Mycopathologia 2017 Jun 23. Epub 2017 Jun 23.

Hospital Universitario de Burgos, Burgos, Spain.

Mucormycosis is a rare infection caused by members included in the subphylum Mucoromycotina. Characterized by the histopathological hallmark of angioinvasion, these infections affect most often patients with certain underlying conditions carrying immunosuppression (haematological neoplasias, diabetic ketoacidosis and other forms of acidosis, and iron overload) or immunocompetent patients with traumatic mucocutaneous barriers breakdown and direct inoculation of the mould. A case is presented in which a rare underlying condition (non-ketotic acidosis) and a rare cause of cutaneous injuries collide. Read More

Biochem J 2017 Jun 23. Epub 2017 Jun 23.

Research and Development, Mahidol University, Division of Medical Molecular Biology and Biotec-Medical Biotechnology unit, Faculty of Medicine Siriraj Hospital, Bangkok, 10700, Thailand

Mutations of solute carrier family 4 member 1 ( SLC4A1 ) gene encoding kidney anion (chloride/bicarbonate ion) exchanger 1 (kAE1) can cause genetic distal renal tubular acidosis (dRTA). Different SLC4A1 mutations give rise to mutant kAE1 proteins with distinct defects in protein trafficking. The mutant kAE1 protein may be retained in endoplasmic reticulum (ER), Golgi apparatus, or mis-targeted to the apical membrane, failing to display its function at the baso-lateral membrane. Read More

Am J Kidney Dis 2017 Jun 21. Epub 2017 Jun 21.

Department of Medicine, Duke University School of Medicine, Durham, NC; Duke Clinical Research Institute, Duke University School of Medicine, Durham, NC; Department of Medicine, Durham Veterans Affairs Medical Center, Durham, NC. Electronic address:

Background: The kidneys maintain acid-base homeostasis through excretion of acid as either ammonium or as titratable acids that primarily use phosphate as a buffer. In chronic kidney disease (CKD), ammoniagenesis is impaired, promoting metabolic acidosis. Metabolic acidosis stimulates phosphaturic hormones, parathyroid hormone (PTH) and fibroblast growth factor 23 (FGF-23) in vitro, possibly to increase urine titratable acid buffers, but this has not been confirmed in humans. Read More

Rev Bras Ginecol Obstet 2017 Jun 23. Epub 2017 Jun 23.

Gynecology and Obstetrics Department, Hospital Geral, Fundação Universidade de Caxias do Sul, Caxias do Sul, Rio Grande do Sul, RS, Brazil.

Purpose To assess the impact of pre-pregnancy obesity (body mass index [BMI] ≥ 30 kg/m(2)) on the gestational and perinatal outcomes. Methods Retrospective cohort study of 731 pregnant women with a BMI ≥ 30 kg/m(2) at the first prenatal care visit, comparing them with 3,161 women with a BMI between 18.5 kg/m(2) and 24. Read More

Am J Physiol Gastrointest Liver Physiol 2017 Jun 22:ajpgi.00110.2017. Epub 2017 Jun 22.

Heidelberg University

Small for size and flow syndrome (SFSF) is one of the most challenging complications following extended hepatectomy (EH). After EH, hepatic artery flow decreases and portal vein flow increases per 100 gram remnant liver volume (RLV). This causes hypoxia followed by metabolic acidosis. Read More

J Trauma Acute Care Surg 2017 Jun 20. Epub 2017 Jun 20.

1Department of Emergency and Critical Care Medicine, School of Medicine, Keio University, Tokyo, Japan2The Center for Molecular Hydrogen Medicine, Keio Advanced Research Centers3Department of Cardiology, School of Medicine, Keio University, Tokyo, Japan.

Background: Mortality of hemorrhagic shock primarily depends on whether or not the patients can endure the loss of circulating volume until radical treatment is applied. We investigated whether hydrogen (H2) gas inhalation would influence the tolerance to hemorrhagic shock and improve survival.

