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    Flexible bronchoscopy and mechanical ventilation in managing Mounier-Kuhn syndrome: a case report.
    Sao Paulo Med J 2017 Apr 20. Epub 2017 Apr 20.
    MD. Professor, Department of Chest Diseases, Ankara University School of Medicine, Ankara, Turkey.
    Context:: Mounier-Kuhn syndrome is a rare congenital condition with distinct dilatation and diverticulation of the tracheal wall. The symptoms may vary and the treatment usually consists of support.

    Case Report:: The patient was a 60-year-old male with recurrent hospital admission. Read More

    Patient with multiple acyl-CoA dehydrogenation deficiency disease and FLAD1 mutations benefits from riboflavin therapy.
    Neuromuscul Disord 2017 Mar 9. Epub 2017 Mar 9.
    Research Programs Unit, Molecular Neurology, Biomedicum Helsinki, University of Helsinki, Helsinki, Finland; Department of Pediatric Neurology, Hospital for Children and Adolescence, Helsinki University Central Hospital, Helsinki, Finland.
    Multiple acyl-CoA dehydrogenation deficiency is genetically heterogenous metabolic disease with mutations in genes involved in electron transfer to the mitochondrial respiratory chain. Disease symptoms vary from severe neonatal form to late-onset presentation with metabolic acidosis, lethargy, vomiting, muscle pain and weakness. Riboflavin therapy has been shown to ameliorate diseases symptoms in some of these patients. Read More

    Role of Coma Acidosis Malaria Score in Patients with Severe Malaria among Indian Population: a Tertiary Care Center Experience.
    Eurasian J Med 2017 Feb;49(1):30-35
    Department of Pathology, Pt B.D. Sharma University of Health Sciences, Haryana, India.
    Objective: Malaria is a prime public health threat in developing countries like India. There is an unmet need of a simplified methodology for the purpose of triage and provision of intensive care to the severely infected patients in these areas.

    Materials And Methods: We did a prospective study in patients (n=60) admitted with severe malaria in a single tertiary care center in the state of Haryana, India. Read More

    The effect of the apneic period on the respiratory physiology of patients undergoing intubation in the emergency department.
    Am J Emerg Med 2017 Apr 2. Epub 2017 Apr 2.
    Department of Emergency Medicine, Lincoln Medical and Mental Health Center, Bronx, NY, USA.
    Objectives: We sought to examine the physiological impact the apneic period has on the respiratory physiology of patients undergoing intubation in the emergency department and whether DAO, the delivery of 15L oxygen by nasal cannula during apnea, can affect the development of respiratory acidosis.

    Methods: This was a prospective observational cohort study conducted at an urban academic level 1 trauma center. A convenience sample of 100 patients was taken. Read More

    [Extracorporeal CO2 Elimination (ECCO2R) for Hypercapnic Respiratory Failure: From Pathophysiology to Clinical Application].
    Pneumologie 2017 Apr 13;71(4):215-220. Epub 2017 Apr 13.
    ARDS und ECMO Zentrum Köln-Merheim, Lungenklinik, Abteilung Pneumologie, Intensiv- und Beatmungsmedizin, Köln.
    Extracorporeal CO2 removal (ECCO2R) is becoming an increasingly established treatment option for patients with acute severe hypercapnic respiratory failure. Technically, pumpless arterio-venous systems using the natural arterio-venous pressure gradient and also pump-driven veno-venous systems are available. Here, veno-venous ECCO2R has become the preferred technique, as settings for arterio-venous ECCO2R are restricted and side effects are more common with arterio-venous ECCO2R. Read More

    Lethal Bentazone Intoxication - A Report of Two Cases.
    J Forensic Sci 2017 Apr 13. Epub 2017 Apr 13.
    Toxicology Laboratory, Department of Forensic Medicine and Criminology, University of Zagreb, School of Medicine, Zagreb, Croatia.
    This study presents two cases of lethal bentazone poisonings, their clinical presentation, the course of the disease and the autopsy findings. The first is a 50-year-old male who had sprayed corn with a solution of bentazone and was admitted to the hospital with sweating, fever, nausea, vomiting of aqueous and hemorrhagic content, and bloody, watery stools. He was treated according to the symptoms including extracorporeal hemodialysis, but eventually suffered from multiorgan failure (acute respiratory failure, acute liver failure, coagulopathy, acute renal failure, metabolic acidosis, and gastrointestinal bleeding) and died 11. Read More

