Sickle cell disease and anesthesia

Anesthesiology

Volumn 101, Issue 3, 2004, Pages 766-785

  Firth, Paul G ^a   Head, C Alvin ^b  

^a TUFTS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b MEDICAL COLLEGE OF GEORGIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BETA GLOBIN; ENDOTHELIAL LEUKOCYTE ADHESION MOLECULE 1; HEME; HEMOGLOBIN A; HEMOGLOBIN C; HEMOGLOBIN F; HEMOGLOBIN S; HYDROGEN PEROXIDE; INTERCELLULAR ADHESION MOLECULE 1; INTERLEUKIN 1; INTERLEUKIN 6; NITRIC OXIDE; NITRIC OXIDE SYNTHASE; PADGEM PROTEIN; SUPEROXIDE; TUMOR NECROSIS FACTOR; VASCULAR CELL ADHESION MOLECULE 1; XANTHINE OXIDASE;

ACUTE CHEST SYNDROME; ALPHA THALASSEMIA; ANESTHESIA; BLOOD VESSEL INJURY; BLOOD VESSEL OCCLUSION; CHRONIC LUNG DISEASE; CLINICAL FEATURE; DEHYDRATION; ERYTHROCYTE MEMBRANE; ERYTHROCYTE TRANSFUSION; GENOTYPE; HUMAN; KIDNEY DISEASE; PAIN; PATHOPHYSIOLOGY; PERIOPERATIVE PERIOD; POINT MUTATION; PREOPERATIVE EVALUATION; PRIORITY JOURNAL; REVIEW; SICKLE CELL ANEMIA; STROKE;

EID: 4344670923     PISSN: 00033022     EISSN: None     Source Type: Journal    
DOI: 10.1097/00000542-200409000-00027     Document Type: Review

References (180)

1. Shapiro ND, Poe MF: Sickle-cell disease: An anesthesiological problem. ANESTHESIOLOGY 1955; 16:771-80
2. Dresbach M: Elliptical human red corpuscles. Science 1904; 19:469-70
3. Edmond MM, Sergent E: On specific bodies in the blood of malaria patients, [in French]. C R Seances Soc Biol Fil 1905; 58:51-4
4. Herrick JB: Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. Trans Assoc Am Physicians 1910; 25:553-6l
5. Mason VR: Sickle cell anemia. JAMA 1927; 79:1318-20
6. Hahn EV, Gillespie EB: Sickle cell anemia. Arch Intern Med 1927; 39:233-54
7. Ham TH, Castle WB: Relationship of increased hypotonic fragility and of erythrostasis to the mechanism of hemolysis in certain anemias. Trans Assoc Am Physicians 1940; 55:127-32
8. Nagel RL, Fabry ME, Pagnier J, Zohoun I, Wajcman H, Baudin V, Labie D: Hematologically and genetically distinct forms of sickle cell anemia in Africa: The Senegal type and the Benin type. N Engl J Med 1985; 312:880-4
9. Lapoumeroulie C, Dunda O, Ducrocq R, Trabechet G, Mony-Lobe M, Bojo JM, Carnevale P, Labie D, Elion J, Krishnamoorthy R: A novel sickle cell mutation of yet another origin in Africa: The Cameroon type. Hum Genet 1992; 89:333-7
10. Miller BA, Olivieri N, Salameh M, Ahmed M, Antognetti G, Huisman TH, Nathan DG, Orkin SH: Molecular analysis of the high-hemoglobin-F phenotype in Saudi Arabian sickle cell anemia. N Engl J Med 1987; 316:244-50
11. Antonarakis SE, Boehm CD, Serjeant GR, Theisen CE, Dover GJ, Kazazian HH, Jr.: Origin of the beta S-globin gene in blacks: The contribution of recurrent mutation or gene conversion or both. Proc Natl Acad Sci U S A 1984; 81:853-6
12. Schiliro G, Russo Mancuso G, Kulozik AE, Serjeant BE, Serjeant GR: Comparison of clinical, hematological and molecular findings of Jamaican and Sicilian patients with sickle cell disease. Haematologica 1990; 75 Suppl 5:42-56
13. Padmos MA, Roberts GT, Sackey K, Kulozik A, Bail S, Morris JS, Serjeant BE, Serjeant GR: Two different forms of homozygous sickle cell disease occur in Saudi Arabia. Br J Haematol 1991; 79:93-8
14. Uddin DE, Dickson LG, Brodine CE: Screening of military recruits for hemoglobin variants. JAMA 1974; 227:1405-7
15. McGrew CJ Jr: Sickle cell trait in the white population. JAMA 1973; 224:1762-3
16. McGrew CJ Jr: Sickle cell trait in the nonblack population. JAMA 1975; 232:1329-30
17. Allison AC: Protection afforded by sickle-cell trait against subtertian malarial infection. BMJ 1954; 1:290-4
18. Bunn HF, Forget B: Hemoglobin structure, Hemoglobin: Molecular, genetic and clinical aspects, 1st edition. Edited by Dyson J. Philadelphia, WB Saunders, 1986, pp13-36
