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Respiratory acidosis

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https://www.readbyqxmd.com/read/28984162/acid-base-regulation-and-sensing-accelerators-and-brakes-in-metabolic-regulation-of-cerebrovascular-tone
#1
Ebbe Boedtkjer
Metabolic regulation of cerebrovascular tone directs blood flow to areas of increased neuronal activity and during disease states partially compensates for insufficient perfusion by enhancing blood flow in collateral blood vessels. Acid-base disturbances frequently occur as result of enhanced metabolism or insufficient blood supply, but despite definitive evidence that acid-base disturbances alter arterial tone, effects of individual acid-base equivalents and the underlying signaling mechanisms are still being debated...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28973171/defective-mitochondrial-rrna-methyltransferase-mrm2-causes-melas-like-clinical-syndrome
#2
Caterina Garone, Aaron R D'Souza, Cristina Dallabona, Tiziana Lodi, Pedro Rebelo-Guiomar, Joanna Rorbach, Maria Alice Donati, Elena Procopio, Martino Montomoli, Renzo Guerrini, Massimo Zeviani, Sarah E Calvo, Vamsi K Mootha, Salvatore DiMauro, Ileana Ferrero, Michal Minczuk
Defects in nuclear-encoded proteins of the mitochondrial translation machinery cause early-onset and tissue-specific deficiency of one or more OXPHOS complexes. Here, we report a 7-year-old Italian boy with childhood-onset rapidly progressive encephalomyopathy and stroke-like episodes. Multiple OXPHOS defects and decreased mtDNA copy number (40%) were detected in muscle homogenate. Clinical features combined with low level of plasma citrulline were highly suggestive of mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome, however, the common m...
August 25, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28969510/acute-mitochondrial-myopathy-with-respiratory-insufficiency-and-motor-axonal-polyneuropathy
#3
Ying Zhou, Jian-Hua Yi, Li Liu, Xiaoping Wang, Liang Dong, Ai-Lian Du
Background Mitochondrial myopathies (MMs) are mainly presented with chronic muscle weakness and accompanied with other syndromes. MM with acute respiratory insufficiency is rare. Aims To reveal the clinical, pathological, and molecular characteristics of a life-threatening mitochondrial myopathy (MM). Methods Muscle biopsy and enzyme staining were performed in skeletal muscles. Mitochondrial DNA sequencing was analyzed and heteroplasmy were quantified by pyrosequencing. Results All three patients had tachycardia, acute lactic acidosis, dyspnea, and sudden severe muscle weakness...
October 2, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/28966145/how-important-is-the-co2-chemoreflex-for-the-control-of-breathing-environmental-and-evolutionary-considerations
#4
Joseph M Santin
Haldane and Priestley (1905) discovered that the ventilatory control system is highly sensitive to CO2. This "CO2 chemoreflex" has been interpreted to dominate control of resting arterial PCO2/pH (PaCO2/pHa) by monitoring PaCO2/pHa and altering ventilation through negative feedback. However, PaCO2/pHa varies little in mammals as ventilation tightly couples to metabolic demands, which may minimize chemoreflex control of PaCO2. The purpose of this synthesis is to (1) interpret data from experimental models with meager CO2 chemoreflexes to infer their role in ventilatory control of steady-state PaCO2, and (2) identify physiological causes of respiratory acidosis occurring normally across vertebrate classes...
September 28, 2017: Comparative Biochemistry and Physiology. Part A, Molecular & Integrative Physiology
https://www.readbyqxmd.com/read/28945122/pulmonary-hemodynamics-and-alveolar-oxygenation-in-healthy-dogs-anesthetized-with-propofol-or-isoflurane-during-one-lung-ventilation-in-a-closed-thoracic-experimental-model
#5
Beatriz P Floriano, Thomas A Trein, Juliana T Wagatsuma, Joana Z Ferreira, Renata H Pinho, Paulo S P Santos, Valéria N L S Oliva
OBJECTIVE To assess pulmonary hemodynamics and alveolar oxygenation in dogs anesthetized with propofol or isoflurane during one-lung ventilation (OLV) in a closed-thoracic experimental model. ANIMALS 6 healthy Beagles. PROCEDURES Dogs were anesthetized with each of 3 protocols (constant rate IV infusion of propofol [0.4 to 1.0 mg/kg/min], isoflurane at the minimum alveolar concentration [MAC], and isoflurane 1.5 MAC), with a 7-day washout period between anesthetic sessions. During each session, dogs were intubated with a double-lumen endotracheal tube, positioned in right lateral recumbency, and administered atracurium (0...
