Original Research Article Acid Based Disorders in Intensive Care Unit: a Hospital-Based Study
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Insurance Coverage of Medical Foods for Treatment of Inherited Metabolic Disorders
ORIGINAL RESEARCH ARTICLE © American College of Medical Genetics and Genomics Open Insurance coverage of medical foods for treatment of inherited metabolic disorders Susan A. Berry, MD1, Mary Kay Kenney, PhD2, Katharine B. Harris, MBA3, Rani H. Singh, PhD, RD4, Cynthia A. Cameron, PhD5, Jennifer N. Kraszewski, MPH6, Jill Levy-Fisch, BA7, Jill F. Shuger, ScM8, Carol L. Greene, MD9, Michele A. Lloyd-Puryear, MD, PhD10 and Coleen A. Boyle, PhD, MS11 Purpose: Treatment of inherited metabolic disorders is accomplished pocket” for all types of products. Uncovered spending was reported by use of specialized diets employing medical foods and medically for 11% of families purchasing medical foods, 26% purchasing necessary supplements. Families seeking insurance coverage for these supplements, 33% of those needing medical feeding supplies, and products express concern that coverage is often limited; the extent of 59% of families requiring modified low-protein foods. Forty-two this challenge is not well defined. percent of families using modified low-protein foods and 21% of families using medical foods reported additional treatment-related Methods: To learn about limitations in insurance coverage, parents expenses of $100 or more per month for these products. of 305 children with inherited metabolic disorders completed a paper survey providing information about their use of medical foods, mod- Conclusion: Costs of medical foods used to treat inherited meta- ified low-protein foods, prescribed dietary supplements, and medical bolic disorders are not completely covered by insurance or other feeding equipment and supplies for treatment of their child’s disorder resources. as well as details about payment sources for these products. -
Pathophysiology of Acid Base Balance: the Theory Practice Relationship
Intensive and Critical Care Nursing (2008) 24, 28—40 ORIGINAL ARTICLE Pathophysiology of acid base balance: The theory practice relationship Sharon L. Edwards ∗ Buckinghamshire Chilterns University College, Chalfont Campus, Newland Park, Gorelands Lane, Chalfont St. Giles, Buckinghamshire HP8 4AD, United Kingdom Accepted 13 May 2007 KEYWORDS Summary There are many disorders/diseases that lead to changes in acid base Acid base balance; balance. These conditions are not rare or uncommon in clinical practice, but every- Arterial blood gases; day occurrences on the ward or in critical care. Conditions such as asthma, chronic Acidosis; obstructive pulmonary disease (bronchitis or emphasaemia), diabetic ketoacidosis, Alkalosis renal disease or failure, any type of shock (sepsis, anaphylaxsis, neurogenic, cardio- genic, hypovolaemia), stress or anxiety which can lead to hyperventilation, and some drugs (sedatives, opoids) leading to reduced ventilation. In addition, some symptoms of disease can cause vomiting and diarrhoea, which effects acid base balance. It is imperative that critical care nurses are aware of changes that occur in relation to altered physiology, leading to an understanding of the changes in patients’ condition that are observed, and why the administration of some immediate therapies such as oxygen is imperative. © 2007 Elsevier Ltd. All rights reserved. Introduction the essential concepts of acid base physiology is necessary so that quick and correct diagnosis can The implications for practice with regards to be determined and appropriate treatment imple- acid base physiology are separated into respi- mented. ratory acidosis and alkalosis, metabolic acidosis The homeostatic imbalances of acid base are and alkalosis, observed in patients with differing examined as the body attempts to maintain pH bal- aetiologies. -
Robust Regression Analysis of GCMS Data Reveals Differential Rewiring of Metabolic Networks in Hepatitis B and C Patients