    Effects of dystocia on blood gas parameters, acid-base balance and serum lactate concentration in heavy draft newborn foals.
    J Equine Sci 2017 28;28(1):27-30. Epub 2017 Mar 28.
    Department of Applied Veterinary Medicine, Obihiro University of Agriculture and Veterinary Medicine, Hokkaido 080-8555, Japan; United Graduate School of Veterinary Sciences, Gifu University, Gifu 501-1193, Japan; Research Center for Global Agromedicine, Obihiro University of Agriculture and Veterinary Medicine, Hokkaido 080-8555, Japan.
    Dystocia is often lethal for neonatal foals; however, its clinicopathological features remain largely unknown. We investigated the effect of dystocia on the foal blood profile. Venous blood samples were collected from 35 foals (5 Percheron and 30 crossbreds between Percheron, Belgian, and Breton heavy draft horses) at 0 hr, 1 hr, 12 hr and 1 day after birth. Read More

    [The topical problems of the application of the TASER electroshock devices].
    Sud Med Ekspert 2017 ;60(2):57-64
    Federal state budgetary institution 'Russian Centre of Forensic Medical Expertise', Russian Ministry of Health, Moscow, Russia, 125284.
    The objective of the present study was the analysis of publications in the foreign medical literature concerning the problems of safety, clinical diagnostics, pathological morphology, and treatment of the patients subjected to the impact by various models of the TASER electroshock devices. The materials for this article were borrowed from the available Internet resources and libraries. The methods of scientific analysis were employed to follow up the dynamics of publication and to determine the number of publications on the issues of interest. Read More

    Acidosis, but Not Alkalosis, Affects Anaerobic Metabolism and Performance in a 4-km Time Trial.
    Med Sci Sports Exerc 2017 Apr 11. Epub 2017 Apr 11.
    1Sports Science Research Group, Department of Physical Education and Sports Science, CAV, Federal University of Pernambuco, Brazil; 2Endurance Performance Research Group (GEDAE-USP), School of Physical Education and Sport, University of São Paulo, Brazil; 3Institute of Sport, Exercise and Active Living, College of Sport and Exercise Science, Victoria University, Australia; 4School of Medical and Health Sciences, Edith Cowan University, Australia; 5Human Performance Research Group, Academic Department of Physical Education, Technological Federal University of Parana, Curitiba, Parana, Brazil.
    Purpose: To determine the effect of pre-exercise metabolic acidosis and alkalosis on power output (PO) and aerobic and anaerobic energy expenditure during a 4-km cycling time trial (TT).

    Methods: Eleven recreationally trained cyclists (VO2peak 54.1 ± 9. Read More

    Pharmacological, but not genetic, alteration of neural Epo modifies the CO2/H(+) central chemosensitivity in postnatal mice.
    Respir Physiol Neurobiol 2017 Apr 8;242:73-79. Epub 2017 Apr 8.
    Université Laval, Faculté de Médecine, Centre de Recherche Institut universitaire de cardiologie et de pneumologie de Québec, Département de Pédiatrie, Québec, QC, Canada; Molecular biology and Biotechnology Institute, Universidad Mayor de San Andres, La Paz, Bolivia. Electronic address:
    Cerebral erythropoietin (Epo) plays a crucial role for respiratory control in newborn rodents. We showed previously that soluble Epo receptor (sEpoR: an Epo antagonist) reduces basal ventilation and hypoxic hyperventilation at postnatal day 10 (P10) and in adult mice. However, at these ages (P10 and adulthood), Epo had no effect on central chemosensitivity. Read More

    Clinical Features, Molecular Heterogeneity, and Prognostic Implications in YARS2-Related Mitochondrial Myopathy.
    JAMA Neurol 2017 Apr 10. Epub 2017 Apr 10.
    Wellcome Trust Centre for Mitochondrial Research, Institute of Neuroscience, The Medical School, Newcastle University, Newcastle upon Tyne, England.
    Importance: YARS2 mutations have been associated with a clinical triad of myopathy, lactic acidosis, and sideroblastic anemia in predominantly Middle Eastern populations. However, the identification of new patients expands the clinical and molecular spectrum of mitochondrial disorders.

