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• W1995544897 abstract "Article1 January 1965Biochemical Studies in ThalassemiaANANDA S. PRASAD, M.B., B.S., PH.D., M. DIWANY, M.D., M.R.C.P., MAMDOUH GABR, M.D., HAROLD H. SANDSTEAD, M.D., NEWAL MOKHTAR, M.B., B.S., ANISA EL HEFNY, M.B., B.S.ANANDA S. PRASAD, M.B., B.S., PH.D.Search for more papers by this author, M. DIWANY, M.D., M.R.C.P.Search for more papers by this author, MAMDOUH GABR, M.D.Search for more papers by this author, HAROLD H. SANDSTEAD, M.D.Search for more papers by this author, NEWAL MOKHTAR, M.B., B.S.Search for more papers by this author, ANISA EL HEFNY, M.B., B.S.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-62-1-87 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptSince the description of a syndrome comprised of erythroblastosis, splenomegaly, and anemia with a familial and racial incidence by Cooley and Lee (1) in 1925, many cases from various parts of the world have been reported (2-4). From Egypt, Diwany (5) first reported two cases in 1944, and since then others have been described (6-9). These reports have documented the presence of this disease in Egypt, but do not provide detailed biochemical and hematological data on the cases.Abnormal iron metabolism in Cooley's anemia (thalassemia) has been established (10-12), and the presence of high serum levels of copper has been...References1. COOLEYLEE TBP: Series of cases of splenomegaly in children with anemia and peculiar bone changes. Trans. Amer. Pediat. Soc. 37: 29, 1925. Google Scholar2. CHERNOFF AI: The distribution of the thalassemia gene: a historical review. Blood 14: 899, 1959. CrossrefMedlineGoogle Scholar3. WENTMACIVER LNJE: Thalassemia in the West Indies. Blood 17: 166, 1961. CrossrefMedlineGoogle Scholar4. WINTROBE MM: Clinical Hematology, 5th ed., Lea & Febiger, Philadelphia, 1961, p. 700. Google Scholar5. DIWANY M: Erythroblastic anemia with bone changes in Egyptian children: possible Cooley's type. Arch. Dis. Child. 19: 163, 1944. CrossrefMedlineGoogle Scholar6. NOUR EL DINAWNY GA: Target cell anemia; description of three cases. J. Roy. Egypt. Med. Ass. 33: 65, 1950. Google Scholar7. EL DEWISEDKIBEBAWI SAE: Study of target oval cell syndrome in three generations of an Egyptian family. J. Egypt. Med. Ass. 37: 941, 1954. Google Scholar8. AWNYZAGHLOUL AYI: Hemolytic anemias in Egypt. Bull. Clin. Sci. Soc. Abbassia Fac. Med. 6: 3, 1955. Google Scholar9. MIALEHOERMANDIWANY AKCM: Thalassemia major in Egypt. Gaz. Egypt. Paediat. Ass. 8: 584, 1960. Google Scholar10. PEARSONMCFARLANDKING HAWER: Erythrokinetic studies in thalassemia trait. J. Lab. Clin. Med. 56: 866, 1960. MedlineGoogle Scholar11. STURGEONFINCH PCA: Erythrokinetics in Cooley's anemia. Blood 12: 64, 1957. CrossrefMedlineGoogle Scholar12. CARTWRIGHTHUGNLEYASHENBRUCKERFAYWINTROBE GECMHJMM: Studies on free erythrocyte protoporphyrin, plasma iron and plasma copper in normal and anemia subjects. Blood 3: 501, 1948. CrossrefMedlineGoogle Scholar13. VALLEE BL: Biochemistry, physiology and pathology of zinc. Physiol. Rev. 39: 443, 1959. CrossrefMedlineGoogle Scholar14. PRASADMIALEFARIDSANDSTEADSCHULERTDARBY ASAZHHARWJ: Biochemical studies on dwarfism, hypogonadism and anemia. Arch. Intern. Med. (Chicago) 111: 407, 1963. CrossrefMedlineGoogle Scholar15. HOERMAN KC: Inhomogeneities in alkali resistant hemoglobins. Blood 17: 