Article Robust Regression Analysis of GCMS Data Reveals Differential Rewiring of Metabolic Networks in Hepatitis B and C Patients Cedric Simillion 1,2, Nasser Semmo 2,3, Jeffrey R. Idle 2,3,4, and Diren Beyoğlu 2,4,* 1 Interfaculty Bioinformatics Unit and SIB Swiss Institute of Bioinformatics, University of Bern, Baltzerstrasse 6, 3012 Bern, Switzerland; [email protected] 2 Department of BioMedical Research, University of Bern, Murtenstrasse 35, 3008 Bern, Switzerland; [email protected] (N.S.); [email protected] (J.R.I.) 3 Department of Visceral Surgery and Medicine, Department of Hepatology, Inselspital, University Hospital of Bern, 3010 Bern, Switzerland 4 Division of Systems Pharmacology and Pharmacogenomics, Samuel J. and Joan B. Williamson Institute, Arnold & Marie Schwartz College of Pharmacy and Health Sciences, Long Island University, Brooklyn, 11201 New York, NY, USA * Correspondence: [email protected]; Tel.: +41-31-632-87-11 Received: 11 September 2017; Accepted: 5 October 2017; Published: 8 October 2017 Abstract: About one in 15 of the world’s population is chronically infected with either hepatitis virus B (HBV) or C (HCV), with enormous public health consequences. The metabolic alterations caused by these infections have never been directly compared and contrasted. We investigated groups of HBV-positive, HCV-positive, and uninfected healthy controls using gas chromatography-mass spectrometry analyses of their plasma and urine. A robust regression analysis of the metabolite data was conducted to reveal correlations between metabolite pairs. Ten metabolite correlations appeared for HBV plasma and urine, with 18 for HCV plasma and urine, none of which were present in the controls. -
Guidelines for Potential Multiple Organ Donors (Adult). Part II
ARTIGO ESPECIAL Glauco Adrieno Westphal, Milton Diretrizes para manutenção de múltiplos órgãos Caldeira Filho, Kalinca Daberkow Vieira, Viviane Renata Zaclikevis, no potencial doador adulto falecido. Parte II. Miriam Cristine Machado Bartz, Ventilação mecânica, controle endócrino metabólico Raquel Wanzuita, Álvaro Réa-Neto, Cassiano Teixeira, Cristiano Franke, e aspectos hematológicos e infecciosos Fernando Osni Machado, Joel de Andrade, Jorge Dias de Matos, Guidelines for potential multiple organ donors (adult). Part II. Alfredo Fiorelli, Delson Morilo Lamgaro, Fabiano Nagel, Felipe Mechanical ventilation, endocrine metabolic management, Dal-Pizzol, Gerson Costa, José hematological and infectious aspects Mário Teles, Luiz Henrique Melo, Maria Emília Coelho, Nazah Cherif RESUMO das alterações hematológicas é igualmente Mohamed Youssef, Péricles Duarte, importante considerando as implicações Rafael Lisboa de Souza A atuação do intensivista durante a da prática transfusional inapropriada. manutenção do potencial doador falecido Ressalta-se ainda o papel da ventilação na busca da redução de perdas de doadores protetora na modulação inflamatória e e do aumento da efetivação de transplantes conseqüente aumento do aproveitamen- não se restringe aos aspectos hemodinâmi- to de pulmões para transplante. Por fim, cos. O adequado controle endócrino-me- assinala-se a relevância da avaliação crite- tabólico é essencial para a manutenção do riosa das evidências de atividade infecciosa aporte energético aos tecidos e do controle e da antibioticoterapia na busca do maior hidro-eletrolítico, favorecendo inclusive a utilização de órgãos de potenciais doadores estabilidade hemodinâmica. A abordagem falecidos. A presente diretriz é uma iniciativa conjunta da Associação de Medicina INTRODUÇAO Intensiva Brasileira (AMIB) e da Associação Brasileira de Transplantes de Órgãos (ABTO) e teve apoio de SC Durante a evolução para a morte encefálica (ME) ocorrem diversas alterações Transplantes - Central de Notificação fisiológicas como resposta à perda das funções do tronco cerebral. -
Adverse Reactions to Foods 2003
AAAAI Work Group Reports Work Group Reports of the AAAAI provide further comment or clarification on appropriate methods of treatment or care. They may be created by committees or work groups, and the end goal is to aid practitioners in making patient decisions. They do not constitute official statements of the AAAAI but serve to bring attention to key clinical or even controversial issues. They contain a bibliography, but typically not one as extensive as that contained within a Position Statement. AAAAI Work Group Report: Current Approach to the Diagnosis and Management of Adverse Reactions to Foods October 2003 The statement below is not to be construed as dictating an exclusive course of action nor is it intended to replace the medical judgment of healthcare professionals. The unique circumstances of individual patients and environments are to be taken into account in any diagnosis and treatment plan. This statement reflects clinical and scientific advances as of the date of publication and is subject to change. Prepared by the AAAAI Adverse Reactions to Foods Committee (Scott H. Sicherer, M.D., Chair and Suzanne Teuber, M.D., Co-Chair) Purpose: To provide a brief overview of the diagnosis and management of adverse reactions to foods. Database: Recent review articles by recognized experts, consensus statements, and selected primary source documents. Definitions “Adverse food reaction” is a broad term indicating a link between an ingestion of a food and an abnormal response. Reproducible adverse reactions may be caused by: a toxin, a pharmacological effect, an immunological response, or a metabolic disorder. Food allergy is a term that is used to describe adverse immune responses to foods that are mediated by IgE antibodies that bind to the triggering food protein(s); the term is also used to indicate any adverse immune response toward foods (e.g., including cell mediated reactions). -