    Objectives: To review the clinical, molecular, and genetic features of YARS2-related mitochondrial disease and to demonstrate a new Scottish founder variant. Read More

    Effect of arterial puncture on ventilation.
    Heart Lung 2017 Apr 6. Epub 2017 Apr 6.
    Division of Pulmonary and Critical Care, John H Stroger Jr Hospital of Cook County, Chicago, IL, USA.
    Background: Clinicians frequently assume that during arterial puncture for measuring arterial blood gases patients hyperventilate from pain and anxiety. This assumption leads clinicians to falsely interpret a PaCO2 and pH near the upper limit of normal as a chronic respiratory acidosis corrected by an acute respiratory alkalosis.

    Objective: Determine if participants hyperventilate during arterial puncture from pain and anxiety. Read More

    An approach to clarify the effect mechanism of glyphosate on body malformations during embryonic development of zebrafish (Daino rerio).
    Chemosphere 2017 Apr 5;180:77-85. Epub 2017 Apr 5.
    Atatürk University, Fisheries Faculty, Aquaculture Department, Erzurum, Turkey; Atatürk University, Fisheries Faculty, Aquatic Biotechnology Laboratory, Erzurum, Turkey. Electronic address:
    In this study, it has been investigated that the effects of glyphosate, which is a herbicide within organophosphate and unselective widely used in agriculture on enzyme activity of carbonic anhydrase, production of reactive oxygen species, cell apoptosis and body morphology during the embryonic development of zebrafish. To this end, it has been treated embryo with 1, 5, 10 and 100 mg/L gyphosate at 96 h. The embryos treated with glyphosate from 4 hpf were evaluated by considering the survival rates, hatching rates, body malformations under the stereo microscope in 24, 48, 72 and 96th hours. Read More

    Extracorporeal CO2 removal by hemodialysis: in vitro model and feasibility.
    Intensive Care Med Exp 2017 Dec 7;5(1):20. Epub 2017 Apr 7.
    Department of Chemical Engineering, University of Pittsburgh, Pittsburgh, PA, USA.
    Background: Critically ill patients with acute respiratory distress syndrome and acute exacerbations of chronic obstructive pulmonary disease often develop hypercapnia and require mechanical ventilation. Extracorporeal carbon dioxide removal can manage hypercarbia by removing carbon dioxide directly from the bloodstream. Respiratory hemodialysis uses traditional hemodialysis to remove CO2 from the blood, mainly as bicarbonate. Read More

    Acidosis Increases the Susceptibility of Respiratory Epithelial Cells to Pseudomonas aeruginosa-Induced Cytotoxicity.
    Am J Physiol Lung Cell Mol Physiol 2017 Apr 6:ajplung.00524.2016. Epub 2017 Apr 6.
    Dartmouth Medical School
    Bacterial infection can lead to acidosis of the local microenvironment. While this is believed to exacerbate disease pathogenesis, the mechanisms by which changes in pH alter disease progression are poorly understood. In this report we test the hypothesis that acidosis enhances respiratory epithelial cell death in response to infection with Pseudomonas aeruginosa. Read More

    Carbon dioxide induced plasticity of branchial acid-base pathways in an estuarine teleost.
    Sci Rep 2017 Apr 5;7:45680. Epub 2017 Apr 5.
    University of Texas at Austin, University of Texas Marine Science Institute, Port Aransas, TX 78373, USA.
    Anthropogenic CO2 is expected to drive ocean pCO2 above 1,000 μatm by 2100 - inducing respiratory acidosis in fish that must be corrected through branchial ion transport. This study examined the time course and plasticity of branchial metabolic compensation in response to varying levels of CO2 in an estuarine fish, the red drum, which regularly encounters elevated CO2 and may therefore have intrinsic resilience. Under control conditions fish exhibited net base excretion; however, CO2 exposure resulted in a dose dependent increase in acid excretion during the initial 2 h. Read More

    Intrapartum and neonatal outcomes in singleton pregnancies following conception by assisted reproduction techniques.
    Aust N Z J Obstet Gynaecol 2017 Apr 3. Epub 2017 Apr 3.
    Mater Mothers' Hospital, South Brisbane, Queensland, Australia.
    Background: To characterise maternal demographics and ascertain whether clinically important differences exist in the intrapartum and neonatal outcomes associated with assisted reproductive technology (ART).

