typically seen in ischemic stroke, but also headache, epilepsy, ataxia, visual impairment, vomiting, and psychiatric abnormalities. Read More

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http://dx.doi.org/10.1016/j.jns.2019.03.021DOI Listing
March 2019
12 Reads

The Lipodystrophy Syndrome in HIV-Infected Children under Antiretroviral Therapy: A First Report from the Central Africa.

Int J Pediatr 2019 3;2019:7013758. Epub 2019 Mar 3.

Division of Cardiology and Infectious Diseases, Department of Pediatrics, University Hospital of Kinshasa, Faculty of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of the Congo.

Background: Despite the high prevalence of the HIV/AIDS, few studies focused on the prevalence of lipodystrophy in pediatric HIV patients on antiretroviral therapy (ARV) in sub-Saharan African countries. The aim of this study was to assess the prevalence and to identify the risk factors of metabolic disorders related to ARV therapy in this population.

Methods: A cross-sectional study was completed in Kinshasa, the Democratic Republic of Congo. Read More

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https://www.hindawi.com/journals/ijpedi/2019/7013758/
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http://dx.doi.org/10.1155/2019/7013758DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6420969PMC
March 2019
3 Reads

A Challenging Case of Coexisting Type A and Type B Lactic Acidosis: A Case Report.

Cureus 2019 Jan 23;11(1):e3944. Epub 2019 Jan 23.

Internal Medicine, Joan C Edwards School of Medicine at Marshall University, Huntington , USA.

The etiology of lactic acidosis can potentially be misleading, especially in a critically ill patient with malignancy. Type B lactic acidosis represents a rare and often lethal complication of malignancy. When differentiating the types of lactic acidosis, Type A is due to marked tissue hypoperfusion and Type B is due to causes in the setting of a normal perfused state. Read More

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http://dx.doi.org/10.7759/cureus.3944DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6433452PMC
January 2019
2 Reads

Cyanide and lactate levels in patients during chronic oral amygdalin intake followed by intravenous amygdalin administration.

Complement Ther Med 2019 Apr 6;43:295-299. Epub 2019 Mar 6.

Department of Urology, Goethe-University, Frankfurt am Main, Germany. Electronic address:

The natural compound amygdalin has gained high popularity among tumor patients as a complementary or alternative treatment option. However, due to metabolization of amygdalin to cyanide (HCN) following oral consumption, there could be a high risk of lactic acidosis caused by cyanide intoxication. The present retrospective study was undertaken to evaluate cyanide blood and lactate plasma levels of tumor patients (n = 55) before and after intravenous (i. Read More

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http://dx.doi.org/10.1016/j.ctim.2019.03.002DOI Listing
April 2019
1 Read

Metabolic disturbances in synovial fluid are involved in the onset of synovitis in heifers with acute ruminal acidosis.

Sci Rep 2019 Apr 1;9(1):5452. Epub 2019 Apr 1.

Laboratory of Inflammation Pharmacology, Faculty of Veterinary Science, Institute of Pharmacology and Morphophysiology, Universidad Austral de Chile, Valdivia, Chile.

Acute ruminal acidosis (ARA) is the result of increased intake of highly fermentable carbohydrates, which frequently occurs in dairy cattle and is associated with aseptic polysynovitis. To characterise the metabolic changes in the joints of animals with ARA, we performed an untargeted gas chromatography-mass spectrometry (GC-MS)-based metabolomic analysis of synovial fluid. Seven heifers were challenged with an intraruminal oligofructose overload (13 g/kg of body weight [BW]) dissolved in water. Read More

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http://www.nature.com/articles/s41598-019-42007-1
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http://dx.doi.org/10.1038/s41598-019-42007-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6443794PMC
April 2019
4 Reads

Identification of human D lactate dehydrogenase deficiency.

Nat Commun 2019 04 1;10(1):1477. Epub 2019 Apr 1.

Department of Genetics, University Medical Center Utrecht, Utrecht, 3584, CX, The Netherlands.