Chapter 15 ENDOCRINE and METABOLIC IMPAIRMENT
Table of Disabilities - Chapter 15 - Endocrine and Metabolic Impairment April 2006 Chapter 15 ENDOCRINE AND METABOLIC IMPAIRMENT Introduction This chapter provides criteria used to rate permanent impairment resulting from endocrine disorders and disorders of metabolism. The endocrine system is composed of the hypothalamic-pituitary axis, the thyroid gland, the parathyroid glands, the adrenal glands, the islet cell tissue of the pancreas and the gonads. Common endocrine disorders and disorders of metabolism assessed within this chapter include: • hyperthyroidism • hypothyroidism • hyperparathyroidism • hypoparathyroidism • hyperadrenocorticism (e.g. Cushing’s disease) • hypoadrenalism (e.g. Addison’s disease) • diabetes mellitus • hyperlipidemia • metabolic bone disease (e.g. osteoporosis). Also assessed within this chapter are hypothalmic-pituitary axis disorders and Paget’s disease of the bone. The pituitary gland, influenced by the hypothalmus, releases several hormones which control the activity of other endocrine glands or directly effect tissues of the body. The hormones released include: • thyrotropin (TSH) controls activity of the thyroid gland • corticotropin (ACTH) controls the activity of the adrenal glands • luteinizing hormone (LH) and follicle-stimulating hormone (FSH) control the activity of the gonads • growth hormone (GH) • prolactin • antidiuretic hormone (ADH) • oxytocin. Veterans Affairs Canada Page 1 Table of Disabilities - Chapter 15 - Endocrine and Metabolic Impairment April 2006 Disorders of the hypothalmic-pituitary axis may affect one or several of these hormones. Each affected hormone may result in permanent impairment. Paget’s disease of the bone is a non-metabolic bone disease; however, for assessment purposes, this condition is rated by using the criteria contained within Table 15.3. A rating is not given from this chapter for conditions listed below. -
Acid-Base Physiology & Anesthesia
ACID-BASE PHYSIOLOGY & ANESTHESIA Lyon Lee DVM PhD DACVA Introductions • Abnormal acid-base changes are a result of a disease process. They are not the disease. • Abnormal acid base disorder predicts the outcome of the case but often is not a direct cause of the mortality, but rather is an epiphenomenon. • Disorders of acid base balance result from disorders of primary regulating organs (lungs or kidneys etc), exogenous drugs or fluids that change the ability to maintain normal acid base balance. • An acid is a hydrogen ion or proton donor, and a substance which causes a rise in H+ concentration on being added to water. • A base is a hydrogen ion or proton acceptor, and a substance which causes a rise in OH- concentration when added to water. • Strength of acids or bases refers to their ability to donate and accept H+ ions respectively. • When hydrochloric acid is dissolved in water all or almost all of the H in the acid is released as H+. • When lactic acid is dissolved in water a considerable quantity remains as lactic acid molecules. • Lactic acid is, therefore, said to be a weaker acid than hydrochloric acid, but the lactate ion possess a stronger conjugate base than hydrochlorate. • The stronger the acid, the weaker its conjugate base, that is, the less ability of the base to accept H+, therefore termed, ‘strong acid’ • Carbonic acid ionizes less than lactic acid and so is weaker than lactic acid, therefore termed, ‘weak acid’. • Thus lactic acid might be referred to as weak when considered in relation to hydrochloric acid but strong when compared to carbonic acid. -
Metabolic Liver Diseases Presenting As Acute Liver Failure in Children
