Leukocyte Peroxidase Test. (A) Identification
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Non-Commercial Use Only
only use Non-commercial 14th International Conference on Thalassaemia and Other Haemoglobinopathies 16th TIF Conference for Patients and Parents 17-19 November 2017 • Grand Hotel Palace, Thessaloniki, Greece only use For thalassemia patients with chronic transfusional iron overload... Make a lasting impression with EXJADENon-commercial film-coated tablets The efficacy of deferasirox in a convenient once-daily film-coated tablet Please see your local Novartis representative for Full Product Information Reference: EXJADE® film-coated tablets [EU Summary of Product Characteristics]. Novartis; August 2017. Important note: Before prescribing, consult full prescribing information. iron after having achieved a satisfactory body iron level and therefore retreatment cannot be recommended. ♦ Maximum daily dose is 14 mg/kg body weight. ♦ In pediatric patients the Presentation: Dispersible tablets containing 125 mg, 250 mg or 500 mg of deferasirox. dosing should not exceed 7 mg/kg; closer monitoring of LIC and serum ferritin is essential Film-coated tablets containing 90 mg, 180 mg or 360 mg of deferasirox. to avoid overchelation; in addition to monthly serum ferritin assessments, LIC should be Indications: For the treatment of chronic iron overload due to frequent blood transfusions monitored every 3 months when serum ferritin is ≤800 micrograms/l. (≥7 ml/kg/month of packed red blood cells) in patients with beta-thalassemia major aged Dosage: Special population ♦ In moderate hepatic impairment (Child-Pugh B) dose should 6 years and older. ♦ Also indicated for the treatment of chronic iron overload due to blood not exceed 50% of the normal dose. Should not be used in severe hepatic impairment transfusions when deferoxamine therapy is contraindicated or inadequate in the following (Child-Pugh C). -
224 Subpart H—Hematology Kits and Packages
§ 864.7040 21 CFR Ch. I (4–1–02 Edition) Subpart H—Hematology Kits and the treatment of venous thrombosis or Packages pulmonary embolism by measuring the coagulation time of whole blood. § 864.7040 Adenosine triphosphate re- (b) Classification. Class II (perform- lease assay. ance standards). (a) Identification. An adenosine [45 FR 60611, Sept. 12, 1980] triphosphate release assay is a device that measures the release of adenosine § 864.7250 Erythropoietin assay. triphosphate (ATP) from platelets fol- (a) Identification. A erythropoietin lowing aggregation. This measurement assay is a device that measures the is made on platelet-rich plasma using a concentration of erythropoietin (an en- photometer and a luminescent firefly zyme that regulates the production of extract. Simultaneous measurements red blood cells) in serum or urine. This of platelet aggregation and ATP re- assay provides diagnostic information lease are used to evaluate platelet for the evaluation of erythrocytosis function disorders. (increased total red cell mass) and ane- (b) Classification. Class I (general mia. controls). (b) Classification. Class II. The special [45 FR 60609, Sept. 12, 1980] control for this device is FDA’s ‘‘Docu- ment for Special Controls for Erythro- § 864.7060 Antithrombin III assay. poietin Assay Premarket Notification (a) Identification. An antithrombin III (510(k)s).’’ assay is a device that is used to deter- [45 FR 60612, Sept. 12, 1980, as amended at 52 mine the plasma level of antithrombin FR 17733, May 11, 1987; 65 FR 17144, Mar. 31, III (a substance which acts with the 2000] anticoagulant heparin to prevent co- agulation). This determination is used § 864.7275 Euglobulin lysis time tests. -
ATSDR Case Studies in Environmental Medicine Nitrate/Nitrite Toxicity