Phenotypic and biochemical categorization of humans with detrimental variants can provide valuable information on gene function. We illustrate this with the identification of two different homozygous variants resulting in enzymatic loss-of-function in LDHD, encoding lactate dehydrogenase D, in two unrelated patients with elevated D-lactate urinary excretion and plasma concentrations. We establish the role of LDHD by demonstrating that LDHD loss-of-function in zebrafish results in increased concentrations of D-lactate. Read More

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http://dx.doi.org/10.1038/s41467-019-09458-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6443703PMC
April 2019
2 Reads

Lactic acidosis due to repressed lactate dehydrogenase subunit B expression down-regulates mitochondrial oxidative phosphorylation via the PDK/PDH axis.

J Biol Chem 2019 Mar 28. Epub 2019 Mar 28.

Biochemistry & Molecular Biology, Ajou University School of Medicine, Korea, Republic of.

Aerobic glycolysis and mitochondrial dysfunction are key metabolic features of cancer cells, but their interplay during cancer development remains unclear. We previously reported that human hepatoma cells with mitochondrial defects exhibit down-regulated lactate dehydrogenase subunit B (LDHB) expression. Here, using several molecular and biochemical assays and informatics analyses, we investigated how LDHB suppression regulates mitochondrial respiratory activity and contributes to liver cancer progression. Read More

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http://www.jbc.org/lookup/doi/10.1074/jbc.RA118.006095
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http://dx.doi.org/10.1074/jbc.RA118.006095DOI Listing
March 2019
9 Reads

Ischemic priapism as a model of exhausted metabolism.

Physiol Rep 2019 Mar;7(6):e13999

Department of Critical Care, University Medical Center Groningen, University of Groningen, Groningen, Netherlands.

In vivo metabolic studies typically concern complex open systems. However, a closed system allows better assessment of the metabolic limits. Ischemic priapism (IP) constitutes a special model of the compartment syndrome that allows direct sampling from a relatively large blood compartment formed by the corpora cavernosa (CC). Read More

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http://dx.doi.org/10.14814/phy2.13999DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6436141PMC
March 2019
2 Reads

[Effect of sodium acetate Ringer injection on perioperative fluid therapy in children with cyanotic congenital heart disease].

Zhonghua Wei Zhong Bing Ji Jiu Yi Xue 2019 Mar;31(3):363-366

Department of Anesthesiology, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China. Corresponding author: Zhang Ruidong, Email:

Objective: To evaluate the efficacy of volume therapy with sodium acetate Ringer solution during the perioperative period in children with cyanotic congenital heart disease (CHD).

Methods: The children who underwent elective surgery for cyanotic CHD admitted to Shanghai Children's Medical Center Affiliated to the Medical School of Shanghai Jiaotong University from September to December 2018 were divided into three groups according to random number table with the informed consent of their legal representatives. All of the children received volume therapy with infusion of sodium acetate Ringer solution intravenously upon anesthesia induction. Read More

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http://dx.doi.org/10.3760/cma.j.issn.2095-4352.2019.03.021DOI Listing
March 2019
1 Read

Expanding and Underscoring the Hepato-Encephalopathic Phenotype of QIL1/MIC13.

Hepatology 2019 Mar 26. Epub 2019 Mar 26.

Robert Debré Hospital, University Paris Diderot-Sorbonne Paris Cité, APHP, Reference Center for Inherited Metabolic Diseases, Paris, FR.

Mitochondrial disease is concerning with rapid infantile liver failure. Two sibling pairs with variants in QIL1, a gene important for mitochondrial contact site and cristae organizing system (MICOS) function, were recently reported. They had intermittent liver disease, mild cardiac hypertrophy, cerebellar atrophy, acquired microcephaly, neurologic impairment and death before age 5 (12mo-5yo). Read More

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http://dx.doi.org/10.1002/hep.30627DOI Listing
March 2019
2 Reads

Causes, consequences, and therapy of tumors acidosis.

Cancer Metastasis Rev 2019 Mar 26. Epub 2019 Mar 26.

Department of Cancer Physiology, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Dr., Tampa, FL, 33602, USA.

