A Bibliography ‘AAT'ON4< LE 90“ C64 < “@OGRP‘J‘ 9S\7ras\o Comprehensive Sickle Cell Centers US. DEPARTMENT OF HEALTH AND HUMAN SERVICES Public Health Service National Institutes of Health lg, ' A BIBLIOGRAPHY Ver r” / LC/ONPREHENSIVE SICKLE CELL .CENTER PROGRAM 1972 - 1980’ Compiled by Calvin Lilgalhoun, Jr., M.D. Marilyn H. Gaston, M.D. National Sickle Cell Disease Program Division of Blood Diseases and Resources National Heart, Lung, and Blood Institute Bethesda, Maryland 20205 Revised September l980 U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES Public Health Service _4 2,.National Institutes of Health 4 NIH Publication No. 8l-l460 ' November l980 ‘ / /4¢ .-‘:"( V ‘1‘ f v {- Q V7€0§0X fluf’yL 75 II. III. IV. VI. VII. VIII. IX. TABLE OF CONTENTS COMPREHENSIVE SICKLE CELL CENTERS INTRODUCTION HEMOGLOBIN DIAGNOSIS CELLULAR GENETICS Counseling IMMUNOLOGY FLOW STUDIES DRUG DEVELOPMENT Sodium Cyanate Carbamyl Phosphate Zinc Fagara Xanthoxyloides Other CLINICAL Genera] Cardiovascular CentraI Nervous System Endocrine Eye Prenatal Diagnosis Rena] SkeTetaI MANAGEMENT 415054; ”A704 7/ 5;} 637/ /7io fl/SL iii iv 20 27 w M 48 51 54 54 56 57 59 60 6T 6T 67 68 69 75 77 78 79 X. XI. XII. SICKLE CELL TRAIT PSYCHOSOCIAL GENERAL ii 82 83 86 COMPREHENSIVE SICKLE CELL CENTERS Comprehensive Sickle Cell Center* University of Chicago 950 East 59th Street Chicago, Illinois 60637 Comprehensive Sickle Cell Center University of Miami School of Medicine P.0. Box 520875, Biscayne Annex Miami, Florida 33l25 Comprehensive Sickle Cell Center * USC School of Medicine 2025 Zonal Avenue Los Angeles, California 90033 Comprehensive Sickle Cell Center SUNY—Downstate Medical Center 450 Clarkson Avenue Brooklyn, New York ll203 Comprehensive Sickle Cell Center* Medical College of Georgia Augusta, Georgia 30902 Comprehensive Sickle Cell Center University of Tennessee 800 Madison Avenue Memphis, Tennessee 38163 Comprehensive Sickle Cell Center* Children's Hospital Research Foundation Elland and Bethesda Avenue Cincinnati, Ohio 45229 Comprehensive Sickle Cell Center Indiana University l2l9 West Michigan Street Indianapolis, Indiana 46202 *Active Comprehensive Sickle Cell Center* University of Illinois l855 West Taylor Street Chicago, Illinois 606l2 Comprehensive Sickle Cell Center* Martin Luther King, Jr. Gen. Hospital l2020 South Wilmington Avenue Los Angeles, California 90059 Comprehensive Sickle Cell Center* Howard University College of Medicine 2l21 Georgia Avenue, N.w. Washington, D.C. 20059 Comprehensive Sickle Cell Center Harlem Hospital Center l35th Street and Lenox Aveue New York, New York l0037 Comprehensive Sickle Cell Center University of Pittsburgh School of Medicine Terrance and DeSoto Streets Pittsburgh, Pennsylvania l52l3 Comprehensive Sickle Cell Center* Boston City Hospital 818 Harrison Avenue Boston, Massachusetts 02ll8 Comprehensive Sickle Cell Center* Children's Hospital of Michigan 390l Beaubien Boulevard Detroit, Michigan 4820l Comprehensive Sickle Cell Center* San Francisco General Hospital 995 Potrero Avenue San Francisco, California 94ll0 INTRODUCTION The National Heart, Lung, and Blood Institute of the National Institutes of Health was presented with the challenge of establishing a multifaceted, national program for the alleviation of the multivariate problems associated with sickle cell anemia and related disorders. The National Sickle Cell Disease Advisory Committee in August l97l, emphasized the need for a coordinated pro- gram responsive to the needs, attitudes and opinions of the client population. The importance of utilizing available sophisticated, scientific competence and capability in the pursuit of investigational efforts with immediate transmission and translation of research findings and innovations for the common weal of the involved public was profoundly evident. To satisfy these recognized disparate needs, an unprecedented concept was promulgated resulting in the establishment of Comprehensive Sickle Cell Centers. These centers incorporate 1) basic and clinical research projects, 2) educational programs for the general public, paraprofessional and professional personnel and practicing physicians, 3) accu- rate diagnosis of hemoglobin types, and 4) appropriate counseling services for all persons diagnosed with hemoglobinopathies. During the 8 years (1972—80) of their existence the Comprehensive Sickle Cell Centers have proven the effectiveness of this administrative mechanism in insuring the informed participation of the client population in these programs and in stimulating research efforts. These investigational studies encompass the total spectrum of studies from the molecular characterization, through cellular, tissue, and organ systems to individual relationships and psychosocial interactions. A significant increase in the cadre of investigators pursuing such studies and total knowledge of sickle hemoglobin and related manifestations has been generated through the activities of these centers. The various efforts of the Comprehensive Sickle Cell Centers Program and resultant publications have been extremely valuable in the acquisition and dissemination of new knowledge in this categorical program's related areas of biomedical research. The bibliographic references presented represent an accumulation of signi- ficant publications from the Comprehensive Sickle Cell Centers resulting from their programs during 1972—80. This document should serve as an authoritative source of information and findings in studies related to sickle cell disease, and provide a foundation for continued investigations. Periodic revision and updating is anticipated to insure currency of listings and dissemination of most recent research results. George B. Riley, Ph.D. 10. 11. 12. HEMOGLOBIN Abraham, E.C. and Huisman, T.H.J.: Differences in Affinity of Variant B Chains for a Chains: A Possible Explanation for the Variation in the percentages of 8 Chain Variants in Heterozygotes. Hemoglobin l(8):86l- 873, 1977. Abu-Sin, A., Felice, A., Gravely, M.E., Wilson, J.B., Reese, A., Lam, H., Miller, A. and Huisman, T.H.J.: Hb P-Nilotic in Association with B - Tha1assemia: Cis-Mutation of a Hemoglobin BA Chain Regulatory Determinant? Journal of Laboratory and Clinical Medicine (In Press). Aksoy, M., Erdenk, S., Efremov, G.D., Wilson, J.B., Huisman, T.H.J., Schroeder, w.A., She1ton, J.R., Shelton, J.B., Ulutih, O.N. and Muftuglu, A.: Hemoglobin Istanbul. Substitution of Glutamine for Histidine in a Proximal Histidine (F-8 (92)B). J. C1in. Invest. 51:2380, 1972. Aksoy, M., Erdem, S., Schroeder, w.A. and Huisman, T.H.J.: Hemoglobins A2 and F in Chronic Benzene Poisoning. Intern. Instanbul Symp. Abn. Hbs. & Thal. Turkey, August, 1974. A1tay, C., Altinoz, N., Wilson, J.B., Bolch, K.C. and Huisman, T.H.J.: Hemoglobin Hacettepe or a282127 (H5) Gln+Glu. Biochim. Biophs. Acta 434:13, 1976. A1tay, C., Niazi, G.A. and Huisman, T.H.J.: The Combination of Hb—S and Hb-E in a Black Female. Hemoglobin 1:100, 1976. Altay, C., Schroeder, w.A. and Huisman, T.H.J.: The GY-GB-Thalassemia and GY—BO-HPFH Conditions in Combination with B-Thalassemia and Hb S. Am. J. Hemat. 321-14, 1977. A1tay, C., Ringelhann, B., Yawson, G.I., Bruce-Tagoe, A.A., Konotey-Ahulu, F.I.D., James, L., Gravely, M. and Huisman, T.H.J.: Hemoglobin a Chain Deficiency in Black Children with Variable Quantities of Hemoglobin Bart's at Birth. Pediatric Research 11:147-152, 1977. Altay, C., Say, 3., Yetgin, S. and Huisman, T.H.J.: a-Thalassemia and B-Thalassemia in a Turkish Family. Amer. J. Haemat. 2:1-15, 1977. A1tay, C., Huisman, T.H.J and Schroeder, w.A.: Another Form of the Hereditary Persistence of Fetal Hemoglobin (The Atlanta Type)? Hemoglobin 1:125-133, 1977. A1tay, C., Huisman, T.H.J. and Moores, R.R.: Probable Inclusion-Body B Tha1assemia in a Chinese Family. Hemoglobin 1:214-217, 1977. Alter, B.P., Rappeport, J.M., Huisman, T.H.J., Schroeder, w.A. and Nathan, D.G.: Fetal Erythropoiesis Following Bone Marrow Transplantation. Blood 48:843-853, l976. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. Amma, E.L., Sproul, G.D., Wong, S. and Huisman, T.H.J.: Mechanism of Sickling in Deer Erythrocytes. Ann. N.Y. Acad. Sci. 241:605-613, 1974. Bank, A., Mears, G., Weiss, R., O'Donnell, J.V. and Natta, C.: Preferential Binding of BS Globin Chains Associated with Stroma in Sickle Cell Disorders. J. Clinical Invest. 