ATSDR Case Studies in Environmental Medicine Nitrate/Nitrite Toxicity Agency for Toxic Substances and Disease Registry Case Studies in Environmental Medicine (CSEM) Nitrate/Nitrite Toxicity Course: WB2342 CE Original Date: December 5, 2013 CE Expiration Date: December 5, 2015 Key • Nitrate toxicity is a preventable cause of Concepts methemoglobinemia. • Infants younger than 4 months of age are at particular risk of nitrate toxicity from contaminated well water. • The widespread use of nitrate fertilizers increases the risk of well-water contamination in rural areas. About This This educational case study document is one in a series of and Other self-instructional modules designed to increase the primary Case Studies care provider’s knowledge of hazardous substances in the in environment and to promote the adoption of medical Environmen- practices that aid in the evaluation and care of potentially tal Medicine exposed patients. The complete series of Case Studies in Environmental Medicine is located on the ATSDR Web site at URL: http://www.atsdr.cdc.gov/csem/csem.html In addition, the downloadable PDF version of this educational series and other environmental medicine materials provides content in an electronic, printable format. Acknowledgements We gratefully acknowledge the work of the medical writers, editors, and reviewers in producing this educational resource. Contributors to this version of the Case Study in Environmental Medicine are listed below. Please Note: Each content expert for this case study has indicated that there is no conflict of interest that would bias the case study content. CDC/ATSDR Author(s): Kim Gehle MD, MPH CDC/ATSDR Planners: Charlton Coles, Ph.D.; Kimberly Gehle, MD; Sharon L. -
Hereditary Spherocytosis: Clinical Features
Title Overview: Hereditary Hematological Disorders of red cell shape. Disorders Red cell Enzyme disorders Disorders of Hemoglobin Inherited bleeding disorders- platelet disorders, coagulation factor Anthea Greenway MBBS FRACP FRCPA Visiting Associate deficiencies Division of Pediatric Hematology-Oncology Duke University Health Service Inherited Thrombophilia Hereditary Disorders of red cell Disorders of red cell shape (cytoskeleton): cytoskeleton: • Mutations of 5 proteins connect cytoskeleton of red cell to red cell membrane • Hereditary Spherocytosis- sphere – Spectrin (composed of alpha, beta heterodimers) –Ankyrin • Hereditary Elliptocytosis-ellipse, elongated forms – Pallidin (band 4.2) – Band 4.1 (protein 4.1) • Hereditary Pyropoikilocytosis-bizarre red cell forms – Band 3 protein (the anion exchanger, AE1) – RhAG (the Rh-associated glycoprotein) Normal red blood cell- discoid, with membrane flexibility Hereditary Spherocytosis: Clinical features: • Most common hereditary hemolytic disorder (red cell • Neonatal jaundice- severe (phototherapy), +/- anaemia membrane) • Hemolytic anemia- moderate in 60-75% cases • Mutations of one of 5 genes (chromosome 8) for • Severe hemolytic anaemia in 5% (AR, parents ASx) cytoskeletal proteins, overall effect is spectrin • fatigue, jaundice, dark urine deficiency, severity dependant on spectrin deficiency • SplenomegalSplenomegaly • 200-300:million births, most common in Northern • Chronic complications- growth impairment, gallstones European countries • Often follows clinical course of affected -
Sickle Cell: It's Your Choice
Sickle Cell: It’s Your Choice What Does “Sickle Cell” Mean? Sickle is a type of hemoglobin. Hemoglobin is the substance that carries oxygen in the blood and gives blood its red color. A person’s hemoglobin type is not the same thing as blood type. The type of hemoglobin we have is determined by genes that we inherit from our parents. The majority of individuals have only the “normal” type of hemoglobin (A). However, there are a variety of other hemoglobin types. Sickle hemoglobin (S) is one of these types. There Are Two Forms of Sickle Cell. Sickle cell occurs in two forms. Sickle cell trait is not a disease; Sickle cell anemia (or sickle cell disease) is a disease. Sickle Cell Trait (or Sickle Trait) Sickle cell trait is found primarily in African Americans, people from areas around the Mediterranean Sea, and from islands in the Caribbean. Sickle cell trait occurs when a person inherits one sickle cell gene from one parent and one normal hemoglobin gene from the other parent. A person with sickle cell trait is healthy and usually is not aware that he or she has the sickle cell gene. A person who has sickle trait can pass it on to their children. If one parent has sickle cell trait and the other parent has the normal type of hemoglobin, there is a 50% (1 in 2) chance with EACH pregnancy that the baby will be born with sickle cell trait. When ONE parent has sickle cell trait, the child may inherit: • 50% chance for two normal hemoglobin genes (normal hemoglobin- AA), OR • 50% chance for one normal hemoglobin gene and one sickle cell gene (sickle cell trait- AS). -