    Materials And Methods: A retrospective study was undertaken between January 2007 and December 2013 of all singleton pregnancies conceived via ART at a major tertiary unit in Brisbane, Australia. Intrapartum outcomes were mode of delivery and indication for emergency caesarean. Read More

    An East Asian Common Variant Vinculin P.Asp841His Was Associated With Sudden Unexplained Nocturnal Death Syndrome in the Chinese Han Population.
    J Am Heart Assoc 2017 Apr 3;6(4). Epub 2017 Apr 3.
    Division of Cardiovascular Medicine, Department of Medicine, University of Wisconsin, Madison, WI.
    Background: We have identified the cardiomyopathy-susceptibility gene vinculin (VCL) mutation M94I may account for a sudden unexplained nocturnal death syndrome (SUNDS) case. We addressed whether VCL common variant D841H is associated with SUNDS.

    Methods And Results: In 8 of 120 SUNDS cases, we detected an East Asian common VCL variant p. Read More

    Decrease in spectral entropy by low tidal volume ventilation-associated severe hypercapnia: a case report.
    Korean J Anesthesiol 2017 Apr 25;70(2):213-216. Epub 2016 Oct 25.
    Department of Anesthesiology and Pain Medicine, Yeungnam University School of Medicine, Daegu, Korea.
    Severe hypercapnia can be predicted by a decrease in cerebral electrical activity. The authors describe a sudden decrease in spectral entropy due to severe hypercapnia-induced respiratory acidosis in a patient with chronic pulmonary obstructive disease during lung resection. After two and a half hours of low tidal volume ventilation in the lateral position, the state entropy suddenly dropped from 45 to 7, without any changes in the effect-site concentration of propofol, end-tidal carbon dioxide (CO2) tension, oxygen saturation, or arterial pressure. Read More

    Effects of alfaxalone administered intravenously to healthy yearling loggerhead sea turtles (Caretta caretta) at three different doses.
    J Am Vet Med Assoc 2017 Apr;250(8):909-917
    OBJECTIVE To compare physiologic and anesthetic effects of alfaxalone administered IV to yearling loggerhead sea turtles (Caretta caretta) at 3 different doses. DESIGN Randomized crossover study. ANIMALS 9 healthy yearling loggerhead sea turtles. Read More

    Ultrasound to Detect Pressure-related Deep Tissue Injuries in Adults Admitted via the Emergency Department: A Prospective, Descriptive, Pilot Study.
    Ostomy Wound Manage 2017 Mar;63(3):36-46
    Biostatistics Unit, Feinstein Institute for Medical Research, North Shore Long Island Jewish Health System, Staten Island University Hospital-North, Staten Island, NY.
    Stage 4 pressure ulcers (PUs) start with tissue death at the level of the bone, also known as deep tissue injury (DTI). Studies have shown the appearance of DTI on the skin is delayed for several days after the original pressure-related injury to the deep soft tissues. Studies also suggest DTI can be seen using ultrasound (US) technology. Read More

    Clinical characteristics of zinc phosphide poisoning in Thailand.
    Ther Clin Risk Manag 2017 14;13:335-340. Epub 2017 Mar 14.
    Ramathibodi Poison Center.
    Objective: The objectives of this study were to describe the clinical characteristics and outcomes of poisoning by zinc phosphide, a common rodenticide in Thailand, and to evaluate whether these outcomes can be prognosticated by the clinical presentation.

    Materials And Methods: A 3-year retrospective cohort study was performed using data from the Ramathibodi Poison Center Toxic Exposure Surveillance System.

    Results: In total, 455 poisonings were identified. Read More

    Eleven years of children methadone poisoning in a referral center: A review of 453 cases.
    J Opioid Manag 2017 Jan/Feb;13(1):27-36
    Department of Epidemiology, School of Public Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran; Center for Educational Research in Medical Sciences, Tehran University of Medical Sciences, Tehran, Iran.
    Objectives: Methadone can be fatal due to respiratory failure even in little doses. This study aimed to evaluate the possible risk factors of death and/or intubation in methadone-poisoned children of 12 years or younger.

