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https://www.readbyqxmd.com/read/29769189/metabolic-acidosis-in-short-bowel-syndrome-think-d-lactic-acid-acidosis
#1
Sorin Stanciu, Aminda De Silva
Short bowel syndrome (SBS) is a condition when a person's gastrointestinal function is insufficient to supply the body with essential nutrients and hydration. Patients with SBS suffer from diarrhoea and symptoms of malabsorption such as weight loss, electrolyte disturbances and vitamin deficiencies. Long-term management of this condition can be complicated by the underlying disease, the abnormal bowel function and issues related to treatment like administration of parenteral nutrition and the use of a central venous catheter...
May 16, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29767723/isca1-mutation-in-a-patient-with-infantile-onset-leukodystrophy-causes-defects-in-mitochondrial-4fe-4s-proteins
#2
Alessandra Torraco, Oliver Stehling, Claudia Stümpfig, Ralf Rösser, Domenico De Rasmo, Giuseppe Fiermonte, Daniela Verrigni, Teresa Rizza, Angelo Vozza, Michela Di Nottia, Daria Diodato, Diego Martinelli, Fiorella Piemonte, Carlo Dionisi-Vici, Enrico Bertini, Roland Lill, Rosalba Carrozzo
Multiple Mitochondrial Dysfunction Syndromes (MMDS) comprise a group of severe autosomal recessive diseases characterized by impaired respiration and lipoic acid metabolism, resulting in infantile-onset mitochondrial encephalopathy, non-ketotic hyperglycinemia, myopathy, lactic acidosis and early death. Four different MMDS have been analyzed in detail according to the genes involved in the disease, MMDS1 (NFU1), MMDS2 (BOLA3), MMDS3 (IBA57), and MMDS4 (ISCA2). MMDS5 has recently been described in a clinical case report of patients carrying a mutation in ISCA1, but with no further functional analysis...
May 14, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29765783/multiorgan-failure-and-refractory-lactic-acidosis-due-to-pasteurella-multocida-septicemia-in-a-patient-with-no-animal-exposure
#3
Damaris Pena, Yaneidy Santana, Jose Perez Lara, Efrain Gonzalez, Misbahuddin Khaja
Introduction: Pasteurella multocida is a gram-negative coccobacillus pathogenic to animals. It can cause infection in humans by a bite, scratch, or lick from a cat or dog. P. multocida can cause a variety of infections in humans, including cellulitis, osteomyelitis, endocarditis, peritonitis, and septic shock. Case Presentation: A 56-year-old male presented to our hospital with a 2-day history of fever, abdominal pain, nausea, and vomiting. He denied exposure to cats, dogs or other pets...
2018: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/29756986/-dietetic-treatment-with-fructose-in-a-5-year-old-girl-with-recurrent-d-lactic-acidosis
#4
Lourdes Travieso Suárez, Pilar Quijada Fraile, Consuelo Pedrón Giner
D-lactic acidosis is an infrequent complication, mainly reported in patients with short bowel syndrome. It is characterized by recurrent episodes of encephalopathy with elevated serum D-lactic acid, usually associating metabolic acidosis. The presence of D-lactate-producing bacteria is necessary for the development of this complication. Other factors, such as the ingestion of large amounts of carbohydrates or reduced intestinal motility, contribute to D-lactic acidosis. We report a case of recurrent D-lactic acidosis in a 5-year-old girl with short bowel syndrome, due to a midgut volvulus...
March 1, 2018: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/29756066/postanaesthetic-shivering-from-pathophysiology-to-prevention
#5
REVIEW
Maria Bermudez Lopez
Postoperative shivering is a common complication of anaesthesia. Shivering is believed to increase oxygen consumption, increase the risk of hypoxemia, induce lactic acidosis, and catecholamine release. Therefore, it might increase the postoperative complications especially in high-risk patients. Moreover, shivering is one of the leading causes of discomfort for postsurgical patients. Shivering is usually triggered by hypothermia. However, it occurs even in normothermic patients during the perioperative period...