19. Marotta CA, Forget BG, Cohne-Solal M, Wilson JT, Weissman SM: Human beta-globin messenger RNA. I. Nucleotide sequences derived from complementary RNA. J Biol Chem 1977; 252:1431-40
20. Neel JV: The inheritance of sickle cell anemia. Science 1949; 110:64-6
21. Platt OS, Brambilla DJ, Rosse WT, Mitner PF, Castro O, Steinberg MH, Klug PP: Mortality in sickle cell disease: Life expectancy and risk factors for early-death. N Engl J Med 1994; 330:1639-44
22. Olson JF, Ware RE, Schultz WH, Kinney TR: Hemoglobin C disease in infancy and childhood. J Pediatr 1994; 125:745-7
23. Baum KF, Dunn DT, Maude GH, Serjeant GR: The painful crisis of homozygous sickle cell disease: A study of the risk factors. Arch Intern Med 1987; 147:1231-4
24. Platt OS, Thorington BD, Brambilla DJ, Milner PF, Rosse WF, Vichinsky E, Kinney TR: Pain in sickle cell disease: Rates and risk factors. N Engl J Med 1991; 325:11-6
25. Powars D, Chan LS, Schroeder WA: The variable expression of sickle cell disease is genetically determined. Semin Hematol 1990; 27:360-76
26. Wood WG, Pembrey ME, Serjeant GR, Perrine RP, Weatherall DJ: HbF synthesis in sickle cell anemia: A comparison of Saudi Arab cases with those of African origin. Br J Haemotol 1980; 45:431-45
27. Embury SH, Dozy AM, Miller J, Davis JR Jr., Kleman KM, Preisler H, Vichinsky E, Lande WN, Lubin BH, Kan YW, Mentzer WC: Concurrent sickle-cell and α-thalassemia: Effect on the severity of anemia. N Eng J Med 1982; 306:270-4
28. Hebbel RP: Beyond hemoglobin polymerization: The red blood cell membrane and sickle disease pathophysiology. Blood 1991; 77:214-37
29. Reiter CD, Wang X, Tanus-Santos JE, Hogg N, Canno RO 3rd, Schechter AN, Gladwin MT: Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease. Nat Med 2002; 8:1383-9
30. Ingram VM: A specific chemical difference between the globins of normal human and sickle-cell hemoglobin. Nature 1956; 178:792-4
31. Shelton JR, Schroeder WA: Further N-terminal sequences in human hemoglobin A, S and F by Edman's phenylthiohydantoin method. J Am Chem Soc 1960; 82:3342-5
32. Pauling L, Itano HA, Singer SJ, Wells IC: Sickle cell anemia. Science 1949; 110:543-8
33. Asakura T, Ohnishi T, Friedman S, Schwanz E. Abnormal precipitation of oxyhemoglobin S by mechanical shaking: Proc Natl Acad Sci USA 1974; 71:1594-8
34. Roth EF, Elbaum D, Bookchin RM, Nagel RL: The conformational requirements for the mechanical precipitation of hemoglobin S and other mutants. Blood 1976; 48:265-71
35. Eaton WA, Hofrichter J: Hemoglobin S gelation and sickle cell disease. Blood 1987; 70:1245-66
36. Hebbel RP: The sickle erythrocyte in double jeopardy; autoxidation and iron decompartmentalization. Sem Hematol 1990; 27:51-69
37. Repka T, Hebbel RP: Hydroxyl radical formation by sickle erythrocyte membranes: Role of pathological iron deposits and cytoplasmic reducing agents. Blood 1991; 78:2753-8
38. Hebbel RP, Eaton JW: Pathobiology of heme interaction with the erythrocyte membrane. Sem Hematol 1989; 26:136-49
39. Hebbel RP, Morgan WT, Eaton JW, Hedlund BE: Accelerated autoxidation and heme loss due to instability of sickle hemoglobin. Proc Natl Acad Sci U S A 1988; 85:273-41
40. Asian M, Thornley-Brown D, Freeman B: Reactive species in sickle cell disease. Ann NY Acad Sci 2000; 889:375-91
41. Hebbel RP: Adhesive interactions of sickle erythrocytes with endothelium. J Clin Invest 1997; 99:2561-4
42. Hebbel RP, Vercelloti GM: The endothelial biology of sickle cell disease. J Lab Clin Med 1997; 129:288-93
43. Platt OS: Sickle cell anemia as an inflammatory disease. J Clin Invest 2000; 106:337-8
44. Gross PL, Aird WC: The endothelium and thrombosis. Sem Thromb Hemost 2000; 26:463-78