While cancer is commonly described as "a disease of the genes," it is also associated with massive metabolic reprogramming that is now accepted as a disease "Hallmark." This programming is complex and often involves metabolic cooperativity between cancer cells and their surrounding stroma. Indeed, there is emerging clinical evidence that interrupting a cancer's metabolic program can improve patients' outcomes. Read More

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http://link.springer.com/10.1007/s10555-019-09792-7
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http://dx.doi.org/10.1007/s10555-019-09792-7DOI Listing
March 2019
3 Reads
7.234 Impact Factor

Metformin-associated Severe Lactic Acidosis in the Setting of Acute Kidney Injury.

Cureus 2019 Jan 16;11(1):e3897. Epub 2019 Jan 16.

Internal Medicine, Seton Hall University-St. Francis Medical Center, Trenton, USA.

Metformin is a first-line biguanide agent for the treatment of diabetes mellitus. It has been known to be associated with lactic acidosis. This side effect especially worsens when being used with other medications affecting the kidney such as angiotensin-converting enzyme inhibitors (ACEi) and loop diuretics. Read More

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http://dx.doi.org/10.7759/cureus.3897DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6424540PMC
January 2019
1 Read

Does reduced oxygen delivery cause lactic acidosis in falciparum malaria? An observational study.

Malar J 2019 Mar 25;18(1):97. Epub 2019 Mar 25.

Mahidol Oxford Tropical Medicine Research Unit, Faculty of Tropical Medicine, Mahidol University, Bangkok, 10400, Thailand.

Background: Lactic acidosis with an elevated lactate-pyruvate ratio suggesting anoxia is a common feature of severe falciparum malaria. High lactate levels are associated with parasitized erythrocyte sequestration in the microcirculation. To assess if there is an additional contribution to hyperlactataemia from relatively inadequate total oxygen delivery, oxygen consumption and delivery were investigated in patients with malaria. Read More

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http://dx.doi.org/10.1186/s12936-019-2733-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6434797PMC
March 2019
1 Read

A novel TUFM homozygous variant in a child with mitochondrial cardiomyopathy expands the phenotype of combined oxidative phosphorylation deficiency 4.

J Hum Genet 2019 Mar 22. Epub 2019 Mar 22.

The Genetics Institute, Rambam Health Care Campus, Haifa, Israel.

Translation of mitochondrial-specific DNA is required for proper mitochondrial function and energy production. For this purpose, an elaborate network of dedicated molecular machinery including initiation, elongation and termination factors exists. We describe a patient with an unusual phenotype and a novel homozygous missense variant in TUFM (c. Read More

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http://dx.doi.org/10.1038/s10038-019-0592-6DOI Listing
March 2019
2 Reads
2.462 Impact Factor

Genetic Data Are a Prerequisite for Interpreting Clinical and Muscle Biopsy Findings in MELAS.

Authors:
Josef Finsterer

Yonsei Med J 2019 04;60(4):399-400

Krankenanstalt Rudolfstiftung, Messerlin Institute, Veterinary University of Vienna, Vienna, Austria.

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http://dx.doi.org/10.3349/ymj.2019.60.4.399DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6433565PMC

Inherited metabolic disorders presenting as hypoxic ischaemic encephalopathy: A case series of patients presenting at a tertiary care hospital in Pakistan.

J Pak Med Assoc 2019 Mar;69(3):432-436

Department of Paediatrics & Child Health, Aga Khan University Hospital, Karachi.

In spite of the efforts and interventions by the Government of Pakistan and The World Health Organization, the neonatal mortality in Pakistan has declined by only 0.9% as compared to the global average decline of 2.1% between 2000 and 2010. Read More

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March 2019
3 Reads
0.403 Impact Factor

A cluster of disseminated small cortical lesions in MELAS: its distinctive clinical and neuroimaging features.

J Neurol 2019 Mar 19. Epub 2019 Mar 19.

Department of Neurology, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan.

Objectives: To investigate a diversity of stroke-like episodes (SLEs) in mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), and report a disseminated form of SLEs (D-SLEs) attributed to a cluster of disseminated small cortical lesions.

