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<< Sickle Cell Disease And Other Hemoglobinopathies: Approaches To Emergency Diagnosis And Treatment


 Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report. To help the reader judge the strength of each reference, pertinent information about the study, such as the type of study and the number of patients in the study, will be included in bold type following the reference, where available. In addition, the most informative references cited in the paper, as determined by the authors, will be noted by an asterisk (*) next to the number of the reference.

  1. Ballas SK. Complications of sickle cell anemia in adults: guidelines for effective management. Cleve Clin J Med 1999;66(1):48-58. (Review)
  2. Rosen P, Barkin R, Danzl DF, et al, eds. Emergency Medicine: Concepts and Clinical Practice. 4th ed. St. Louis: Mosby; 1998. (Textbook)
  3. * Steinberg MH. Management of sickle cell disease. N Engl J Med 1999;340(13):1021-1030. (Review; 103 references)
  4. Davis H, Moore RM Jr, Gergen PJ. Cost of hospitalizations associated with sickle cell disease in the United States. Public Health Rep 1997 Jan-Feb;112(1):40-43. (Data analysis)
  5. Mandelberg JH, Kuhn RE, Kohn MA. Epidemiologic analysis of an urban, public emergency department's frequent users. Acad Emerg Med 2000 Jun;7(6):637-646. (Cross-sectional, retrospective cohort study; 348,858 visits to the San Francisco General Hospital ED during a five-year period)
  6. * Steinberg MH, Forget BG, Higgs DR, et al, eds. Disorders of Hemoglobin. Cambridge: Cambridge University Press; 2001.(Textbook)
  7. Wethers DL. Sickle cell disease in childhood. Am Fam Physician 2000;62(5):1013-1020. (Review)
  8. Herrick JB. Peculiar elongated and sickle-shaped red blood corpuscles in a case of severe anemia. Arch Intern Med 1910;6:517-521. (Case report; historical reference)
  9. Hagar RW, Vichinsky EP. Major changes in sickle cell disease. In: Advances in Pediatrics. St Louis: Mosby-Yearbook; 2000:47;249-272. (Textbook chapter)
  10. * Yaster H, Kost-Byerly S, Maxwell LG. The management of pain in sickle cell disease. Pediatr Clin North Am 2000;47(3):699-710. (Review)
  11. Bjornson AB, Lobel JS, Harr KS. Relation between serum opsonic activity for Streptococcus pneumoniae and complement function in sickle cell disease. J Infect Dis 1985;153:701-709. (Basic science)
  12. Magnus SA, Humbleton IR, Moosdeen F, et al. Recurrent infections in homozygous sickle cell disease. Arch Dis Child 1999;80:537-541. (Prospective; 176 patients)
  13. * Sears DA. The morbidity of sickle cell trait. Am J Med 1978;64:1021-1036. (Review)
  14. Ballas SK, Kocher W. The erythrocytes in HbSC disease are microcytic and hyperchromic. Am J Hematol 1988;28:37-39. (Basic science)
  15. Rakel RE, Bope ET, eds. Conn's Current Therapy. 53rd ed. Philadelphia: WB Saunders; 2001. (Textbook)
  16. No authors listed. Acute complications of sickle cell disease in children. Drug Ther Bull 2001;39(5):33-37. [Erratum appears in Drug Ther Bull 2001 Aug;39(8):64.] (Review; 21 references)
  17. Leikin SL, Gallagher D, Kinney TR, et al. Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease. Pediatrics 1989;84:500-508. (Prospective; 2824 patients)
  18. Neonato MG, Guilloud-Bataille M, Beauvais P, et al. Acute clinical events in 299 homozygous sickle cell patients living in France. French Study Group on Sickle Cell Disease. Eur J Haematol 2000;65(3):155-164. (Cohort; 299 patients)
  19. * Gill FM, Sleeper LA, Weiner SJ, et al. Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease. [see comments] Blood 1995;86:776-783. (Prospective; 649 patients)
  20. * Miller ST, Sleeper LA, Pegelow CH, et al. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med 2000;342(2):83-89. (Retrospective; 392 children)
  21. No authors listed. A review of evidence about factors affecting quality of pain management in sickle cell disease. Database of Abstracts of Reviews of Effectiveness. Issue 1, July 2001.