October 2017: American Journal of Veterinary Research
https://www.readbyqxmd.com/read/28942965/biallelic-c1qbp-mutations-cause-severe-neonatal-childhood-or-later-onset-cardiomyopathy-associated-with-combined-respiratory-chain-deficiencies
#6
René G Feichtinger, Monika Oláhová, Yoshihito Kishita, Caterina Garone, Laura S Kremer, Mikako Yagi, Takeshi Uchiumi, Alexis A Jourdain, Kyle Thompson, Aaron R D'Souza, Robert Kopajtich, Charlotte L Alston, Johannes Koch, Wolfgang Sperl, Elisa Mastantuono, Tim M Strom, Saskia B Wortmann, Thomas Meitinger, Germaine Pierre, Patrick F Chinnery, Zofia M Chrzanowska-Lightowlers, Robert N Lightowlers, Salvatore DiMauro, Sarah E Calvo, Vamsi K Mootha, Maurizio Moggio, Monica Sciacco, Giacomo P Comi, Dario Ronchi, Kei Murayama, Akira Ohtake, Pedro Rebelo-Guiomar, Masakazu Kohda, Dongchon Kang, Johannes A Mayr, Robert W Taylor, Yasushi Okazaki, Michal Minczuk, Holger Prokisch
Complement component 1 Q subcomponent-binding protein (C1QBP; also known as p32) is a multi-compartmental protein whose precise function remains unknown. It is an evolutionary conserved multifunctional protein localized primarily in the mitochondrial matrix and has roles in inflammation and infection processes, mitochondrial ribosome biogenesis, and regulation of apoptosis and nuclear transcription. It has an N-terminal mitochondrial targeting peptide that is proteolytically processed after import into the mitochondrial matrix, where it forms a homotrimeric complex organized in a doughnut-shaped structure...
October 5, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28932601/the-role-of-sodium-bicarbonate-in-the-management-of-some-toxic-ingestions
#7
REVIEW
Aibek E Mirrakhimov, Taha Ayach, Aram Barbaryan, Goutham Talari, Romil Chadha, Adam Gray
Adverse reactions to commonly prescribed medications and to substances of abuse may result in severe toxicity associated with increased morbidity and mortality. According to the Center for Disease Control, in 2013, at least 2113 human fatalities attributed to poisonings occurred in the United States of America. In this article, we review the data regarding the impact of systemic sodium bicarbonate administration in the management of certain poisonings including sodium channel blocker toxicities, salicylate overdose, and ingestion of some toxic alcohols and in various pharmacological toxicities...
2017: International Journal of Nephrology
https://www.readbyqxmd.com/read/28930639/50-years-of-research-in-ards-tidal-volume-selection-in-the-acute-respiratory-distress-syndrome
#8
Sarina K Sahetya, Jordi Mancebo, Roy G Brower
Mechanical ventilation (MV) is critical in the management of many patients with the acute respiratory distress syndrome (ARDS). However, MV can also cause ventilator-induced lung injury (VILI). The selection of an appropriate tidal volume is an essential part of a lung-protective MV strategy. Since the publication of a large randomized clinical trial demonstrating the benefit of lower tidal volumes, the use of tidal volumes of 6 mL/kg predicted body weight (PBW, based on sex and height) has been recommended in clinical practice guidelines...
September 20, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28923612/deceased-organ-donation-from-pediatric-donors-does-the-literature-really-help-us-implication-for-more-powerful-guidelines
#9
M Mojtabaee, F Sadegh Beigee, F Ghorbani
BACKGROUND: Brain-dead pediatric donors have always been the focus of attention because of the higher quality, utility, and possibility of their organ donation. However, donors under the age of 5 years always necessitate making more challenging management efforts, which are not clearly implied in most parts of the guidelines. METHODS: The data obtained from 79 brain-dead pediatric donors of the Organ Procurement Unit of Masih Daneshvari Hospital, Tehran, Iran, were assessed...
October 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28914359/changes-in-acid-base-and-ion-balance-during-exercise-in-normoxia-and-normobaric-hypoxia
#10
Olaf Lühker, Marc Moritz Berger, Alexander Pohlmann, Lorenz Hotz, Tilmann Gruhlke, Marcel Hochreiter
PURPOSE: Both exercise and hypoxia cause complex changes in acid-base homeostasis. The aim of the present study was to investigate whether during intense physical exercise in normoxia and hypoxia, the modified physicochemical approach offers a better understanding of the changes in acid-base homeostasis than the traditional Henderson-Hasselbalch approach. METHODS: In this prospective, randomized, crossover trial, 19 healthy males completed an exercise test until voluntary fatigue on a bicycle ergometer on two different study days, once during normoxia and once during normobaric hypoxia (12% oxygen, equivalent to an altitude of 4500 m)...