Sickle Cell Disease Brochure
What is sickle cell trait? Who can have sickle cell disease and sickle cell trait? Sickle Cell Trait (AS) is an inherited condition which affects the hemoglobin in your red blood cells. » It is estimated that SCD affects 90,000 to 100,000 people in the United States, mainly Blacks or It is important to know if you have sickle cell trait. African Americans. All About: Sickle cell trait is inherited from your parents, » The disease occurs in about 1 of every 500 Black like hair or eye color. If one parent has sickle cell or African American births and in about 1 of every trait, there is a 50% (1 in 2) chance with each 36,000 Hispanic American births. Sickle Cell pregnancy of having a child with sickle cell trait. Sickle cell trait rarely causes any health problems. » SCD affects millions of people throughout the Some people may develop health problems under world and is particularly common among those certain conditions, such as: whose ancestors come from sub-Saharan Africa, Disease & regions in the Western Hemisphere (South » Dehydration – from not drinking enough water America, the Caribbean, and Central America), » Low oxygen – from over-exertion Saudi Arabia, India, and Mediterranean countries » High altitudes – from low oxygen levels such as Turkey, Greece, and Italy. Sickle Cell » About 1 of every 12 African Americans has sickle How do you know if you have sickle cell cell trait and about 1 of every 100 Hispanics has trait or disease? sickle cell trait. Trait » It is possible for a person of any race or nationality to have sickle cell trait. -
The Formation of Methemoglobin and Sulfhemoglobin During Sulfanilamide Therapy
THE FORMATION OF METHEMOGLOBIN AND SULFHEMOGLOBIN DURING SULFANILAMIDE THERAPY J. S. Harris, H. O. Michel J Clin Invest. 1939;18(5):507-519. https://doi.org/10.1172/JCI101064. Research Article Find the latest version: https://jci.me/101064/pdf THE FORMATION OF METHEMOGLOBIN AND SULFHEMOGLOBIN DURING SULFANILAMIDE THERAPY By J. S. HARRIS AND H. 0. MICHEL (From the Departments of Pediatrics and Biochemistry, Duke University School of Medicine, Durham) (Received for publication April 8, 1939) Cyanosis almost invariably follows the admin- during the administration of sulfanilamide. Wen- istration of therapeutic amounts of sulfanilamide del (10) found spectroscopic evidence of met- (1). This cyanosis is associated with and is due hemoglobin in every blood sample containing over to a change in the color of the blood. The dark- 4 mgm. per cent sulfanilamide. Evelyn and Mal- ening of the blood is present only in the red cells loy (11) have found that all patients receiving and therefore must be ascribed to one of two sulfanilamide show methemoglobinemia, although causes, a change in the hemoglobin itself or a the intensity is usually very slight. Finally Hart- staining of the red cells with some product formed mann, Perley, and Barnett (12) found cyanosis during the metabolism of sulfanilamide. It is the associated with methemoglobinemia in almost ev- purpose of this paper to assay quantitatively the ery patient receiving over 0.1 gram sulfanilamide effect of sulfanilamide upon the first of these fac- per kilogram of body weight per day. They be- tors-that is, upon the formation of abnormal lieved that the intensity of the methemoglobinemia heme pigments. -
The Role of Nonhemoglobin Proteins and Reduced Glutathione in the Protection of Hemoglobin from Oxidation in Vitro