  22. * American Pain Society. Guideline for the management of acute and chronic pain in sickle cell disease. APS Clinical Practice Guideline no. 1. Glenview, IL: American Pain Society (APS); August 1999. (Clinical practice guideline, 98 references)
  23. * Silbergleit R, Jancis MO, McNamara RM. Management of sickle cell pain crisis in the emergency department at teaching hospitals. J Emerg Med 1999;17(4):625-630. (Survey; 549 emergency physicians)
  24. Overturf GD. Infections and immunizations in children with sickle cell disease. Adv Pediatr Dis 1999;14:191-218. (Review)
  25. * Wong AL, Sakamoto KM, Johnson EE. Differentiating osteomyelitis from bone infarction in sickle cell disease. Pediatr Emerg Care 2001;17(1):60-63. (Review)
  26. Landesman SH, Rao SP, Ahonkhai VI. Infections in children  with sickle cell anemia. Am J Pediatr 1982;4:407-415. (Review)
  27. Buchanan GR. Differentiation of bone infarct from infection in a child with sickle cell disease. Pediatr Inf Dis J 1996;15:725. (Case report)
  28. * Crowley JJ, Sarnaik S. Imaging of sickle cell disease. Pediatr Radiol 1999;29:646-661. (Review)
  29. * No authors listed. National Institutes Of Health. Division of blood diseases and resources. Management and Therapy of Sickle Cell Disease. 3rd rev. Bethesda, MD: National Heart, Lung and Blood Institute; 1995; NIH Publ no. 95-2117. (Clinical guideline)
  30. * Baumgartner F. Klein S. The presentation and management of the acute abdomen in the patient with sickle-cell anemia. Am Surg 1989;55(11):660-664. (Retrospective; 53 patients)
  31. Sheey TW. Sickle cell hepatopathy. South Med J 1977;70:533-538. (Review)
  32. Betrosian A, Balla M, Kafirig G, et al. Reversal of liver failure in sickle cell vaso-occlusive crisis. Am J Med Sci 1996;311:292-295. (Case report)
  33. Shao SH, Orringer EP. Sickle cell intrahepatic cholestasis: approach to a difficult problem. Am J Gastroenterol 1995;90:2048-2050. (Review)
  34. Haberkern CM, Neumayr LD, Orringer EP, et al. Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the national preoperative transfusion study. Blood 1997;89:1533-1542. (Prospective; 364 cases)
  35. Lachman BS, Lazerson J, Starshak RJ, et al. The prevalence of cholelithiasis in sickle cell disease as diagnosed by ultrasound and cholecystography. Pediatrics 1979;64:601-603. (Prospective; 31 patients)
  36. Sarnaik S, Slovis TL, Corbett DP, et al. Incidence of cholelithiasis in sickle cell anemia using the ultrasonic gray-scale technique. J Pediatr 1980;96:1005-1008. (Prospective; 226 patients)
  37. Walker TM, Hambleton IR, Serjeant GR. Gallstones in sickle cell disease: observations from the Jamaican cohort study. J Pediatr 2000;136:80-85. (Review)
  38. Alexander-Reindorf C, Nwaneri RU, Worrel RG, et al. The significance of gallstones in children with sickle cell anemia. J Nat Med Assoc 1990;83:645-650. (Retrospective; 86 patients)
  39. Nelson MS, Chisolm JJ Jr. Lead toxicity masquerading as sickle cell crisis. Ann Emerg Med 1986;15(6):748-750. (Case report)
  40. Antal P, Gauderer M, Koshy M, et al. Is the incidence of appendicitis reduced in patients with sickle cell disease? Pediatrics 1998 Jan;101(1):E7. (Multicenter retrospective chart review)
  41. Walker BK, Ballas SK, Burka ER. The diagnosis of pulmonary thromboembolism in sickle cell disease. Am J Hematol 1979;7:219-232. (Review)
  42. * Martin L, Buonomo C. Acute chest syndrome of sickle cell disease: radiographic and clinical analysis of 70 cases. Pediatr Radiol 1997;27:637-641. (Retrospective; 70 cases)
  43. Bhalla M, Abboud MR, McLoud TC, et al. Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion. Radiology 1993;187(1):45-49. (Retrospective; 10 patients)
