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Acidosis metabolic

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https://www.readbyqxmd.com/read/28820737/when-one-disease-is-not-enough-succinyl-coa-3-oxoacid-coenzyme-a-transferase-scot-deficiency-due-to-a-novel-mutation-in-oxct1-in-an-infant-with-known-phenylketonuria
#1
Jan-Niclas Schwade, Matthias Endmann, Thomas Hofmann, Stephan Rust, Jörn Oliver Sass, Frank Rutsch
A 9-month-old Turkish girl was admitted several times within 3 months to the hospital in reduced general condition and with extreme tachypnea. The patient had been diagnosed with phenylketonuria (PKU) in newborn screening and has been treated with a low phenylalanine diet and amino acid supplements. Each time an unexplained pronounced metabolic acidosis was noted, and the child was treated with sodium-bicarbonate and glucose-electrolyte infusions. The acidosis with only slightly abnormal glucose, normal lactate levels and pronounced ketonuria suggested a defect in ketone body utilization...
August 18, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28820736/long-qt-syndrome-diagnosed-in-two-sisters-with-propionic-acidemia-a-case-report
#2
Ensar Duras, Ahmet İrdem, Ozan Özkaya
Propionic acidemia (PA) is a rare autosomal recessive metabolic disorder caused by deficiency of the mitochondrial enzyme propionyl-CoA carboxylase (PCC). This disorder mostly progresses with episodes of metabolic acidosis. Cardiomyopathy is among the cardiac complications known to occur during metabolic decompensation episodes. However, several recent papers emphasized the association of PA and long QT syndrome (LQTS) which may lead to extremely serious and fatal consequences. In this report, we describe two sisters with PA who have prolonged QT duration that were incidentally detected in an outpatient setting...
August 18, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28817693/clinical-signs-profound-acidemia-hypoglycemia-and-hypernatremia-are-predictive-of-mortality-in-1-400-critically-ill-neonatal-calves-with-diarrhea
#3
Florian M Trefz, Ingrid Lorenz, Annette Lorch, Peter D Constable
Profound acidemia impairs cellular and organ function and consequently should be associated with an increased risk of mortality in critically ill humans and animals. Neonatal diarrhea in calves can result in potentially serious metabolic derangements including profound acidemia due to strong ion (metabolic) acidosis, hyper-D-lactatemia, hyper-L-lactatemia, azotemia, hypoglycemia, hyperkalemia and hyponatremia. The aim of this retrospective study was to assess the prognostic relevance of clinical and laboratory findings in 1,400 critically ill neonatal calves with diarrhea admitted to a veterinary teaching hospital...
2017: PloS One
https://www.readbyqxmd.com/read/28812381/retrospective-review-of-sglt2-inhibitor-exposures-reported-to-13-poison-centers
#4
Scott E Schaeffer, Carol DesLauriers, Henry A Spiller, Alfred Aleguas, Salvador Baeza, Mark L Ryan
BACKGROUND: SGLT2 inhibitors are a new class of oral antidiabetics prescribed in the United States since 2013. They act by inhibiting reabsorption of glucose in the proximal convoluted tubule of the kidney, allowing excess glucose to be excreted. Little has been reported regarding effects of non-therapeutic exposure to this class of medication. METHODS: Retrospective records from 13 poison centers were examined for human exposures to SGLT2 inhibitors between 1st January 2013 and 31st December 2016...