409, 1961. CrossrefMedlineGoogle Scholar16. SINGERCHERNOFFSINGER KAIL: Studies on abnormal hemoglobins. Blood 6: 413, 1951. CrossrefMedlineGoogle Scholar17. JONXISHUISMAN JHTH: A Laboratory Manual on Abnormal Hemoglobins, Blackwell Publications, Oxford, 1958. Google Scholar18. PRASADMIALESCHULERTFARIDSANDSTEAD ASAARZHH: Zinc metabolism in patients with the syndrome of iron deficiency anemia, hepatosplenomegaly, dwarfism and hypogonadism. J. Lab. Clin. Med. 61: 537, 1963. MedlineGoogle Scholar19. GUBLERLAHEYASHENBRUCKERCARTWRIGHTWINTROBE CJMEHGEMM: A method for the determination of copper in whole blood, red blood cells and plasma. J. Biol. Chem. 196: 209, 1952. CrossrefMedlineGoogle Scholar20. GARNER RJ: Colorimetric determination of magnesium in plasma or serum by means of titan yellow. Biochem. J. 40: 828, 1946. CrossrefMedlineGoogle Scholar21. MEITESFAULKNER SWR: Serum Iron, Manual of Practical Micro and General Procedures in Clinical Chemistry, Charles C Thomas, Publisher, Springfield, Ill., 1962, p. 267. Google Scholar22. WEBSTER WW: A simple microspectrophotometric method for the determination of serum calcium. Techn. Bull. Regist. Med. Techn. 32: 32, 1962. MedlineGoogle Scholar23. FISKESUBBAROW CHY: The colorimetric determination of phosphorus. J. Biol. Chem. 66: 375, 1925. CrossrefGoogle Scholar24. : Manual for Nutrition Surveys, Plasma or Serum Vitamin A and Carotene (Carr-Price), U. S. Government Printing Office, Washington, D. C., 1957. Google Scholar25. Ibid., p. 69. Google Scholar26. LUHBYCOOPERMANTELLER ALJMDN: Urinary excretion of formiminoglutamic acid; application in diagnosis of clinical folic acid deficiency. Amer. J. Clin. Nutr. 7: 397, 1959. CrossrefMedlineGoogle Scholar27. WASHSTEINGUADAITIS MMT: Detection of vitamin B6 deficiency: utilization of xanthurenic acid in urine. Amer. J. Clin. Path. 22: 652, 1952. CrossrefMedlineGoogle Scholar28. Sigma Technical Bulletin, No. 505, Serum Glutamic Oxalacetic Transaminase and Serum Glutamic Pyruvic Transaminase, Sigma Chemical Company, St. Louis, Mo., 1956. Google Scholar29. Sigma Technical Bulletin, No. 500, Serum Lactic Dehydrogenase, Sigma Chemical Company, St. Louis, Mo., 1957. Google Scholar30. Sigma Technical Bulletin, No. 104, Serum Alkaline Phosphatase, Sigma Chemical Company, St. Louis, Mo., 1957. Google Scholar31. BERFENSTAM R: Studies on blood zinc, Acta Paediat. (Stockholm) 87 suppl.: 7, 1952. Google Scholar32. NELSON W: Textbook of Paediatrics, 7th ed., W. B. Saunders, Philadelphia, 1960, p. 64. Google Scholar33. ABOUDALFIHEFNYEL MAZNY MAOAAR: An anthropometric study of Arabian school children. Gaz. Egypt. Paediat. Ass., 5 & 6: 493, 1957-1958. Google Scholar34. PRASADHALSTEDNADIMI ASJAM: Syndrome of iron deficiency anemia, hepatosplenomegaly, hypogonadism, dwarfism and geophagia. Amer. J. Med. 31: 432, 1961. CrossrefGoogle Scholar35. VALLEEWACKERBARTHOLMAYROBIN BLWEAFED: Zinc metabolism in hepatic dysfunction. New Eng. J. Med. 255: 403, 1956. CrossrefMedlineGoogle Scholar36. PRASADFLINK ASEB: The base binding property of serum protein with respect to calcium. J. Lab. Clin. Med. 51: 345, 1958. MedlineGoogle Scholar37. NICHOLWELCH CAAD: Synthesis of citrovorum factor from folic acid by liver slices; augmentation by ascorbic acid. Proc. Soc. Exp. Biol. Med. 74: 52, 1950. CrossrefMedlineGoogle Scholar38. CROSBYSACKS WHHJ: The coincidence of Mediterranean