The Role of Nonhemoglobin Proteins and Reduced Glutathione in the Protection of Hemoglobin from Oxidation In Vitro A. S. Hill Jr., … , G. E. Cartwright, M. M. Wintrobe J Clin Invest. 1964;43(1):17-26. https://doi.org/10.1172/JCI104889. Research Article Find the latest version: https://jci.me/104889/pdf Journal of Clinical Investigatiox Vol. 43, No. 1, 1964 The Role of Nonhemoglobin Proteins and Reduced Glutathione in the Protection of Hemoglobin from Oxidation In Vitro * A. S. HILL, JR., A. HAUT, G. E. CARTWRIGHT, AND M. M. WINTROBE (From the Department of Medicine, University of Utah College of Medicine, Salt Lake City, Utah) A deficiency of reduced glutathione in erythro- human erythrocytes by Allen and Jandl (10) led cytes of freshly shed blood (1-4) or in erythro- these authors to conclude that GSH per se pro- cytes exposed to certain chemical compounds (5) tects hemoglobin from oxidation and that no ad- has been associated with shorter red cell survival ditional factors are required; conversely, Szein- and, in most cases, with the accumulation of oxi- berg and Marks (11) found that GSH affords no dation products of hemoglobin, including methe- protection to human hemoglobin against oxidative moglobin, sulfhemoglobin, and Heinz bodies. changes. The earlier report of Foulkes and Studies of "primaquine-sensitive" hemolytic dis- Lemberg (12) that GSH might oxidize hemo- ease have emphasized as a characteristic feature globin, along with the studies by Szeinberg and an "unstable" reduced glutathione (GSH) in the Marks (11) and Allen and Jandl (10), casts red cells, attributed to limited availability of re- doubt on the applicability to humans of the con- duced TPN (TPNH), the result, in turn, of a cept (13, 14), based on studies of rat blood (7, deficiency of the enzyme, glucose-6-phosphate 8), that an adequate amount of reduced glutathi- dehydrogenase - (G6PD). -
Inborn Defects in the Antioxidant Systems of Human Red Blood Cells
Free Radical Biology and Medicine 67 (2014) 377–386 Contents lists available at ScienceDirect Free Radical Biology and Medicine journal homepage: www.elsevier.com/locate/freeradbiomed Review Article Inborn defects in the antioxidant systems of human red blood cells Rob van Zwieten a,n, Arthur J. Verhoeven b, Dirk Roos a a Laboratory of Red Blood Cell Diagnostics, Department of Blood Cell Research, Sanquin Blood Supply Organization, 1066 CX Amsterdam, The Netherlands b Department of Medical Biochemistry, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands article info abstract Article history: Red blood cells (RBCs) contain large amounts of iron and operate in highly oxygenated tissues. As a result, Received 16 January 2013 these cells encounter a continuous oxidative stress. Protective mechanisms against oxidation include Received in revised form prevention of formation of reactive oxygen species (ROS), scavenging of various forms of ROS, and repair 20 November 2013 of oxidized cellular contents. In general, a partial defect in any of these systems can harm RBCs and Accepted 22 November 2013 promote senescence, but is without chronic hemolytic complaints. In this review we summarize the Available online 6 December 2013 often rare inborn defects that interfere with the various protective mechanisms present in RBCs. NADPH Keywords: is the main source of reduction equivalents in RBCs, used by most of the protective systems. When Red blood cells NADPH becomes limiting, red cells are prone to being damaged. In many of the severe RBC enzyme Erythrocytes deficiencies, a lack of protective enzyme activity is frustrating erythropoiesis or is not restricted to RBCs. Hemolytic anemia Common hereditary RBC disorders, such as thalassemia, sickle-cell trait, and unstable hemoglobins, give G6PD deficiency Favism rise to increased oxidative stress caused by free heme and iron generated from hemoglobin. -
Genetics: Sickle Beta Plus Thalassemia
Genetics: Sickle beta plus thalassemia Sickle beta plus thalassemia (THAL-UH-SEE-ME-AH) is a blood condition that is similar to sickle cell anemia. Sickle cell anemia is a disease that causes red blood cells (RBCs) to have an abnormal shape. Sickle red blood cells can get stuck in blood vessels and block the flow of blood and oxygen in the body. When this happens is can cause severe pain, serious infections, organ damage, or even stroke. What is hemoglobin and what does it do? Red blood cells contain hemoglobin (HEE-MUH-GLOW-BIN). Hemoglobin is a protein that carries oxygen around the body. There are several types of abnormal hemoglobin. Sickled hemoglobin is the type that causes sickle cell anemia. It is usually written as Hb-S. Beta thalassemia causes your child's body to make less normal hemoglobin (Hb-A). When this happens, your child's body makes more sickled cells and has symptoms similar to sickle cell anemia. The amount of sickled cells is different in each child with beta thalassemia. When a person has one copy of Hb-S and one copy of beta thalassemia, it is called sickle beta thalassemia. In general, people who have sickle beta plus thalassemia make more normal hemoglobin than people who have sickle beta zero thalassemia. How does a person get sickle beta plus thalassemia? Sickle beta thalassemia is genetic disorder, meaning it is passed on from parents to their children just like hair, eye, and skin color. You are born with sickle beta thalassemia disease. It is not contagious. -