August 16, 2017: Clinical Toxicology
https://www.readbyqxmd.com/read/28811944/successful-resuscitation-of-a-patient-with-life-threatening-metabolic-acidosis-by-hemodialysis-a-case-of-ethylene-glycol-intoxication
#5
Ikuyo Narita, Michiko Shimada, Norio Nakamura, Reiichi Murakami, Takeshi Fujita, Wakako Fukuda, Hirofumi Tomita
BACKGROUND: Ethylene glycol intoxication causes severe metabolic acidosis and acute kidney injury. Fomepizole has become available as its antidote. Nevertheless, a prompt diagnosis is not easy because patients are often unconscious. Here we present a case of ethylene glycol intoxication who successfully recovered with prompt hemodialysis. CASE PRESENTATION: A 52-year-old Japanese male was admitted to a local hospital due to suspected food poisoning. The patient presented with nausea and vomiting, but his condition rapidly deteriorated, with worsening conscious level, respiratory distress requiring mechanical ventilation, hypotension, and severe acute kidney injury...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28801177/uses-and-misuses-of-sodium-bicarbonate-in-the-neonatal-intensive-care-unit
#6
Amélie Collins, Rakesh Sahni
Over the past several decades, bicarbonate therapy continues to be used routinely in the treatment of acute metabolic acidosis in critically ill neonates despite the lack of evidence for its effectiveness in the treatment of acid-base imbalance, and evidence indicating that it may be detrimental. Clinicians often feel compelled to use bicarbonate since acidosis implies a need for such therapy and thus the justification for its use is based on hearsay rather than science. This review summarizes the evidence and refutes the clinical practice of administering sodium bicarbonate to treat metabolic acidosis associated with several specific clinical syndromes in neonates...
August 8, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28797958/quantification-of-paracetamol-and-5-oxoproline-in-serum-by-capillary-electrophoresis-implication-for-clinical-toxicology
#7
Tomáš Hložek, Tomáš Křížek, Petr Tůma, Miroslava Bursová, Pavel Coufal, Radomír Čabala
High anion gap metabolic acidosis frequently complicates acute paracetamol overdose and is generally attributed to lactic acidosis or compromised hepatic function. However, metabolic acidosis can also be caused by organic acid 5-oxoproline (pyroglutamic acid). Paracetamol's toxic intermediate, N-acetyl-p-benzoquinoneimine irreversibly binds to glutathione and its depletion leads to subsequent disruption of the gamma glutamyl cycle and an excessive 5-oxoproline generation. This is undoubtedly an underdiagnosed condition because measurement of serum 5-oxoproline level is not readily available...
July 26, 2017: Journal of Pharmaceutical and Biomedical Analysis
https://www.readbyqxmd.com/read/28797572/hyperosmolar-metabolic-acidosis-in-burn-patients-exposed-to-glycol-based-topical-antimicrobials-a-systematic-review
#8
REVIEW
Tom Leibson, Paige Davies, Cheri Nickel, Gideon Koren
BACKGROUND: The well documented susceptibility of burn patients to acquired infections via damaged skin mandates application of antimicrobial agents. These agents are dissolved in various vehicles that augment skin absorption thus allowing greater efficacy. Polyethylene glycol (PEG) and Propylene glycol (PropG) are among the most commonly used vehicles, and both have been used in numerous medications and cosmetic products over the past few decades. Rarely, burn patients treated with agents containing these glycols present with a life threatening systemic toxidrome of hyperosmolar metabolic acidosis...
August 7, 2017: Burns: Journal of the International Society for Burn Injuries
https://www.readbyqxmd.com/read/28797049/effect-of-sodium-bicarbonate-on-prolonged-running-performance-a-randomized-double-blind-cross-over-study
#9
Tanja Freis, Anne Hecksteden, Ulf Such, Tim Meyer
BACKGROUND: The ability to sustain intense exercise seems to be partially limited by the body's capability to counteract decreases in both intra- and extracellular pH. While the influence of an enhanced buffering capacity via sodium bicarbonate (BICA) on short-term, high-intensity exercise performance has been repeatedly investigated, studies on prolonged endurance performances are comparatively rare, especially for running. The aim of the following study was to assess the ergogenic effects of an oral BICA substitution upon exhaustive intensive endurance running performance...
2017: PloS One
https://www.readbyqxmd.com/read/28794844/a-case-series-on-simultaneous-liver-and-kidney-transplantation-do-we-need-intraoperative-renal-replacement-therapy
#10
Wongook Wi, Tae Soo Hahm, Gaab-Soo Kim
Since the implementation of the model for end-stage liver disease (MELD) scoring system in 2002, the liver transplantation (LT) society has observed a substantial increase in the number of recipients with renal dysfunction. Intraoperative renal replacement therapy (ioRRT) has emerged as one of the solutions available to manage high-MELD score recipients; however, its usefulness has not yet been proven. To date, we have experienced five cases of simultaneous liver and kidney transplantation (SLKT). Recipients of SLKT tend to have a lower pre-transplant kidney function and the longer operation time mandates a larger amount of fluid than LT alone...