anemia and pernicious anemia in a young Sicilian. Blood 4: 1267, 1949. CrossrefMedlineGoogle Scholar39. GOLDBERGSCHWARTZ MASU: Mediterranean anemia in a Negro complicated by pernicious anemia of pregnancy. Blood 9: 648, 1954. CrossrefMedlineGoogle Scholar40. WESTTODD ESWR: Textbook of Biochemistry, The Macmillan Company, N. Y., 1955. Google Scholar41. CHOREMISECONOMOU-MARROUGREGORIOUS CCM: Blood plasma levels and urinary excretion of ascorbic acid before and after a test dose in children with severe thalassemia. J. Pediat. 59: 361, 1961. CrossrefMedlineGoogle Scholar42. LUHBYCOOPERMAN ALJM: Folic acid deficiency in thalassemia major. Lancet (no. 7200) 2: 490, 1961. MedlineGoogle Scholar43. WACKERULNERVALLEE WEDDBL: Metalloenzymes and myocardial infarction. New Eng. J. Med. 255: 449, 1956. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: From the Medical Department, U. S. Naval Medical Research Unit No. 3, Cairo, Egypt, U. A. R., the Division of Nutrition, Departments of Medicine and Biochemistry, Vanderbilt University, Nashville, Tenn., and the Pediatric Department, Faculty of Medicine, Munira Children's Hospital, Cairo University, Cairo, Egypt, U.A.R.This study was supported by research contract Nonr 2149 from the Navy Department, Office of Naval Research to Vanderbilt University, Nashville, Tenn., and by an agreement with the National Institutes of Health under Public Law 480.This paper was presented at the Tenth Congress of the International Society of Hematology in Stockholm, Sweden, September 2, 1964.Requests for reprints should be addressed to Ananda S. Prasad, M.D., Wayne State University School of Medicine, Department of Medicine, 1400 Chrsyler Expressway, Detroit 7, Mich. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByApplication of Acyzol in the Context of Zinc Deficiency and PerspectivesThalassemia SyndromesZinkbedarf und Risiko und Nutzen einer ZinksupplementierungZinc supplements for treating thalassaemia and sickle cell diseaseHuman Zinc Deficiency: Discovery to Initial TranslationZinc supplementation decreases oxidative stress, incidence of infection, and generation of inflammatory cytokines in sickle cell disease patientsZinc**Dr. Carl-Gustaf Elinder was the author of this chapter in the 2nd edition of the Handbook on Toxicology of Metals; his text provided guidance.Zinc requirements and the risks and benefits of zinc supplementationEffect of desferrioxamine on urinary copper and zinc excretion in ?-thalassemia major patientsNutritional factors and thalassaemia major.Effects of zinc supplementation on linear growth in beta-thalassemia (A new approach)ReplyZinc in Human NutritionPLASMA-COPPER IN SICKLE-CELL ANÆMIAINCREASED COPPER/ZINC RATIOS IN ACRODERMATITIS ENTEROPATHICAPATIENT RESPONSE TO INDUCTION OF LABOURTowards a Reborn G.M.C.Copper and Steely HairSERUM-COPPER IN SICKLE-CELL ANÆMIAORAL ZINC SULPHATE IN SICKLE-CELL ULCERSPLASMA-ZINC IN HEALTH AND DISEASEMethodology of Zinc Determinations and The Role of Zinc in Biochemical ProcessesPlasma Zinc Concentration in Liver DiseasesHemopoietic System 1 January 1965Volume 62, Issue 1Page: 87-96KeywordsBeta-thalassemiaBiochemistryBlood plasmaDehydrogenasesGel electrophoresisHematology and oncologyHemoglobinHospital medicineThalassemiaZinc Issue Published: 1 January 1965 PDF DownloadLoading ..." @default.
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