R E V I E W A R T I C L E Metabolic Liver Diseases Presenting as Acute Liver Failure in Children SEEMA A LAM AND BIKRANT BIHARI LAL From Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India. Correspondence to: Prof Seema Alam, Professor and Head, Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi 110 070, India. [email protected] Context: Suspecting metabolic liver disease in an infant or young child with acute liver failure, and a protocol-based workup for diagnosis is the need of the hour. Evidence acquisition: Data over the last 15 years was searched through Pubmed using the keywords “Metabolic liver disease” and “Acute liver failure” with emphasis on Indian perspective. Those published in English language where full text was retrievable were included for this review. Results: Metabolic liver diseases account for 13-43% cases of acute liver failure in infants and young children. Etiology remains indeterminate in very few cases of liver failure in studies where metabolic liver diseases were recognized in large proportion. Galactosemia, tyrosinemia and mitochondrial disorders in young children and Wilson’s disease in older children are commonly implicated. A high index of suspicion for metabolic liver diseases should be kept when there is strong family history of consanguinity, recurrent abortions or sibling deaths; and history of recurrent diarrhea, vomiting, failure to thrive or developmental delay. Simple dietary modifications and/or specific management can be life-saving if instituted promptly. Conclusion: A high index of suspicion in presence of red flag symptoms and signs, and a protocol-based approach helps in timely diagnosis and prompt administration of life- saving therapy. -
Metabolic Alkalosis Is the Most Common Acid-Base Disorder in ICU
Mæhle et al. Critical Care 2014, 18:420 http://ccforum.com/content/18/2/420 LETTER Metabolic alkalosis is the most common acid–base disorder in ICU patients Kjersti Mæhle1*, Bjørn Haug2, Hans Flaatten3,4 and Erik Waage Nielsen1,5,6 Publications give diverging information as to which alkalosis is a complication of mechanical ventilation metabolic acid–base disorder is the most common in in patients with chronic obstructive pulmonary dis- the ICU [1,2]. We explored the distribution of base ease [4]. excess (BE) values in a large number of ICU patients If the count of repetitive sampling influenced our re- and evaluated if this distribution was related to rising sults, we assume they are skewed towards acidosis, as sodium values after admission. BE values were ob- unstable and acidotic patients tend to have acid–base tained during ICU admission in selected periods samples drawn more frequently. from a first level small community hospital, a second Data from the Norwegian National Intensive Care level central hospital with university affiliations, and Registry [5] suggest that the difference in BE values be- a third level large Norwegian university/regional tween the three hospitals in our study may partly stem hospital. Sodium values were from ICU patients in from difference in patients’ lengths of stay. In our study, the second level hospital. Laboratory values were an- the second level hospital had the longest median length onymously retrieved from databases in each hospital, of stay (2.7 days). aggregated and analyzed in Qlikview or Excel and A coupling of metabolic alkalosis to rising sodium exported to GraphPad Prism for column statistics and values proposed by Lindner and colleagues [6] did not analysis of variance and for preparing graphs and seem to apply to patients in our study, as the day- frequency histograms. -
Parenteral Nutrition Primer: Balance Acid-Base, Fluid and Electrolytes
Parenteral Nutrition Primer: Balancing Acid-Base, Fluids and Electrolytes Phil Ayers, PharmD, BCNSP, FASHP Todd W. Canada, PharmD, BCNSP, FASHP, FTSHP Michael Kraft, PharmD, BCNSP Gordon S. Sacks, Pharm.D., BCNSP, FCCP Disclosure . The program chair and presenters for this continuing education activity have reported no relevant financial relationships, except: . Phil Ayers - ASPEN: Board Member/Advisory Panel; B Braun: Consultant; Baxter: Consultant; Fresenius Kabi: Consultant; Janssen: Consultant; Mallinckrodt: Consultant . Todd Canada - Fresenius Kabi: Board Member/Advisory Panel, Consultant, Speaker's Bureau • Michael Kraft - Rockwell Medical: Consultant; Fresenius Kabi: Advisory Board; B. Braun: Advisory Board; Takeda Pharmaceuticals: Speaker’s Bureau (spouse) . Gordon Sacks - Grant Support: Fresenius Kabi Sodium Disorders and Fluid Balance Gordon S. Sacks, Pharm.D., BCNSP Professor and Department Head Department of Pharmacy Practice Harrison School of Pharmacy Auburn University Learning Objectives Upon completion of this session, the learner will be able to: 1. Differentiate between hypovolemic, euvolemic, and