    Design: Retrospective routine database study. Read More

    Persistent pulmonary hypertension of the newborn.
    Semin Fetal Neonatal Med 2017 Mar 23. Epub 2017 Mar 23.
    Departments of Pediatrics and Obstetrics, Gynecology and Women's Health, Albert Einstein College of Medicine and the Children's Hospital at Montefiore, Bronx, NY, USA. Electronic address:
    Failure of the normal circulatory adaptation to extrauterine life results in persistent pulmonary hypertension of the newborn (PPHN). Although this condition is most often secondary to parenchymal lung disease or lung hypoplasia, it may also be idiopathic. PPHN is characterized by elevated pulmonary vascular resistance with resultant right-to-left shunting of blood and hypoxemia. Read More

    Continuous-Infusion Etomidate in a Patient Receiving Extracorporeal Membrane Oxygenation.
    J Pediatr Pharmacol Ther 2017 Jan-Feb;22(1):65-68
    We describe a 16-year-old, 65-kg male deployed on extracorporeal membrane oxygenation (ECMO) for refractory respiratory failure secondary to ingestion of multiple substances. During his ECMO course, standard sedative and analgesic strategies failed and alternative medications were used. The patient received various dosages of fentanyl, morphine, hydromorphone, clonidine patches, dexmedetomidine, lorazepam, methadone, pentobarbital, olanzapine, and propofol. Read More

    Maternal, Labor, Delivery, and Perinatal Outcomes Associated with Placental Abruption: A Systematic Review.
    Am J Perinatol 2017 Mar 22. Epub 2017 Mar 22.
    Maternal and Child Health Program, University of Maryland, College Park, Maryland.
    Objective Risk factors for placental abruption have changed, but there has not been an updated systematic review investigating outcomes. Methods We searched PubMed, EMBASE, Web of Science, SCOPUS, and CINAHL for publications from January 1, 2005 through December 31, 2016. We reviewed English-language publications reporting estimated incidence and/or risk factors for maternal, labor, delivery, and perinatal outcomes associated with abruption. Read More

    Hypercapnia Caused by a Therapeutic Dosage of Pregabalin in a Tetraplegic Patient With Cervical Spinal Cord Injury.
    Am J Phys Med Rehabil 2017 Mar 20. Epub 2017 Mar 20.
    From the Department of Physical Medicine and Rehabilitation, Veterans Health Service Medical Center, Seoul, Korea (KHD, EJC, H-EY); and Department of Physical Medicine and Rehabilitation, College of Medicine, Yeungnam University, Daegu, Korea (MCC).
    Pregabalin is often used for the treatment of neuropathic pain in patients with spinal cord injury (SCI). We reported a patient with C5 [S(C5/C6)] ASIA Impairment Scale C SCI due to cervical myelopathy who presented CO2 retention when taking a therapeutic dosage of pregabalin. An 88-year-old patient with cervical SCI was transferred to the department of physical medicine and rehabilitation. Read More

    Extracellular acidosis and very low [Na(+) ] inhibit NBCn1- and NHE1-mediated net acid extrusion from mouse vascular smooth muscle cells.
    Acta Physiol (Oxf) 2017 Mar 20. Epub 2017 Mar 20.
    Department of Biomedicine, Aarhus University, Aarhus, Denmark.
    Aim: The electroneutral Na(+) , HCO3- cotransporter NBCn1 and Na(+) /H(+) exchanger NHE1 regulate acid-base balance in vascular smooth muscle cells (VSMCs) and modify artery function and structure. Pathological conditions - notably ischaemia - can dramatically perturb intracellular (i) and extracellular (o) pH and [Na(+) ]. We examined effects of low [Na(+) ]o and pHo on NBCn1 and NHE1 activity in VSMCs of small arteries. Read More

    Effects of sevoflurane and clonidine on acid base status and long-term emotional and cognitive outcomes in spontaneously breathing rat pups.
    PLoS One 2017 20;12(3):e0173969. Epub 2017 Mar 20.
    Department of Physiology and Pharmacology, Sapienza University of Rome, Rome, Italy.
    Background: Numerous experiments in rodents suggest a causative link between exposure to general anaesthetics during brain growth spurt and poor long-lasting neurological outcomes. Many of these studies have been questioned with regard of their translational value, mainly because of extremely long anaesthesia exposure. Therefore, the aim of the present study was to assess the impact of a short sevoflurane anaesthesia, alone or combined with clonidine treatment, on respiratory function in spontaneously breathing rat pups and overall effects on long-lasting emotional and cognitive functions. Read More

