Acute chest syndrome in sickle cell disease: Pathophysiology and management

Journal of Intensive Care Medicine

Volumn 15, Issue 3, 2000, Pages 159-166

  Claster, Susan ^a   Vichinsky, Elliott ^b  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b CHILDREN S HOSPITAL OAKLAND   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

DEXAMETHASONE; HYDROXYUREA; NITRIC OXIDE; STEROID; VASODILATOR AGENT;

BLOOD TRANSFUSION; HUMAN; LUNG EMBOLISM; MORTALITY; PATHOGENESIS; PRIORITY JOURNAL; REVIEW; SICKLE CELL ANEMIA; SPIROMETRY; THORAX PAIN;

EID: 0034120827     PISSN: 08850666     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1525-1489.2000.00159.x     Document Type: Review

References (69)

1. Thomas AN, Pattison C, Serjeant GR. Causes of death in sickle-cell disease in Jamaica. Br Med J Clin Res Ed 1982; 285:633-635
2. Vichinsky E. Comprehensive care in sickle cell disease: its impact on morbidity and mortality. Semin Hematol 1991; 28:220
3. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death [see comments]. N Engl J Med 1994;330:1639-1644
4. Vichinsky EP, Styles LA, Colangelo LH, et al. Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease. Blood 1997;89:1787-1792
5. 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
6. Vichinsky E. Acute chest syndrome (ACS) in sickle cell disease (SCD). Blood 1994;10(suppl):219a
7. 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. Blood 1995;86: 776-783
8. Powars D, Weidman JA, Odom-Maryon T, et al. Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine 1988;67:66-76
9. Embury SH, Dozy AM, Miller J, et al. Concurrent sickle-cell anemia and alpha-thalassemia: effect on severity of anemia. N Engl J Med 1982;306:270-274
10. Powars D. Sickle cell anemia: beta S-gene-cluster haplotypes as prognostic indicators of vital organ failure. Semin Hematol 1991;28:202
11. Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med 1995;332:1317-1322
12. Cole TB, Sprinkle RH, Smith SJ, et al. Intravenous narcotic therapy for children with severe sickle cell pain crisis. Am J Dis Child 1986;140:1255-1259
13. Curtis EM. Pregnancy in sickle cell anemia, sickle-cell-hemoglobin C disease and variants thereof. Am J Obstet Gynecol 1959;77:1312
14. Haynes J Jr, Allison RC. Pulmonary edema. Complication in the management of sickle cell pain crisis. Am J Med 1986; 80:833-840
15. Rucknagel DL, Kalinyak KA, Gelfand MJ. Rib infarcts and acute chest syndrome in sickle cell diseases. Lancet 1991; 337:831-833
16. Barrett-Connor E. Pneumonia and pulmonary infarction in sickle cell anemia. JAMA 1973;224:997-1000
17. Charache S, Scott JC, Charache P. "Acute chest syndrome" in adults with sickle cell anemia. Microbiology, treatment, and prevention. Arch Intern Med 1979;139:67-69
18. Barrett-Connor E. Acute pulmonary disease and sickle cell anemia. Am Rev Respir Dis 1971;104:159
19. Wong WY, Powars DR, Chan L, et al. Polysaccharide encapsulated bacterial infection in sickle cell anemia: a thirty year epidemiologic experience. Am J Hematol 1992;39:176-182
20. Poncz M, Kane E, Gill FM. Acute chest syndrome in sickle cell disease: etiology and clinical correlates. J Pediatr 1985; 107:861-866
21. Sprinkle RH, Cole T, Smith S, et al. Acute chest syndrome in children with sickle cell disease. A retrospective analysis of 100 hospitalized cases. Am J Pediatr Hematol Oncol 1986; 8:105-110
22. Miller ST, Hammerschlag MR, Chirgwin K, et al. Role of Chlamydia pneumoniae in acute chest syndrome of sickle cell disease. J Pediatr 1991;118:30-33