  44. * Elander J, Midencek K. A review of evidence about factors affecting the quality of pain management in sickle cell disease. Clin J Pain 1996;12:180-193. (Review)
  45. Shaprio BS. The management of pain in sickle cell disease. Pediatr Clin North Am 1989;36:1029-1045. (Review)
  46. * Platt OS, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease. Rates and risk factors. N Engl J Med 1991;325:11-16. (Prospective; 3578 patients)
  47. Adams RJ, McKie V, Hsu L, et al. Prevention of first stroke by transfusions in children with sickle cell disease and abnormal results on transcranial ultrasonography. N Engl J Med 1998;339:5-11. (Prospective; 30 patients)
  48. Smith J. Bone disorders in sickle cell disease. Hematol Oncol Clin North Am 1996;10:1345-1355. (Review)
  49. * Sporrer KA, Jackson SM, Agner S, et al. Pain in children and adolescents with sickle cell anemia: a prospective study utilizing self-reporting. Am J Pediatr Hematol Oncol 1994 Aug;16(3):219-224. (Prospective; 17 patients)
  50. Kudsk KA, Tranbaugh RF, Sheldon GF. Acute surgical illness in patients with sickle cell anemia. Am J Surg 1981;142(1):113-117. (7 patients)
  51. * Bonadio WA. Clinical features of abdominal painful crisis in sickle cell anemia. J Pediatr Surg 1990;25(3):301-302. (Retrospective; 106 cases of abdominal painful crisis in 43 children)
  52. Fitzgerald RK, Johnson A. Pulse oximetry in sickle cell anemia. Crit Care Med 2001;29(9):1803-1806. (Prospective; comparative; 24 patients)
  53. Blaisdell CJ, Goodman S, Clark K, et al. Pulse oximetry is a poor predictor of hypoxemia in stable children with sickle cell disease. Arch Pediatr Adolesc Med 2000;154(9):900-903. (Comparative; 21 children)
  54. Ernst AA, Weiss SJ, Johnson WD, et al. Blood pressure in acute vaso-occlusive crises of sickle cell disease. South Med J 2000;93(6):590-592. (Retrospective; 459 SCD-related visits in 106 patients)
  55. Simmons BE, Santhanam V, Castaner A, et al. Sickle cell heart disease. Two-dimensional echo and Doppler ultrasonographic findings in the hearts of adult patients with sickle cell anemia. Arch Intern Med 1988;148(7):1526-1528. (40 patients)
  56. Karayalcin G, Rosner F, Kim KY, et al. Sickle cell anemia— clinical manifestations in 100 patients and review of the literature. Am J Med Sci 1975;269(1):51-68. (Review, case report; 100 patients)
  57. Serjeant GR, Ceulaer CD, Lethbridge R, et al. The painful crisis of homozygous sickle cell disease: clinical features. Br J Haematol 1994; 87(3):586-591. (Prospective; 183 painful crises in 118 patients)
  58. Zimmerman SA, Ware RE. Palpable splenomegaly in children with haemoglobin SC disease: haematological and clinical manifestations. Clin Lab Haematol 2000;22(3):145-150. (Retrospective; 100 patients)
  59. * Pollack CV Jr. Jorden RC. Kolb JC. Usefulness of empiric chest radiography and urinalysis testing in adults with acute sickle cell pain crisis. Ann Emerg Med 1991;20(11):1210-1214. (Prospective; 134 presentations in 71 patients)
  60. * Ander DS, Vallee PA. Diagnostic evaluation for infectious etiology of sickle cell pain crisis. Am J Emerg Med 1997;15(3):290-292. (Retrospective, observational; 94 visits)
  61. * Morris C, Vichinsky E, Styles L. Clinician assessment for acute chest syndrome in febrile patients with sickle cell disease: is it accurate enough? Ann Emerg Med 1999;34(1):64-69. (Prospective; 96 febrile events in 73 patients)
  62. Leong CS, Stark P. Thoracic manifestations of sickle cell disease. J Thorac Imag 1998;13(2):128-134. (Review; 8 references)
  63. Magid D, Fishman EK, Charache S, et al. Abdominal pain in sickle cell disease: the role of CT. Radiology 1987;163(2):325-328. (30 patients)
  64. Al-Salem AH, Qaisaruddin S, Al Jam'a A, et al. Splenic abscess and sickle cell disease. Am J Hematol 1998; 58(2):100-104. (Retrospective; 10 cases)