45. Verrier ED, Boyle E: Endothelial cell injury in cardiovascular surgery. Ann Thorac Surg 1996; 62:915-22
46. Steudel WS, Hurford WE, Zapol WM: Inhaled nitric oxide: Basic biology and clinical implications. ANESTHESIOLOGY 1999; 91:1090-121
47. Lia JC: Blood feud: keeping hemoglobin from nixing NO. Nat Med 2002; 8:1350-1
48. Lancaster JR: Reaping of nitric oxide by sickle cell disease. Proc Natl Acad Sci U S A 2002; 99:552-3
49. Asian M, Ryan TM, Adler B, Townes TM, Parks DA, Thompson JA, Tousson A. Gladwin MT, Patel RP, Tarpey MM, Batinic-Haberle I, White CR, Freeman BA: Oxygen radical inhibition of nitric oxide-dependent vascular function in sickle cell disease. Proc Natl Acad Sci U S A 2001; 98:15215-20
50. Gladwin MT, Schechter AN, Shelhamer KH, Ognibene FP: The acute chest syndrome in sickle cell disease: Possible role of nitric oxide in its pathophysiology and treatment. Am J Respir Crit Care Med 1999; 159:1368-76
51. Stuart MJ, Setty BNY: Acute chest syndrome of sickle cell disease: New light on an old problem. Curr Opin Hematol 2001; 8:111-22
52. Kual DK, Hebbel RP: Hypoxia/reoxygenation causes inflammatory response in transgenic sickle cell mice but not in normal mice. J Clin Invest 2000; 106:411-20
53. Keaney JF, Vita JA: The value of inflammation for predicting unstable angina. N Eng J Med 2002; 347:55-7
54. Buffon A, Biasucci LM, Liuzzo G, D'Onofrio G, Crea F, Maseri A: Widespread coronary inflammation in unstable angina. N Eng J Med 2002; 347:5-12
55. Henderson AB, Thornell, HE: Observations on the effect of lowered oxygen tension on sicklemia and sickle cell anemia among military flying personnel. J Lab Clin Med 1946; 31:769-76
56. Sproule BJ, Halden RE, Miller WF: A study of the cardiopulmonary alterations in patients with sickle-cell disease and its variants. J Clin Invest 1958; 37:486-95
57. Stein RE, Urbaniak J: Use of the tourniquet during surgery in patients with sickle cell hemoglobinopathies. Clin Orthop 1980; 151:231-3
58. Adu-Gyamfi Y, Sankarankutty M, Marwa S: Use of a tourniquet in patients with sickle-cell disease. Can J Anaesth 1993; 40:24-7
59. Oginni LM, Rufai MB: How safe is tourniquet use in sickle-cell disease? Afr J Med Med Sci 1996; 25:3-6
60. Harris LC, Haggard ME, Travis LB: The coexistence of sickle cell disease and congenital heart disease; a report of three cases with repair under cardiopulmonary bypass in two. Pediatrics 1964; 33:562-70
61. Pearson HA, Schiebler GL, Krovetz J, Hartley TD, David JK: Sickle-cell anemia associated with tetralogy of Fallot. N Engl J Med 1965; 273:1079-83
62. O'Keefe JD, LePere R, Britton HA: Blalock-Taussig shunt for tetralogy of Fallot in a patient with sickle cell anemia: A case report. JAMA 1967; 200:252-4
63. Szentpetery S, Robertson L, Lower RR: Complete repair of tetralogy associated with sickle cell anemia and G-6- PD deficiency. J Thorac Cardiovasc Surg 1976; 72:276-9
64. Hudson RL, Castro O, Spivak JL, Sampson C, Downing JW: Sickle cell anemia and transposition of the great vessels. Am J Dis Child 1978; 132:149-51
65. Powars D, Weidman JA, Odom-Maryon T, Niland JC, Johnson C: Sickle cell chronic lung disease: Prior morbidity and the risk of pulmonary failure. Medicine (Baltimore) 1988; 67:66-76
66. Lonsdorfer J, Bogui P, Otayeck A, Bursaux E, Poyart C, Cabannes R: Cardiorespiratory adjustments in chronic sickle cell anemia. Bull Eur Physiopathol Respir 1983; 19:339-44
67. Green RL, Huntsman RG, Sergeant GR: Sickle-cell and altitude: BMJ 1972; 1:803-4
68. Githens JH, Phillips CR, Humbert JR, Bonner SE, Ewing PC: Effects of altitude in persons with sickle hemoglobinopathies. Rocky Mt Med J 1975; 72:515-9
