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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. -
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. -
Spatial and Temporal Dynamics of the Endothelium
Journal of Thrombosis and Haemostasis, 3: 1392–1406 REVIEW ARTICLE Spatial and temporal dynamics of the endothelium W. C. AIRD Division of Molecular and Vascular Medicine, Department of Medicine, and Center for Vascular Biology Research, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA To cite this article: Aird WC. Spatial and temporal dynamics of the endothelium. J Thromb Haemost 2005; 3: 1392–1406. particular emphasis on: (i) the mechanisms of phenotypic Summary. The endothelium is a highly metabolically active heterogeneity; (ii) the bench-to-bedside gap in endothelial organ that is involved in many physiological processes, biomedicine; (iii) endothelial cell activation and dysfunction; including the control of vasomotor tone, barrier function, and (iv) the need for new diagnostic and therapeutic approa- leukocyte adhesion and trafficking, inflammation, and hemos- ches in endothelial-based diseases. tasis. Endothelial cell phenotypes are differentially regulated in space and time. Endothelial cell heterogeneity has important implications for developing strategies in basic research, diag- Scales of investigation nostics and therapeutics. The goals of this review are to: The term ÔvascularÕ refers to blood vessels, the elaborate (i) consider mechanisms of endothelial cell heterogeneity; series of blood-filled hollow tubes that deliver oxygen and (ii) discuss the bench-to-bedside gap in endothelial biomedicine; nutrients to all tissues of the human body. The vascular (iii) revisit definitions for endothelial cell activation and system comprises both blood vessels and lymphatic vessels. dysfunction; and (iv) propose new goals in diagnosis and For purposes of this review, we will focus on the former. For therapy. Finally, these themes will be applied to an under- more information about lymphangiogenesis and lymphatic standing of vascular bed-specific hemostasis. -
Angiocrine Endothelium: from Physiology to Cancer Jennifer Pasquier1,2*, Pegah Ghiabi2, Lotf Chouchane3,4,5, Kais Razzouk1, Shahin Rafi3 and Arash Rafi1,2,3
Pasquier et al. J Transl Med (2020) 18:52 https://doi.org/10.1186/s12967-020-02244-9 Journal of Translational Medicine REVIEW Open Access Angiocrine endothelium: from physiology to cancer Jennifer Pasquier1,2*, Pegah Ghiabi2, Lotf Chouchane3,4,5, Kais Razzouk1, Shahin Rafi3 and Arash Rafi1,2,3 Abstract The concept of cancer as a cell-autonomous disease has been challenged by the wealth of knowledge gathered in the past decades on the importance of tumor microenvironment (TM) in cancer progression and metastasis. The sig- nifcance of endothelial cells (ECs) in this scenario was initially attributed to their role in vasculogenesis and angiogen- esis that is critical for tumor initiation and growth. Nevertheless, the identifcation of endothelial-derived angiocrine factors illustrated an alternative non-angiogenic function of ECs contributing to both physiological and pathological tissue development. Gene expression profling studies have demonstrated distinctive expression patterns in tumor- associated endothelial cells that imply a bilateral crosstalk between tumor and its endothelium. Recently, some of the molecular determinants of this reciprocal interaction have been identifed which are considered as potential targets for developing novel anti-angiocrine therapeutic strategies. Keywords: Angiocrine, Endothelium, Cancer, Cancer microenvironment, Angiogenesis Introduction of blood vessels in initiation of tumor growth and stated Metastatic disease accounts for about 90% of patient that in the absence of such angiogenesis, tumors can- mortality. Te difculty in controlling and eradicating not expand their mass or display a metastatic phenotype metastasis might be related to the heterotypic interaction [7]. Based on this theory, many investigators assumed of tumor and its microenvironment [1]. -
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. -
GLOSSARY of MEDICAL and ANATOMICAL TERMS