  65. Serafini AN, Spoliansky G, Sfakianakis GN, et al. Diagnostic studies in patients with sickle cell anemia and acute abdominal pain. Arch Intern Med 1987;147(6):1061-1062. (Retrospective; 28 patients)
  66. Awogu AU. Leucocyte counts in children with sickle cell anaemia usefulness of stable state values during infections. West Afr J Med 2000 Jan-Mar;19(1):55-58. (Case-control; 200 steady state SCA children, 60 age- and sex-matched AA genotype controls)
  67. Lopez BL, Griswold SK, Navek A, et al. The complete blood count and reticulocyte count—are they necessary in the evaluation of acute vaso-occlusive sickle-cell crisis? Acad Emerg Med 1996;3(8):751-757. (Part 1: retrospective chart review; Part 2: prospective)
  68. Ahmed YF, Abbag FI, Al-Qahtani JM, et al. Erythrocyte sedimentation rate during steady state, painful crisis and infection in children with sickle cell disease. Saudi Med J 2000;21(5):461-463. (Comparative; 95 children)
  69. Sellors JW, Mahony JB, Pickard L, et al. Screening urine with a leukocyte esterase strip and subsequent chlamydial testing of asymptomatic men attending primary care practitioners. Sex Transm Dis 1993;20(3):152-157. (882 men)
  70. Beyer JE. Judging the effectiveness of analgesia for children and adolescents during vaso-occlusive events of sickle cell disease. J Pain Symptom Manage 2000 Jan;19(1):63-72. (21 patients)
  71. * Brookoff D, Polomano R. Treating sickle cell pain like cancer pain. [see comments]. Ann Intern Med 1992;116(5):364-368. (Cohort)
  72. Friedman EW, Webber AB, Osborn HH, et al. Oral analgesia for treatment of painful crisis in sickle cell anemia. Ann Emerg Med 1986 Jul;15(7):787-791. (Comparative; 100 patients)
  73. Gonzalez ER, Bahal N, Hansen LA, et al. Intermittent injection vs patient-controlled analgesia for sickle cell crisis pain. Comparison in patients in the emergency department. Arch Intern Med 1991;151(7):1373-1378. (Prospective; 20 patients)
  74. Trentadue NO, Kachoyeanos MK. Lea G. A comparison of two regimens of patient-controlled analgesia for children with sickle cell disease. J Pediatr Nurs 1998;13(1):15-19. (Retrospective; 60 visits, 26 children)
  75. Shapiro BS, Cohen DE, Howe CJ. Patient-controlled analgesia for sickle-cell-related pain. J Pain Symptom Manage 1993;8(1):22-28. (Retrospective; 46 patients)
  76. McPherson E, Perlin E, Finke H, et al. Patient-controlled analgesia in patients with sickle cell vaso-occlusive crisis. Am J Med Sci 1990;299(1):10-12. (Pilot study; 16 patients)
  77. Cole TB, Sprinkle RH, Smith SJ, et al. Intravenous therapy for children with severe sickle cell pain crisis. Am J Child 1986;140:1255-1259. (Retrospective; 98 painful episodes in 38 patients)
  78. Battenhorst RL, Maurer HS, Bertch KA, et al. Patient controlled analgesia in uncomplicated sickle cell pain crisis. Blood 1987;70:558A.
  79. Hagmeyer KO, Mauro LS, Mauro VF. Meperidine-related seizures associated with patient-controlled analgesia pumps. Ann Pharmacother 1993; 27(1):29-32. (Review; 8 references)
  80. Stephens CR, Linton R, Cole K. Sickle cell disease: a review of state of the art emergency management and outcome-effective therapy. Emerg Med Rep 1999;20:18. (Review)
  81. Physicians' Desk Reference. 55th ed. Montvale, NJ: Medical Economics; 2001.
  82. Jacobson SJ, Kopecky EA, Joshi P, et al. Randomised trial of oral morphine for painful episodes of sickle-cell disease in children. Lancet 1997;350(9088):1358-1361. (Randomized controlled trial; 56 children)
  83. Eckman JR, Platt A. Problem Oriented Management of Sickle Syndromes. Atlanta: Emory University School of Medicine; 1991. See: http://www.emory.edu/PEDS/SICKLE/prod04.htm.