23. Shulman ST, Bartlett J, Clyde WA Jr, Ayoub EM. The unusual severity of Mycoplasmal pneumonia in children with sickle-cell disease. N Engl J Med 1972;287:164-167
24. Solanki DL, Berdoff RL. Severe mycoplasma pneumonia with pleural effusions in a patient with sickle cell-hemoglobin C (SC) disease. Case report and review of the literature. Am J Med 1979;66:707-710
25. Kirkpatrick MB, Haynes J. Sickle cell disease and the pulmonary circulation. Respir Crit Care Med 1994;15:473
26. Gowda N, Rao SP, Cohen B, et al. Human parvovirus infection in patients with sickle cell disease with and without hypoplastic crisis. J Pediatr 1987;110:81-84
27. Haddad JD, John JF Jr, Pappas AA. Cytomegalovirus pneumonia in sickle cell disease. Chest 1984;86:265-266
28. Lowenthal EA, Wells A, Emanuel PD, et al. Sickle cell acute chest syndrome associated with parvovirus B19 infection: case series and review. Am J Hematol 1996;51:207-213
29. Conrad ME, Studdard H, Anderson LJ. Aplastic crisis in sickle cell disorders: bone marrow necrosis and human parvovirus infection. Am J Med Sci 1988;295:212-215
30. Leong MA, Dampier C, Varlotta L, et al. Airway hyperreactivity in children with sickle cell disease. J Pediatr 1997;131: 278-283
31. Godeau B, Bachir D, Randrianjohanny A, et al. [Extensive bone marrow necrosis and major sickle cell syndromes]. Rev Med Interne 1994;15:25-29
32. Gong H Jr. Fat embolism in sickle cell disease. Rev Med Interne 1977;62:40
33. Johnson K, Stastny JF, Rucknagel DL. Fat embolism syndrome associated with asthma and sickle cell-beta(+)-thalassemia. Am J Hematol 1994;46:354-357
34. Shapiro MP, Hayes JA. Fat embolism in sickle cell disease. Report of a case with brief review of the literature. Arch Intern Med 1984;144:181-182
35. Lehmann EP, McNathin RF. Fat embolism, incidence at post mortem. Arch Surg 1928;17:79
36. Desselle BC, O'Brien T, Bugnitz M, et al. Fatal fat embolism in a patient with sickle-beta + thalassemia. Pediatr Hematol Oncol 1995;12:159-162
37. Horton DP, Ferriero DM, Mentzer WC. Nontraumatic fat embolism syndrome in sickle cell anemia. Pediatr Neurol 1995; 12:77-80
38. Hutchinson RM, Merrick MV, White JM. Fat embolism in sickle cell disease. J Clin Pathol 1973;26:620-622
39. Vichinsky E, Williams R, Das M, et al. Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia. Blood 1994;83:3107-3112
40. Weil JV, Castro O, Malik AB, et al. NHLBI workshop summary. Pathogenesis of lung disease in sickle hemoglobinopathies. Am Rev Respir Dis 1993;148:249-256
41. Styles LA, Schalkwijk CG, Aarsman AJ, et al. Phospholipase A2 levels in acute chest syndrome of sickle cell disease. Blood 1996;87:2573-2578
42. Grisham J, Vichinsky E. Ketorolac versus meperidine in vaso-occlusive crisis: a study of safety and efficacy. Int J Pediatr Hematol Oncol 1996;3:239
43. Palmer J, Broderick KA, Naiman JL. Acute lung syndrome during painful sickle cell crisis - relation to site of pain and narcotic requirement. Blood 1983;62(suppl):59a
44. Ballas SK, Park CH. Severe hypoxemia secondary to acute sternal infarction in sickle cell anemia. J Nucl Med 1991;32: 1617-1618
45. Bellet PS, Kalinyak KA, Shukla R, et al. Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. N Engl J Med 1995;333:699-703
46. Gelfand MJ, Daya SA, Rucknagel DL, et al. Simultaneous occurrence of rib infarction and pulmonary infiltrates in sickle cell disease patients with acute chest syndrome. J Nucl Med 1993;34:614-618