August 2017: Korean Journal of Anesthesiology
https://www.readbyqxmd.com/read/28789699/oral-pyridoxine-can-substitute-for-intravenous-pyridoxine-in-managing-patients-with-severe-poisoning-with-isoniazid-and-rifampicin-fixed-dose-combination-tablets-a-case-report
#11
M D S A Dilrukshi, C A P Ratnayake, C A Gnanathasan
BACKGROUND: Fixed drug combination of isoniazid and rifampicin is a rare cause of poisoning even in endemic countries for tuberculosis infection. Severe poisoning can cause severe morbidity and mortality if not treated promptly. Though intravenous pyridoxine is the preferred antidote for severe standard isoniazid poisoning it is not freely available even in best of care centers. We describe a case of severe poisoning with fixed drug combination of isoniazid and rifampicin successfully managed with oral pyridoxine at national hospital of Sri Lanka...
August 8, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28782093/metabolic-acidosis-caused-by-concomitant-use-of-paracetamol-acetaminophen-and-flucloxacillin-a-case-report-and-a-retrospective-study
#12
J K Berbee, L A Lammers, C T P Krediet, J C Fischer, E M Kemper
PURPOSE: A patient was identified with severe metabolic acidosis, a high anion gap and 5-oxoproline accumulation, probably caused by the simultaneous use of paracetamol (acetaminophen) and flucloxacillin. We wanted to investigate the necessity to control the interaction between both drugs with an automatic alert system. METHODS: To investigate the relevance of the interaction of paracetamol and flucloxacillin, a retrospective study was conducted. Data on paracetamol and flucloxacillin prescriptions and laboratory data (pH, Na(+), HCO3(-), Cl(-), albumin and 5-oxoproline levels) were combined to assess the prevalence of acidosis, calculate the anion gap and analyse 5-oxoproline levels in clinically admitted patients using both drugs simultaneously...
August 7, 2017: European Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28774273/thrombotic-microangiopathy-associated-with-valproic-acid-toxicity
#13
Sean A Hebert, Timothy P Bohan, Christian L Erikson, Rita D Swinford
BACKGROUND: Thrombotic microangiopathy (TMA) is a serious, sometimes life-threatening disorder marked by the presence of endothelial injury and microvascular thrombi. Drug-induced thrombotic microangiopathy (DI-TMA) is one specific TMA syndrome that occurs following drug exposure via drug-dependent antibodies or direct tissue toxicity. Common examples include calcineurin inhibitors Tacrolimus and Cyclosporine and antineoplastics Gemcitabine and Mitomycin. Valproic acid has not been implicated in DI-TMA...
August 3, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28765741/experience-with-mechanical-circulatory-support-for-medically-intractable-low-cardiac-output-in-a-pediatric-intensive-care-unit
#14
Jung Bin Park, Jae Gun Kwak, Hong-Gook Lim, Woong-Han Kim, Jeong Ryul Lee, Yong Jin Kim
BACKGROUND AND OBJECTIVES: Mechanical circulatory support with extracorporeal membrane oxygenation (ECMO) and ventricular assist device has always been the optimal choice for treating the majority of medically intractable low cardiac output case. We retrospectively investigated our institution's outcomes and variables associated with a high risk of mortality. SUBJECTS AND METHODS: From 1999 to 2014, 86 patients who were of pediatric age or had grown-up congenital heart disease underwent mechanical circulatory support for medically intractable low cardiac output in our pediatric intensive care unit...