  84. Ballas SK. Management of sickle pain. Curr Opin Hematol 1997;4:104-111. (Review)
  85. Pryle BJ, Grech H, Stoddart PA, et al. Toxicity of norpethidine in sickle cell crisis. BMJ 1992;304:1478-1479.
  86. Nadvi SZ, Sarnaik S, Ravindranath Y. Low frequency of meperidine-associated seizures in sickle cell disease. Clin Pediatr (Phila) 1999;38(8):459-462. (Retrospective)
  87. Barsan WG, Tomassoni AJ, Seger D, et al. Safety assessment of high-dose narcotic analgesia for emergency department procedures. Ann Emerg Med 1993;22:1444-1449. (Prospective, multicenter; 72 patients)
  88. Christensen ML, Wang WC, Harris S, et al. Transdermal fentanyl administration in children and adolescents with sickle cell pain crisis. J Pediatr Hematol Oncol 1996;18(4):372-376. (Controlled; 10 patients)
  89. Beiter JL Jr, Simon HK, Chambliss CR, et al. Intravenous ketorolac in the emergency department management of sickle cell pain and predictors of its effectiveness. Arch Pediatr Adolesc Med 2001;155(4):496-500. (Prospective; 51 children)
  90. Hardwick WE Jr, Givens TG, Monroe KW, et al. Effect of ketorolac in pediatric sickle cell vaso-occlusive pain crisis. Pediatr Emerg Care 1999;15(3):179-182. (Randomized controlled trial; 41 episodes in 29 children)
  91. Wright SW, Norris RL, Mitchell TR. Ketorolac for sickle cell vaso-occlusive crisis pain in the emergency department: lack of a narcotic-sparing effect. [see comments] Ann Emerg Med 1992;21(8):925-928. (Randomized controlled trial; 24 crises in 18 patients)
  92. Simckes AM, Chen SS, Osorio AV, et al. Ketorolac-induced irreversible renal failure in sickle cell disease: a case report. Pediatr Nephrol 1999;13(1):63-67. (Case report)
  93. Haynes J Jr, Allison RC. Pulmonary edema. Complication in the management of sickle cell pain crisis. Am J Med 1986;80(5):833-840. (Case report; 4 cases of pulmonary edema among 51 SC admissions)
  94. Ballas SK. Neurobiology and treatment of pain. In: Embury SH, Hebbel RP, Mohandas N, et al, eds. Sickle Cell Disease: Basic Principles and Clinical Practice. New York: Raven Press; 1994:745-772. (Textbook chapter)
  95. Robieux IC, Kellner JD, Coppes MJ, et al. Analgesia in children with sickle cell crisis: comparison of intermittent opioids vs. continuous intravenous infusion of morphine and placebocontrolled study of oxygen inhalation. Pediatr Hematol Oncol 1992;9(4):317-326. (Prospective; 66 children)
  96. Zipursky A, Robieux IC, Brown EJ, et al. Oxygen therapy in sickle cell disease. Am J Pediatr Hematol Oncol 1992;14(3):222-228. (Randomized controlled trial; 25 patients)
  97. Griffin TC, McIntire D, Buchanan GR. High-dose intravenous methylprednisolone therapy for pain in children and adolescents with sickle cell disease. [see comments] N Engl J Med 1994;330(11):733-737. (Randomized controlled trial; 36 children and adolescents)
  98. Frush K, Ware RE, Kinney TR. Emergency department visits by children with sickle hemoglobinopathies: factors associated with hospital admission. Pediatr Emerg Care 1995;11(1):9-12. (Retrospective; 146 visits by 56 children)
  99. Quinn CT, Buchanan GR. The acute chest syndrome of sickle cell disease. J Pediatr 1999;135:416-422. (Review)
  100. Vichinsky E. Comprehensive care in sickle cell disease: its impact on morbidity and mortality. Semin Hematol 1991;28:220- 226. (Review)
  101. Castro O, Brambilla DJ, Thorington B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood 1994;84:643-649.(Prospective; 3751 patients)
  102. * Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med 1994;330:1639-1644. (Prospective; 3764 patients)
  103. * Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med 2000;342(25):1855-1865. (Prospective; 538 patients)
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  108. * Vichinsky EP, Styles LA, Colangelo LH, et al. Acute chest syndrome in sickle cell disease: clinical presentation and course. Blood 1997;89(5):1787-1792. (Prospective, longitudinal; 3751 patients)
  109. Lisbona R, Derbekyan V, Norales-Diaz JA. Scintigraphic evidence of pulmonary vascular occlusion in sickle cell disease. J Nucl Med 1997;38:1151-1153. (Case report)
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  112. Bainbridge R, Higgs DR, Maude GH, et al. Clinical presentation of sickle cell disease. J Pediatr 1985;106:881-885. (Retrospective chart review; 305 patients)
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  118. Sheth S, Ruzal-Shapiro C, Piomelli S, et al. CT imaging of splenic sequestration in sickle cell disease. Pediatr Radiol 2000;30(12):830-833. (7 patients)
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  123. Vichinsky EP. Current issues with blood transfusions in sickle cell disease. Semin Hematol 2001 Jan;38(1,):14-22. (Review)
  124. Wierenga KJ, Hambleton IR, Wilson RM, et al. Significance of fever in Jamaican patients with homozygous sickle cell disease. Arch Dis Child 2001;84(2):156-159. (Retrospective; 165 events in 144 patients)
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  133. Adams RJ. Stroke prevention and treatment in sickle cell disease. Arch Neurol 2001;58:565-568. (Review)
  134. Fabian RH, Peters BH. Neurological complications of hemoglobin SC disease. Arch Neurol 1984 Mar;41(3):289-292. (Retrospective chart review; 136 cases)
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  139. Powars DR, Elliot Mills DD, Chan L. Chronic renal failure in sickle cell disease: risk factors, clinical course and mortality. Ann Intern Med 1991;115:614-620. (Prospective; 934 patients)
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