Methods: Hemorrhagic shock was achieved by withdrawing blood until the mean arterial blood pressure reached 30-35 mmHg. Read More

Medicine (Baltimore) 2017 Jun;96(25):e7133

Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Republic of Korea.

Rationale: Recently tenofovir disoproxil fumarate (TDF) has been widely used as a first-line therapy for chronic hepatitis B (CHB) infection. Although TDF demonstrates successful viral suppression, the possibility of renal failure and lactic acidosis has been proposed with TDF administration, especially in human immunodeficiency virus co-infected patients. However, TDF induced lactic acidosis has never been reported in CHB mono-infected patients. Read More

Magn Reson Med 2017 Jun 21. Epub 2017 Jun 21.

Division of MR Research, Department of Radiology, Johns Hopkins University, Baltimore, Maryland, USA.

Purpose: To quantify amide protein transfer (APT) effects in acidic ischemic lesions and assess the spatial-temporal relationship among diffusion, perfusion, and pH deficits in acute stroke patients.

Methods: Thirty acute stroke patients were scanned at 3 T. Quantitative APT (APT(#) ) effects in acidic ischemic lesions were measured using an extrapolated semisolid magnetization transfer reference signal technique and compared with commonly used MTRasym (3. Read More

Sci China Life Sci 2017 Jun 16. Epub 2017 Jun 16.

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

Mitochondrial disease was a clinically and genetically heterogeneous group of diseases, thus the diagnosis was very difficult to clinicians. Our objective was to analyze clinical and genetic characteristics of children with mitochondrial disease in China. We tested 141 candidate patients who have been suspected of mitochondrial disorders by using targeted next-generation sequencing (NGS), and summarized the clinical and genetic data of gene confirmed cases from Neurology Department, Beijing Children's Hospital, Capital Medical University from October 2012 to January 2015. Read More

Clin Kidney J 2017 Feb 31;10(1):135-140. Epub 2016 Aug 31.

Division of Nephrology and Vascular Biology Research Center, Department of Medicine, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, USA.

Distal renal tubular acidosis caused by missense mutations in kidney isoform of anion exchanger 1 (kAE1/SLC4A1), the basolateral membrane Cl(-)/HCO3(-) exchanger of renal alpha-intercalated cells, has been extensively investigated in heterologous expression systems but rarely in human kidneys. The preferential apical localization of distal renal tubular acidosis (dRTA)-associated kAE1 mutants R901X, G609R and M909T in cultured epithelial monolayers has not been examined in human kidney. Here, we present kidney tissues from dRTA-affected siblings heterozygous for kAE1 G609R, characterized by predominant absence rather than mistargeting of kAE1 in intercalated cells. Read More

Rinsho Shinkeigaku 2017 Jun 22. Epub 2017 Jun 22.

Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University.

Cochrane Database Syst Rev 2017 Jun 21;6:CD009569. Epub 2017 Jun 21.

Department of Hepatobiliary Surgery, Yongchuan Hospital, Chongqing Medical University, No. 439, Quxuanhua Road, Chongqing, China, 402160.

Background: This is an update of the review published in 2013.Laparoscopic surgery is now widely performed to treat various abdominal diseases. Currently, carbon dioxide is the most frequently used gas for insufflation of the abdominal cavity (pneumoperitoneum). Read More

Intern Emerg Med 2017 Jun 20. Epub 2017 Jun 20.

UOC Pronto Soccoso e Medicina d'Urgenza, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122, Milan, Italy.

Int J STD AIDS 2017 Jan 1:956462417695995. Epub 2017 Jan 1.

5 Department of Pharmacy, Virginia Commonwealth University Health System, Richmond, VA, USA.

An analysis of the interaction between dolutegravir and metformin was conducted in the HIV ambulatory clinic setting. This was a multicenter, retrospective case series evaluating adult, HIV-infected patients concurrently prescribed dolutegravir and metformin. Historical electronic medical records were utilized to collect case-specific data. Read More

Oncol Res 2017 Jun 19. Epub 2017 Jun 19.