November 2017: European Journal of Applied Physiology
https://www.readbyqxmd.com/read/28905505/biallelic-variants-in-wars2-encoding-mitochondrial-tryptophanyl-trna-synthase-in-six-individuals-with-mitochondrial-encephalopathy
#11
Saskia B Wortmann, Sharita Timal, Hanka Venselaar, Liesbeth T Wintjes, Robert Kopajtich, René G Feichtinger, Carla Onnekink, Mareike Mühlmeister, Ulrich Brandt, Jan A Smeitink, Joris A Veltman, Wolfgang Sperl, Dirk Lefeber, Ger Pruijn, Vesna Stojanovic, Peter Freisinger, Francjan V Spronsen, Terry Gj Derks, Hermine E Veenstra-Knol, Johannes A Mayr, Agnes Rötig, Mark Tarnopolsky, Holger Prokisch, Richard J Rodenburg
Mitochondrial protein synthesis involves an intricate interplay between mitochondrial DNA encoded RNAs and nuclear DNA encoded proteins, such as ribosomal proteins and aminoacyl-tRNA synthases. Eukaryotic cells contain 17 mitochondria-specific aminoacyl-tRNA synthases. WARS2 encodes mitochondrial tryptophanyl-tRNA synthase (mtTrpRS), a homodimeric class Ic enzyme (mitochondrial tryptophan-tRNA ligase; EC 6.1.1.2). Here, we report six individuals from five families presenting with either severe neonatal onset lactic acidosis, encephalomyopathy and early death or a later onset, more attenuated course of disease with predominating intellectual disability...
September 14, 2017: Human Mutation
https://www.readbyqxmd.com/read/28893805/melas-syndrome-associated-with-a-new-mitochondrial-trna-val-gene-mutation-m-1616a-g
#12
Yuka Toyoshima, Yuji Tanaka, Kazuo Satomi
We describe the case of a 40-year-old-man with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome, with cardiomyopathy and severe heart failure. He had a mitochondrial transfer RNA (tRNA) mutation (m.1616A>G) of the (tRNA-Val) gene, and it was not found in MELAS syndrome ever before. The presence of this newly observed tRNA-Val mutation (m.1616A>G) may induce multiple respiratory chain enzyme deficiencies and contribute to MELAS syndrome symptoms that are associated with mitochondrial DNA (mtDNA) mutations...
September 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28878349/peptide-mediated-delivery-of-donor-mitochondria-improves-mitochondrial-function-and-cell-viability-in-human-cybrid-cells-with-the-melas-a3243g-mutation
#13
Jui-Chih Chang, Fredrik Hoel, Ko-Hung Liu, Yau-Huei Wei, Fu-Chou Cheng, Shou-Jen Kuo, Karl Johan Tronstad, Chin-San Liu
The cell penetrating peptide, Pep-1, has been shown to facilitate cellular uptake of foreign mitochondria but further research is required to evaluate the use of Pep-1-mediated mitochondrial delivery (PMD) in treating mitochondrial defects. Presently, we sought to determine whether mitochondrial transplantation rescue mitochondrial function in a cybrid cell model of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) disease. Following PMD, recipient cells had internalized donor mitochondria after 1 h, and expressed higher levels of normal mitochondrial DNA, particularly at the end of the treatment and 11 days later...
September 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28874237/-drowning-induced-hyperfibrinolytic-disseminated-intravascular-coagulation
#14
Andreas Lund Hattesen, Henrik Karsten Berg, Lars Folkersen, Anne-Mette Hvas
This article summarizes the current knowledge of drowning-induced hyperfibrinolytic disseminated intravascular coagulation. Drowning induces respiratory failure with ensuing cardiac arrest, hypoxaemia and ischaemia. A coagulopathy is induced by ischaemia, acidosis and hypothermia, and clinically the patient develops uncontrolled bleeding due to hyperfibrinolysis. A rapid diagnostic approach is required to recognize this hyperfibrinolytic state, since initiation of treatment with antifibrinolytics and fibrinogen concentrate may bring this life-threatening condition to cessation...