54:805, 1974. Bank, A., Gambino, R., Ramirez, F., Maniatis, G., Natta, C., Kacian, D., Spiegelman, S., and Marks, P.A.: Regulation of Globin Synthesis in the Tha1assemias. Ann. N.Y. Acad. Sci. 241:246, 1974. Bank, A., Dow, L.w., Farace, M.D., O‘Donnell, J.V., Fors, S. and Natta, C.: Changes in Globin Synthesis with Erythroid Cell Maturation in Sickle Thalassemia. Blood 41:353, 1973. Bannister, J.V., Bannister, w.H., Wilson, J.B., Lam, H., Miller, A. and Huisman, T.H.J.: The Structure of Goat Hemoglobins V. A Fourth 8 Chain Variant (B-D-Malta; 69 Asp + Gly) with Decreased Oxygen Affinity and Occurring at a High Frequency in Malta. Hemoglobin (In press). Bannister, N.H., Grech, J.L., Plese, C.F., Smith, L.L., Barton, B.P., Wilson, J.B., Reynolds, C.A. and Huisman, T.H.J.: Hemoglobin St. Luke's or a2 95 Arg (GZ) 82. Europ. J. Biochem. 29:301, 1972. Bannister, w.A., Grech, J.L., Huisman, T.H.J. and Schroeder, w.A.: Abnormal Haemoglobins in Malta. The Significance of Two Fetal and Adult Variant. Saint Luke's Gazette 7:69, 1972. Beaudoin, A.G. and Mizukami, H.: ESR Correlation Times of 2,2,6,6,- Tetramethyl Piperidone-N-Oxyl (tempone) in Solutions of Hemoglobin A and Hemoglobin 5, Biochim. Biophys. Acta 532:41-47, 1978. Bernstein, S.C., Bowman, J.E. and Noche, L.K.: Genetic Variation in Cameroon. Thermostability Variants of Hemoglobin and of Glucose-6- Phospate Dehydrogenase. Biochemical Genetics (In press). Bernstein, S.C., Bowman, J.E. and Noche, L.K.: Interaction of Sickle Cell Trait and Glucose-6-Phosphate Dehydrogenase Deficiency in Cameroon. Human Heredity (In press). Bernstein, S.C., Bowman, J.E. and Noche, L.K.: Population Studies in Cameroon. Hemoglobin S, Glucose-6-Phosphate Dehydrogenase Deficiency and Falciparum Malaria. Human Heredity (In press). Bernstein, S.C. and Bowman, J.E.: The Demonstration of Asymmetric Hemoglobin Hybrids by Polyacrylamide Electrophoresis. Biochim. et Biophysica Acta 427:512-519, 1976. Bhattacharya, S.P., Anyaibe, 5.1.0 and Headings, V.E.: Biological Variation in the Heterogenous Distribution of Haemoglobin F Among Erythrocytes. Brit. J. Haemat. 33:401-408, 1976. 26. 27. 28. 29. 30. 31. 32. 33. 34. 35. 36. 37. 38. Blunt, M.H. and Huisman, T.H.J.: The Haemoglobins of Sheep in The Blood of Sheep Composition and Function, pp. 155-183, ed. M.H. Blunt. Springer- Verlag Berlin-Heidelberg and New York, publ. 1975. Bowman, J.E.: The Population Genetics of Abnormal Haemoglobins in the United States in Haemoglobinopathy "Sickle Cell", Reproduction and Contraception, ed. B. Kwaku Adedevah, M.D. (In press). Breen, J.J., Bertoli, D.A., Dakok, J. and Ho, C.: Proton Magnetic Resonance Studies of Human Adult Hemoglobin in Water. Biophys. Chem. 2:49, 1974. Brittenham, G., Lozoff, 8., Harris, J.W., Nayudu, N.V.S., Gravely, M., Wilson, J.B., Lam, H. and Huisman, T.H.J.: Hemoglobin Hofu or a282 [126 (H4) Val + Glu] Found in Combination with Hemoglobin S. Hemoglobin (In press). Brown, W.J., Niazi, G.A., Jayalakshmi, M., Abraham, E.C. and Huisman, T.H.J.: Hemoglobin Athens-Georgia, or agsg 40 (C6) Arg + Lys, A. Hemoglobin Variant with an Increased Oxygen Affinity. Biochim. Biophys. Acta 439:70, 1976. C rcassi, U.E.F., Pintus, A., Gravely, M.E. and Huisman, T.H.J.: B -Thalassemia in Association with Hb Leslie (a282 131 Gln + 0) in a Sardinian Family. Hemoglobin (In press). Castro, 0., Lee, T.C.K., Headings, V.E. and Winter, W.P.: Prevalence of a Chain Variants at Birth. Blood 50:103, 1977. Castro, 0., Winter, W.P., Doan, R.J., et al: Heat Stability Studies in Hemoglobin Agenogi. Clinical Research 24:630A, 1976. Castro, 0., Winter, W.P., Bullock, W.H., Jilly, P.N. Gvozden, A.B. and Rucknagel, D.L.: Hemoglobin D Ibadan Trait in Combination with 68 Thalassemia. Hemoglobin 3:77-82, 1979. Chay, T.R. and Ho, C.: Statistical Mechanics Applied to Cooperative Ligand Binding to Proteins. Proc. Nat. Acad. Sci., U.S.A. 70:3914, 1973. DeSimone, J. and Adams, J.G. III: Evidence for Rapid Loss of Newly Synthesized Hemoglobin S Molecules in Sickle Cell Anemia. First National Symposium on Sickle Cell Disease. DHEW Pub. No. (NIH) 75-723zl83, 1974. DeSimone, J., Kleve, L., Longley, M.A. and Shaeffer, J.: Rapid Turnover of Newly Synthesized BS Chains in Reticulocytes from Individuals with Sickle Cell Trait. Biochem. Biophys. Res. Commun. 57:248, 1974. DeSimone, J., Kleve, L., Longley, M.A. and Shaeffer, J.: Unbalanced Globin Chain Synthesis in Reticulocytes of Sickle Cell Trait Individuals with Low Concentrations of Hemoblogin S. Biochem. Biophys. Res. Commun., 59:564, 1975. 39. 40. 41. 42. 43. 44. 45. 46. 47. 48. 49. 50. Dill, K., Satterlee, J., Richards, J.H.: 13C Nuclear Magnetic Resonance Studies of the Binding of Isocyanides to Various Hemoglobins and Myoglobins. Biochemistry l7z429l-4298, l978. Dingle, B., Farquharson, H., Williams, 0., Wilson, J.B., and Huisman, T.H.J.: Hb Rainier or @282 (l45(HC2)Tyr + Cys) Observed in Members of a Canadian Family of Greek Origin. Hemoglobin (In press). Efremov, G.D., Aksoy, M. and Huisman, T.H.J.: A Note on Heme Loss in Hemoglobin Istanbul. Intern. Istanbul Symp. Abn. Hbs & Thal., Turkey, August, l974. Efremov, G.D., Huisman, T.H.J., Stanulovic, M., Zurovec, M., Duma, H., Wilson, J.B. and Jeremic, V.: Hemoglobin M Saskatoon and Hemoglobin M Hyde Park in Two Yugoslavian Families. Scand. J. Haemat. l3:48, l974. Efremov, G.D., Ruvidic, R., Niazi, G.A., Hunter, E., Jr. and Huisman, T.H.J. and Schroeder, W.A.: An Individual with Hb-Lepore—Baltimore- GB-Thalassemia in a Yugoslavian Family. Scand. U. Haemat. l6:8l, l976. Efremov, G.D., Apostolova, S., Duma, H., Wilson, J.B. and Huisman, T.H.J.: Hemoglobin O-Arab in a Gypsy Family of SR Macedonia, Yugoslavia. Acta Med. Jug. 27:359, l973. Efremov, G.D., Duma, H., Ruvidic, R., Rolovic, 2., Wilson, J.B. and Huisman, T.H.J.: Hemoglobin Beograd or agsg lZl Glu + Val (GH4). Biochim. Bi0phys. Acta 328:81, l973. Efremov, G.D., Sodikario, A., Stojmirovic, E., Schroeder, W.A., Shelton, J.R., Shelton, J.B., Apell, G., Wilson, J.B., Brodie, A.R. and Huisman, T.H.J.: Chemical Heterogeneity of Foetal Haemoglobin in the Lepore Haemoglobinopathy. Brit. J. Haemat. 27:319-329, l974. Efremov, G.D., Nikolov, N., Duma, H., Schroeder, W.A., Miller, A. and Huisman, T.H.J.: SB-Thalassemia in Two Yugoslavian Families. Scand. J. Haemat. 14:226, l975. Efremov, G.D., Sadikario, A., Stojancov, A., Dojcinov, D. and Huisman, T.H.J.: Homozygous Hemolgobin O Arag in a Gypsy Family in Yugoslavia. Hemoglobin l(4):389-394, 1977. Efremov, G.D., Stojmirovic, E., Lam, H.L., Wilson, J.B. and Huisman, T.H.J.: Hb Beth Israel ( BlOZ (G4) Asn + Ser) Observed in a Yugoslavian Teenager. Hemoglobin 2(l):75-77, l978. Efremov, G.D., Wilson, J.B. and Huisman, T.H.J.: The Chemical Heterogeneity of Human Hemoglobin F: Direct Evidence for the Existence of Three Types of Y Chains, the IGY, IAY, and TAY, Chains. Biochim. Biophys. Acta (In press). 51. 52. 53. 54. 55. 56. 57. 58. 59. 60. 61. 62. Farace, M.G. and Bank, A.: Control of Human Hemoglobin Synthesis. Trans- lation of Globin Chain in Heterozygotes with Hemoglobin Riverdale-Bronx. Biochim. Biophys. Acta 3l2:59l, 1973. Felice, A., Abraham, E.C., Miller, A., Cope, N., Gravely, M. and Huisman, T.H.J.: Post-Translational Control of Human Hemoglobin Synthesis: The Number of a Chain Genes and the Synthesis of Hb S. "Brewer Symposium" The Red Cell. Alan R. Liss, New York, NY, pp. l3l—l54, l978. Felice, A., Webber, B., Miller, A., Mayson, S.M., Harris, H.F., Henson, J.B., Gravely, M.E. and Huisman, T.H.J.: The Association of Sickle Cell Anemia with Heterozygous and Homozygous a-Thalassemia-Z: In vitro Hb Chain Synthesis. Amer. J. Hemat. (In press). Felice, A., Abraham, E.C., Miller, A., Stallings, M. and Huisman, T.H.J.: Is the Trimodality of Hb Leslie (G282 l3l.Glu + O) in Heterozygotes the Result of a Variable Number of Active a Chain Genes? Evidence for Post- Translational Control of Hemoglobin Synthesis. Amer. J. Hemat. 5:l-9, l978. Felice, A. and Huisman, T.H.J.: Observations on the Calculated Content of Variant and Normal a Chains in Adult and Fetal Erythrocytes. Hemoglobin (In press). Frischer, H. and Bowman, J.: Hemoglobin E and Oxidatively Unstable Mutation. Journal of Laboratory and Clinical Medicine 85:531-539, l975. Fung, L.w.M. and Ho, C.: A Proton Nuclear Magnetic Resonance Study of the Quaternary Structure of Human Hemoglobins in Water. Biochemistry 14:2526, l975. Fung, L.N.M., Ho, C., Roth, E.R. and Nagel, R.L.: The Alkylation of Hemoglobin S by Nitrogen Mustard, High-Resolution Proton Nuclear Magnetic Resonance Studies. J. Biol. Chem. 250:4786, l975. Fung, L.w.M., Lin, K.L.C. and Ho, C.: High-Resolution Proton Nuclear Magnetic Resonance Studies of Sickle Cell Hemoglobin. Biochemistry l4: 3424, l975. Geerdink, R.A., Schillhorn Van Veen, J.M., de Leeuw, R., Hamelink, M.L., Stoop, J.M., Wilson, J.B. and Huisman, T.H.J.: Hemoglobin D-Los Angeles (1232 lZl Glu+ Gln) and J—Baltimore (azsg l6 Gly+ Asp) in Two Dutch Families. The Netherlands Journal of Medicine l8:lO6, l975. Gidari, A.S. and Levere, R.D.: Stimulation of Heme and Hemoglobin in Cultured Sickle Cell Marrow in Response to Erythropoetin. Clinical Research 22:391a, l974. Girling, R.L., Schmidt, W.C., Jr., Houston, T.E., Amma, E.L. and Huisman, T.H.J.: The Molecular Packing and Intermolecular Contacts of Sickling Deer Type III Hemoglobin. J. Mol. Biol. l3l:4l7-433, 1979. 