References for Sickle Cell Disease Review


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10.  Ingram VM. Gene mutations in human haemoglobin: The chemical difference between normal and sickle cell haemoglobin. Nature 180:326,1957.

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24.  Motulsky AG. Frequency of sickling disorders in U.S. blacks. New Eng. J Med. 288:31, 1973.

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32.  Sullivan LW. The risks of sickle cell trait: caution and common sense. New Eng. J Med 317:830, 1987.

33.  Charache S. Fetal Hemoglobin, Sickling, and Sickle Cell Disease. Advances in Pediatrics 37:1,1990.

34.  Adekile AD and Huisman THJ. Hb F in sickle cell anemia. Experientia 49:16,1993.

35.  Steinberg M. The interactions of a -thalassemia with hemoglobinopathies [Review]. Hematology/Oncology Clinics of North America 5:453, 1991.                                                

36.  Steinberg MH. Dreiling BJ. Clinical, hematologic and biosynthetic studies in sickle cell-beta 0-thalassemia: a comparison with sickle cell anemia. Am. J  Hematol 1:35-44, 1976.

37.  Serjeant GR, Serjeant BE. Comparison of sickle cell beta o-thalassemia and sickle cell beta +-thalassemia in black populations. Birth Defects: Original Article Series. 18:223, 1982.

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40.  Powars DR. Natural History of Sickle Cell Disease: The First Ten Years. Sem. in Hematol. 12:267,1975.

41.  Barrett DH, Wisotzek IE, Abel GG et al. Assessment of psychosocial functioning of patients with sickle cell disease. Southern Med. J. 81:745, 1988.

42.  Thomas AN, Pattison C and Serjeant GR. Causes of death in sickle cell disease in Jamaica. Brit. Med. J 285:633,1982.

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45.  National Institute of Health Consensus Development Conference Statement. Newborn screening for sickle cell disease and other hemoglobinopathies. J Am. Med. Assoc. 258: 1205, 1987.

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47.  Perutz MF. Hemoglobin Structure and Respiratory Transport. Scientific American  :92, 1978.

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51.  Bunn HF  and McDonald MJ. Electrostatic interactions in the assembly of haemoglobin. Nature 306:498,1983.

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53.  Perutz MF and Mitchison JM. State of haemoglobin in sickle cell anemia. Nature 166:677,1950.

54.  Noguchi CT and Schecter AN. The intracellular polymerization of sickle hemoglobin and its relevance to sickle cell disease[Review]. Blood 58:1057,1981.

55.  Bunn F and Forget B. Sickle Cell Disease- Molecular and Cellular Pathogenesis. in Hemoglobin: Molecular, Genetic and Clinical Aspects. (Philadelphia :W.B.Saunders Co., 1986),p.453-501.

56.  SanBagio PL, Hofrichter J, Mozzarelli A, Henry ER and Eaton WA. Current perspectives on the kinetics of hemoglobin S gelation. Ann. New York  Acad. Sci.565 :53, 1989.

57.  Finch JT, Perutz MF, Bertles JF and Dobler J. Structure of sickled erythrocytes and of sickle-cell hemoglobin fibers. Proc. Natl. Acad. Sci. 70:718,1973.

58.  Wishner BC, Ward KB, Lattman EE and Love WE. Crystal structure of sickle-cell deoxyhemoglobin at 5 Å resolution. J Mol. Biol. 98:179,1975.

59.  Padlan EA and Love WE. Refined crystal structure of deoxyhemoglobin S:I. Refined least-squares refinement at 30 Å resolution. J Biol. Chem. 260:8272,1985. 

60.  Padlan EA and Love WE. Refined crystal structure of deoxyhemoglobin S:II. Molecular interactions in the crystal. J Biol. Chem. 260:8280,1985.

61.  Edelstein SJ. Molecular topology in crystals and fibers of hemoglobin S. J Mol. Biol. 150:557,1981.

62.  Acquaye C, et al. Electron microscopic studies of the intracellular polymerization of sickle hemoglobin. Blood Cells 13 :1359,1988.

63.  Kaul DK, Fabry ME and Nagel RL. The pathophysiology of vascular obstruction in the sickle syndromes [Review]. Blood Reviews 10:29,1996. 

64.  Asakura T, Mattiello JA, Obata K, Asakura K, Reilly MP, Tomassini N, Schwartz E and Ohene-Frempong K. Patially oxygenated sickled cells: Sickle-shaped red cells found in circulating blood of patients with sickle cell disease. Proc. Natl. Acad. Sci 91: 12589, 1994.

65.  Bertles JF and Milner PF. Irreversibly sickled erythrocytes: A consequence of the heterogeneous distribution of hemoglobin types in sickle-cell anaemia. J Clin. Invest. 47:1731,1968.

66.  Serjeant GR, Serjeant BE and Milner PF. The Irreversibly sickled cell; a determinant of haemolysis in sickle-cell anaemia. Brit. J  Haematol. 17:527,1969.

67.  Hofrichter J, Ross PD and Eaton WA. Kinetics and mechanism of deoxyhemoglobin gelation-a new approach to understanding sickle cell disease. Proc. Natl. Acad. Sci. 71:14864, 1974.

68.  Eaton WA, Hofrichter J, and Ross P.D. Delay time of gelation: A possible determinant of clinical severity in sickle cell disease[Editorial]. Blood 47; 621, 1976.