Julius-Bernstein-Institut für Physiologie, Universität Halle-Wittenberg, Germany.

Carbonic anhydrase IX has emerged as a promising target for cancer therapy. It is highly up-regulated in hypoxic regions and mediates pH regulation critical for tumor cell survival as well as extracellular acidification of the tumor microenvironment which promotes tumor aggressiveness via various mechanisms, such as augmenting metastatic potential. Therefore, the aim of this study was to analyze the complex interdependency between CA IX and the tumor microenvironment in prostate tumor cells in concern of potential therapeutic implications. Read More

Paediatr Anaesth 2017 Jun 20. Epub 2017 Jun 20.

Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada.

Background: Intraoperative hypercapnia and acidosis have been associated with thoracoscopic repair of both congenital diaphragmatic hernia and esophageal atresia/tracheoesophageal fistula.

Aim: The aim of the present study was to investigate whether thoracoscopic repair of congenital diaphragmatic hernia or esophageal atresia/tracheoesophageal fistula was associated with acidosis and hypercapnia in a large group of neonates, and to analyze the effects of acidosis and hypercapnia on early postoperative outcomes.

Methods: We reviewed the charts of neonates who underwent open or thoracoscopic congenital diaphragmatic hernia or esophageal atresia/tracheoesophageal fistula repair (2004-2014). Read More

J Trop Med 2017 24;2017:7868535. Epub 2017 May 24.

Malaria Group, Faculty of Medicine, University of Antioquia, Calle 70, No. 52-21, Medellin, Colombia.

Background: Urinalysis is a poorly reviewed diagnostic tool in malaria patients; its application can show the presence of severe malaria.

Methods: Urinalysis was performed in a total of 620 patients diagnosed with malaria by thick blood smear; complications were classified according to WHO major criteria for severity and minor criteria according to the Colombian malaria guideline.

Results: Severe or moderate clinical complications were diagnosed in 31. Read More

J Am Soc Nephrol 2017 Jun 19. Epub 2017 Jun 19.

Wake Forest School of Medicine; and Section of General Internal Medicine, Winston Salem, North Carolina

Comp Biochem Physiol B Biochem Mol Biol 2017 Jun 16. Epub 2017 Jun 16.

Department of Wildlife & Fisheries Sciences, Texas A&M University, 210 Nagle Hall, 2258 TAMU, College Station, TX 77843, USA.

The handheld point of care analyzer is a quick and feasible option to obtain hematology data from individuals. The iSTAT-1® was used to evaluate select venous blood analytes obtained via jugular venipuncture from 238 passerine birds from South Texas. These data were used to assess the health of birds in the area while taking into consideration life history (migratory or sedentary), locale, seasonality, sex, and age. Read More

J Matern Fetal Neonatal Med 2017 Jun 19:1-20. Epub 2017 Jun 19.

a Department of Obstetrics and Gynecology "L. Mangiagalli" , Fondazione IRCCS 'Ca' Granda' - Ospedale Maggiore Policlinico , Milan , Italy.

Purpose: To identify obstetric risk factors of delivering a neonate with poor neonatal adaptation at birth.

Material And Methods: Nested-case control study. Poor neonatal adaptation was defined for presence of at least: umbilical cord artery pH < 7. Read More

Oncol Rep 2017 Jun 13. Epub 2017 Jun 13.

Department of Obstetrics and Gynecology, Medical Center - University of Freiburg, Freiburg, Germany.

Due to their post-transcriptional regulatory impact on gene expression, microRNAs (miRNA, miRs) influence decisively cellular processes of differentiation, proliferation and apoptosis. In oncogenic pathways various miRNAs exert either oncogenic or tumor suppressor activities in a stage-specific manner. Dysregulation of miRNA expression pattern has been associated with several human cancers including endometrial cancer (EC). Read More

Eur J Pharm Sci 2017 Jun 13;106:313-327. Epub 2017 Jun 13.