Methods: We retrospectively reviewed the clinical information of 27 MELAS patients seen at Kitasato University Hospital between January 1990 and April 2018. Among those, we selected 13 patients with m. Read More

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http://dx.doi.org/10.1007/s00415-019-09283-3DOI Listing
March 2019
1 Read

Low-dose 2-deoxyglucose and metformin synergically inhibit proliferation of human polycystic kidney cells by modulating glucose metabolism.

Cell Death Discov 2019 11;5:76. Epub 2019 Mar 11.

Department of Nephrology, Chinese PLA General Hospital, Chinese PLA Institute of Nephrology, State Key Laboratory of Kidney Diseases, National Clinical Research Center for Kidney Diseases, Beijing Key Laboratory of Kidney Diseases, Beijing, 100853 China.

Polycystic kidney disease (PKD) is a common hereditary kidney disease with abnormal proliferation and apoptosis of kidney cystic epithelial cells, eventually leading to chronic renal failure. Currently, there are no effective treatment methods. Similar to tumor cells, cystic epithelial cells have abnormal glycolysis and over-activation of proliferation signaling pathways. Read More

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http://dx.doi.org/10.1038/s41420-019-0156-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6411866PMC
March 2019
4 Reads

DNA polymerase-γ hypothesis in nucleoside reverse transcriptase-induced mitochondrial toxicity revisited: A potentially protective role for citrus fruit-derived naringenin?

Eur J Pharmacol 2019 Mar 12;852:159-166. Epub 2019 Mar 12.

Molecular and Clinical Pharmacology Research Laboratory, Department of Pharmacology, Discipline of Pharmaceutical, School of Health Sciences, University of KwaZulu-Natal, Westville Campus, Private Bag X5400, Durban 3629, South Africa. Electronic address:

Nucleoside reverse transcriptase inhibitors (NRTIs) form the backbone in combination antiretroviral therapy (cARVs). They halt chain elongation of the viral cDNA by acting as false substrates in counterfeit incorporation mechanism to viral RNA-dependent DNA polymerase. In the process genomic DNA polymerase as well as mitochondrial DNA (mtDNA) polymerase-γ (which has a much higher affinity for these drugs at therapeutic doses) are also impaired. Read More

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http://dx.doi.org/10.1016/j.ejphar.2019.03.017DOI Listing
March 2019
1 Read

Combined-modality therapy for pulmonary alveolar proteinosis in a remote setting: a case report.

BMC Pulm Med 2019 Mar 12;19(1):61. Epub 2019 Mar 12.

Department of Medicine, Tripler Army Medical Center, 1 Jarrett White Rd, Tripler AMC, Hawaii, 96859, USA.

Background: Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by accumulation of phospholipoproteinaceous material in the alveoli. The presentation is nonspecific but typically includes dyspnea; the spectrum of disease includes rapidly progressive hypoxic respiratory failure. Whole lung lavage (WLL) is the treatment of choice in symptomatic PAP, but transient worsening of oxygenation sometimes requires salvage modalities of support such as extracorporeal membrane oxygenation (ECMO). Read More

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http://dx.doi.org/10.1186/s12890-019-0822-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6417025PMC
March 2019
1 Read

Near-Infrared Spectroscopy in the Diagnostic Evaluation of Mitochondrial Disorders: A Neonatal Intensive Care Unit Case Series.

J Pediatr 2019 Mar 7. Epub 2019 Mar 7.

Department of Pediatrics, Division of Neonatology, Stanford University, Stanford, CA.

We assessed the utility of near-infrared spectroscopy to evaluate neonates with mitochondrial disorders. We observed abnormally high cerebral oxygen saturation levels indicating insufficient tissue oxygen utilization. We propose that near-infrared spectroscopy may be an additional tool in the diagnostic evaluation of a suspected mitochondrial disorder. Read More

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http://dx.doi.org/10.1016/j.jpeds.2019.01.022DOI Listing
March 2019
1 Read

Chorea-ballism as a dominant clinical manifestation in heteroplasmic mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome with A3251G mutation in mitochondrial genome: a case report.

J Med Case Rep 2019 Mar 6;13(1):63. Epub 2019 Mar 6.