69. Mahony BS, Githens JH: Sickling crises and altitude: Occurrence in the Colorado patient population. Clin Pediatr (Phila) 1979; 18:431-8
70. Claster S, Godwin MJ, Embuty SH: Risk of altitude exposure in sickle cell disease. West J Med 1981; 135:364-7
71. Heffher JE, Sahn SA: High-altitude pulmonary infarction (letter). Arch Intern Med 1981; 141:1721
72. Hackett PH, Roach RC: High-altitude illness. N Eng J Med 2001; 345:107-14
73. Serjeant GR, Ceulaer CD, Lethbridge R, Morris J, Singhal A, Thomas PW: The painful crisis of homozygous sickle cell disease: Clinical features. Br J Haematol 1994; 87:586-91
74. Rao VM, Fishman M, Mitchell DG, Steiner RM, Ballas SK, Axel L, Dalinka MK, Gefter W, Kressel HY: Painful sickle cell crisis: Bone marrow patterns observed with MR imaging. Radiology 1986; 161:211-5
75. Rao SP, Miller S, Solomon N: Acute bone and joint manifestations of sickle cell disease in children. N Y State J Med 1986; 86:254-60
76. Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P, Vera JC, Levy PS: The acute chest syndrome in sickle cell disease: Incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood 1994; 84:643-9
77. Vichinsky EP, Styles LA, Colangelo LH, Wright EC, Castro O, Nickerson B: Acute chest syndrome in sickle cell disease: Clinical presentation and course. Cooperative Study of Sickle Cell Disease. Blood 1997; 89:1787-92
78. Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, Nickerson B, Orringer E, McKie V, Bellevue R, Daeschner C, Manci EA: Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000; 342:1855-65
79. Koumbourlis AC, Zar HJ, Hurlet-Jensen A, Goldberg MR: Prevalence and reversibility of lower airway obstruction in children with sickle cell disease. J Pediatr 2001; 138:188-92
80. Sutton LL, Castro O, Cross DJ, Spencer JE, Lewis JF: Pulmonary hypertension in sickle cell disease. Am J Cardiol 1994; 74:626-8
81. Ohene-Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury S, Moohr JW, Wethers DL, Pegelow CH, Gill FM: Cerebrovascular accidents in sickle cell disease: Rates and risk factors. Blood 1998; 91:288-94
82. Oyesiku NM, Barrow DL, Eckman JR, Tindall SC, Colohan AR: Intracranial aneurysms in sickle-cell anemia: Clinical features and pathogenesis. J Neurosurg 1991; 75:356-63
83. Stockman JA, Nigro MA, Mishkin MM, Oski FA: Occlusion of large cerebral vessels in sickle-cell anemia. N Eng J Med 1972; 287:846-9
84. Bruno D, Wigfall DR, Zimmerman SA, Rosoff PM, Wiener JS: Genitourinary complications of sickle cell disease. J Urol 2001; 166:803-11
85. Atlas SA: The sickle cell trait and surgical complications: A matched-pair patient analysis. JAMA 1974; 229:1078-80
86. Heller P, Best WR, Nelson RB, Becktel J: Clinical implications of sickle-cell trait and glucose-6-phosphate dehydrogenase deficiency in hospitalized black male patients. N Engl J Med 1979; 300:1001-5
87. Koshy M, Weiner SJ, Miller ST, Sleeper LA, Vichinsky E, Brown AK, Khakoo Y, Kinney TR: Surgery and anesthesia in sickle cell disease. Cooperative Study of Sickle Cell Diseases. Blood 1995; 86:3676-84
88. Vichinsky EP, Haberkern CM, Neumayr L, Earles AN, Black D, Koshy M, Pegelow C, Abboud M, Ohene-Frempong K, Iyer RV: A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group. N Engl J Med 1995; 333:206-13
89. Haberkern CM, Neumayr LD, Orringer EP, Earles AN, Robertson SM, Black D, Abboud MR, Koshy M, Idowu O, Vichinsky EP: Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the National Preoperative Transfusion Study. Preoperative Transfusion in Sickle Cell Disease Study Group. Blood 1997; 89:1533-42
90. Koshy M: Sickle cell disease and pregnancy. Blood Rev 1995; 9:157-64
91. Miller ST, Sleeper LA, Pegelow CH, Enos LE, Wang WC, Weiner SJ, Wethers DL, Smith J, Kinney TR: Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med 2000; 342:83-9