Key Lab of Drug Metabolism and Pharmacokinetics, China Pharmaceutical University, Nanjing, Jiangsu, China. Electronic address:

Entecavir (ETV) is a superior nucleoside analogue used to treat hepatitis B virus (HBV) infection. Although its advantages over other agents include low viral resistance and the elicitation of a sharp decrease in HBV DNA, adverse effects such as hepatic steatosis, hepatic damage and lactic acidosis have also been reported. Glycyrrhizin has long been used as hepato-protective medicine. Read More

Best Pract Res Clin Anaesthesiol 2017 Mar 8;31(1):57-68. Epub 2017 Jan 8.

Duke University Medical Center, Durham, NC, USA. Electronic address:

Hypotension commonly occurs in parturients undergoing cesarean delivery under spinal anesthesia. This leads to maternal and neonatal adverse outcomes, including maternal nausea and vomiting and fetal acidosis, and might even lead to cardiovascular collapse if not treated. Arterial dilatation and reduction in systemic vascular resistance are the major contributors to spinal-induced hypotension. Read More

Inflammation 2017 Jun 15. Epub 2017 Jun 15.

Institute of Physiological Chemistry, University Hospital Essen, Essen, Germany.

Melatonin improves survival and functional impairment including hemolysis, thrombocytopenia, and hypotension when administered in a prophylactic manner or early after initiation of sepsis or endotoxemia. In the present study, melatonin was given not before first symptoms of systemic inflammation became manifest. Lipopolysaccharide was infused at a rate of 0. Read More

Case Rep Crit Care 2017 23;2017:6148350. Epub 2017 May 23.

Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Ave, Boston, MA 02215, USA.

Background: Elevated lactate levels in critically ill patients are most often thought to be indicative of relative tissue hypoxia or type A lactic acidosis. Shock, severe anemia, and thromboembolic events can all cause elevated lactate due to tissue hypoperfusion, as well as the mitochondrial dysfunction thought to occur in sepsis and other critically ill states. Malignancy can also lead to elevation in lactate, a phenomenon described as type B lactic acidosis, which is much less commonly encountered in the critically ill. Read More

Case Rep Cardiol 2017 23;2017:9473917. Epub 2017 May 23.

Department of Cardiology, Japanese Red Cross Ashikaga Hospital, Ashikaga, Japan.

We report a case with 46-year-old man diagnosed with mitochondrial cardiomyopathy in the dilated phase of hypertrophic cardiomyopathy. Since cardiac magnetic resonance imaging, beta-methyl-p-(123)I-iodophenyl-pentadecanoic myocardial scintigraphy, and positron emission tomography/computed tomography revealed no remarkable findings, we performed electron microscopic examination, which aided in diagnosing mitochondrial cardiomyopathy. Muscle biopsy was also compatible with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes and DNA analysis also concluded it. Read More

SAGE Open Med Case Rep 2017 6;5:2050313X17713149. Epub 2017 Jun 6.

Interfaith Medical Center, Brooklyn, NY, USA.

Objective: We present a rare case of pernicious anemia presented as multi-organ dysfunction syndrome, later found to have pseudo-thrombotic thrombocytopenic purpura.

Methods: An 86-year-old female presented with respiratory distress, altered mental status, acute renal failure and was intubated in emergency room. She was found to have severe anemia, thrombocytopenia, high lactate, high lactate dehydrogenase and low haptoglobin. Read More

Tumour Biol 2017 Jun;39(6):1010428317705750

1 Department of Pharmacology, School of Basic Medical Sciences, Anhui Medical University, Hefei, P.R. China.

Acid-sensing ion channels, a proton-gated cation channel, can be activated by low extracellular pH and involved in pathogenesis of some tumors such as glioma and breast cancer. However, the role of acid-sensing ion channels in the growth of lung cancer cell is unclear. In this study, we investigated the expression of acid-sensing ion channels in human lung cancer cell line A549 and their possible role in proliferation and migration of A549 cells. Read More

Ann Intensive Care 2017 Dec 14;7(1):66. Epub 2017 Jun 14.