63. 64. 65. 66. 67. 68. 69. 70. 7l. 72. 73. Girling, R.L., Houston, T.E., Schmidt, W.C., Jr., Amma, E.L. and Huisman, T.H.J.: Macromolecular Structure Refinement by Restrained Least-Squares and Interactive Graphics as Applied to Sickling Deer Type III Hemoglobin. Acta Crystallogr. (In press). Girling, R.L., Houston, T.E. and Amma, E.L.: An X-ray Determination of the Molecular Interactions in Hemoglobin C: A Disease Characterized by Intraerythrocytic Crystals. Biochem. Biophys. Res. Commun. 88:768-773, l979. Goldberg, M., Bunn, F.H., Muss, H. and Harkness, D.: Studies on the Viscosity of Sickle Hemoglobin. Evidence for an Intermediate Phase in Gelation. Proceedings of the Symposium on Molecular and Cellular Aspects of Sickle Cell Disease, p. l33, Dallas, December, l975, DHEW Publication No. (NIH) 76-l007. Gorecki, M., Votano, J.R. and Rich, A.: "Peptide Inhibitors of Sickle Hemoglobin Aggregation: Effect of Hydrophobicity. Biochemistry (In press) l979. Gravely, M.E., Harris, H.F., Stallings, M., Lam, H., Wilson, J.B. and Huisman, T.H.J.: Hb Suresnes or a2 l4l (HC3) Arg + His 82 in a Black Family. Hemoglobin 2(2):l87-l89, l978. Gunay, U., Pauli, C., Shamsuddin, M., Mason, R.G., Heinze, W.J. and Hong, E6R'l Sickle Hemoglobin in Combinationwith HB JBangkok (“282 g Y'+ aSp). Blood 44:638-690, 1974. Hanash, S.M., Rucknagel, D.L., Sing, C.F., Winter, W.P., Whitten, C.F. and Prasad, A.S.: Age Distribution and Correlation of Hemoglobins F and A2 in Sickle Cell Anemia in Cellular and Molecular Regulation of Hemo- globin Switching, Stamatoyannopoulos, G. and Nienhius, A., ed. erne & Stratton, New York, l979. Harkness, D.R. Villa, L., Berman, 1., Wilson, J.B. and Huisman, T.H.J.: Biosynthetic and Structural Studies of Hemoglobin in a Patient with Congenital Dyserythropoietic Anemia Type I. Hemoglobin l(7):663-677, l977. Harris, M.J., Huisman, T.H.J. and Hayes, F.A.: Geographic Distribution of Hemoglobin Variants in the White-Tailed Deer. J. Mammalogy 54:27l-274, l973. Harris, M.J., Wilson, J.B. and Huisman, T.H.J.: Two Hemoglobin Phenotypes in the American Bison (Bison bison): a Possible Genetic Explanation Based on Structural Studies. Biochemical Genetics 9:l-l2, 1973. Harris, M.J., Wilson, J.B. and Huisman, T.H.J.: Structural Studies of Hemoglobin a Chains from Virginia White-Tailed Deer. Arch. Biochem. Bi0phys. l5l:540-548, l972. 74. 75. 76. 77. 78. 79. 80. 81. 82. 83. 84. 85. Headings, V., Anyaibe, 5.1.0 and Bhattacharya, 5.: Diagnosis of Hemoglobin Phenotype in Individual Fetal Red Cells. Perinatal Approaches to the Diagnosis of Fetal Hemoglobinopathies. Proceedings of a Research Workshop 1978 (In press). Henson, J. and Huisman, T.H.J.: Possible Relationship Between the Level of Hb Bart's (Y4) and the Relative Amount of Hb S or Hb C in Black Heterozygous Newborn. Hemoglobin 2(4):393-398, 1978. Ho, C., Lindstrom, T.R., Baldassare, J.J. and Breen, J.J.: Magnetic Resonance Studies of Human Hemoglobins and their Implications to the Structure-Function Relationships in Human Normal and Abnormal Hemoglobins. Ann. N.Y. Acad. Sci. 222:31, 1973. Ho, C., Lin, K.A., Fung, L.N.M. and Johnson, M.E.: Proton Nuclear Magnetic Resonance Studies of Sickle Cell Hemoglobin. Proceedings of the First National Symposium on Sickle Cell Disease, Washington, D.C., June 26-29, 1974, DHEN Pub. No. (NIH) 75-723, pp. 117-119, 1974. Ho, C., Fung, L.N.M., Niechelman, K.J., Pifat, G. and Johnson, M.E.: Recent NMR Studies on Quarternary Structural Transitions in Human Hemoglobins in Erythrocyte Structure and Function, eds. Brewer, G.J. and Liss, A.R., Inc., New York, pp. 43-64, 1975. Honig, G.R., Green, D., Shamsuddin, M., Vida, J.N., Mason, R.G. and Maurer, H.S.: Hemoglobin Abraham Lincoln, B32 (814) Leucine Proline, An Unstable Variant Producing Severe Hemolytic Disease. J. Clin. Invest. 52:1746-1755, 1973. Honig, G.R., Mason, R.G., Vida, L.N. and Shamsuddin, M.: Synthesis of Hemoglobin Abraham Lincoln (B32 Leu + Pro). Blood 43:657-664, 1974. Honig, G.R., Mason, R.G. and Vida, L.N.: Globin Chain Synthesis in Sickle Cell Trait Under Conditions of Folate Antagonism. Acta Haemat. 51:236- 239, 1974. Honig, G.R., Shamsuddin, M., Mason, R.G. and Vida, L.N.: Hemoglobin Lincoln Park: A 86 Fusion (anti-Lepore) Variant with an Amino Acid Deletion in the 5 Chain-Derived Segment. Proc. Natl. Acad. Sci. U.S.A. 75:1475-1479, 1978. Honig, G.R., Mason, R.G., Tremaine, L.M. and Vida, L.N.: Sickle Cell Syndromes III. Silent-Carrier a-Thalassemia in Combination with Hemoglobin S and Hemoglobin C. Pediatric Research 13:1109-1111, 1979. Honig, G.R., Mason, R.G., Shamsuddin, M., Vida, L.N., Rao, K.R.P. and Patel, A.R.: Two New Sickle Cell Syndromes: Hb S, Hb Camden, and a-Thalassemia; and Hb S in Combination with Hb Tacoma. Blood (In press), 1980. Honig, G.R., Shamsuddin, M., Mason, R.G., Vida, L.N., Tremaine, L.M., Tarr, G.E. and Shahidi, N.: Hemoglobin Nigeria (9-81 Ser'+ Cys): A New Variant Associated with a-Thalassemia. Blood (In press), 1980. 86. 87. 88. 89. 90. 91. 92. 93. 94. 95. 96. 97. 98. Houston, T.E., Girling, R.L., Amma, E.L. and Huisman, T.H.J.: Structure of Human Hemoglobin C: A Disease with Intraerythrocytic Crystals. Biochim. Biophys. Acta 576:497-50l, l979. Houston, T.E., Girling, R.L., Bridges, A.B., Amma, E.L. and Huisman, T.H.J.: The X-ray Structure Solution and Electron Microscopy of Hemoglobin C Crystals. Biochim. Biophys. Acta (In press). Houston, T.E., Girling, R.L., Amma, E.L. and Huisman, T.H.J.: Structure of Human Hemoglobin C: A Disease with Intraerythrocytic Crystals. Biochim Biophys. Acta 576:479-59l, l979. Houston, T.E., Plese, C.F., Girling, R.L. and Amma, E.L.: Crystallography and Oriented Single Crystal Electron Microsc0py of Hemoglobin Deer II, A Hemoglobin that Exhibits Matchstick-Shaped Erythrocytes. Biochim Biophys. Acta 535:413-4l7, l978. Hubbard, M., Winton, E.F., Lindeman, J.G., Dessauer, P.L., Wilson, J.B., Wrightstone, R.N. and Huisman, T.H.J.: Hemoglobin Atlanta or a282 75 Leu + Pro (El9). An Unstable Variant Found in Several Members of a Caucasian Family. BBA 386:538, l975. Huestis, W.H. and Raferty, M.A.: Conformation and Cooperativity in Hemoglobin. Biochemistry l4:l886, l975. Huisman, T.H.J.: Normal and Abnormal Human Hemoglobins. Adv. Clin. Chem. l5:l50—253, l972. Huisman, T.H.J.: Structural Aspects of Fetal and Adult Hemoglobins from Nonanemic Ruminants. Ann. N.Y. Acad. Sci. 24l:392-4l0, l974. Huisman, T.H.J.: The in vivo Production of Hemoglobin C in Ruminants. Ann. N.Y. Acad. Sci. 24l:549-555, l974. Huisman, T.H.J.: Trimodality in the Percentages of 8 Chain Variants in Heterozygotes: The Effect of the Number of Active Hba Structural Loci. Hemoglobin l(4):349-382, 1977. Huisman, T.H.J.: The Hemoglobin P—Galveston-Hb-C Condition in Members of a Black Family from South Carolina. FEBS Letters 94:68-72, l978. Huisman, T.H.J., Gravely, M.E., Henson, J., Felice, A., Wilson, J.B., Abraham, E.C., Vella, F. and Little, M.W.: Variability in the Interaction of B-Thalassemia with a Chain Variants Hb G-Philadelphia and Hb Rampa. Journal of Laboratory and Clinical Medicine 92:3ll-320, l978. Huisman, T.H.J., Gravely, M.E., Wilson, J.B., Webber, B., Felice, A.E. and Miller, A.: Interaction of the 8 Chain Variant Hemoglobin Leslie and the a Chain Variant Hemoglobin Montgomery in a Black Female. The American Journal of Hematology (In press). 99. 100. 101. 102. 103. 104. 105. 106. 107. 108. 109. Huisman, T.H.J., Harris, H., Grave1y, M., Schroeder, N.A., She1ton, J.R., She1ton, J.B. and Evans, L.: The Chemica1 Heterogeneity of the Feta1 HemogTobin in Norma1 Newborn Infants and in Adu1ts. Mo1ecu1ar and Ce11u1ar Biochemistry 17(1):45-55, 1977. Huisman, T.H.J. and Mi11er, A.: Hb-Grady and a-Tha1assemia. A Contribution to the Prob1em of the Number of Hb Structura1 Loci in Man. Am. J. Hum. Genet. 