69.  Eaton WA and Hofrichter J. Hemoglobin S Gelation and Sickle Cell Disease [Review]. Blood 70:1245, 1987.

70.  Mozzarelli A, Hofrichter J and Eaton WA. Delay time of hemoglobin polymerization prevents most cells from sickling in vivo. Science 237:500, 1987.

71.  Noguchi CT, Torchia DA and Schecter AN. Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenation. Proc. Natl. Acad. Sci. 77: 5487, 1980.

72.  Brittenham GM, Schecter AN and Noguchi CT. Hemoglobin S polymerization: Primary determinant of the hemolytic and clinical severity of the sickling syndromes. Blood 65:183, 1985.

73.  Noguchi CT, Rodgers GP and Schecter AN. Intracellular polymerization: Disease severity and therapeutic predictions. Ann. New York  Acad. Sci.565 :75,1989.

74.  Hiruma H, Noguchi CT, Uyesaka N, Hasegawa S, Blanchette-Mackie EJ, Schecter A and Rodgers GP. Sickle cell rheology is determined by polymer fraction-not cell morphology. Am. J  Hematol 48:19, 1995.

75.  Hebbel RP.Beyond Hemoglobin Polymerization: The red cell membrane and sickle cell disease pathophysiology [Review]. Blood 77:214,1991.

76.  Lux SE, John KM and Karnovsky MJ. Irreversible deformation of the spectrin-actin lattice in irreversibly sickled cells. J Clin. Invest. 58: 955, 1976.

77.  Jensen WN, Bromberg PA and Barefield K. Membrane deformation: A cause of irreversibly sickled cells (ISC). Clin. Res. 17:464, 1969.

78.  McCurdy PR, Sherman AD. Irreversibly sickled cells and red cell survival in sickle cell anemia. Am J Med 64:253,1978.

79.  Zago MA and Bottura C. Splenic function in sickle cell disease. Clinical Science 65: 297,1983.

80.  Connor J, Pak CC and Schroit AJ. Exposure of phosphatidylserine in the outer leaflet of human red blood cells. Relationship to cell density, cell age, and clearance by mononuclear cells. J  Biol. Chem. 269:2399, 1994.

81.  Grasso JA, Sullivan AL and Sullivan LW. Ultrastructural studies of the bone marrow in sickle cell anaemia. Brit. J Haematol. 31:135, 1975.

82.  Ashby W. The determination of the length of life of transfused blood corpuscles in man. J Exp. Med. 29: 267, 1919.

83.  Ballas SK and Mohandas N. Pathophysiology of vaso-occlusion [Review]. Hematology /Oncology Clinics of North America 10:1221,1996.

84.  Schwartz RS, Musto S, Fabry ME and Nagel RL. Two distinct pathways mediate the formation of intermediate density cells and hyperdense cells from normal density sickle red blood cells. Blood 92:4844, 1998.

85.  Ballas SK, Larnar J, Smith ED, Surrey S, Schwartz E and Rappaport EF. The xyerocytosis of Hb SC disease. Blood 69:124,1987.

86.  Sutera SP, et al. Rheoscopic investigation of red cell deformability in sickle cell anemia. ISA Transactions 25: 1, 1986.

87.  Clark MR,  Mohandas N and Shohet SB. Deformability of oxygenated irreversibly sickled cells. J Clin. Invest. 65:189, 1980.

88.  Kuypers FA. Red cell membrane damage. J Heart Valve Disease 7:387, 1998.

89.  Nash GB, Johnson CS and Meiselman H. Mechanical properties of oxygenated red blood cells in sickle cell (Hb SS) disease. Blood 63:73,1984.

90.  Hoover R, Rubin R, Wise G and Warren R. Adhesion of normal and sickle erythrocytes to endothelial monolayer cultures. Blood 54: 872, 1979.

91.  Hebbel RP et al. Abnormal Adherence of Sickle Erythrocytes to Cultured Vascular Endothelium. J Clin. Invest. 65: 155, 1980.

92.  Hebbel RP et al. Erythrocyte Adherence To Endothelium In Sickle-Cell Anemia. New Eng. J Med. 302: 993, 1980.

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94.  Ballas SK et al. Rheological Predictors of the Severity of the Painful Sickle cell Crisis. Blood 72: 1217, 1988.

95.  Lande WM et al. The Incidence of Painful crisis in Homozygous Sickle Cell Disease: Correlation With Red Cell Deformability. Blood 72: 2056, 1988.

96.  Powars D, Chan LS and Schroeder WA. The variable expression of sickle cell disease is genetically determined. Semin. in Hematol. 27:361,1990.

97.  Nagel RL. Sickle cell anemia is a multigene disease: Sickle painful crises, a case in point. [Editorial]. Am. J Hematol. 42:96,1993.

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102.Dover GJ, Smith KD, Chang YC, Purvis S, Mays A, Meyers DA, Sheils C and Serjeant GR. Fetal hemoglobin levels in sickle cell disease and normal individuals are partially controlled by an X-linked gene located at Xp22.2. Blood 80:816,1992.

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127.Walters MC, Patience M, Leisenring W, Eckman JR, Scott JP, Mentzer WC, Davies SC, Ohene-Frempong K, Bernaudin F, Matthews DC, Storb R, Sullivan KM. Bone marrow transplantation for sickle cell disease. New Eng. J  Med. 335:369, 1996.

128.Vermylen C and Cornu G. Bone marrow transplantation for sickle cell anemia [Review]. Current Opinion in Hematology 3:163, 1996.

129.Bowman JE. Is a national program to prevent sickle cell disease possible? Am. J Ped. Hem. Onc. 5:367, 1983.