CHU d'Angers, 4 rue Larrey, 49000, Angers, France.

Background: According to international guidelines, volume expansion with crystalloids is the first-line treatment for hemodynamic management in patients with severe sepsis or septic shock. Compared to balanced crystalloids, 0.9% sodium chloride (0. Read More

Clin Kidney J 2017 Jun 28;10(3):301-304. Epub 2017 Mar 28.

Division of Nephrology, Kaiser Permanente, Southern California Permanente Medical Group, Fontana, CA, USA.

Metformin is one of the oldest and most widely prescribed antidiabetic medicines worldwide. It is the only such medicine that has shown a reduction of cardiovascular mortality in diabetes mellitus type 2. Since many diabetic patients have chronic kidney disease, its use is often curtailed by practitioners due to fear of lactic acidosis and the US Food and Drug Administration (FDA) warnings that, until recently, had been in place for decades. Read More

Am J Respir Cell Mol Biol 2017 Jun 14. Epub 2017 Jun 14.

Tokyo Medical University Ibaraki Medical Center , Department of Respiratory Medicine, Ibaraki, Japan ;

Obesity is a major risk factor for the development of obstructive sleep apnea (OSA) and obesity hypoventilation syndrome (OHS), which manifest as intermittent hypercapnia and sustained plus intermittent hypercapnia, respectively. In this study, we investigated whether CO2 affects adipocyte differentiation (adipogenesis) and maturation (hypertrophy). Human visceral or subcutaneous preadipocytes were grown to confluence and then induced to differentiate to adipocytes under hypocapnia, normocapnia, and hypercapnia with or without hypoxia. Read More

Biomed Res Int 2017 22;2017:5383574. Epub 2017 May 22.

Institute of Physiological Chemistry, University Hospital Essen, University of Duisburg-Essen, Hufelandstrasse 55, Essen, 45147 North Rhine-Westphalia, Germany.

Background: Postoperative acid-base imbalances, usually acidosis, frequently occur after cardiac surgery. In most cases, the human body, not suffering from any severe preexisting illnesses regarding lung, liver, and kidney, is capable of transient compensation and final correction. The aim of this study was to correlate the appearance of postoperatively occurring acidosis with renal ammonium excretion. Read More

J Anim Physiol Anim Nutr (Berl) 2017 Jun 13. Epub 2017 Jun 13.

Institute for Veterinary Physiology, University of Leipzig, Leipzig, Germany.

The carbon dioxide released and dissolved in rumen fluid may easily permeate across the epithelial cell membrane. Thus, we hypothesized that CO2 may act as proton carrier and induce epithelial damage under acidotic conditions. Ovine ruminal epithelia were mounted in Ussing chambers under short-circuit conditions. Read More

Int Urol Nephrol 2017 Jun 12. Epub 2017 Jun 12.

Department of Medicine, Geffen School of Medicine, UCLA, Los Angeles, CA, USA.

Chronic hepatic patients, and particularly those suffering from cirrhosis, are predisposed to different sort of water, electrolyte, acid-base, and trace elements disorders due to their altered liver function, and also to their exposition to infectious, inflammatory, oncologic, and pharmacologic variables whose combination undermines their homeostatic capability. Hyponatremia, hypokalemia, hyperkalemia, hypocalcemia, metabolic acidosis, respiratory, and metabolic alkalosis are the main internal milieu alterations in this group. Read More

Mol Cell Biol 2017 Jun 12. Epub 2017 Jun 12.

Department of Biochemistry, Department of Anesthesiology, and Translational Research Center, Keio University School of Medicine, Tokyo, Japan.