28:363, 1976. Huisman, T.H.J. and Mi11er, A.: Hemog1obin Types in Barbary Sheep (Ammotsagus Lervia Pa11as, 1777); Absence of a BC Production in a Homozygous BC (na Anima1 During Severe Anemia. Proc. Soc. Exp. Bio1. Med. 140:815- 819, 1972. Huisman, T.H.J., Mi11er, A., Cook, L., Gordon, S. and Schroeder, w.A.: The Mo1ecu1ar Heterogeneity of Some Types of Hereditary Persistence of Feta1 Hemog1obin (HPFH). Intern. Istanbu1 Symp. Abn. Hbs & Tha1., Turkey, August, 1974. Huisman, T.H.J., Mi11er, A. and Schroeder, w.A.: A Gv-Type of the Hereditary Persistence of Feta1 Hemog1obin with 8 Chain Production in gig. Amer. J. Hum. Gen. 27:765-777, 1975. Huisman, T.H.J., Jones, R.T., Labie, D., Lehmann, H., McCurdy, P.R., Rose, J. and Weathera11, D.J.: Mechanisms for Reporting and Recording Second Subse- quent Findings of Mutant Hemog1obins. Int. Instanbu1 Symp. Abn. Hbs & Tha1., Turkey, August, 1974. Huisman, T.H.J. and Schroeder, w.A.: Feta1 Hemog1obin Synthesis in Some Forms of Leukemia. Protides of the Bio1ogica1 F1uids. H. Peeters, ed. Pergamon Press, 1976. Huisman, T.H.J., Schroeder, w.A., Bannister, w.H. and Grech, J.L.: Evidence for Four Nona11e1ic Structura1 Genes for the v Chain of Human Feta1 Hemo- g1obin. Biochemica1 Genetics 7:131—139, 1972. Huisman, T.H.J., Schroeder, w.A., Brown, A.K., Hyman, C.B., Ortega, J.A. and Sukumaran, P.K.: Further Studies of the Postnata1 Change in Chemica1 Heterogeneity of Human Feta1 Hemog1obin in Severa1 Abnorma1 Conditions. Pediatric Research 9:1-6, 1975. Huisman, T.H.J., Schroeder, w.A., Efremov, G.D., Duma, H., M1adenovsky, B., Hyman, C.B., Rachmi1ewitz, E.A., Bouver, N., Mi11er, A., Brodie, A., She1ton, J.R., She1ton, J.B. and Ape11, G.: The Present Status of the Heterogeneity of Feta1 Hemog1obin in B-Tha1assemia. An Attempt to Unify Some Observations in Tha1assemia and Re1ated Conditions. Ann. N.Y. Acad. Sci. 232:107, 1974. Huisman, T.H.J., Schroeder, w.A., Fe1ice, A., Powars, D. and Ringe1hann, B.: An Anoma1y in the Y Chain Heterogeneity of the Newborn. Nature 265:63, 1977. 110. 111. 112. 113. 114. 115. 116. 117. 118. 119. 120. 121. Huisman, T.H.J., Schroeder, N.A., Bouver, N.G., Mi11er, A., She1ton, J.R., She1ton, J.B. and Ape11, G.: Chemica1 Heterogeneity of Feta1 Hemog1obin in Subjects with Sick1e Ce11 Anemia, Homozygous Hb-C Disease, SC-Disease, and Various Combinations of Hemogiobin Variants. CTin. Chim. Acta 38:5-16, 1972. Huisman, T.H.J., Schroeder, w.A., Reese, A., WiISon, J.B., Lam, H., She1ton, J.R., She1ton, J.B. and Baker, 3.: The Ty Chain of Human Feta1 Hemog1obin at Birth and in SeveraT Abnorma1 HematoTogicaT Conditions. Pediatric Research II(10):1102-1105, 1977. Huisman, T.H.J., Ni1son, J.B., Grave1y, M. and Hubbard, M.: Hemog1obin Grady. The First Examp1e of a Variant with E1ongated a Chains Due to an Insertion of Residues. Proc. Nat1. Acad. Sci. 71:3270, 1974. Huisman, T.H.J., Wrightstone, R.N., Wi1son, J.B., Schroeder, W.A. and Kenda11, A.G.: Hemog1obin Kenya, the Product of Fusion of 6 and B Poiypeptide Chains. Arch. Biochem. Biophys. 1532850, 1972. Johnson, C., Powars, D. and Schroeder, w.A.: An Individua1 with Both Hemogiobin C and N—Ba1timore. Acta Haemato1ogica 56:183-188, 1976. Johnson, C.S., Moyes, D., Schroder, w.A., She1ton J.B. She1ton, J.R., and Beut1er, E.: HemogIobin Pasadena d28275 E19)Eeu.+ Arg. Identifica- tion by HPLC of a New Unstab1e Variant with Increased Oxygen Affinity. Biochim. Biophys. Acta (In press). Johnson, M.E. and Ho, C.: Effects of Ligands and Organic Phosphates on Functiona1 Properties of Human Adu1t Hemog1obin. Biochemistry 13:3653, 1974. Johnson, M.E.: Librationa1 Motion of an ”ImmobiTized" Spin Labe1: HemogTobin Spin Labe1ed by a Ma1eimide Derivative. Biochemistry 1721223- 1228, 1978. Johnson, M.E.: Spin Labe1 Techniques for Monitoring Macromo1ecu1ar Rotationa1 Motion: Empirica1 Ca1ibration Under Nonidea1 Conditions. Biochemistry 18:378-384, 1979. Johnson, M.E.: A Temperature Induced Variation in the Intrinsic Hyperfine Separation of a Tight1y Bound Nitroxide Spin Labe1. FEBS Letters 97:363- 366, 1979. Johnson, M.E. and Dany1uk, S.S.: Spin Labe1 Detection of Intermo1ecu1ar Interactions in Carbonmonoxy Sick1e Hemog1obin. Biophysica1 Journa1 24: 517-524, 1978. Kan, Y.w., Dozy, A., Stamatoyannopou1os, G., Hadjiminas, M.G., Zachariades, A., Furbetta, M. and Cao, A.: Mo1ecu1ar Basis of Hemog1obin-H Disease in the Mediterranean Popu1ation. B1ood (In press), 1979. 10 122. 123. 124. 125. 126. 127. 128. 129. 130. 131. 132. 133. 134. 135. Kenda11, A.G., Ten Pas, A., Wi1son, J.B., Cope, N., Bo1ch, K. and Huisman, T.H.J.: Hb-Vaasa or a282 (39(C5)G1n + G1u), a Mi1d1y Unstab1e Variant Found in a Finnish Fami1y. Hemog1obin 1(3):292-295, 1977. Kenda11, A.G., ijang, P.J., Schroeder, w.A. and Huisman, T.H.J.: Hemo- g1obin Kenya. A Product of a Gamma-Beta Fusion Gene. Studies of the Fami1y. Amer. J. Human Gen. 25:548, 1973. Ki11ion, P.J. and Cameron, B.F.: Water Sorption on Deoxygenated Hemog1obins CC, SC, SS, AS, and AA. Biopo1m. 13:1653-1659, 1974. Ki1martin, J.V., Breen, J.J., Roberts, G.C.K and Ho, C.: Direct Measurement of the pK Va1ues of an A1ka1ine Bohr Group in Human Hemoglobin. Proc. Nat1. Acad. Sci. U.S.A. 70:1246, 1973. K1eckner, H.B., Wi1son, J.B., Lindeman, J.G., Stevens, P.D., Niazi, G.A., Hunter, E., Jr., Chen, C.J. and Huisman, T.H.J.: Hemog1obin Fort Gordon, or 0£282 145 Tyr + Asp. A New High Oxygen Affinity Hemdg1obin Variant. Biochim. BiOphys. Acta 400:343, 1975. Kraus, L.M., Rasad, A., Ohba, Y. and Patterson, M.T.: Mouse Feta1 Hemo- b1ogin. Ann. N.Y. Acad. of Sci. 241:683-389, 1974. Kuis-Reerink, J.D., Jonix, J.H.P., Niazi, G.A., Ni1son, J.B., Bo1ch, K.C., Grave1y, M. and Huisman, T.H.J.: Hb-Vo1ga or a282 27 (89) A1a + Asp. An Unstab1e Hemog1obin Variant in Three Generations of a Dutch Fami1y. Biochim. Biophys. Acta 439263, 1976. Kumpati, J., Huisman, T.H.J. and Efremov, G.D.: Interaction Between Human Hemog1obin Variants and Hemog1obin S. Proceedings Of the Society of Experimenta1 Bio1ogy and Medicine 157:250-252, 1978. Lam, H., Ni1son, J.B., Harris, H.F., Grave1y, M.E. and Huisman, T.H.J. Hemog1obin A1amo (G282 19(B1) Asn + Asp). Hemog1obin 1(7):703-706, 1977. Lee, T.C.K.: A Rapid Method for Hemog1obin Chain Recombination. Ana1. Biochem. 91:646-650, 1978. Lee, C.K., Graves, D.G., Nerurkar, 5.6. and Kim, B.C.: Affinity Difference Among Hemog1obin non-a Chains Toward a Chains in the Assemb1y of Hemog1obins. Fed. Proc. 37:1390, 1978. Lee, D.K., 0de11, C.V., E1iot, F.P., Jones, E.w.: A Comparison of Hemog1o- bins from the Adu1t and Newborn Dog. Res. Vet. Sci. 152335-337, 1973. Levere, R.D. and Gidari, A.S.: Steroid Metabo1ites and the Contro1 of Hemog1obin Synthesis. Bu11, N.Y. Acad. Med. 50:563, 1974. Levere, R.D. and Mizoguchi, H.: Effects of Certain SB-H Steroid Metabo1ites on Hemog1obin Synthesis in Cu1tured Human Marrow Ce11s. Chapter 3 in Androgens in the Anemia of Bone Marrow Factors. T.F. Neche1es ed. Syntex Laboratories, Pa1o A1to, CA, 1973. 11 136. 137. 138. 139. 140. 141. 142. 143. 144. 145. 146. 147. Lindenbaum, J.: Hemog1obin Munchausen. JAMA 228:498, 1974. Lindstrom, T.R., B1adassare, J.J., Bunn, H.F., Ho, C.: Nuc1ear Magnetic Resonance and Spin Labe1 Studies of Hemog1obin Kempsey. Biochemistry 12:4214, 1973. Lindstrom, T.R. and Ho, C.: Effects of Anions and Ligands in the Tertiary Structure Around Ligand Binding Site in Human Adu1t Hemog1obin. Biochemistry 12:134, 1973. Lutcher, C.L., Wi1son, J.B., Grave1y, M.E., Stevens, P.D., Chen, C.J., Lindeman, J.G., Wong, S.C., Mi11er, A., Got1ieb, M. and Huisman, T.H.J.: Hb-Les1ie, an Unstab1e Hemog1obin Due to e1etion of G1utaminy1 Residue 8131 (H9) Occurring in Association with B —Tha1assemia, Hb-C and Hb-S. B1ood 47:99-112, 1976. Mant, M.J., Sa1kie, M.L., Cope, N., App1ing, F., Bo1ch, K., Jaya1akshmi, M., Grave1y, M., Wi1son, J.B. and Huisman, T.H.J.: Hb A1berta or G282 (101 (G3) G1u +~G1y), A New High Oxygen Affinity Hemoglobin Variant Causing Erythrocytosis. Hemog1boin 1:183-194, 1977. Matthay, K.K., Mentzer, w.c., Dozy, A.M., Kan, Y.w. and Bainton, D.F.: Modification of Hemog1obin-H Disease by Sick1e Trait. J. C1in. Invest. 64:1024-1032, 1979. Mi1ner, P.F., Gor1ey, C.C., Pomeroy, M.L., Wi1son, J.B., Grave1y, M. and Huisman, T.H.J.: Tha1assemia Intermedia Caused by Heterozygosity for Both B-Tha1assemia and Hemog1obin Saki (B14 (A11) Leu+-Pro). Amer. J. Hema. 1:283, 1976. Mi1ner, P.F. and Huisman, T.H.J.: Studies on the Proportion and Synthesis of Hemog1obin G Phi1ade1phia in Red Ce11s of Heterozygotes. A Homozygote and a Heterozygote for Both Hemog1obin G and a Tha1assemia. Brit. J. Haemat. 34:207, 1976. Moon, R.B. and Richards, J.H.: Nuc1ear Magnetic Resonance Studies of 13C0 Binding to Various Heme G1obins. Journa1 of the American C1inica1 Society 94:5093-5095, 1972. Moon, R.B. and Richards, J.H.: Conformationa1 Studies of Various Hemo- g1obins by Natura1-Abundance 13C NMR Spectroscopy. Proceedings of the Nat1. Acad. Sci. 69:2193-2197, 1973. Moon, R.B. and Richards, J.H.: 13C Magnetic Resonance Studies of the Binding of Carbon Monoxide to Various Hemog1obins. Biochemistry 13:3437, 1974. Moon, R.B., Ne1son, J.J., Powars, D. and Richards, J.: C Magnetic Resonance Studies of Carbamy1ated Sick1e Ce11 Hemog1obin. Physio1. Chem. and Phys. 6:31, 1974. 