GLOSSARY of MEDICAL and ANATOMICAL TERMS Abbreviations: • A. Arabic • abb. = abbreviation • c. circa = about • F. French • adj. adjective • G. Greek • Ge. German • cf. compare • L. Latin • dim. = diminutive • OF. Old French • ( ) plural form in brackets A-band abb. of anisotropic band G. anisos = unequal + tropos = turning; meaning having not equal properties in every direction; transverse bands in living skeletal muscle which rotate the plane of polarised light, cf. I-band. Abbé, Ernst. 1840-1905. German physicist; mathematical analysis of optics as a basis for constructing better microscopes; devised oil immersion lens; Abbé condenser. absorption L. absorbere = to suck up. acervulus L. = sand, gritty; brain sand (cf. psammoma body). acetylcholine an ester of choline found in many tissue, synapses & neuromuscular junctions, where it is a neural transmitter. acetylcholinesterase enzyme at motor end-plate responsible for rapid destruction of acetylcholine, a neurotransmitter. acidophilic adj. L. acidus = sour + G. philein = to love; affinity for an acidic dye, such as eosin staining cytoplasmic proteins. acinus (-i) L. = a juicy berry, a grape; applied to small, rounded terminal secretory units of compound exocrine glands that have a small lumen (adj. acinar). acrosome G. akron = extremity + soma = body; head of spermatozoon. actin polymer protein filament found in the intracellular cytoskeleton, particularly in the thin (I-) bands of striated muscle. adenohypophysis G. ade = an acorn + hypophyses = an undergrowth; anterior lobe of hypophysis (cf. pituitary). adenoid G. " + -oeides = in form of; in the form of a gland, glandular; the pharyngeal tonsil. adipocyte L. adeps = fat (of an animal) + G. kytos = a container; cells responsible for storage and metabolism of lipids, found in white fat and brown fat. -
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. -
Prostaglandin E2 Breaks Down Pericyte–Endothelial Cell Interaction Via EP1 and EP4-Dependent Downregulation of Pericyte N-Cadh
www.nature.com/scientificreports OPEN Prostaglandin E2 breaks down pericyte–endothelial cell interaction via EP1 and EP4‑dependent downregulation of pericyte N‑cadherin, connexin‑43, and R‑Ras Carole Y. Perrot1,2, Jose L. Herrera1,2, Ashley E. Fournier‑Goss1,2 & Masanobu Komatsu1,2* A close association between pericytes and endothelial cells (ECs) is crucial to the stability and function of capillary blood vessels and microvessels. The loss or dysfunction of pericytes results in signifcant disruption of these blood vessels as observed in pathological conditions, including cancer, diabetes, stroke, and Alzheimer’s disease. Prostaglandin E2 (PGE2) is a lipid mediator of infammation, and its tissue concentration is elevated in cancer and neurological disorders. Here, we show that the exposure to PGE2 switches pericytes to a fast‑migrating, loosely adhered phenotype that fails to intimately interact with ECs. N‑cadherin and connexin‑43 in adherens junction and gap junction between pericytes and ECs are downregulated by EP‑4 and EP‑1‑dependent mechanisms, leading to breakdown of the pericyte–EC interaction. Furthermore, R‑Ras, a small GTPase important for vascular normalization and vessel stability, is transcriptionally repressed by PGE2 in an EP4‑dependent manner. Mouse dermal capillary vessels lose pericyte coverage substantially upon PGE2 injection into the skin. Our results suggest that EP‑mediated direct disruption of pericytes by PGE2 is a key process for vascular destabilization. Restoring pericyte–EC interaction using inhibitors of PGE2 signaling may ofer a therapeutic strategy in cancer and neurological disorders, in which pericyte dysfunction contributes to the disease progression. Pericytes are mesenchyme-derived mural cells that surround endothelial cells (ECs) of capillary blood vessels and microvessels. -
Dengue Glossary and Acronyms
Dengue Clinical Case Management E-learning Dengue Glossary and Acronyms Merriam-Webster, PubMed Health, and Mosby’s Medical Dictionary were consulted in the compilation of this glossary. Afebrile: not marked by or having a fever Agonal breathing: irregular breathing associated with respiratory failure Antibody-dependent enhancement (ADE): occurs when nonneutralizing antiviral antibodies enhance viral entry into host cells. Once inside the white blood cell, the virus replicates undetected, eventually generating very high virus titers which is thought to lead to more severe disease Arthralgia: pain in one or more joints Ascites: abnormal accumulation of serous fluid in the spaces between tissues and organs in the cavity of the abdomen—called also hydroperitoneum Asystole: lack of heart beat or electrical activity Atrioventricular: 1: of, relating to, or situated between an atrium and ventricle 2: of, involving, or being the atrioventricular node Auscultation: the act of listening to sounds arising within organs (as the lungs or heart) as an aid to diagnosis and treatment Bolus: a large amount of a substance such as a drug or fluid given intravenously over a short period of time Bradycardia: slow heart rate Cerebral edema: the accumulation of fluid in, and resultant swelling of, the brain Cholecystitis: inflammation of the gallbladder Colloid: a fluid containing insoluble molecules such as albumin that are incapable of passing through capillary walls, thereby maintaining or increasing osmotic pressure in the blood Cytokines: any of a class -