92. Kinney TR, Sleeper LA, Wang WC, Zimmerman RA, Pegelow CH, Ohene-Frempong K, Wethers DL, Bello JA, Vichinsky EP, Moser FG, Gallagher DM, DeBaun MR, Platt OS, Miller ST: Silent cerebral infarcts in sickle cell anemia: A risk factor analysis. The Cooperative Study of Sickle Cell Disease. Pediatrics 1999; 103:640-5
93. Schatz J, Brown RT, Pascual JM, Hsu L, DeBaun MR: Poor school and cognitive functioning with silent cerebral infarcts and sickle cell disease. Neurology 2001; 56:1109-11
94. Miller ST, Macklin EA, Pegelow CH, Kinney TR, Sleeper LA, Bello JA, Dewitt LD, Gallagher DM, Guarini L, Moser FG, Ohene-Frempong K, Sanchez N, Vichinsky EP, Wang WC, Wethers DL, Younkin DP, Zimmerman RA, Debaun MR: Silent infarction as a risk factor for overt stroke in children with sickle cell anemia: A report from the Cooperative Study of Sickle Cell Disease. J Pediatr 2001; 139:385-90
95. Esseltine DW, Baxter MRN, Bevan JC: Sickle cell states and the anaesthetist. Can J Anaesth 1988; 35:385-403
96. Griffin TC, Buchanan GR: Elective surgery in children with sickle cell disease without preoperative blood transfusion. J Pediatr Surg 1993; 28:681-5
97. Cohen MM, Duncan PG, Tate RB: Does anesthesia contribute to operative mortality? JAMA 1988; 260:2859-63
98. Vichinsky EP, Neumayr LD, Haberkern C, Earles AN, Eckman J, Koshy M, Black DM: The perioperative complication rate of orthopedic surgery in sickle cell disease: Report of the National Sickle Cell Surgery Study Group. Am J Hematol 1999; 62:129-38
99. Djaiani GN, Cheng DC, Carroll JA, Yudin M, Karski JM: Fast-track cardiac anesthesia in patients with sickle cell abnormalities. Anesth Analg 1999; 89:598-603
100. Metras D, Ouezzin Coulibaly A, Oattara K, Longechaud A, Miler P, Chauvet J: Open-heart surgery in sickle-cell hemoglobinopathies: Report of 15 cases. Thorax 1982; 37:486-91
101. Frimpong-Boateng K, Amoah AG, Barwasser HM, Kallen C: Cardiopulmonary bypass in sickle cell anemia without exchange transfusion. Eur J Cardiothorac Surg 1998; 14:527-9
102. Firth PG, Peterfreund RA: Management of multiple intracranial aneurysms: Neuroanesthetic considerations of sickle cell disease. J Neurosurg Anesthesiol 2000; 12:366-71
103. Davies SC: Blood transfusion in sickle cell disease. Curr Opin Hematol 1996; 3:485-91
104. Cox JV, Steane E, Cunningham G, Frenkel EP: Risk of alloimmunization and delayed hemolytic transfusion reactions in patients with sickle cell disease. Arch Intern Med 1988; 148:2485-9
105. Castellino SM, Combs MR, Zimmerman SA, Issitt PD, Ware RE: Erythrocyte autoantibodies in paediatric patients with sickle cell disease receiving transfusion therapy: frequency, characteristics and significance. Br J Haematol 1999; 104:189-94
106. Rosse WF, Gallagher D, Kinney TR, Castro O, Dosik H, Moohr J, Wang W, Levy PS: Transfusion and alloimmunization in sickle cell disease. The Cooperative Study of Sickle Cell Disease. Blood 1990; 76:1431-7
107. Moreira Junior G, Bordin JO, Kuroda A, Kerbauy J: Red blood cell alloimmunization in sickle cell disease: the influence of racial and antigenic pattern differences between donors and recipients in Brazil. Am J Hematol 1996; 52:197-200
108. Vichinsky EP, Luban NL, Wright E, Olivieri N, Driscoll C, Pegelow CH, Adams RJ, Stroke Prevention Trial in Sickle Cell Anemia: Prospective RBC phenotype matching in a stroke-prevention trial in sickle cell anemia: A multicenter transfusion trial. Transfusion 2001; 41:1086-92
109. Castro O, Sandler SG, Houston-Yu P, Rana S: Predicting the effects of transfusing only phenotype-matched RBCs to patients with sickle cell disease: Theoretical and practical implications. Transfusion 2002; 42:684-90