    Benefits of 21% Oxygen Compared with 100% Oxygen for Delivery of Isoflurane to Mice (Mus musculus) and Rats (Rattus norvegicus).
    J Am Assoc Lab Anim Sci 2017 Mar;56(2):148-154
    Unit for Laboratory Animal Medicine, Department of Pathology, University of Michigan, Ann Arbor, Michigan.
    At research institutions, isoflurane delivered by precision vaporizer to a face mask is the standard for rodent surgery and for procedures with durations that exceed a few minutes. Pure oxygen is often used as the carrier gas for isoflurane anesthesia, despite documented complications from long-term 100% oxygen use in humans and known occupational safety risks. We therefore examined the effect of anesthetic delivery gas on physiologic variables in mice and rats. Read More

    Intravenous 15% isoflurane lipid nanoemulsion for general anesthesia in dogs.
    Vet Anaesth Analg 2017 Jan 7. Epub 2017 Jan 7.
    Faculty of Agronomy and Veterinary Medicine, Central Institute of Sciences, University of Brasilia, Brasilia, DF, Brazil.
    Objective: To investigate the efficacy of a new intravenous (IV) nanoemulsified isoflurane formulation for maintenance of general anesthesia in dogs.

    Study Design: Prospective, crossover, experimental study.

    Animals: Seven healthy, mature, mixed-breed dogs, three male and four female, weighing 11. Read More

    [Severe imported malaria in adults: a retrospective study of thirteen cases admitted to the Intensive Care Unit in Marrakech].
    Pan Afr Med J 2016 21;25:179. Epub 2016 Nov 21.
    Service de Parasitologie Mycologie, Hôpital Militaire Avicenne Marrakech, Maroc.
    Imported malaria is being seen with increasing frequency in non-endemic areas. Severe forms represent 10% of cases of Plasmodium falciparum malaria. In Morocco, more than 50 cases of malaria occur each year, 83% of which with Plasmodium falciparum malaria. Read More

    Acid-base disturbances in nephrotic syndrome: analysis using the CO2/HCO3 method (traditional Boston model) and the physicochemical method (Stewart model).
    Clin Exp Nephrol 2017 Mar 13. Epub 2017 Mar 13.
    Division of Nephrology and Rheumatology, Department of Internal Medicine, Aichi Medical University School of Medicine, Nagakute, Japan.
    Background: The Stewart model for analyzing acid-base disturbances emphasizes serum albumin levels, which are ignored in the traditional Boston model. We compared data derived using the Stewart model to those using the Boston model in patients with nephrotic syndrome.

    Methods: Twenty-nine patients with nephrotic syndrome and six patients without urinary protein or acid-base disturbances provided blood and urine samples for analysis that included routine biochemical and arterial blood gas tests, plasma renin activity, and aldosterone. Read More

    White sturgeon (Acipenser transmontanus) acid-base regulation differs in response to different types of acidoses.
    J Comp Physiol B 2017 Mar 11. Epub 2017 Mar 11.
    Department of Zoology, University of British Columbia, Vancouver, BC, Canada.
    White sturgeon (Acipenser transmontanus) completely protect intracellular tissue pH (pHi) despite large reductions in extracellular (blood) pH (pHe), termed preferential pHi regulation, in response to elevated environmental PCO2 (hypercarbia) and in general appear to be relatively resilient to stressors. Preferential pHi regulation is thought to be associated with hypercarbia tolerance in general, but has also recently been observed to protect pHi against metabolic acidoses induced by exhaustive exercise and anoxia in a tropical air breathing catfish. We hypothesized that preferential pHi regulation may also be a general strategy of acid-base regulation in sturgeon. Read More

    Guillain-Barré Syndrome Presenting with Sinus Node Dysfunction and Refractory Shock.
    Am J Case Rep 2017 Mar 11;18:251-254. Epub 2017 Mar 11.
    Sands-Constellation Heart Institute, Rochester General Hospital, Rochester, NY, USA.
    BACKGROUND Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy that is usually associated with preceding respiratory or gastrointestinal infection and has the hallmark manifestation of ascending flaccid paralysis. We report an atypical presentation of GBS. CASE REPORT A 76-year-old male presented with acute onset of diaphoresis and altered mental status. Read More