April 2018: Romanian Journal of Anaesthesia and Intensive Care
https://www.readbyqxmd.com/read/29755998/application-of-a-pharmacokinetic-model-of-metformin-clearance-in-a-population-with-acute-myeloid-leukemia
#6
Alice C Ceacareanu, Geoffrey W Brown, Hoda A Moussa, Zachary A P Wintrob
Objective: We aimed to estimate the metformin-associated lactic acidosis (MALA) risk by assessing retrospectively the renal clearance variability and applying a pharmacokinetic (PK) model of metformin clearance in a population diagnosed with acute myeloid leukemia (AML) and diabetes mellitus (DM). Methods: All adults with preexisting DM and newly diagnosed AML at Roswell Park Cancer Institute were reviewed (January 2003-December 2010, n = 78). Creatinine clearance (CrCl) and total body weight distributions were used in a two-compartment PK model adapted for multiple dosing and modified to account for actual intra- and inter-individual variability...
January 2018: Journal of Research in Pharmacy Practice
https://www.readbyqxmd.com/read/29746311/evaluating-mortality-risk-adjustment-among-children-receiving-extracorporeal-support-for-respiratory-failure
#7
Ryan P Barbaro, Philip S Boonstra, Kevin W Kuo, David T Selewski, David K Bailly, Cheryl L Stone, Chin Ying Chow, Gail M Annich, Frank W Moler, Matthew L Paden
This study evaluates whether three commonly used pediatric intensive care unit (PICU) severity of illness scores, pediatric risk of mortality score (PRISM) III, pediatric index of mortality (PIM) 2, and pediatric logistic organ dysfunction (PELOD), are the appropriate tools to discriminate mortality risk in children receiving extracorporeal membrane oxygenation (ECMO) support for respiratory failure. This study also evaluates the ability of the Pediatric Risk Estimate Score for Children Using Extracorporeal Respiratory Support (Ped-RESCUERS) to discriminate mortality risk in the same population, and whether Ped-RESCUERS' discrimination of mortality is improved by additional clinical and laboratory measures of renal, hepatic, neurologic, and hematologic dysfunction...
May 8, 2018: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/29738540/risk-of-lactic-acidosis-in-type-2-diabetes-patients-using-metformin-a-case-control-study
#8
Abdellatif Aharaz, Anton Pottegård, Daniel Pilsgaard Henriksen, Jesper Hallas, Henning Beck-Nielsen, Annmarie Touborg Lassen
BACKGROUND: Metformin constitutes first-line treatment of type 2 diabetes mellitus. It is presumed to have lactic acidosis as a dangerous, but rare, side effect. OBJECTIVES: To estimate the incidence rate of lactic acidosis in patients with type 2 diabetes mellitus as well as to estimate the relative risk of lactic acidosis associated with metformin treatment. METHODS: This is a population-based combined cohort and case-control study among patients with type 2 diabetes mellitus who were acutely admitted with lactic acidosis at Odense University Hospital, Denmark; in the period from 1st June 2009 to 1st October 2013...
2018: PloS One
https://www.readbyqxmd.com/read/29737362/-regional-citrate-anticoagulation-in-renal-replacement-therapy-in-the-intensive-care-station-recommendations-from-the-renal-section-of-the-dgiin-%C3%A3-giain-and-divi
#9
REVIEW
M Schmitz, M Joannidis, D Czock, S John, A Jörres, S J Klein, M Oppert, V Schwenger, J Kielstein, A Zarbock, D Kindgen-Milles, C Willam
BACKGROUND: Regional citrate anticoagulation (RCA) in continuous renal replacement therapy can effectively anticoagulate dialysis circuits without having adverse effects on systemic heparin application. In particular, in continuous renal replacement therapy RCA is well established and represents a safe procedure with longer filter lifetimes and fewer bleeding complications. OBJECTIVES: To provide guidance on the indications, advantages and disadvantages, and use of RCA, current recommendations from the renal section of the DGIIN (Deutschen Gesellschaft für Internistische Intensivmedizin und Notfallmedizin), ÖGIAIN (Österreichischen Gesellschaft für Internistische und Allgemeine Intensivmedizin und Notfallmedizin) and DIVI (Deutschen Interdisziplinären Vereinigung für Intensiv- und Notfallmedizin) are stated...
May 8, 2018: Medizinische Klinik, Intensivmedizin und Notfallmedizin
https://www.readbyqxmd.com/read/29715193/severe-lactic-acidosis-in-an-extremely-low-birth-weight-infant-due-to-thiamine-deficiency
#10
Hulya Ozdemir, Hulya Bilgen, Zeynep Alp Unkar, Ertugrul Kiykim, Asli Memisoglu, Eren Ozek
BACKGROUND: In this case report, we present a preterm newborn with persistent lactic acidosis who received total parenteral nutrition (TPN) that lacked thiamine. CASE PRESENTATION: A 28-week-old, 750 g female infant was born with an Apgar score of 8 at the 5th minute. Umbilical cord blood gas levels, including lactate level, were normal, and she was admitted to our neonatal intensive care unit (NICU). Achieving full enteral feeding was not possible due to gastric residues and abdominal distention, making the patient dependent on TPN during the first 2 weeks of life...