Department of Neurology, Bangur Institute of Neurosciences, IPGMER and SSKM Hospital, Kolkata, 700025, India.

Background: Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes, the most common maternally inherited mitochondrial disease, can present with a wide range of neurological manifestations including both central and peripheral nervous system involvement. The most frequent genetic mutation reported in mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome is A3243G in MT-TL1 gene. Stroke-like episodes, dementia, epilepsy, lactic acidemia, myopathy, recurrent headaches, hearing impairment, diabetes, and short stature constitute the known presentations in this syndrome. Read More

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http://dx.doi.org/10.1186/s13256-018-1936-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6402098PMC
March 2019
6 Reads

Mitochondrial Disease (MELAS Syndrome) Discovered at the Start of Pregnancy in a Patient with Advanced CKD: A Clinical and Ethical Challenge.

J Clin Med 2019 Mar 4;8(3). Epub 2019 Mar 4.

Department of Clinical and Biological Sciences, University of Torino, 10100 Torino, Italy.

Pregnancy is a challenge in the life of a woman with chronic kidney disease (CKD), but also represents an occasion for physicians to make or reconsider diagnosis of kidney disease. Counselling is particularly challenging in cases in which a genetic disease with a heterogeneous and unpredictable phenotype is discovered in pregnancy. The case reported regards a young woman with Stage-4 CKD, in which "Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes" (MELAS syndrome), was diagnosed during an unplanned pregnancy. Read More

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http://dx.doi.org/10.3390/jcm8030303DOI Listing
March 2019
1 Read

Inconsistencies in Reporting Studies of Lactic Acidosis-Reply.

JAMA Intern Med 2019 Mar;179(3):456

Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland.

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http://dx.doi.org/10.1001/jamainternmed.2018.8594DOI Listing
March 2019
5 Reads

Inconsistencies in Reporting Studies of Lactic Acidosis.

Authors:
Frank de Vries

JAMA Intern Med 2019 Mar;179(3):455-456

Division of Pharmacoepidemiology and Pharmacotherapy, Utrecht Institute for Pharmaceutical Sciences, Utrecht University, Utrecht, the Netherlands.

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http://dx.doi.org/10.1001/jamainternmed.2018.8627DOI Listing
March 2019
1 Read

Long-term outcomes of ultrashort bowel syndrome due to malrotation with midgut volvulus managed at an interdisciplinary pediatric intestinal rehabilitation center.

J Pediatr Surg 2019 Feb 2. Epub 2019 Feb 2.

Center for Advanced Intestinal Rehabilitation, Department of Surgery; Boston Children's Hospital and Harvard Medical School, Boston, MA, USA. Electronic address:

Purpose: The purpose of this study was to describe long-term outcomes of pediatric-onset ultrashort bowel syndrome owing to midgut volvulus managed at an interdisciplinary intestinal rehabilitation center.

Methods: Patients with a history of malrotation and pediatric-onset midgut volvulus causing extensive bowel loss (<20% residual small bowel length expected for postconception age) and treated between 2010 and 2017 were reviewed. Data are expressed as median (IQR). Read More

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http://dx.doi.org/10.1016/j.jpedsurg.2019.01.025DOI Listing
February 2019
2 Reads

[Phenotype and genotype of twelve Chinese children with mitochondrial DNA depletion syndromes].

Zhonghua Er Ke Za Zhi 2019 Mar;57(3):211-216

Department of Neurology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.

To explore the phenotype and genotype of mitochondrial DNA depletion syndromes (MDS) in Chinese children. The clinical and genetic data of 12 MDS patients (8 were boys and 4 were girls) diagnosed in the Department of Neurology in Beijing Children's Hospital, Capital Medical University from October 2010 to April 2018 were retrospectively collected and analyzed. The developmental milestones were normal or mildly retardated before disease onset. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2019.03.011DOI Listing
March 2019
10 Reads

Targeting NAD Metabolism as Interventions for Mitochondrial Disease.

Sci Rep 2019 Feb 28;9(1):3073. Epub 2019 Feb 28.

Mitochondria and Metabolism Center, University of Washington, Seattle, WA, 98109, USA.