Hemolytic Anemia and the Reactive Sulfhydryl Groups of the Erythrocyte Membrane
HEMOLYTIC ANEMIA AND THE REACTIVE SULFHYDRYL GROUPS OF THE ERYTHROCYTE MEMBRANE by Erwin Peter GABOR, M.D., C.M. A thesis presented to the Faculty of Graduate Studies and Research ~n partial fulfillment of the requirements for 1 the degree of Master of Science in Experimental Medicine. McGill University Clinic and Royal Victoria Hospital, Montreal, Quebec. April, 1964. HEMOLITIC ANEMIA AND THE REACTIVE SULFHYDR!L GROUPS OF THE ER'YTHROCITE MEMBRANE by Erwin Peter Gabor ( Abstract ) Membrane sul.fhydryl ( SH) groups have been reported to be important for the maintenance of red cell integrity E, ~ ( Jacob and Jandl, 1962 ). A technique has been developed for the determination of reactive membrane sulfhydryl content in intact erythrocytes, utilizing sub hemolytic concentrations of p-chloromercuribenzoate (PMB). The erythrocyte membrane of 52 healthy subjects contained 2.50 - 2.85 x lo-16 moles of reactive SH groups ( mean 2.50 ·~ 0.20 ) per erythrocyte, when determined by this method. A 27-56% reduction of erythrocyte membrane SH content was observed in various conditions characterized by accelerated red cell destruction, including glucose- 6-phosphate dehydrogenase ( G6PD ) deficiency, drug-induced, auto- immune and other acquired hemolytic anemias and congenital spherocytosis. Normal membrane sulfhydryl content was found in iron deficiency anemia, pernicious anemia in relapse, and in other miscellaneous hematological conditions. Inhibition of membrane SH groups with PMB caused marked potassium leakage from the otherwise intact cells. The possible role of membrane suli'hydryl groups in the development of certain types of hemolytic anemias,and in the maintenance of active transmembrane cation transport in the erythrocyte is discussed. -
An Unusual Case of Methemoglobinemia
ISSN: 2639-4553 Madridge Journal of Case Reports and Studies Case Report Open Access An Unusual Case of Methemoglobinemia Devika Mishra*, Kirti N Saxena, and Bharti Wadhwa Department of Anesthesiology and Intensive care, Maulana Azad medical college and associated LokNayak hospital, New Delhi, India Article Info Abstract *Corresponding author: Methemoglobinemia is a condition in which an abnormal proportion of the iron in Devika Mishra heme moiety of the hemoglobin is oxidized to the ferric state leading to impaired Senior Resident Department of Anesthesiology and oxygen transport and anemic hypoxia and can cause tissue ischemia and death. A 21 year Intensive care old male, known case of Hansen’s disease was posted for surgicalneurolysis of ulnar Maulana Azad medical college and nerve. His surgery was already postponed once as SpO2 waslow on the operation table. associated Lok Nayak hospital, New Delhi India He then underwent complete systemic evaluation for the cause of low saturation but Phone: +91 9811889531 was found normal. On pulse oximetry it was seen that SpO2 was 91-93% on room air E-mail: [email protected] without any signs and symptoms of hypoxemia. This suggestedmethemoglobinemia due to treatment with dapsone which was being taken by the patient for treatment of Received: July 23, 2018 Hansen’s disease. The surgery was conducted uneventfully under general anesthesia. Accepted: July 26, 2018 Published: July 31, 2018 The diagnosis was confirmed by laboratory testing. Keywords: Methemoglobinemia; Dapsone. Citation: Mishra D, Saxena KN, Wadhwa B. An unusual Case of Methemoglobinemia. Madridge J Case Rep Stud. 2018; 2(1): 53-54. Introduction doi: 10.18689/mjcrs-1000113 There are many causes of low oxygen saturation (SpO2) of hemoglobin in a patient Copyright: © 2018 The Author(s).