110. Dzik WH: Leukoreduction of blood components. Curr Opin Hematol 2002; 9:521-6
111. Milner PF: Oxygen transport in sickle cell anemia. Arch Intern Med 1974; 133:565-72
112. Rodgers GP, Schlechter AN, Noguchi CT, Klein HG, Nienhuis AW, Bonner RF: Periodic microciculatory flow in patients with sickle-cell disease. N Eng J Med 1984; 311:1534-8
113. Herold S, Brozovic M, Gibbs J, Lammertsma AA, Leenders KL, Carr D, Fleming JS, Jones T: Measurement of regional cerebral blood flow, blood volume and oxygen metabolism in patients with sickle cell disease using positron emission tomography. Stroke 1986; 17:692-8
114. Fitzgerald RK, Johnson A: Pulse oximetry in sickle cell anemia. Crit Care Med 2001; 29:1803-6
115. Chaplin H, Mischeaux JR, Inkster MD, Sherman LA: Frozen storage of 11 units of sickle cell red cells for autologous transfusion of a single patient. Transfusion 1986; 26:341-5
116. Cook A, Hanowell LH: Intraoperative autotransfusion for a patient with homozygous sickle cell disease. ANESTHESIOLOGY 1990; 73:177-9
117. Fox JS, Amaranath L, Hoeltge GA, Andrish JT: Autologous blood transfusion and intraoperative cell salvage in a patient with homozygous sickle cell disease. Cleve Clin J Med 1994; 61:137-40
118. Cheatam ML, Brackett CE: Problems in the management of subarachnoid hemorrhage in sickle cell anemia. J Neurosurg 1965; 23:488-93
119. Close RA, Buchheit WA: The management of ruptured intracranial aneurysm in sickle cell anemia: Case report. J Neurosurg 1977; 47:761-5
120. Overby MC, Rothman AS: Multiple intracranial aneurysms in sickle cell anemia: Report of two cases. J Neurosurg 1985; 62:430-4
121. Love LC, Mickle JP, Sypert GW: Ruptured intracranial aneurysms in cases of sickle cell anemia. Neurosurgery 1985; 16:808-12
122. Lewin P, Goodell RA: Post-operative blindness with complete recovery in a patient with sickle cell anemia (letter). BMJ 1962; 5316:1373
123. Shenk E: Sickle cell trait and superior longitudinal sinus thrombosis. Ann Intern Med 1964; 60:465-70
124. Leachman RD, Miller WT, Atias IM: Sickle cell trait complicated by sickle cell thrombi after open-heart surgery. Am Heart J 1967; 74:268-70
125. Howels TH, Humtsman RG, Boys JE, Mahmood A: Anaesthesia and sickle-cell hemoglobin. Br J Anaesth 1972; 44:975-87
126. Reinhard EH, Moore CV, Dubach R, Wade LJ: Depressant effect of high concentrations of oxygen on erythrogenesis: Observations on patients with sickle cell anemia with a description of the observed toxic manifestations of oxygen. J Clin Invest 1944; 23:682-98
127. Embury SH, Garcia JF, Mohandas N, Pennathur-Das R, Clark MR: Effects of oxygen inhalation on endogenous erythropoietin kinetics, erythropoiesis, and properties of blood cells in sickle-cell anemia. N Engl J Med 1984; 311:291-5
128. Charache S: Preoperative transfusion in sickle cell anemia. Am J Hematol 1991; 38:156-7
129. Bookchin RM, Balazs T, Landau LC: Determinants of red cell sickling: Effects of varying pH and of increasing intracellular hemoglobin concentration by osmotic shrinkage. J Lab Clin Med 1976; 87:597-616
130. Anson JA, Koshy M, Ferguson L, Crowell RM: Subarachnoid hemorrhage in sickle-cell disease. J Neurosurg 1991; 75:552-8
131. Rubenstein E: Studies on the relationship of temperature to sickle cell anemia. Am J Med 1961; 42:570-80
132. Ratner SJ, Athanasian EA: Water sports and sickle cell anemia (letter). Ann Intern Med 1986; 105:971
133. Murray N, May A: Painful crises in sickle cell disease: Patients' perspectives. BMJ 1988; 297:452-4
134. Resar LM, Oski FA: Cold water exposure and vaso-occlusive crises in sickle cell anemia. J Pediatr 1991; 118:407-9