May 1, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29715184/mutations-in-surf1-are-important-genetic-causes-of-leigh-syndrome-in-slovak-patients
#11
Daniel Danis, Katarina Brennerova, Martina Skopkova, Timea Kurdiova, Jozef Ukropec, Juraj Stanik, Miriam Kolnikova, Daniela Gasperikova
OBJECTIVES: Leigh syndrome is a progressive early onset neurodegenerative disease typically presenting with psychomotor regression, signs of brainstem and/or basal ganglia disease, lactic acidosis, and characteristic magnetic resonance imaging findings. At molecular level, deficiency of respiratory complexes and/or pyruvate dehydrogenase complex is usually observed. Nuclear gene SURF1 encodes an assembly factor for cytochrome c-oxidase complex of the respiratory chain and autosomal recessive mutations in SURF1 are one of the most frequent causes of cytochrome c-oxidase-related Leigh syndrome cases...
April 1, 2018: Endocrine Regulations
https://www.readbyqxmd.com/read/29695298/pyruvate-is-a-prospective-alkalizer-to-correct-hypoxic-lactic-acidosis
#12
REVIEW
Ying Wang, Ya Huang, Jing Yang, Fang-Qiang Zhou, Lian Zhao, Hong Zhou
Type A lactic acidosis resulted from hypoxic mitochondrial dysfunction is an independent predictor of mortality for critically ill patients. However, current therapeutic agents are still in shortage and can even be harmful. This paper reviewed data regarding lactic acidosis treatment and recommended that pyruvate might be a potential alkalizer to correct type A lactic acidosis in future clinical practice. Pyruvate is a key energy metabolic substrate and a pyruvate dehydrogenase (PDH) activator with several unique beneficial biological properties, including anti-oxidant and anti-inflammatory effects and the ability to activate the hypoxia-inducible factor-1 (HIF-1α) - erythropoietin (EPO) signal pathway...
April 26, 2018: Military Medical Research
https://www.readbyqxmd.com/read/29692773/lactobacilli-are-prominent-members-of-the-microbiota-involved-in-the-ruminal-digestion-of-barley-and-corn
#13
Hee E Yang, Claiton A Zotti, John J McKinnon, Tim A McAllister
The chemical composition of barley grain can vary among barley varieties (Fibar, Xena, McGwire, and Hilose) and result in different digestion efficiencies in the rumen. It is not known if compositional differences in barley can affect the microbiota involved in the ruminal digestion of barley. The objective of this study was to characterize the in situ rumen degradability and microbiota of four barley grain varieties and to compare these to corn. Three ruminally cannulated heifers were fed a low (60% barley silage, 37% barley grain, and 3% supplement) or high grain (37% barley silage, 60% barley grain, and 3% supplement) diet...
2018: Frontiers in Microbiology
https://www.readbyqxmd.com/read/29687945/severe-lactic-acidosis-and-hypoglycemia-due-to-acute-metformin-intoxication-in-a-dog
#14
Yu Ueda, Christine Wong, Angela Borchers
OBJECTIVE: To report a case of severe lactic acidosis and hypoglycemia due to acute metformin intoxication in a dog. CASE SUMMARY: A female neutered Rat Terrier was presented for an acute onset of seizure-like episodes, weakness, and vomiting approximately 14 hours after ingestion of 198 mg/kg of metformin. The dog was found to be laterally recumbent, paddling, and unresponsive shortly before presentation. On physical exam, the dog was in hypovolemic shock and hypothermic...
April 24, 2018: Journal of Veterinary Emergency and Critical Care
https://www.readbyqxmd.com/read/29685833/-metabolic-lactic-acidosis-as-a-sign-of-voluntary-poisoning-in-adolescents
#15
Anna Habimana-Jordana, Victoria López-Corominas, Bernardino Barceló-Martín, Isabel Gomila-Muñiz, Lidia Martínez-Sánchez
No abstract text is available yet for this article.