Metformin is one of the most widely used therapeutics for type 2 diabetes mellitus and also has anti-cancer and anti-aging properties. However, it is known to induce metformin-associated lactic acidosis (MALA), a severe medical condition with poor prognosis, especially in individuals with renal dysfunction. Inhibition of prolyl hydroxylase (PHD) is known to activate transcription factor HIF (hypoxia-inducible factor) that increases lactate efflux as a result of enhanced glycolysis, but it also enhances gluconeogenesis from lactate in the liver that contributes to reducing circulating lactate levels. Read More

Biomol Ther (Seoul) 2017 Jun 14. Epub 2017 Jun 14.

College of Pharmacy, Ajou University, Suwon 16499, Republic of Korea.

The Na(+)/H(+) exchanger-1 (NHE-1) is a ubiquitously expressed pH-regulatory membrane protein that functions in the brain, heart, and other organs. It is increased by intracellular acidosis through the interaction of intracellular H(+) with an allosteric modifier site in the transport domain. In the previous study, we reported that glutamate-induced NHE-1 phosphorylation mediated by activation of protein kinase C-β (PKC-β) in cultured neuron cells via extracellular signal-regulated kinases (ERK)/p90 ribosomal s6 kinases (p90RSK) pathway results in NHE-1 activation. Read More

Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2017 Jun;34(3):382-386

Department of Genetics and Metabolism, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310052, China.

Objective: To delineate the clinical, biochemical and genetic mutational characteristics of a child with mitochondrial complex III deficiency.

Methods: Clinical information and results of auxiliary examination of the patient were analyzed. Next-generation sequencing of the mitochondrial genome and related nuclear genes was carried out. Read More

JAAPA 2017 Jul;30(7):20-23

Corinne I. Alois is an assistant professor-industry professional in the Department of Clinical Health Professions at St. John's University in Queens, N.Y., and a guest lecturer and adjunct faculty member in the PA program at Pace University-Lenox Hill Hospital in New York, N.Y. Denise Rizzolo is an associate professor in the PA program at Kean University in Union, N.J., and assistant clinical professor at the Pace Completion Program, New York, N.Y. The authors have disclosed no potential conflicts of interest, financial or otherwise.

Despite advances in diabetes management, diabetic ketoacidosis (DKA) continues to be a leading cause of morbidity and mortality. Up to one-third of children may present with DKA as the original presentation of type 1 diabetes. These children develop clinical manifestations of DKA including hyperglycemia, acidosis, and ketosis. Read More

Neuroimage 2017 Jun 7;157:341-350. Epub 2017 Jun 7.

Center for Neuroscience Imaging Research, Institute for Basic Science (IBS), Suwon, South Korea; Department of Biomedical Engineering, Sungkyunkwan University, Suwon, South Korea.

Amide-proton-transfer weighted (APTw) MRI has emerged as a non-invasive pH-weighted imaging technique for studies of several diseases such as ischemic stroke. However, its pH-sensitivity is relatively low, limiting its capability to detect small pH changes. In this work, computer simulations, protamine phantom experiments, and in vivo gas challenge and experimental stroke in rats showed that, with judicious selection of the saturation pulse power, the amide-CEST at 3. Read More

Eur J Pediatr 2017 Jun 10. Epub 2017 Jun 10.

Institute for Medical and Biomedical Education, St. George's University of London, London, SW17 0QT, UK.

This study assessed the agreement between arterial and venous blood lactate and pH levels in children with sepsis. This retrospective, three-year study involved 60 PICU patients, with data collected from electronic or paper patient records. The inclusion criteria comprised of children (≤17 years old) with sepsis and those who had a venous blood gas taken first with an arterial blood gas taken after within one hour. Read More

Abdom Radiol (NY) 2017 Jun 9. Epub 2017 Jun 9.

Department of Radiology, Beaumont Hospital, Dublin 9, Ireland.