Leigh syndrome is a mitochondrial disease characterized by neurological disorders, metabolic abnormality and premature death. There is no cure for Leigh syndrome; therefore, new therapeutic targets are urgently needed. In Ndufs4-KO mice, a mouse model of Leigh syndrome, we found that Complex I deficiency led to declines in NAD levels and NAD redox imbalance. Read More

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http://dx.doi.org/10.1038/s41598-019-39419-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395802PMC
February 2019
3 Reads

Type B Lactic Acidosis: A Rare Initial Presentation of Childhood Acute Lymphoblastic Leukemia.

Indian J Nephrol 2019 Jan-Feb;29(1):71-73

Division of Pediatric Hemato Oncology, Sri Ramachandra Medical University, Porur, Chennai, Tamil Nadu, India.

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http://www.indianjnephrol.org/preprintarticle.asp?id=249861
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http://dx.doi.org/10.4103/ijn.IJN_181_16DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6375013PMC
March 2019
4 Reads

Acidosis and cancer: from mechanism to neutralization.

Cancer Metastasis Rev 2019 Feb 26. Epub 2019 Feb 26.

Department of Cancer Physiology, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Avenue, Tampa, FL, 33612, USA.

The extracellular pH of solid tumors is unequivocally acidic due to a combination of high rates of lactic acid production (a consequence of fermentative glycolytic metabolism) and poor perfusion. This has been documented by us and others in a wide variety of solid tumor models, primarily using magnetic resonance spectroscopic imaging (MRSI). This acidity contributes to tumor progression by inducing genome instability, promoting local invasion and metastases, inhibiting anti-tumor immunity, and conferring resistance to chemo- and radio-therapies. Read More

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http://dx.doi.org/10.1007/s10555-019-09787-4DOI Listing
February 2019
3 Reads

-Related Mitochondrial DNA Depletion Syndrome 13 (MTDPS13): A Case Report With a Comprehensive Mutation Review.

Front Genet 2019 5;10:39. Epub 2019 Feb 5.

Division of Pediatric Neurology, Department of Pediatrics, American University of Beirut Medical Center, Beirut, Lebanon.

Mitochondrial DNA depletion syndromes (MTDPS) are a group of rare genetic disorders caused by defects in multiple genes involved in mitochondrial DNA (mtDNA) maintenance. Among those, mutations result in the encephalomyopathic mtDNA depletion syndrome 13 (MTDPS13; OMIM #615471), which commonly presents as a combination of failure to thrive, neurodevelopmental delays, encephalopathy, hypotonia, and persistent lactic acidosis. We report here the case of a Lebanese infant presenting to us with profound neurodevelopmental delays, generalized hypotonia, facial dysmorphic features, and extreme emaciation. Read More

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http://dx.doi.org/10.3389/fgene.2019.00039DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6370620PMC
February 2019
1 Read

Severe Encephalopathy, Lactic Acidosis and Hyperammonaemia With FOLFIRI Plus Aflibercept After Two-stage Hepatectomy: A Case Report.

In Vivo 2019 Mar-Apr;33(2):563-565

Department of Gastroenterological Surgery, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

Background/aim: Recent advances in chemotherapy have increased the possibility of conversion hepatectomy for patients with initially unresectable liver metastases. Although long-term chemotherapy and subsequent extensive hepatectomy are becoming more common, the toxicities of such chemotherapies are unclear.

Patients And Methods: We present a case report of a patient with metastatic colorectal cancer who developed severe encephalopathy with lactic acidosis and hyperammonaemia caused by 5-fluorouracil-based chemotherapy. Read More

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http://dx.doi.org/10.21873/invivo.11511DOI Listing
November 2018

Cerebellar Hyperintensity Lesions on Diffusion-weighted MRI in MELAS.

Intern Med 2019 Feb 25. Epub 2019 Feb 25.

Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences, Japan.

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http://dx.doi.org/10.2169/internalmedicine.2190-18DOI Listing
February 2019
1 Read

Metformin-associated hyperlactacidaemia acidosis: Diagnosis rate in standard clinical practice and its relationship with renal failure.