    Lifetime exercise intolerance with lactic acidosis as key manifestation of novel compound heterozygous ACAD9 mutations causing complex I deficiency.
    Neuromuscul Disord 2017 May 14;27(5):473-476. Epub 2017 Feb 14.
    Department of Neurology, Medical Academy, Lithuanian University of Health Sciences, Kaunas, Lithuania. Electronic address:
    We report a 36-year-old female having lifetime exercise intolerance and lactic acidosis with nausea associated with novel compound heterozygous Acyl-CoA dehydrogenase 9 gene (ACAD9) mutations (p.Ala390Thr and p.Arg518Cys). Read More

    A Rare Cause of Neonatal Hemolytic Anemia: Glutathione Synthetase Deficiency.
    J Pediatr Hematol Oncol 2017 Mar 6. Epub 2017 Mar 6.
    *Pediatric Nutrition and Metabolism Clinic †Pediatric Hematology and Oncology Clinic ‡Genetic Clinic §Neonatalogy Clinic ∥Pediatric Nefrology Clinic, Health Sciences University, Kayseri Training and Research Hospital, Kayseri, Turkey.
    Background: Isolated hemolysis or hemolytic anemia and 5-oxoprolinuria are 2 distinct medical conditions in the clinical spectrum associated with glutathione synthetase deficiency.

    Clinical Observation: A 1-day-old female baby presented with anemia and respiratory distress. Her hemoglobin level was 9. Read More

    JSA guideline for the management of malignant hyperthermia crisis 2016.
    • Authors:
    J Anesth 2017 Apr 28;31(2):307-317. Epub 2017 Feb 28.
    Malignant hyperthermia (MH) can be fatal if the crisis is not appropriately treated. It is an inherited disease usually triggered by the administration of volatile inhalational anesthetics and/or succinylcholine, a muscle relaxant. In a patient with suspected MH, the mechanism of calcium release from storage in the sarcoplasmic reticulum in the skeletal muscle is abnormally accelerated. Read More

    Effects of three methadone doses combined with acepromazine on sedation and some cardiopulmonary variables in dogs.
    Vet Anaesth Analg 2017 Jan 7. Epub 2017 Jan 7.
    School of Veterinary Medicine, University of Vila Velha, Vila Velha, ES, Brazil. Electronic address:
    Objective: To evaluate the sedative and cardiopulmonary effects of three methadone doses, combined with acepromazine, in dogs.

    Study Design: Prospective, randomized, complete block study.

    Animals: Six healthy, adult, cross-bred dogs weighing 17. Read More

    Vinculin variant M94I identified in sudden unexplained nocturnal death syndrome decreases cardiac sodium current.
    Sci Rep 2017 Feb 20;7:42953. Epub 2017 Feb 20.
    Division of Cardiovascular Medicine, Department of Medicine, University of Wisconsin, Madison, WI 53792, USA.
    Sudden unexplained nocturnal death syndrome (SUNDS) remains an autopsy negative disorder with unclear etiology. Vinculin (VCL) was linked to sudden arrhythmia death in VCL knockout mice prior to the appearance of cardiomyopathy. We hypothesized VCL mutations underlie risk for SUNDS. Read More

    The influence of mechanical ventilation on physiological parameters in ball pythons (Python regius).
    Comp Biochem Physiol A Mol Integr Physiol 2017 May 10;207:30-35. Epub 2017 Feb 10.
    Centre for Zoo and Wildlife Health, Copenhagen Zoo, Roskildevej 32, 2000 Frederiksberg, Denmark.
    Mechanical ventilation is widely recommended for reptiles during anesthesia, and while it is well-known that their low ectothermic metabolism requires much lower ventilation than in mammals, very little is known about the influence of ventilation protocol on the recovery from anesthesia. Here, 15 ball pythons (Python regius) were induced and maintained with isoflurane for 60min at one of three ventilation protocols (30, 125, or 250mlmin(-1)kg(-1) body mass) while an arterial catheter was inserted, and ventilation was then continued on 100% oxygen at the specified rate until voluntary extubation. Mean arterial blood pressure and heart rate (HR) were measured, and arterial blood samples collected at 60, 80, 180min and 12 and 24h after intubation. Read More