Over the last 5 years, several key trends have emerged which challenge the established paradigm of inpatient computed tomography (CT) services. Many widespread practices seem intuitive and have become dogmatic; these include pre-hydration for renal injury, holding medications which predispose to metabolic acidosis, steroid administration for allergies, oral contrast administration, and fasting. The efficacy and cost-benefit of all of these measures have been challenged by recent publications. Read More

Am J Physiol Heart Circ Physiol 2017 Jun 9:ajpheart.00222.2017. Epub 2017 Jun 9.

University of Szeged

Spreading depolarization (SD) events contribute to lesion maturation in the acutely injured human brain. Neurodegeneration related to SD is thought to be caused by the insufficiency of the cerebral blood flow (CBF) response; yet, the mediators of the CBF response, or their deficiency in the aged or ischemic cerebral cortex remain the target of intensive research. Here we postulate that tissue pH effectively modulates the magnitude of hyperemia in response to SD, which coupling is prone to be dysfunctional in the aged or ischemic cerebral cortex. Read More

Am J Kidney Dis 2017 Jun 6. Epub 2017 Jun 6.

Section of Nephrology, Department of Medicine, Yale University School of Medicine, New Haven, CT.

Hypobicarbonatemia, or a reduced bicarbonate concentration in plasma, is a finding seen in 3 acid-base disorders: metabolic acidosis, chronic respiratory alkalosis and mixed metabolic acidosis and chronic respiratory alkalosis. Hypobicarbonatemia due to chronic respiratory alkalosis is often misdiagnosed as a metabolic acidosis and mistreated with the administration of alkali therapy. Proper diagnosis of the cause of hypobicarbonatemia requires integration of the laboratory values, arterial blood gas, and clinical history. Read More

J Pediatr Endocrinol Metab 2017 May 23. Epub 2017 May 23.

.

Fructose-1,6-bisphosphatase (FBPase) enzyme deficiency is one of the treatable autosomal recessive inherited metabolic disorders. If diagnosed early, FBPase deficiency has a favorable prognosis. We report the clinical and biochemical findings of a 9. Read More

Clin Hemorheol Microcirc 2017 Jun 3. Epub 2017 Jun 3.

Department of Bioengineering, University of California, San Diego, CA, USA.

Background: Small rodent models are routinely used to evaluate the safety and efficacy of blood transfusions. Limited comprehensive literature exists about effect of different storage solutions in rat red blood cells (RBCs) characteristics. RBCs undergo time dependent biochemical and biophysical changes during storage known as hypothermic storage lesions (HSLs). Read More

Oncotarget 2017 May 24. Epub 2017 May 24.

College of Veterinary Medicine, Nanjing Agricultural University, Nanjing, P.R. China.

Rumen-derived lipopolysaccharide (LPS) is translocated from the rumen into the bloodstream when subacute ruminal acidosis (SARA) occurs following long-term feeding with a high-concentrate (HC) diet in dairy cows. The objective of this study was to investigate the mechanism of inflammatory responses in the liver caused by HC diet feeding. We found that SARA was induced in dairy cows when rumen pH below 5. Read More

PLoS One 2017 8;12(6):e0178125. Epub 2017 Jun 8.

Genetic Metabolic Disorders Research Unit, Western Sydney Genetics Program, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.

Glycyl-tRNA synthetase (GARS; OMIM 600287) is one of thirty-seven tRNA-synthetase genes that catalyses the synthesis of glycyl-tRNA, which is required to insert glycine into proteins within the cytosol and mitochondria. To date, eighteen mutations in GARS have been reported in patients with autosomal-dominant Charcot-Marie-Tooth disease type 2D (CMT2D; OMIM 601472), and/or distal spinal muscular atrophy type V (dSMA-V; OMIM 600794). In this study, we report a patient with clinical and biochemical features suggestive of a mitochondrial respiratory chain (MRC) disorder including mild left ventricular posterior wall hypertrophy, exercise intolerance, and lactic acidosis. Read More