12 148. 149. 150. 151. 152. 153. 154. 155. 156. 157. 158. 159. Murari, J., Smith, L.L., Ni1son, J.B., Schneider, R.G. and Huisman, T.H.J.: Some Properties of Hemog1obin Gun Hi11. HemogIobin 1(3):267-282, 1977. Nage1, R.L., Bookchin, R.M., Johnson, J., Labie, D., Wajcman, H., Isaac- Sodeye, w.A., Honig, G.R., Schi1iro, G., Crookston, J.H. and Matsutomo, K.: Structura1 Bases of the Inhibitory Effects of Hemog1obin F and Hemoglobin A2 on the Po1ymerization of Hemoglobin S. Proc. Nat1. Acad. Sci., U.S.A. 76:670-672, 1979. Natta, C., Niazi, 6., Ford, S. and Bank, A.: Ba1anced G1obin Chain Synthesis in Hereditary Persistence of Feta1 Hemog1obin. J. C1in. Invest. 54:453, 1974. Niazi, G.A. and Huisman, T.H.J.: Erythrocytosis and Hemog1obin Variants. Proceedings Roya1 Dutch Academy of Science. 80(1):13-34, 1977. Niazi, G.A., Efremov, G.D., Niko1ov, N., Hunter, E., Jr. and Huisman, T.H.J.: Hemog1obin Strumica fig 112 (G19) His + Arg 82, (with an addendum: Hb-J-Paris-I, a2 12(A10) A1a + Asp 82 in the Same Popu1ation). Biochim. Biophys. Acta 412:181, 1975. Noren, I.B.E., Berto1i, D.A., Ho, C. and Casassa, E.F.: On the Tetramer- dimer Equi1ibrium of Carbon Monoxyhemog1obin in 2M Sodium Ch1oride. Biochemistry 13:2163, 1974. Oberiter, V., Bajanic, D., Efremov, 6.0. and Huisman, T.H.J.: Hb Beograd (asz 121 (GH4) G1u i Va1) Observed in a Third Yugos1avian Fami1y. Hemog1obin 2(5):453-455, 1978. Orringer, E.P., Fe1ice, A., Reese, A., Wi1son, J.B., Lam, H., Grave1y, M.E. and Huisman, T.H.J.: Hb Nottingham (G282 (F65) 98 Va1 + G1y) in a Caucasian Ma1e: C1inica1 and Biosynthesis Studies. Hemog1obin 2(4): 315-332, 1978. Orringfir, E.P., Wi1son, J.B. and Huisman, T.H.J.: Hemog1obin Chape1 Hi1 or me Asp + G1y 82. FEBS Letters 65:297-300, 1976. Pedersen, P.R., McCurdy, P.R., Wrightstone, R.N., Wi1son, J.B., Smith, L.L. and Huisman, T.H.J.: Hemog1obin Ko1n in a Negro: Pre- and Post- Sp1enectomy Red Ce11 Surviva1 (DF32p and 51Cr) and the Pathogenesis of Hemog1obin Instabi1ity. B1ood 42:771, 1973. Pennathur-Das, R., Vickery, L.E., Mentzer, w.c. and Lubin, B.H.: Modification of Hemog1obin-A with Dimethy1 Adipemidate - Contribution of Individua1 Reacted Subunits to Changes in Oxygen Affinity. Biochim. Biophys. Acta 580:356—365, 1979. Perutz, M.F., Heidner, E.J., Ladner, J.E., Bett1estone, J.G., Ho, C. and S1ade, E.F.: Inf1uence of G1obin Structure on the State of the Heme. III, Changes in the Heme Spectra Accompanying A11osteric Transitions in Methemog1obin and Their Imp1ications for Heme—Heme Interactions. Biochemistry 13:2187, 1974. 13 160. 161. 162. 163. 164. 165. 166. 167. 168. 169. 170. 171. 172. Perutz, M.D., Ladner, J.E., Simon, S.R. and Ho, C.: Influence of Globin Structure on the State of the Heme. I. Human Deoxyhemoglobin. Biochemistry 13:2163, 1974. Pinkerton, P.H., Wilson, J.B., Lam, H., Williams, D. and Huisman, T.H.J.: Hemoglobin Riyadh-BO-Thalassemia in an Indian Family. Hemoglobin (In press). Plese, C.F., Jr. and Amma, E.L.: Nature of the Negative Ellipticity of Human Fetal Hemoglobin in the 280 nm Region. Biochem. Biophys. Res. Commun. (In press). Plese, C.F. and Amma, E.L.: Circular Dichroism as a Probe of the Allosteric R 2 T Transformation in Hemoglobins and Modified Hemoglobins. Biochem. Biophys. Res. Commun. 76:691-97, l977. Plese, C.F., Amma, E.L. and Rodesi1er, P.F.: Conformational State and R 2 T Transformation in Mn(II) and Mn(III) Hemoglobins and Azide Mn(III) Hemo- globin. Biochem. Biophys. Res. Commun. 77:837—44, 1977. Powars, D., Schroeder, w.A., Shelton, J.R., Evans, L. and Vinetz, R.: An Individual with Hemoglobin S and Deer Lodge. Hemoglobin l:97-l02, 1976. Powars, D., Schroeder, w.A., Weiss, J. and Chan, L.S.: The Influence of Fetal Hemoglobin on the Risk of Complications of Sickle Cell Anemia. Sym- posium on the Molecular Basis of Mutant Hemoglobin Dysfunction. University of Chicago Press (In press). Powars, D., Schroeder, w.A., Weiss, J., Chan, L.S. and Azen, S.P.: The Lack of Influence of Fetal Hemoglobin Levels or Red Cell Indices on the Severity of Sickle Cell Anemia. J. Clin. Invest. (In press). Premachandra, B.R., Smith, A. and Kalra, V.K.: Cooperativity of HbAA and HbSS Binding to Inside-out Vesicles. Fed. Proc. (Abstract), 1979. Rachmilewitz, E., Huisman, T.H.J. and Schroeder, w.A.: Heterogeneity of Fetal Hemoglobin Among Israel Families with B-Thalassemia. International Symposium on Genetic Polymorphism and Disease in Human Populations. Israel J. Med. Sci. 9 l464-l468, l973. Rachmilewitz, E., Levi, S. and Huisman, T.H.J.: High Frequency of Haemo- globin C in a Large Bedouin Tribe. Israel J. Med. Sci. 10:219, 1974. Raybourne, S.R., Stallings, M.B., Gravely, M.E. and Huisman, T.H.J.: Oxygen Equibibrium Analyses of Isolated Hemoglobins A2, Lepore-Washington and P-Nilotic. Biochim. Biophys. Acta 535(1):78-84, 1978. Ringelhann, B., Acquaye, C.T.A., Oldham, J.H., Konotey-Ahulu, F.I.D., Yawson, G., Sukumaran, P.K., Schroeder, w.A. and Huisman, T.H.J.: Homozygotes for the Hereditary Persistence of Fetal Hemoglobin: The Ratio of GY and AY Chains and Biosynthetic Studies. Biochem. Gen. 15(ll/12): 1083-1096, l977. 14 173. 174. 175. 176. 177. 178. 179. 180. 181. 182. 183. 184. 185. Romain, P.L., Schwartz, A.D., Shamsuddin, M., Adams, J.G., Mason, R.G., Vida, L.N. and Honig, G.R.: Hemoglobin JChicago (876 (E20) A1a + Asp); A New HemogIobin variant Resu1ting from Substitution of an Externa1 Residue. B1ood 45:387, 1975. Rucknage1, D.L., Seid-Akhavan, M., Ayres, M., Sa12ano, F.M. and Winter, w.P.: Two More Examp1es of Hb Porto A1egre a28 Ser + Cys in Be1em, Brazi1. Human Heredity 23:175-181, 1973. Rucknage1, D.L., Reed, 1. and Winter, w.P.: HaemogIobin Inkster a285 Asp+Va1 82, Coexisting with B-Tha1assemia in a Caucasion Fami1y. Brit. J. Haemat. 26:475-484, 1974. Rucknage1, D.L. and Winter, w.P.: Peptide Mapping of Hemog1obin. Critica1 Rev. C1in. Lab. Sci. 5:81-100, 1974. Ruis-Reyes, G., Pina-Camara, A., Fe1ice, A.E., Grave1y, M.E. and Huisman, T.H.J.: 68-Tha1assemia in a Mexican Fami1y: C1inica1 Differences Among Homozygotes. Hemog1obin. Satter1ee, J., Teintze, M. and Richards, J.H.: Spectroscopic Studies of the Nature of Ligand Bounding in Carbon Monoxyhemog1obins: Evidence of a Specific Function for Histidine-E7 from Infrared and Nuc1ear Magnetic Resonance Intensities. Biochemistry 17:1456-146 Schmidt, N.C. Jr., Gir1ing, R.L., Houston, T.E., Sprou1, G.D., Amma, E.L. and Huisman, T.H.J.: Structure of Sick1ing Deer Type III Hemog1obin by Mo1ecu1ar Rep1acement. Acta Crysta11ogr. B33 335-343, 1977. Schmidt, w.c. Jr., Gir1ing, R.L. and Amma, E.L.: App1ication of a Restrained Least-Squares Refinement Procedure to Sick1ing Deer Hemog1obin. Acta Crysta11ogr. 833:3618-3620, 1977. Schroeder, w.A., Bannister, w.H., Grech, J.L., Brown, A.K., Wrightstone, R.N. and Huisman, T.H.J.: Nonsyncronized Suppression of Post-Nata1 Activity in Nona11e1ic Genes which Synthesize the Gy Chain in Human Foeta1 Haemog1obin. Nature 244:89-90, 1973. Schroeder, w.A., Fe1ice, A., Powars, D. and Ringe1hann, B.: An Anoma1y in the 5 Chain Heterogeneity of the Newborn. Nature 265:a63-65, January, 1977. Schroeder, w.A. and Huisman, T.H.J.: Hereditary Persistence of Foeta1 Haemog1obin. Letter to the Editor, Nature 257:70, 1975. Schroeder, w.A. and Huisman, T.H.J.: Mu1tip1e cistrons for Feta1 Hemog1obin in Man. Ann. N.Y. Acad. Sci. 241:70-79, 1974. Schroeder, w.A. and Huisman, T.H.J.: Human Gamma Chains: Structura1 Features. Seatt1e Meeting, June 19-20, 1978. Grune & Stratton, 1979. 15 186. 187. 188. 189. 190. 191. 192. 193. 194. 195. 196. 197. 198. Schroeder, M.A., Huisman, T.H.J., Efremov, G.D., Shelton, J.R., Shelton, J.B., Phillips, R., Reese, A., Gravely, M., Harrison, J.M. and Lam, H.: Further Studies of the Frequency and Significance of the 1v Chain of Human Fetal Hemoglobin. J. Clin. Invest. (In press). 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Chem. 247:1433-1439, 1972. Stathakis, N., Gidari, A.S. and Levere, R.S.: Erythropoietin Induced Stimu1ation of a, BS and Chain Synthesis in Cu1tured Human Marrow Ce11s. C1in. Res. 23:283a, 1975. Sukumaran, P.K., Huisman, T.H.J., Schroeder, W.A., McCurdy, P.R., Freehafer, J.T., Bouver, N., She1ton, J.R., She1ton, J.B. and Ape11, G.: A Homozygote for the HbGY Type of Foeta1 Haemog1obin in India: A Study of Two Indian and Four Negro Fami1ies. Brit. J. Haemato1. 23:403-417, 1972. Ve11a, F., Ga1braith, P., Wi1son, J.B., Wong, S.C., Fo1ger, G.C. and Huisman, T.H.J.: Hemog1obin St. C1aude or @2127 (H10) Lys + Thr 82. Biochim. Biophys. Acta 365:318, 1974. Ve11a, F., Wong, S.C., Wi1son, J.B. and Huisman, T.H.J.: Hemog1obins A2- Spakia and Ag-NYU in Canada. Canada J. Biochem. 