    Dynamics of acid-base and hematological regulation in day 15 chicken embryos (Gallus gallus domesticus) exposed to graded hypercapnia and hypoxia.
    Respir Physiol Neurobiol 2017 May 9;239:55-63. Epub 2017 Feb 9.
    Developmental Integrative Biology, Department of Biological Sciences, University of North Texas, 1155 Union Circle #305220, Denton, TX 76203, USA.
    Most experiments examining acid-base regulation of chicken embryos have employed static, single time point measurements rather than dynamic, multiple time point measurements that might reveal additional components of developing acid-base regulation. Thus, we studied blood acid-base balance and hematology of day 15 chicken embryos under 24h exposure to graded hypercapnia (1%-7% CO2) accompanied by graded hypoxia (20% O2 down to 13% O2). Across all hypercapnic/hypoxic environments, respiratory acidosis occurred 2h after exposure in proportion to the magnitude of hypercapnia. Read More

    [Systemic lupus erythematosus presenting as severe alveolar hypoventilation and the shrinking lung syndrome].
    Rev Mal Respir 2017 Feb 8. Epub 2017 Feb 8.
    Service des maladies respiratoires, unité Inserm 903, hôpital Maison-Blanche, CHU de Reims, 45, rue Cognacq-Jay, 51092 Reims cedex, France. Electronic address:
    Introduction: The shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus.

    Case Report: A 69-year-old man presented with exertional dyspnoea, muscle weakness, and weight loss of 15kg in 6months. Pulmonary function tests revealed a restrictive lung disorder, with a dramatic decrease in maximal inspiratory pressure (17% of theoretical value), and alveolar hypoventilation (pH 7. Read More

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

    Hereditary myopathies with early respiratory insufficiency in adults.
    Muscle Nerve 2017 Feb 9. Epub 2017 Feb 9.
    Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
    Introduction: Hereditary myopathies with early respiratory insufficiency as a predominant feature of the clinical phenotype are uncommon and underestimated in adults.

    Methods: We reviewed the clinical and laboratory data of patients with hereditary myopathies who demonstrated early respiratory insufficiency before the need for ambulatory assistance. Only patients with disease-causing mutations or a specific histopathological diagnosis were included. Read More

    Blast-Burn Combined Injury Followed by Immediate Seawater Immersion Induces Hemodynamic Changes and Metabolic Acidosis: An Experimental Study in a Canine Model.
    Clin Lab 2016 Jul;62(7):1193-1199
    Background: Severe burn-blast combined injury often causes systematic dysfunction related to blood coagulation, anticoagulation, and fibrinolysis. However, studies of burn-blast combined injury followed by immersion in seawater are rarely reported.

    Methods: A canine burn-blast combined injury model was established including blast injury caused by explosion immediately followed by burning with gelatinized gasoline flames. Read More

    Pulmonary gas exchange and acid-base status during immobilisation of black rhinoceroses (Diceros bicornis) in Zimbabwe.
    J S Afr Vet Assoc 2016 Dec 2;87(1):e1-e9. Epub 2016 Dec 2.
    Department of Clinical Sciences, Swedish University of Agricultural Sciences.
    When immobilising wildlife, adverse side effects can include hypoxaemia, acidosis and hypertension. Pulmonary gas exchange and acid-base status were evaluated during immobilisation of 25 free-ranging and one boma-held black rhinoceros (Diceros bicornis) in Zimbabwe. The effect of different body positions on arterial oxygenation was evaluated. Read More

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

    Materials And Methods: Whole exome sequencing was performed as part of a "diagnostic odyssey" for suspected mitochondrial condition in 2 patients, presenting congenital cataracts, progressive encephalomyopathy and hypotrophy and detected unreported compound heterozygous variants in GFER. Read More

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

    Bilateral putaminal necrosis in a comatose patient with metabolic acidosis.
    Indian J Crit Care Med 2016 Dec;20(12):745-748
    Department of Neurology, Institute of Neurological Sciences, Apollo Hospitals, Hyderabad, Telangana, India.
    We present a case of acute-onset coma in a young woman, associated with metabolic acidosis, respiratory distress, and hypotension. Magnetic resonance imaging of the brain done on day 2 of admission showed features of bilateral putaminal necrosis. History of methanol ingestion, though not forthcoming at admission, was confirmed later after the patient regained consciousness. Read More

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