Renin-Angiotensin System in Pathogenesis of Atherosclerosis and Treatment of CVD
International Journal of Molecular Sciences Review Renin-Angiotensin System in Pathogenesis of Atherosclerosis and Treatment of CVD Anastasia V. Poznyak 1,* , Dwaipayan Bharadwaj 2,3, Gauri Prasad 3, Andrey V. Grechko 4, Margarita A. Sazonova 5 and Alexander N. Orekhov 1,5,6,* 1 Institute for Atherosclerosis Research, Skolkovo Innovative Center, 121609 Moscow, Russia 2 Academy of Scientific and Innovative Research, CSIR-Institute of Genomics and Integrative Biology Campus, New Delhi 110067, India; [email protected] 3 Systems Genomics Laboratory, School of Biotechnology, Jawaharlal Nehru University, New Delhi 110067, India; [email protected] 4 Federal Research and Clinical Center of Intensive Care Medicine and Rehabilitology, 14-3 Solyanka Street, 109240 Moscow, Russia; [email protected] 5 Laboratory of Angiopathology, Institute of General Pathology and Pathophysiology, 125315 Moscow, Russia; [email protected] 6 Institute of Human Morphology, 3 Tsyurupa Street, 117418 Moscow, Russia * Correspondence: [email protected] (A.V.P.); [email protected] (A.N.O.) Abstract: Atherosclerosis has complex pathogenesis, which involves at least three serious aspects: inflammation, lipid metabolism alterations, and endothelial injury. There are no effective treatment options, as well as preventive measures for atherosclerosis. However, this disease has various severe complications, the most severe of which is cardiovascular disease (CVD). It is important to note, that CVD is among the leading causes of death worldwide. The renin–angiotensin–aldosterone system (RAAS) is an important part of inflammatory response regulation. This system contributes to Citation: Poznyak, A.V.; Bharadwaj, the recruitment of inflammatory cells to the injured site and stimulates the production of various D.; Prasad, G.; Grechko, A.V.; cytokines, such as IL-6, TNF-a, and COX-2. -
Nomina Histologica Veterinaria, First Edition
NOMINA HISTOLOGICA VETERINARIA Submitted by the International Committee on Veterinary Histological Nomenclature (ICVHN) to the World Association of Veterinary Anatomists Published on the website of the World Association of Veterinary Anatomists www.wava-amav.org 2017 CONTENTS Introduction i Principles of term construction in N.H.V. iii Cytologia – Cytology 1 Textus epithelialis – Epithelial tissue 10 Textus connectivus – Connective tissue 13 Sanguis et Lympha – Blood and Lymph 17 Textus muscularis – Muscle tissue 19 Textus nervosus – Nerve tissue 20 Splanchnologia – Viscera 23 Systema digestorium – Digestive system 24 Systema respiratorium – Respiratory system 32 Systema urinarium – Urinary system 35 Organa genitalia masculina – Male genital system 38 Organa genitalia feminina – Female genital system 42 Systema endocrinum – Endocrine system 45 Systema cardiovasculare et lymphaticum [Angiologia] – Cardiovascular and lymphatic system 47 Systema nervosum – Nervous system 52 Receptores sensorii et Organa sensuum – Sensory receptors and Sense organs 58 Integumentum – Integument 64 INTRODUCTION The preparations leading to the publication of the present first edition of the Nomina Histologica Veterinaria has a long history spanning more than 50 years. Under the auspices of the World Association of Veterinary Anatomists (W.A.V.A.), the International Committee on Veterinary Anatomical Nomenclature (I.C.V.A.N.) appointed in Giessen, 1965, a Subcommittee on Histology and Embryology which started a working relation with the Subcommittee on Histology of the former International Anatomical Nomenclature Committee. In Mexico City, 1971, this Subcommittee presented a document entitled Nomina Histologica Veterinaria: A Working Draft as a basis for the continued work of the newly-appointed Subcommittee on Histological Nomenclature. This resulted in the editing of the Nomina Histologica Veterinaria: A Working Draft II (Toulouse, 1974), followed by preparations for publication of a Nomina Histologica Veterinaria.