50:841-843, 1972. Votano, J.R., Gorecki, M. and Rich, A.: Sick1e Hemog1obin Aggregation: A New C1ass of Inhibitors. Science 196:1216—1219, 1977. 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Wilson, J.B., Lam, H., Williams, D., Huisman, T.H.J., Espinosa-Turcott, J. and Ruiz-Reyes, G.: Hemoglobin G-San Jose (azgz 7 (H4) Glu + Gly) in a Mexican Family. Hemoglobin (In press). Wilson, S.M. and Makinen, M.W.: An Electron Microscope Study of the Fiber to Crystal Transition of Sickle Cell Hemoglobin. Proc. Natl. Acad. Sci. U.S.A. 77 (In press). Winter, W.P.: Is the Glutathione 8 Chain Mixed Disulfide a Natural Inter- mediate in Hemoglobin Synthesis? Gordon Research Conference Proceedings, P1ymouth, New Hampshire, June 10-15, 1979. Winter, W.P.: The GSH 85 Mixed Disulfide as a Natural Intermediate in Hemoglobin Assembly. Blood 52:121, 1978. Winter, W.P., Rucknagel, D.L., Baine, R. and Hanash, S.M.: Polymorphism in the Number of Hemoglobin a-Chain Loci and Mechanism Governing the Balance of Chain Synthesis. In Brewer, G.D., ed., Progress in Clinical and Biological Research Vol. 21, "The Red Cell“. 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Zucker, R.M., Wu, N.C. and Sikkema, D.A.: Isolation and Characterization of Multiple Hamster Hemoglobins. Federation Proceedings, 35:1423, 1976. 19 6. 7. 9. 10. 11. 12. DIAGNOSIS Abraham, E.C., Carver, J., Ddbler, J., Milner, P.F. and Huisman, T.H.J.: Microchromatographic Qauntitation of Fetal Hemoglobin in Patients with Sickle Cell Disease. Hemoglobin 3:341-351,1979. Abraham, E.C., Cope, N.C., Braziel, N.N. and Huisman, T.H.J.: 0n the Chromatographic Heterogeneity of Human Fetal Hemoglobin. Biochim. Biophys. Acta 547:159-169, 1979. Abraham, E.C., Huff, T.A., Cope, N.D., Wilson, J.B. Jr., Bransome, E.D. Jr., and Huisman, T.H.J. Detenmination of the Glycosylated Hemoglobins (Hb-Al) with a New Microcolumn Procedure. Diabetes 27:931-937, 1978. Abraham, E.C., Husiman, T.H.J., Schroeder, w.A., Pace, L.A. and Grussing, L.: Michrochromatography of Hemoglobins VII. Detection of Some Uncommon Hemoglobin Variants and Two Rapid Methods for the Quantitation of Hb—Ag in the Presence of Hb-C. J. Chromatogr. 143:59, 1976. Abraham, E.C., Reese, A., Stallings, M., Garver, F.A. and Huisman, T.H.J.: An Improved Chromatographic Procedure for Quantitation of Human Fetal Hemoglobin. Hemoglobin 1(16):547-560, 1977. Abraham, E.C., Reese, A. Stallings, M. and Huisman, T.H.J.: Separation of Human Hemoglobins by DEAE-Cellulose Chromatography using Glycerine-KCN—NaCl Developers. Hemoglobin 1:27, 1976. Abraham, E.C., Walker, 0., Gravely, M. and Huisman, T.H.J.: Minor Hemoglobins in Sickle Cell Anemia, B-Thalassemia, and Related Conditions: A Study of Red Cell Fractions Isolated by Density Gradient Centrifugation. Biochemical Medicine 13:56-77, 1975. Altay, G., Garver, F., Bannister, w.H., Grech, J.L., Felice, A. and Huisman, T.H.J.: Detection and Quantitation of the Fetal Hemoglobin Variant Hb F- Malta—I in Adults. Biochem. Genet. 15:915-923, 1977. Alter, B.P. and Nathan, D.G.: Antenatal Diagnosis of Haemotologic Disorders — '1978'. Clinics in Hematology. 1978. 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Proceedings Symp. Sickle Cell Anemia. INSERM 44:200, 1975. Efremov, 6.0. and Huisman, T.H.J.: Studies on the Heterogeneity of Hemoglobin XV. Separation of Fetal Hemoglobin and the Normally Occurring Minor Adult Hemoglobins by Chromatography on DEAE-Cellulose. J. Chromatog. 89:191-196, l974. Efremov, G.D., and Huisman T.H.J.: The Laboraroty Diagnosis of Hemoglo- binopathies, Clinics in Haematology 3:527, 1974. Efremov, G.D., Wrightstone, R.N., Braun, R.C., Bodie, A.R., Mayson, S.M. and Huisman, T.H.J.: The Use of Microchromatography in Mass-testing Programs for Hemoglobinopathies . Intern. Istabnul Symp. Abn. Hbs and Thal., Turkey. August, 1974. Erbe, R.N.: Screening for the Hemoglobinopathies. In Milunsky, A. ed. The Prevention of Mental Retardation & Genetic Dis. Saunders Co. p.204, 1975. Felice, A.E., Mayson, S.M., Webber, B.B., Miller, A., Gravely, M.E. and Huisman, T.H.J.: HbS, Hb G-Philadelphia and a-Thalessemia-Z in a Black Family. Pediatric Research (In press). 21 26. 27. 28. 29. 31. 32. 33. 34. 35. 36. 37. 38. Felice, A.E., Nebber, B., Miller, A., Mayson, S., Harris, H.F., Henson, J.B., Gravely, M.E. and Huisman, T.H.J.: The Association of Sickle Cell Anemia with Heterozygous and Homozygous a-Thalassemia-Z: In vitro Hb Chain Synthesis. Amer. J. Hematology 6:91-106, 1979. Garver, F.A., Applying RIA to Identify and Quantitate Normal and Abnormal Hemoglobins. Lab Management 16: 25—34, 1978. Garver, F.A., Altay,G., Baker, M.M., Gravely, M. and Huisman, T.H.J.: Radioimmunochemical Characterization of Hemoglobins Lepore and Kanya: Unique Antigenic Determinants Located on Hybrid Hemoglobins. J. Immunol. 121 1297-1301, 1978. Garver, F., Baker, M., Duvall,R., McDonough, P. and Mende, T.: Quantitation of Sickle Cell and Normal Hemoglobins in Aminiotic Fluid by Radioimmunoassay. Am. J. Obstet. Gyn. 132:697-698, 1978. Garver, F.A., Baker, M.M., Jones, S.C., Gravely, M., Altay, G. and Huisman, T.H.J.: Radioimmunoassay for Abnormal Hemoglobins. Science 196:1334-1337, 1977. Garver, F.A., Jones, S.C., Baker, M.M., Altay, G., Barton, B.P., Gravely, M. and Huisman, T.H.J.: Specific Radio-immunochemical Identification and Quantitation of Hemoglobins A2 and F. Amer. J. Haemat. 1:459-469, 1976. Garver, F.A.: Radioimmunoassay for Human Erythropoietin. Huisman, T.H.J. and Jonxis: The Hemoglobinopathies, Techniques of Identification. Eds., Dekker Publishing Co., New York, 181-185, 1977. Gbedemak, K.A., Acquaye, C.T.A., Konotey-Ahulu, F.I.D. and Reindorf, C.A.: Haemoglobin Phenotype Patterns in more than 1,000 Consecutive Newborn Babies in Ghana. Ghana Medical Journal 15(4):254, 1976. Giorgio, A.J. and Boggs, D.R.: Large Scale Screening for Hemoglobinopathies Utilizing Electrophoresis. Am. J. Public Health Briefs 64:993, 1974. 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AC5 Symposium Series, No. 36. Clinical Chemistry; Foreman and Mattoon, editors. American Chemical Society, Washington, DC 1976. Huisman, T.H.J.: Sickle Cell Anemia as a Syndrome: A Review of Diagnostic Features. Am. J. Hemat. 6:173-184, 1979. 23 53. 54. 55. 56. 57. 58. 59. 60. 61. 62. 63. 64. 65. 66. Huisman, T.H.J. and Efremov, 0.0.: Recent Advances in Methodology Used in the Detection of Hemoglobinopathies. Proc. Natl. Meeting on Sickle Cell Disease, 1974. Huisman, T.H.J. and Jonxis, J.H.P.: Immunological Identification of Hemoglobin Variant by Radioimmunoassay. In The Hemoglobinopathies, Techniques of Identification. Dekker Publishing Co., New York, 188-192, 1977. Huisman, T.H.J., Schroeder, w.A., Brodie, A.R., Mayson, S.M. and Jakway, J.: Microchromatography of Hemoglobins III. A Simple Procedure for the Determination of Hemoglobin A2. J. Lab. Clin. Med. 802700, 1975. Huisman, T.H.J. and Jonxis, J.H.P.: The Hemoglobinopathies, Techniques of Identification. 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Critical Rev. Clinic. Lab. Sci. 5:81-100, 1974. Rucknagel, D.L. and Chudwin, D.S.: Immunological Quantification of Hemoglobin F and A. Clinical Chimica Acta 50:413-418, 1974. Schleider, C.T.H., Mayson, S.M. and Huisman, T.H.J.: Further Modification of the Microchromatographic Determination of Hemoglobin A2. Hemoglobin 1(5): 503-504, 1977. Schmidt, R.M., Huisman, T.H.J. and Lehmann, H., editors: Standardization of Laboratory Reagents and Methods for the Detection of Hemoglobinopathies. Proceedings International Conference of Standardization of Diagnostic Materials 131-159, October, 1974. 24 67. 68. 69. 70. 71. 72. 73. 74. 75. 76. 77. 78. Schmidt, R.M., Rucknagel, D.L. and Necheles, T.F.: Comparison of Methodologies for Thalassemia Screening by Hb A2 Quantitation. J. Lab. Clin. Med. 86:873-882, 1975. 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Journal of Laboratory and Clinical Medicine. (In press). Pitcock, J.A., Hatch, F.E., Roy, S., Etteldorf, J.N. and Muirhead, E.E.: Renal Changes in Sickle Cell Disease, Supplement in Thrombosis et Diasthesis Haemorrhagica, 231-242, 1973. Also published as a Chapter in Sickle Cell Disease a monograph of Transactions of Twentieth Annual Symposium on Blood, Wayne State Univ., Detroit, MI, Edited by: K.L. Schattauer Verlag, NY, 1973. 77 Skeletal 1. Gelfand, M.J. and Harcke, H.T.: "Skeletal Imaging in Sickle Cell Disease: Jour. Nucl. Med. 19:698, 1978. Hruby, M.A., Honig, G.R., Lolekha, S. and Gotoff, S.P.: Arizona Hinshawii Osteomyelitis in Sickle Cell Anemia. Am. J. Dis. Child., 125:867-868, 1973. Ti, M., Moadel, E., Dosik, H. and Seligman, B.R.: Bone Changes in Sickle Cell Anemia and Its Genetic Variants: Proceedings of the First National Symposium on Sickle Cell Disease. DHEw Publication, 369, 1974. Walker, R.D., Schenck, K.L.J.: Infarct of the Mandible in Sickle Cell Anemia: Report of Case. J. Am. Dental Assoc. 