135. Mohan JS, Marshall JM, Reid HL, Thomas PW, Hambleton I, Serjeant GR: Comparison of responses evoked by mild indirect cooling and by sound in the forearm vasculature in patients with homozygous sickle cell disease and in normal subjects. Clin Auton Res 1998; 8:25-30
136. Sessler DI: Perioperative heat balance. ANESTHESIOLOGY 2000; 92:578-96
137. Kark JA, Ward FT: Exercise and hemoglobin S. Semin Hematol 1994; 31:181-225
138. Greenberg M, Kass EH: Studies on the destruction of red blood cells. Arch Intern Med 1958; 101:355-63
139. Oster JR, Lespier LE, Lee SM, Pellegrini EL, Vaamonde CA: Renal acidification in sickle-cell disease. J Lab Clin Med 1976; 88:389-401
140. Kong HH, Alleyne GA: Acid-base status of adults with sickle-cell anaemia. BMJ 1969; 3:271-3
141. Holzmann L, Finn H, Lichtman HC, Harmel MH: Anesthesia in patients with sickle cell disease: A review of 112 cases. Anesth Analg 1969; 48:566-72
142. Yaster M, Tobin JR, Billett C, Casella JF, Dover G: Epidural analgesia in the management of severe vaso-occlusive sickle cell crisis. Pediatrics 1994; 93:310-5
143. Vijay V, Cavenagh JD, Yate P: The anaesthetist's role in acute sickle cell crisis. Br J Anaesth 1998; 80:820-8
144. Robieux IC, Kellner JD, Coppes MJ, Shaw D, Brown E, Good C, O'Brodovich H, Manson D, Olivieri NF, Zipursky A: Analgesia in children with sickle cell crisis: Comparison of intermittent opioids vs. continuous intravenous infusion of morphine and placebo-controlled study of oxygen inhalation. Pediatr Hematol Oncol 1992; 9:317-26
145. Pryle BJ, Grech H, Stoddart PA, Carson R, O'Mahoney T, Reynolds F: Toxicity of norpethidine in sickle cell crisis. BMJ 1992; 304:1478-9
146. Bellet PS, Kallinyak KA, Shukla R, Gelfand MJ, Rucknagel DL: Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. N Eng J Med 1995; 333:699-703
147. Griffin TC, McIntire D, Buchanan GR: High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease. N Engl J Med 1994; 330:733-7
148. Zipursky A, Robieux IC, Brown EJ, Shaw D, O'Brodovich H, Kellner JD, Coppes MJ, Koren G, Olivieri NF: Oxygen therapy in sickle cell disease. Am J Pediatr Hematol Oncol 1992; 14:222-8
149. Pegelow CH: Survey of pain management therapy provided for children with sickle cell disease. Clin Pediatr (Phil) 1992; 31:211-4
150. Barrett DH, Wisotzek IE, Abel GG, Pouleau JL, Platt AF, Pollard WE, Eckman JR: Assessment of psychosocial functioning of patients with sickle cell disease. South Med J 1988; 81:735-50
151. Steinberg MH: Management of sickle cell disease. N Engl J Med 1999; 340:1021-30
152. Huehns ER, Davies SC, Brozovic M: Fresh frozen plasma for vaso-occlusive crises in sickle cell disease. Lancet 1981; 1:1310-1
153. Bolanos-Meade J, Keung YK, Lopez-Arvzu C, Florendo R, Cobos E: Thrombotic thrombocytopenic purpura in a patient with sickle cell crisis. Ann Hematol 1999; 78:558-9
154. Gigel EJ, Francis CW: Reversal of multiorgan system dysfunction in sickle cell disease with plasma exchange. Acta Anesthesiol Scand 1997; 41:647-50
155. Svarch E, Gonzalez A, Villaescusa R, Basanta P: Plasma exchange for acute cholelithiasis in homozygous sickle cell disease. Haematologia 1986; 19:49-51
156. Setty BNY, Chen D, Stuart MJ: Sickle cell vaso-occlusive crisis is associated with abnormalities in the ratio of vasoconstrictor to vasodilator prostanoids. Pediatr Res 1995; 23:458-61
157. Atz AM, Wessel DL: Inhaled nitric oxide in sickle cell disease with acute chest syndrome. ANESTHESIOLOGY 1997; 87:988-90
158. Sullivan KJ, Goodwin SR, Evangelist J, Moore RD, Mehta P: Nitric oxide successfully used to treat acute chest syndrome of sickle cell disease in a young adolescent. Crit Care Med 1999; 27:2563-8
159. Bernini JC, Rogers ZR, Sandler ES, Reisch JS, Quinn CT, Buchanan GR: Beneficial effects of intravenous dexamethasone in children with mild to moderate acute chest syndrome complicating sickle cell disease. Blood 1998; 92:3082-9