47. Durant JR, Cortes FM. Occlusive pulmonary vascular disease associated with hemoglobin SC disease. A case report. Am Heart J 1966;17:100
48. Kaul DK, Baez S, Nagel RL. Flow properties of oxygenated HBS and HbC erythrocytes in the isolated microvasculature of the rat. A contribution to the hemorheology of hemoglobinopathies. Clin Hemorheol 1981;1:73
49. Walter BK, Ballas SK, Burka ER. The diagnosis of pulmonary thromboembolism in sickle cell disease. Am J Hematol 1979; 7:219
50. Lisbona R, Derbekyan V, Novales-Diaz JA. Scintigraphic evidence of pulmonary vascular occlusion in sickle cell disease. J Nucl Med 1997;38:1151-1153
51. Francis RB Jr. Elevated fibrin D-dimer fragment in sickle cell anemia: evidence for activation of coagulation during the steady state as well as in painful crisis. Haemostasis 1989; 19:105-111
52. Francis RB Jr. Platelets, coagulation, and fibrinolysis in sickle cell disease: their possible role in vascular occlusion. Blood Coagul Fibrinolysis 1991;2:341-353
53. Hammerman SI, Kourembanas S, Conca TJ, et al. Endothelin-1 production during the acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med 1997;156:280-285
54. Rackoff WR, Kunkel N, Silber JH, et al. Pulse oximetry and factors associated with hemoglobin oxygen desaturation in children with sickle cell disease. Blood 1993;81:3422-3427
55. Emre U, Miller ST, Gutierez M, et al. Effect of transfusion in acute chest syndrome of sickle cell disease. J Pediatr 1995; 127:901-904
56. Mallouh AA, Asha M. Beneficial effect of blood transfusion in children with sickle cell chest syndrome. Am J Dis Child 1988;142:178-182
57. Bhalla M, Abboud MR, McLoud TC, et al. Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion. Radiology 1993;187:45-49
58. 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: 64-69
59. Emre U, Miller ST, Rao SP, et al. Alveolar-arterial oxygen gradient in acute chest syndrome of sickle cell disease. J Pediatr 1993;123:272-275
60. Embury SH, Garcia JF, Mohandas N, et al. Effects of oxygen inhalation on endogenous erythropoietin kinetics, erythropoiesis, and properties of blood cells in sickle-cell anemia. N Engl J Med 1984;311:291-295
61. Castro O. Systemic fat embolism and pulmonary hypertension in sickle cell disease. Hematol Oncol Clin N Am 1996; 10:1289-1303
62. Bernini JC, Rogers ZR, Sandler ES, et al. Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease. Blood 1998;92:3082-3089
63. 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;330:733-737
64. Lanzkowsky P, Shende A, Karayalcin G, et al. Partial exchange transfusion in sickle cell anemia. Use in children with serious complications. Am J Dis Child 1978;132:1206-1208
65. Wayne AS, Kevy SV, Nathan DG. Transfusion management of sickle cell disease. Blood 1993;81:1109-1123
66. Abboud MR, Habib D, Jackson SM, et al. Elevated serum and bronchoalveolar lavage fluid levels of IL-8 and G-CSF associated with the acute chest syndrome in patients with sickle cell disease. Blood 1998;92(suppl 1):497a
67. Atz AM, Wessel DL. Inhaled nitric oxide in sickle cell disease with acute chest syndrome. Anesthesiology 1997;87:988-990
68. Baird JS, Johnson JL, Escudero J, et al. Combined pressure control/high frequency ventilation in adult respiratory distress syndrome and sickle cell anemia. Chest 1994;106: 1913-1916
69. Sullivan KM, Reid CD. Introduction to a symposium on sickle cell anemia: current results of comprehensive care and the evolving role of bone marrow transplantation. Semin Hematol 1991;28:177-179