78:661-664, September, 1973. 78 10. 11. 12. 13. 14. MANAGEMENT Desforges, J.F. and Warth, J.: The Management of Sickle Cell Disease in Pregnancy. Clin. in Perinatol, pp. 385-394, September, 1974. Diggs, L.w.: Home Management of Recurrent Painful Crises in Sickle Cell Disease. Supplement 53 in Thrombosis et. Diathesis Hemorrhagacia, 1973. Edmonds, Jr. and Randolph, S.: A Sickle Cell Disease Manual for Community Physicians. 1973. Gaston, M.H.: Management of Children Between Sickle Cell Crises. Urban Health 2:24, December, 1973. Halder, R.M. and Scott, R.B.: Causes of Sickle Cell Crises: Patients Point of View. Proceedings of International Conference on Sickle Cell Disease: A World Health Problem, Washington, D.C. (In press). Harkness, D.R.: Treatment of Sickle Cell Anemia. Current Therapy, pp. 266-270, 1976. Harkness, D.R.: Critique on Sickling Dynamics and Other Physiologic Studies in Anesthetized Patients with Sickle Cell Disease. Edited by: A. Maduska et al. Anesth. Anlg. 54:361-365, 1975. Jilly, P.N., Uy, C.G., Hudson, R.L., Scott, R.B. and Bullock, w.H.: Intravenous Urea in Control of Pain from Vaso-Occlusive Crises in Sickle Cell Anemia. Pediatric Research 8:128, 1974. Johnson, C.S.: Intravenous Iron Dextran in the Treatment of Iron Deficient Anemia. Journal of the National Medical Association 71:1101-1105, 1979. Johnson, C.S.: Sickle Cell Disease and Vocational Rehabilitation in Medical Aspects of Severe Disabilities for the Rehabilitation Counselor. Edited by: w.c. Stolov and M.R. Clovers, DHEw 1979 (In press). Kraus, A.P.: Sickle Cell Disease. Current Therapy, Philadelphia, Edited by: w.B. Saunders, Co., Howard 1972. Kraus, A.0., Cooper, M.R., Grush, D.C., Felts, J.H., Porter, F.S., Rhyne, A.L., Robinson, H. and Rosse, w.F.: Clinical Trials of Therapy for Sickle Cell Vase-Occlusive Crises. A Cooperative Study. JAMA 228:1120-1124, 1974. Lee, S., and Powars, D.: Sickle Cell Disease and the Emergency Nurse. Journal of Emergency Nursing 2:15-21, 1976. Levine, A.M., Overturf, G.D., Field, R. and Feinstein, L.A.: Use and Efficacy of Pneumococcal Vaccine in Patients with Hodgkin's Disease. Blood, October, 1979. 79 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. McCurdy, P.R., Binder, R.A., Bullock, W.H., Hudson, R.L., Jilly, R.N., Mahmood, L., Schmidt, T.H., Scott, R.B. and Westerman, M.P.: Treatment of Sickle Cell Crisis with Urea in Invert Sugar: A Controlled Trial, J.A.M.A. 22821125, 1974. Morrison, J.C., Fort, A.T., Wiser, W.L. and Fish, S.A.: The Modern Management of Pregnant Sickle Cell Patient: A Preliminary Report. South Med. J. 65:533, 1972. Murray, R.F., Jr.: Drug Treatment of Drug Addiction: (Can A Physician Differentiate Between a True Sickle Cell Crisis and Iatrogenic Narcotic Addiction?) Case Studies in Bioethics, The Hastings Center Report, Hastings-on-Hudson, 4:11-12, New York, 1974. Overturf, G.D., Field, R. and Edmonds, R.: Vaccines Against Pneumococcal Infections in Sickle Cell Disease. New Engl. J. Med. 300:143, 1979. Pegelow, C.H., Powars, D. and Overturf, G.D.: White Blood Counts in Sickle Cell Disease. Amer. J. Dis. Child. 133:448, 1979. Pegelow, C.H., Wilson, B. and Overturf, G.D., et al: Splenectomy in Sickle Cell Disease. Clinical Pediatrics 1979 (In press). Powars, D.R.: Sickle Cell Disease: Current Therapy, 263-271, 1978. Powars, D.R., Lightbourne, R.E. and Benson, 5.6.: A Comprehensive Approach to the Care of the Patient with Sickle Cell Anemia. Sickle Cell Anemia-- The Neglected Disease, Health Center Seminar Program, No. 5, University of California, Berkeley 1973. Prasad, A.S., Rabbani, P. and Warth, J.A.: Effect of Zinc Hyperammonemia in Sickle Cell Anemia Subjects, Am. J. Hematology (In press). Reindorf, C.A.: Management of Crises in Sickle Cell Disease: Proceedings of International Conference on Sickle Cell Disease: A World Health Problem, held on November 14—17, in Washington, D.C. 1976. Rucknagel, D.L.: The Therapy of Genetic Diseases. The Science Teacher, 40:20—23, 1973. Sarnaik, S., Slovis, T.L., Corbett, D.P., Emami, A. and Whitten, C.: Incidence of Cholelithiasis in Sickle Cell Anemia Using the Gray-Scale Technique. Journal of Pediatrics, 1979 (In press). Sarnaik, S., Soorya, D., Kim, J., Ravindranath, Y. and Lusher, J.: "Periodic Transfusions for Children with Sickle Cell Anemia Complicated by CNS Infarction. Am. J. Dis. Child. 1979 (In press). Satcher, D.: Emergency Room Protocol, Emergency Evaluation and Management of Persons with Sickle Cell Disease. Edited by: R.N. Pope and N.P. Leo, September, 1975. 80 29. 30. 31. 32. 33. 34. 35. 36. 37. Scott, R.B.: Clinical Manifestations of Sickle Cell Disease, in Proceedings of Conference on A Humanistic Approach to Sickle Cell Disease, Silver Spring, Maryland, Edited by: Waverly Press, Baltimore, MD, 1975 (In press). Scott, R.B.: Surgical Considerations in Patients with Sickle Cell Disease. Les Editions de INSERM, LA DREPANOCYTOSE/Sickle Cell Anemia INSERM, 44:75, 1975. Scott, R.R.: Treatment of the Crisis in Sickle Cell Anemia, Urban Health, Vol. 2, No. 6, p.22, December, 1973. Scott, R.B.: Treatment of Sickle Cell Disease: Current Pediatric Therapy, Edited by: w.B. Saunders, Co., Philadelphia, PA, 1973—1974. Scott, R.B., with McCurdy, P.R., Binder, R.A., Bullock, w.H., Hudson, R.L., Jilly, P.N., Nohmond, L., Schmidt, T.H. and Nesterman, M.P.: Treatment of Sickle Cell Crises with Urea in Invert Sugar: A Controlled Trial, J.A.M.A., 22:1125, May 26, 1974. Seligman, B.R., Rosner, F. and Dosik, H.: Splenic Calcifications in Sickle Cell Anemia. Amer. J. of Med. Sci. 265:495-499, 1973. Silberman, H., Overturf, 6.0. and Field, R.J., et al: Response of Renal Allograft Recipients to Pneumococcal Vaccination. Surgical Forum 1979. Sinnette, C.H. and Gilman, R.M.A.: Vocational Rehabilitation and Sickle Cell Anemia, Urban Health 3:38, February, 1974. Tigner-weekes, L., Pegelow, C., Lee, S. and Powars, 0.: Lead Screening in Sickle Cell Disease. J. Pediatrics 95:738-740, November, 1979. 81 SICKLE CELL TRAIT Adams, J.G., DeSimone, J., 0hrbsch, H., Blanksma, L., Westiclita, M. and Heller, P.: The Effect of Lead on the Proportion of HbS Individuals with Sickle Cell Trait. Blood 44:944, l974. Bowman, J.E. and Bernstein, S.C.: 0n the Physical and Intellectual Develop- ment of Black Children with and Without Sickle Cell Trait. Pediatrics 59(4):639-64l, l977. Castro, 0. and Finch, S.C.: Splenic Infarction in Sickle Cell Trait. Are Whites More Susceptible? New England Journal Medicine 29lza630-63l, l974. Diggs, L.W.: The Sickle Cell Trait Should not be Called a Disease. Clinical Medicine 8l:23-26, l974. Diggs, L.W. and Flowers, E.: High School Athletes with Sickle Cell Trait. J. National Medical Association 68:892—893, l976. Oster, J.R., Lee, S.M., Lespier, L.E., Pellegrini, E.L. and Vasmonde, C.A.: Renal Acidification in Sickle Cell Trait. Archives of International Medicine (In press) Pochedly, C., Ente, G., Nalbandian, R., Francis, Y.F. and Smith, J.A.: Is Sickle Cell Trait Harmless? Medical Counterpoint, p. 38, June, l973. Ramirez, A., Hartley, L, Redraza, H., Rhodes, W.D. and Abelmann, W.H.: Morphological Features of Red Blood Cells in Subjects with Sickle Cell Trait: Changes During Exercise. Arch. Intern. Med. l36(a):lD64, September, 1976. Satcher, D. and Brown, F.: The Implications of Sickle Cell Trait in the Hospital Population. Proceedings of the First National Symposium on Sickle Cell Disease, Washington, D.C., June, l974. 82 10. 11. 12. 13. 