August 28, 2017: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/28847139/swimming-three-ice-miles-within-fifteen-hours
#15
Mirko Stjepanovic, Pantelis T. Nikolaidis, Beat Knechtle
Ice Mile swimming (1608 m in water of below 5 °Celsius) is becoming increasingly popular. This case study aimed to identify body core temperature and selected haematological and biochemical parameters before and after repeated Ice Miles. An experienced ice swimmer completed three consecutive Ice Miles within 15 h. Swim times, body core temperatures, and selected urinary and haematological parameters were recorded. Body core temperature reached its maximum between 5, 8 and 15 min after immersion (37.7°C, 38...
August 31, 2017: Chinese Journal of Physiology
https://www.readbyqxmd.com/read/28846437/oxygen-administration-improves-survival-but-worsens-cardiopulmonary-functions-in-chlorine-exposed-rats
#16
Obiefuna C Okponyia, Matthew D McGraw, Marilyn M Dysart, Rhonda B Garlick, Jacqueline S Rioux, Angela L Murphy, Gates B Roe, Carl W White, Livia A Veress
RATIONALE: Chlorine is a highly reactive gas that can cause significant injury when inhaled. Unfortunately, its use as a chemical weapon has increased in recent years. Massive chlorine inhalation can cause death within 4h of exposure. Survivors usually require hospitalization after massive exposure. No countermeasures exist for massive chlorine exposure, and supportive care measures lack controlled trials. METHODS: Adult rats were exposed to chlorine gas (LD58-67) in a whole-body exposure chamber, and given oxygen (0...
August 28, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28834137/evaluation-of-the-expected-ventilatory-response-to-metabolic-acidosis-in-chronic-hemodialysis-patients
#17
Marco Marano
INTRODUCTION: Expected pCO2 during metabolic acidosis should be computed to rule out superimposing respiratory acid-base disorders, the latter being featured by too high or too low pCO2 than expected. At least 4 different and inconsistent formulas exist. Based on the common practical rule, the reduction of pCO2 equals 1.2 multiplied by the reduction of serum bicarbonate ([HCO3-]), whereas pCO2 equals to the 2 digit after pH decimal point according to Fulop. Instead, based on Winters' formula, pCO2 equals to 1...
August 23, 2017: Hemodialysis International
https://www.readbyqxmd.com/read/28822390/physiologic-features-of-brain-death
#18
Eno-Obong Essien, Kristina Fioretti, Thomas M Scalea, Deborah M Stein
Brain death is known to be associated with physiologic derangements but their incidence is poorly described. Knowledge of the changes that occur during brain death is important for management of the potential organ donor. Thus, we sought to characterize the pathophysiology that occurs during brain death in patients with traumatic injuries. All brain-dead patients over a 10-year period were identified from the trauma registry at a level 1 urban trauma center. Patient demographics, injury characteristics, and clinical data for defining organ dysfunction were reviewed for the 24 hours surrounding brain-death declaration...
August 1, 2017: American Surgeon
https://www.readbyqxmd.com/read/28811944/successful-resuscitation-of-a-patient-with-life-threatening-metabolic-acidosis-by-hemodialysis-a-case-of-ethylene-glycol-intoxication
#19
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/28804536/combined-respiratory-chain-deficiency-and-uqcc2-mutations-in-neonatal-encephalomyopathy-defective-supercomplex-assembly-in-complex-iii-deficiencies
#20
René G Feichtinger, Michaela Brunner-Krainz, Bader Alhaddad, Saskia B Wortmann, Reka Kovacs-Nagy, Tatjana Stojakovic, Wolfgang Erwa, Bernhard Resch, Werner Windischhofer, Sarah Verheyen, Sabine Uhrig, Christian Windpassinger, Felix Locker, Christine Makowski, Tim M Strom, Thomas Meitinger, Holger Prokisch, Wolfgang Sperl, Tobias B Haack, Johannes A Mayr
Vertebrate respiratory chain complex III consists of eleven subunits. Mutations in five subunits either mitochondrial (MT-CYB) or nuclear (CYC1, UQCRC2, UQCRB, and UQCRQ) encoded have been reported. Defects in five further factors for assembly (TTC19, UQCC2, and UQCC3) or iron-sulphur cluster loading (BCS1L and LYRM7) cause complex III deficiency. Here, we report a second patient with UQCC2 deficiency. This girl was born prematurely; pregnancy was complicated by intrauterine growth retardation and oligohydramnios...
2017: Oxidative Medicine and Cellular Longevity
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