July 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28765189/expanding-the-differential-toluene-induced-toxicity
#15
Lavanya Dharmarajan, Hussam Ammar
A 31-year-old woman presented to the hospital with generalised weakness and lower back and abdominal pain. The only significant finding on physical examination was the weakness of the legs. Laboratory analysis in the emergency department revealed that she was hypokalaemic with metabolic acidosis. She was treated with oral and intravenous potassium, which resolved her weakness. During the search for an explanation for her renal tubular acidosis, she said she was exposed to paint exposure while working on a friend's house...
August 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28764826/minding-the-gap-utility-of-the-anion-gap-in-the-differential-diagnosis-of-metabolic-acidosis
#16
Susan Givens Bell
The anion gap, in conjunction with other laboratory results, can be a useful clue in the differential diagnosis of metabolic acidosis. There are three primary causes of metabolic acidosis: loss of base, decreased renal excretion of acid, and increased acid production. Depending on the cause of metabolic acidosis, the anion gap may be elevated or normal.
July 1, 2017: Neonatal Network: NN
https://www.readbyqxmd.com/read/28762684/-the-retroperitoneal-space
#17
Andrea Mancini, Mario Meola, Giuseppe Tarantino, Ilaria Petrucci, Giovanna Berardi, Cristoforo Cuzzola
We describe the case of a 45-year-old woman with a clinical history of breast cancer presenting with anuric renal failure, metabolic acidosis and bilateral grade 2-3 hydronephrosis. Following insertion of bilateral ureteral stents, urinary output was 5000 ml in the subsequent 24 hours with frankly bloody urine, after which anuria recurred. A new ultrasound examination showed hydronephrotic kidneys with properly positioned stents, a distended bladder free of clots and a hypo-anechoic, well-demarcated mass enveloping the aorta...
August 1, 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28762682/-use-of-citrate-in-patients-with-nephrolithiasis
#18
REVIEW
Martino Marangella
Citrate is a tricarboxylic acid and an intermediate metabolite of Krebs cycle. It contributes to oxidative metabolism of both kidney and liver. Alkaline sodium or potassium salts have the potential to increase alkaline reserve. In the kidney citrate is completely filtered at the glomerulus, undergoing to 10-40% tubular resorption. Renal insufficiency, even early, metabolic acidosis, potassium depletion induce hypocitraturia. Its importance in nephrolithiasis stems from its ability to form soluble complexes with calcium and to interfere with crystal formation, thus exerting a dual inhibition, thermodynamic and kinetic...
August 1, 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28749752/high-anion-gap-metabolic-acidosis-induced-by-cumulation-of-ketones-l-and-d-lactate-5-oxoproline-and-acute-renal-failure
#19
Laura Heireman, Boris Mahieu, Mark Helbert, Wim Uyttenbroeck, Jan Stroobants, Marian Piqueur
INTRODUCTION: Frequent causes of high anion gap metabolic acidosis (HAGMA) are lactic acidosis, ketoacidosis and impaired renal function. In this case report, a HAGMA caused by ketones, L- and D-lactate, acute renal failure as well as 5-oxoproline is discussed. CASE PRESENTATION: A 69-year-old woman was admitted to the emergency department with lowered consciousness, hyperventilation, diarrhoea and vomiting. The patient had suffered uncontrolled type 2 diabetes mellitus, underwent gastric bypass surgery in the past and was chronically treated with high doses of paracetamol and fosfomycin...
July 27, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28747361/metabolic-acidosis-stimulates-the-production-of-the-antimicrobial-peptide-cathelicidin-in-rabbit-urine
#20
Hu Peng, Jeffrey M Purkerson, Andrew L Schwaderer, George J Schwartz
Intercalated cells of the collecting duct (CD) are critical for acid-base homeostasis and innate immune defense of the kidney. Little is known about the impact of acidosis on innate immune defense in the distal nephron. Urinary tract infections are mainly due to Escherichia coli and are an important risk factor for development of chronic kidney disease. While the effect of urinary pH on growth of E. coli is well established, in this study we demonstrate that acidosis increases urine antimicrobial activity due, at least in part, to induction of cathelicidin expression within the CD...
July 26, 2017: American Journal of Physiology. Renal Physiology
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