14. PSYCHOSOCIAL Bowman, J.E.: Ethica1, Lega1 and Humanistic Implications of Sick1e Ce11 Program. INSERM 44:353-378, 1975. Bowman, J.E.: Socia1 Imp1ications of Sick1e Ce11 Programs. Urban Hea1th 3:16, 1974. Bowman, J.E.: Socia1, Lega1 and Economic Issues in Sick1e Ce11 Programs. Chapter in Ethica1 and Socia1 Issues in Genetic Engineering and Counse1ing. Edited by J.J. Buck1ey, Jr. (In press). Bowman, J.E.: Genetic Screening Programs and Pub1ic Po1icy. Phy1on 38:117- 142, 1977. Bowman, J.E.: Socia1, Lega1 and Economic Issues in Sick1e Ce11 Programs. In Genetics Now: Ethica1 Issues in Genetic Research. Edited by J.J. Buck1ey, Jr., University Press of America, pp. 141—171, Washington, D.C., 1978. Burks, C.M., Banet, A.G. and Ant1ey, R.M.: Psycho1ogica1 Consequences of Having Sick1e Ce11 Anemia. American Journa1 of Human Genetics 25:12a, 1973. Chand1er, L., Dunca, D. and Tetrau1t, S.: Socio-Economic Aspects of Sick1e Ce11 Disease. Proceedings of Conference on A Humanistic Approach to Sick1e Ce11 Disease he1d in Si1ver Spring, Mary1and, October, 1975. Duncan, 0., Chand1er, L. and Tetrau1t, S.: Socio-Economic Aspects of Sick1e Ce11 Disease. Proceedings of Conference on Internationa1 Conference on Sick1e Ce11 Disease, November 14-17, 1976. Ha1der, R.M. and Scott, R.B.: Causes of Sick1e Ce11 Crises. Patients Point of View. Proceedings of Internationa1 Conference on Sick1e Ce11 Disease. A Wor1d Hea1th Prob1em, Washington, D.C., November 14-17, 1976. Hudson, R.L.: Sick1e Ce11 Anemia. A Perspective. B1ack Bag. Journa1 of the Student Nationa1 Medica1 Association 2(1):1-3, 1973. Kosacoff, M.I., Dosik, H. and Se1igman, B.R.: Psycho1ogica1 Study of Sick1e Ce11 Anemia and its Variants. Proceedings of the First Nationa1 Symposium on Sick1e Ce11 Disease, p. 365, 1974. Kumar, 5., Powars, D., A11en, J. and Haywood, L.J.: Anxiety, Se1f-Concept and Persona1 and Socia1 Adjustments in Chi1dren with Sick1e Ce11 Anemia. Journa1 of Pediatrics 88:859-863, May, 1976. Murray, R.F. Jr.: Mass Genetic Screening Programs and Human Va1ues: Another View. Medica1 Dimensions 22(2), February, 1973. Murray, R.F. Jr.: The S-Hemog1obinopathies. An Eva1uation of Their Status in the Armed Forces. Nationa1 Academy of Sciences. Nationa1 Research Counci1, 1973. 83 15. 16. 17. 19. 19. 20. 21. 22. 23. 24. 25. 26. 27. Murray, R.F. Jr.: Soliloquoy on Screening. Correspondence, New England J. Med. 291, 1974. Murray, R.F. Jr.: The Practitioner's View of the Values Involved in Genetic Screening and Counseling. Individual vs. Societal Imperatives in Ethical, Social and Legal Dimensions of Screening for Human Genetic Disease. Birth Defects. Orig. Art. Ser. National foundation March of Dimes, 10:185-200, 1974. Murray, R.F. Jr.: Ethical and Moral Aspects of Genetic Knowledge and Coun- seling. In To Create a Different Future. Edited by K. Vaux, New York, Friendship Press, pp. 67-83, 1972. Murray, R.F. Jr.: Psycho-social Aspects of Genetic Counseling. Social Work in Health Care 2:1, 1976. Murray, R.F. Jr.: Teaching the Ethics of Genetic Counseling in the Framework of a Medical Genetics Curriculum. The Teaching of Medical Ethics. Edited by R.M. Veatch, W. Gayling and Morgan, C.A. Hastings Center Publication, Hastings- on-Hudson, New York, 1973. Murray, R.F. Jr.: The Humanization of Medical Practice. Medical Dimensions 2:9, 1973. Murray, R.F. Jr.: The Ethical and Moral Values of Black Americans and Popu- lation Policy. In Population Policy and Ethics: The American Experience. Edited by R.M. Veatch, Irvington Publisher, Inc., New York, pp. 197-210, 1977. Nishiuria, E., Whitten, C.F. and Jenkins, 0.: Screening for Problems Needing Social Work Intervention. Journal of Health and Social Work (In press). Scott, R.B.: Testimony Before the Senate Health Subcommittee on Sickle Cell Disease. J. Natl. Med. Assoc. 67:498, 1975. Scott, R.B. and Tetrault, S.M.: Medical & Psychological Aspects of Sickle Cell'Disease in the Adolescent. 2nd International Symposium on Adolescent Medicine. Book of Abstracts, May, 1979. Sinette, C.H. and Smith, J.A.: Legislative and Socio-Economic Aspects of Sickle Cell Disease. Eds. Proceedings of Two Symposia, Harlem Hospital, October, 1973. Smith, J.A.: Response to Presentations on Family Planning and Genetic Counseling in the Proceedings of the First National and International Con— ference on Mental Health Impacts of Sickle Cell Anemia. Meharry Medical College, June, 1972. Smith, J.A.: Genetic Counseling and Being Black in America. Proceedings of Workshop held at the National Conference on Mental Health Aspects of Sickle Cell Anemia. Meharry Medical College, June, 1972. 84 28. 29. 30. 3l. 32. 33. 34. 35. Tetrault, S.M. and Scott, R.B.: Urban Hospitalization Cost Analysis of Patients with Sickle Cell Disease. Pediatric Research 8:l36, l974. Tetrault, S.M. and Scott, R.B.: A Child with Sickle Cell Anemia - His Recreation and Hobbies as Developmental Support. Clinical Pediatrics l3:496, June, l974. Tetrault, S.M. and Scott, R.B.: Five Year Retrospective Study on the Hospitalization and Treatment of Patients with Sickle Cell Anemia. Southern Medical Journal 69(l0):l3l4-l3l6, October, l976. Tetrault, S.M.: Patient Profile and Medical Cost of Sickle Cell Disease. Center for Sickle Cell Disease. Proceedings of a Conference on Humanistic Approach to Sickle Cell Disease, l975. Tetrault, S.M.: The School-aged Child with Sickle Cell Disease. Psycho- logic Aspects and Preventive Intervention. Center for Sickle Cell Disease. Proceedings of a Conference on Humanistic Approach to Sickle Cell Disease, l975. Vavasseur, J.: A Comprehensive Program for Meeting Psychosocial Needs of Sickle Cell Anemia Patients. Journal of National Medical Association 69(5):335, l977. Vavasseur, J.: Sickle Cell Disease - A Psychological Perspective. The Journal of Family Practice (In press). Whitten, C.F. and Fischhoff, J.: Psychosocial Effects of Sickle Cell Disease. Archives of Int. Med. l33(4):68l-689, l974. 85 10. 11. 12. 13. GENERAL Brewer, G.J.: Detours on the Road to Successful Treatment of Sickle Cell Anemia. Perspectives in Biology and Medicine, Vol. 22, No. 2, Part 1, 1979, by the University of Chicago. Bullock, w.H. and Jilly, P.N.: Hematology; Textbook of Black Related Diseases. Edited by R. Williams, McGraw Hill, New York. pp. 199—316, 1975. Desforges, J.F., Milner, P., Nethers, D. and Whitten, C.F.: SCA: Tell the Facts, Quell the Fables. Patient Care, Vol. 12, No. 11, pp. 164-216, June, 1978. Duncan, 0., Scott, R.B. and Castro, 0.: Use of a Mobile Health Unit as an Adjunct to a Community Outreach Program. Proceedings of National Sickle Cell Conference, San Juan, Puerto Rico, 1978. Gaston, M.H.: Screening For Sickle Cell Disease, Ohio’s Health, June, 1976. Gaston, M.H.: Commentary - Screening for Sickle Cell Disease. Southern Medical Journal 672257, March, 1974. Gaynon, P. and Honig, G.R.: A Computer-Based System for Management of Clinical and Laboratory Data of a Comprehensive Sickle Cell Center. Meth. Inform. Med. 13:216-221, 1974. Hicks, E.J.: Screening for Sickle Cell Anemia in Indianapolis, IN. Urban Health 1:16, 1972. Hicks, E.J., Ragatz, B.H. and Harper, E.T.: Medical Students and Health Care in the Black Community. A Project Laboratory on Sickle Cell Disease. Urban Health 3, 1:36-37, 1974. Jones, R.L., Smith, C.H. and Cox, E.: Integrating the Teaching of Sickle Cell Anemia into the Curriculum of the Cincinnati Public School System. J. Nat. Med. Assoc. (In press). Lutcher, C.L., Dorsey, M.M., Mayson, S., Huisman, T.H.J., Indvigsen, E. and Smith, A.T.: The Role of a Sickle Cell Center in Comprehensive Screening and Counseling for Sickle Cell and Related Disorders. Southern Medical Journal 67:259-264, 1974. Satcher, D.: Present Status of Sickle Cell Screening. Proceedings of International Symposium on Sickle Cell Disease, Washington, D.C., 1976. Scott, R.B.: Introductory Remarks at Opening of Scientific Session of Conference on Hemoglobins: Comparative Molecular Biology. Models for the Study of Disease, Silver Spring, MD, 1973. Also published by N.Y. Acad. Sci. 2412714, 1974. 86 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. Scott, R.B.: Some Observations on the Status of Sickle Cell Disease in 1973. Pediatric Sectional Newsletter or Nat. Med. Assoc. 2:3, 1973. Scott, R.B.: Introduction (Preface) to Perspectives on Sickle Cell Anemia, Urban Health, Urban Publishing Co., 1976. Scott, R.B.: Reflections on Contributions of Howard University Toward Control of Sickle Cell Anemia. Ivy Leaf Magazine of Alpha Kappa Alpha Sorority 52:24, 1976. Scott, R.B.: Reflections on the Current Status of the National Sickle Cell Disease Program in the United States. J. Natl. Med. Assoc. 71:679-681, l979. Scott, R.B.: Editorial: Hard Times, Children and Politics. J. Natl. Med. Assoc. 68:149, 1976. Scott, R.B.: Editorial: International Year of the Child. J. Natl. Med. Assoc. 71:221, 1979. Scott, R.B.: How a Comprehensive Center for Sickle Cell Disease Can Contribute to Continuing Medical Education. J. Natl. Med. Assoc. 69:67, 1977. Scott, R.B. and Castro, 0.: Screening for Sickle Cell Hemoglobinopathies. Journal of the American Medical Assocaition 241:1145, 1979. Scott, R.B. and Winston, M.R.: The Health & Welfare of the Black Family in the United States: A Historical and Institutional Analysis. Amer. J. Dis. Child 130:703, 1976. Whitten, C.F.: The Need For and an Approach to Parent Education. Proceed- ings of the First National Sickle Cell Educational Symposium, May, l976. Whitten, C.F.: Sickle-Cell Programming - An Imperiled Promise. (An Editorial) New Eng. J. Med. 283(6):318-319, 1973. Whitten, C.F.: A Primer on Sickle Cell Conditions in Children For Physicians. Pediatric Basis 22:4, 1978. 87 0 U S. GOVERNMENT PRINTING OFFICE : 1980 720-020/4605 GENERAL UBHABY-ILC.BEHKELEY BUDDSLHLSE