Rev Clin Esp 2019 Feb 18. Epub 2019 Feb 18.

Unidad de Investigación, Hospital Universitario de Canarias, Complejo Hospitalario Universitario de Canarias, Servicio Canario de la Salud, San Cristóbal de La Laguna, Tenerife, Islas Canarias, España.

Background And Objectives: Observational study on the difference between the number of cases of acidosis with hyperlactacidaemia suspected of being caused by metformin diagnosed in standard clinical practice and the incidence of this condition according to the datasheet. The study also explored the relationship between renal function and metformin-associated hyperlactacidaemia acidosis.

Patients: We identified cases of acidosis between 2013 and 2014 by analysing the minimum basic data set and laboratory requests. Read More

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http://dx.doi.org/10.1016/j.rce.2018.11.006DOI Listing
February 2019
5 Reads

[Metformin-associated lactic acidosis : myth or reality ?]

Rev Med Suisse 2019 Feb;15(639):422-426

Service de néphrologie, HUG, 1211 Genève 14.

Metformin is the first line therapy for patients with diabete type 2. However, the molecule is known to be responsible for lactic acidosis through its inhibition of the mitochondrial respiratory chain complex, a pathology called MALA (metformin associated lactic acidosis). This complication has been widely discussed in the literature because its development is usually the result of a multifactorial and complex process. Read More

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February 2019
7 Reads

Metformin-associated lactic acidosis, a ghost or a murderer?

Rev Clin Esp 2019 Feb 16. Epub 2019 Feb 16.

Unidad de Endocrinología y Nutrición, Hospital General de Segovia, Segovia, España.

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https://linkinghub.elsevier.com/retrieve/pii/S00142565193001
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http://dx.doi.org/10.1016/j.rce.2019.01.001DOI Listing
February 2019
4 Reads

A Case of Renal Involvement in B Lymphoblastic Lymphoma Leukemia.

Clin Lab 2019 Jan;65(1)

Background: Renal involvement is rare in B lymphoblastic lymphoma (B-LBL). The authors describe a rare case of renal involvement in a 21-year-old male patient with B lymphoblastic lymphoma leukemia, presenting with severe lactic acidosis.

Methods: Hematologic investigation, bone marrow aspirate and biopsy, cytogenetic analysis and renal biopsy were performed. Read More

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http://dx.doi.org/10.7754/Clin.Lab.2018.180726DOI Listing
January 2019
4 Reads
1.084 Impact Factor

Inhibitory ASIC2-mediated calcineurin/NFAT against colorectal cancer by triterpenoids extracted from Rhus chinensis Mill.

J Ethnopharmacol 2019 May 14;235:255-267. Epub 2019 Feb 14.

Department of Pharmaceutics, Shanghai Eighth People's Hospital, Jiangsu University, Shanghai 200235, China.

Ethnopharmacological Relevance: Studies have shown that the etiology and pathogenesis of colorectal cancer are closely related to the tumor microenvironment, and the cancer tissue is still in the state of "energy deficit" and has to promote energy generation through high glycolysis. Rhus chinensis Mill is a Chinese herbal medicine used to treat various types of solid tumors in China. Colorectal cancer (CRC) is a heterogeneous disease group caused by abnormal changes in glucose metabolism resulted in lactic acid production, which remodels acidosis. Read More

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http://dx.doi.org/10.1016/j.jep.2019.02.029DOI Listing
May 2019
1 Read

Whole exome sequencing revealed mutations in FBXL4, UNC80, and ADK in Thai patients with severe intellectual disabilities.

Gene 2019 May 14;696:21-27. Epub 2019 Feb 14.

Center of Excellence for Medical Genomics, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand; Excellence Center for Medical Genetics, King Chulalongkorn Memorial Hospital, the Thai Red Cross Society, Bangkok 10330, Thailand.

Intellectual disabilities (ID) are etiologically heterogeneous. Advanced molecular techniques could be helpful in identification of the underlying genetic defects. We aimed to characterize clinical and molecular features of three Thai patients with ID. Read More

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http://dx.doi.org/10.1016/j.gene.2019.01.049DOI Listing