hypervolemic hyponatremia 2. Recommend appropriate changes in nutrition support formulations when hyponatremia occurs 3. Identify drug-induced causes of hypo- and hypernatremia No sodium for you! Presentation Outline . Overview of sodium and water . Dehydration vs. Volume Depletion . Water requirements & Equations . Hyponatremia • Hypotonic o Hypovolemic o Euvolemic o Hypervolemic . Hypernatremia • Hypovolemic • Euvolemic • Hypervolemic Sodium and Fluid Balance . Helpful hint: total body sodium determines volume status, not sodium status . Examples of this concept • Hypervolemic – too much volume • Hypovolemic – too little volume • Euvolemic – normal volume Water Distribution . Total body water content varies from 50-70% of body weight • Dependent on lean body mass: fat ratio o Fat water content is ~10% compared to ~75% for muscle mass . -
Residency Essentials Full Curriculum Syllabus
RESIDENCY ESSENTIALS FULL CURRICULUM SYLLABUS Please review your topic area to ensure all required sections are included in your module. You can also use this document to review the surrounding topics/sections to ensure fluidity. Click on the topic below to jump to that page. Clinical Topics • Gastrointestinal • Genitourinary • Men’s Health • Neurological • Oncology • Pain Management • Pediatrics • Vascular Arterial • Vascular Venous • Women’s Health Requisite Knowledge • Systems • Business and Law • Physician Wellness and Development • Research and Statistics Fundamental • Clinical Medicine • Intensive Care Medicine • Image-guided Interventions • Imaging and Anatomy Last revised: November 4, 2019 Gastrointestinal 1. Portal hypertension a) Pathophysiology (1) definition and normal pressures and gradients, MELD score (2) Prehepatic (a) Portal, SMV or Splenic (i) thrombosis (ii) stenosis (b) Isolated mesenteric venous hypertension (c) Arterioportal fistula (3) Sinusoidal (intrahepatic) (a) Cirrhosis (i) ETOH (ii) Non-alcoholic fatty liver disease (iii) Autoimmune (iv) Viral Hepatitis (v) Hemochromatosis (vi) Wilson's disease (b) Primary sclerosing cholangitis (c) Primary biliary cirrhosis (d) Schistosomiasis (e) Infiltrative liver disease (f) Drug/Toxin/Chemotherapy induced chronic liver disease (4) Post hepatic (a) Budd Chiari (Primary secondary) (b) IVC or cardiac etiology (5) Ectopic perianastomotic and stomal varices (6) Splenorenal shunt (7) Congenital portosystemic shunt (Abernethy malformation) b) Measuring portal pressure (1) Direct -
Endocrinology and Reproduction Part 2
Endocrinology and Reproduction Part 2 Elisabet Stener-Victorin, Professor, PhD Reproductive Endocrinology and Metabolism (REM) group Department of Physiology and Pharmacology Karolinska Institutet, Stockholm, Sweden [email protected] Understand function in the different hormonal systems by dysfunction i.e. endocrine disorders: 1. Dwarfism, gigantism, acromegaly 2. Cretinism 3. Goiter 4. Hyperthyroidism 5. Hypothyroidism 6. Cushings syndrome and Cushings disorder 7. Type 1 diabetes and Type 2 diabetes 8. Polycystic Ovary Syndrome 9. Hypothalamic insufficiency 10.Osteoporosis Growth Hormone (GH) → IGF-1 Childhood - before closure of epiphysis: . Growth hormone deficiency → pituitary dwarfism . Excessiv secretion of GH → gigantism . GH can be used to treat children that are more than 2 SD below their growht curve Adulthood: . Excessiv secretion of GH → acromegali . Long bones cannot growth in adults, instead abnormal growth of bones in the face, hands, feet, and certain organs such as liver . Cause: adenoma Thyroid dysfunction(s) . Cretinism . Iodine deficiency during fetal development to childhood . Leads to: . Short stature . Skeletal growth is more (”stocky” & obese appearance, enlarged tongue) . Mental retardation . Goiter – iodine deficiency (indigenous hypothyroidism) Hypothyroidism . Primary hypothyroidism . Hashimoto disease,an autoimmune disease causing impaired hormone synthesis . Secondary hypothyroidism . Can be heritable and affect the biosynthesis of thyroid hormones . Surgery . Radioiodine . Symptoms . Extreme tiredness, muscle weakness . Frozen . Weight gain – low metabolic rate . Dry skin . Slow in mind . Treatment . Supplement with levaxin Hyperthyroidism • Primary hyperthyroidism • Graves Disease, autoimmune disorder is the most common cause • Adenoma, less common – secrete thyroid hormone • Symptoms • Exopthalmos • High metabolic rate • Increased appetite, but decrease in weight • Tachycardia Goiter • Tremor, nervous etc • Treatment • Thyreostatics and radioactive iodin • Β-blocker • Surgery • Supplement with levaxin Hypercalcemia .