April 20, 2018: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/29682452/extending-the-phenotypic-spectrum-of-sengers-syndrome-congenital-lactic-acidosis-with-synthetic-liver-dysfunction
#16
David B Beck, Kristina Cusmano-Ozog, Nickie Andescavage, Eyby Leon
Sengers syndrome is a rare autosomal recessive mitochondrial disease characterized by lactic acidosis, hypertrophic cardiomyopathy and bilateral cataracts. We present here a case of neonatal demise, within the first day of life, who initially presented with severe lactic acidosis, with evidence of both chorioamnionitis and cardiogenic shock. Initial metabolic labs demonstrated a severe lactic acidosis prompting genetic testing which revealed a homozygous pathogenic variant for Sengers syndrome in AGK, c.979A >  T; p...
April 13, 2018: Translational Science of Rare Diseases
https://www.readbyqxmd.com/read/29681094/a-neurodegenerative-mitochondrial-disease-phenotype-due-to-biallelic-loss-of-function-variants-in-pnpla8-encoding-calcium-independent-phospholipase-a2%C3%AE
#17
Anju Shukla, Russell P Saneto, Malavika Hebbar, Ghayda Mirzaa, Katta M Girisha
Animal studies have demonstrated the critical roles of the patatin-like protein family plays in cellular growth, lipid homeostasis, and second messenger signaling the nervous system. Of the nine known calcium-independent phospholipase A2γ, only PNPLA2, PNLPA6, PNPLA9 and most recently a single patient with PNPLA8 are associated with mitochondrial-related neurodegeneration. Using whole exome sequencing, we report two unrelated individuals with variable but similar clinical features of microcephaly, severe global developmental delay, spasticity, lactic acidosis, and progressive cerebellar atrophy with biallelic loss-of-function variants in PNPLA8...
May 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29681092/lipt1-deficiency-presenting-as-early-infantile-epileptic-encephalopathy-leigh-disease-and-secondary-pyruvate-dehydrogenase-complex-deficiency
#18
Robert C Stowe, Qin Sun, Sarah H Elsea, Fernando Scaglia
Lipoic acid is an essential cofactor for the mitochondrial 2-ketoacid dehydrogenase complexes and the glycine cleavage system. Lipoyltransferase 1 catalyzes the covalent attachment of lipoate to these enzyme systems. Pathogenic variants in LIPT1 gene have recently been described in four patients from three families, commonly presenting with severe lactic acidosis resulting in neonatal death and/or poor neurocognitive outcomes. We report a 2-month-old male with severe lactic acidosis, refractory status epilepticus, and brain imaging suggestive of Leigh disease...
May 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29673406/inflammatory-mechanism-of-rumenitis-in-dairy-cows-with-subacute-ruminal-acidosis
#19
Chenxu Zhao, Guowen Liu, Xiaobing Li, Yuan Guan, Yazhou Wang, Xue Yuan, Guoquan Sun, Zhe Wang, Xinwei Li
BACKGROUND: Subacute ruminal acidosis (SARA) is a metabolic disease in high-producing dairy cattle, and is accompanied by rumenitis. However, the mechanism of rumenitis remains unclear. Therefore, the aim of this study was to investigate the molecular mechanism of rumenitis in dairy cows with SARA. RESULTS: The results showed that SARA cows displayed high concentrations of ruminal volatile fatty acids, lactic acid and lipopolysaccharide (LPS). Furthermore, the blood concentrations of LPS and acute phase proteins haptoglobin, serum amyloid-A, and LPS binding protein were significantly higher in SARA cows than in control cows...
April 19, 2018: BMC Veterinary Research
https://www.readbyqxmd.com/read/29666206/taurine-supplementation-for-prevention-of-stroke-like-episodes-in-melas-a-multicentre-open-label-52-week-phase-iii-trial
#20
Yutaka Ohsawa, Hiroki Hagiwara, Shin-Ichiro Nishimatsu, Akihiro Hirakawa, Naomi Kamimura, Hideaki Ohtsubo, Yuta Fukai, Tatsufumi Murakami, Yasutoshi Koga, Yu-Ichi Goto, Shigeo Ohta, Yoshihide Sunada
OBJECTIVE: The aim of this study was to evaluate the efficacy and safety of high-dose taurine supplementation for prevention of stroke-like episodes of MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes), a rare genetic disorder caused by point mutations in the mitochondrial DNA that lead to a taurine modification defect at the first anticodon nucleotide of mitochondrial tRNALeu(UUR) , resulting in failure to decode codons accurately. METHODS: After the nationwide survey of MELAS, we conducted a multicentre, open-label, phase III trial in which 10 patients with recurrent stroke-like episodes received high-dose taurine (9 g or 12 g per day) for 52 weeks...
April 17, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
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