160. Rennels MB, Dunne MG, Grossman NJ, Schwanz AD: Cholelithiasis in patients with major sickle hemoglobinopathies. Am J Dis Child 1984; 138:66-7
161. Smith JA, Espeland M, Bellevue R, Bonds D, Brown AK, Koshy M: Pregnancy in sickle cell disease: Experience of the Cooperative Study of Sickle Cell Disease. Obstet Gynecol 1996; 87:199-204
162. Powars DR, Sandhu M, Niland-Weiss J, Johnson C, Bruce S, Manning PR: Pregnancy in sickle cell disease. Obstet Gynecol 1986; 67:217-28
163. Perry KG Jr, Morrison JC: Hematologic disorders in pregnancy. Obstet Gynecol Clin North Am 1992; 19:783-99
164. Koshy M, Burd L, Wallace D, Moawad A, Baron J: Prophylactic red-cell transfusions in pregnant patients with sickle cell disease: A randomized cooper ative study. N Engl J Med 1988; 319:1447-52
165. Anyaegbunam A, Morel MI, Merkatz IR: Antepartum fetal surveillance tests during sickle cell crisis. Am J Obstet Gynecol 1991; 165:1081-3
166. Finer P, Blair J, Rowe P: Epidural analgesia in the management of labor pain and sickle cell crisis: A case report. ANESTHESIOLOGY 1988; 68:799-800
167. Milner PF, Kraus AP, Sebes JI, Sleeper LA, Dukes KA, Embury SH, Bellevue R, Koshy M, Moohr JW, Smith J: Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med 1991; 325:1476-81
168. McQuaker IG, Jaspan T, McConachie NS, Dolan G: Coil embolization of cerebral aneurysms in patients with sickling disorders. Br J Haematol 1999; 106:388-90
169. Rao KR: Angiography in hemoglobin S-C disease (letter). Ann Intern Med 1986; 105:971
170. Moran CJ, Siegel MJ, DeBaun MR: Sickle cell disease: Imaging of cerebrovascular complications. Radiology 1998; 206:311-21
171. Bomfim V, Ribeiro A, Gouvea F, Pereira J, Bjork V: Sickle cell anemia and mitral valve replacement: Case report. Scand J Thorac Cardiovasc Surg 1989; 23:75-7
172. Yacoub NH, Baron J, Et-Etr A, Kittle CF: Aortic homograft replacement of the mitral valve in sickle cell trait. J Thorac Cardiovasc Surg 1970; 59:568-73
173. Covitz W, Espeland M, Gallagher D, Hellenbrand W, Leff S, Talner N: The heart in sickle cell anemia. The Cooperative Study of Sickle cell Disease. Chest 1995; 108:1214-9
174. Wong WY, Powars DR, Chan L, Hiti A, Johnson C, Overturf G: Polysaccharide encapsulated bacterial infection in sickle cell anemia: A thirty year epidemiologic experience. Am J Hematol 1992; 39:176-82
175. Charace S, Terrin ML, Moore RD, Dover GJ, Barton FB, Ekhert SV, McMahon RP, Bonds DR: Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxurea in Sickle Cell Anemia. N Eng J Med 1995; 332:1317-22
176. Styles LA, Vichinsky E: Effects of a long-term transfusion regimen on sickle cell-relate illnesses. J Pediatrics 1994; 125:909-11
177. Falk JR, Scheinman J, Phillips G, Orringer E, Johnson A, Jennette JC: Prevalence and pathological features of sickle cell nephropathy and response to inhibition of angiotensin-converting enzyme. N Eng J Med 1992; 236:910-5
178. Head CA, Brugnara C, Martinez-Ruiz R, Kacmarek RM, Bridges KR, Kuter D, Bloch KD, Zapol WM: Low concentrations of nitric oxide increase oxygen affinity of sickle erythrocytes in vitro and in vivo. J Clin Invest 1997; 100:1193-8
179. La Nasa G, Giardini C, Argiolu F, Locatelli F, Arras M, De Stefano P, Ledda A, Pizzati A, Sanna MA, Vacca A, Lucarelli G, Contu L: Unrelated bone marrow transplant for thalassemia: the effect of extended haplotypes. Blood 2002; 99:4350-6
180. Pawliuk R, Westerman KA, Fabry ME, Payen E, Tighe R, Bouhassira EE, Acharya SA, Ellis J, London IM, Eaves CJ, Humphries RK, Beuzard Y, Nagel RL, Leboulch P: Correction of sickle cell disease in transgenic mouse models by gene therapy. Science 2001; 294:2368-71