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Sickle Cell Disease, Acute Chest Syndrome
Alexander J. Towbin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Acute chest syndrome (ACS)
            • Sickle cell disease (SCD)
          • Definitions

            • New pulmonary opacity on chest radiograph + ≥ 1 additional symptom (such as fever, cough, sputum production, tachypnea, dyspnea, or hypoxia) in setting of SCD

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • Nuclear Medicine Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  Selected References

                                  1. Knight-Madden J et al: Acute pulmonary complications of sickle cell disease. Paediatr Respir Rev. 15(1):13-6, 2014
                                  2. Abbas HA et al: A review of acute chest syndrome in pediatric sickle cell disease. Pediatr Ann. 42(3):115-20, 2013
                                  3. Desai PC et al: The acute chest syndrome of sickle cell disease. Expert Opin Pharmacother. 14(8):991-9, 2013
                                  4. Miller AC et al: Pulmonary complications of sickle cell disease. Am J Respir Crit Care Med. 185(11):1154-65, 2012
                                  5. Mekontso Dessap A et al: Pulmonary artery thrombosis during acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 184(9):1022-9, 2011
                                  6. Cohen RT et al: Smoking is associated with an increased risk of acute chest syndrome and pain among adults with sickle cell disease. Blood. 115(18):3852-4, 2010
                                  7. Laurie GA: Acute chest syndrome in sickle cell disease. Intern Med J. 40(5):372-6, 2010
                                  8. Sobota A et al: Corticosteroids for acute chest syndrome in children with sickle cell disease: variation in use and association with length of stay and readmission. Am J Hematol. 2010 Jan;85(1):24-8. Erratum in: Am J Hematol. 85(5):399, 2010
                                  9. Kokoska ER et al: Risk factors for acute chest syndrome in children with sickle cell disease undergoing abdominal surgery. J Pediatr Surg. 39(6):848-50, 2004
                                  10. Vichinsky EP et al: Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 342(25):1855-65, 2000
                                  11. Vichinsky EP et al: Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease. Blood. 89(5):1787-92, 1997
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Pathology

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Abbreviations

                                            • Acute chest syndrome (ACS)
                                            • Sickle cell disease (SCD)
                                          • Definitions

                                            • New pulmonary opacity on chest radiograph + ≥ 1 additional symptom (such as fever, cough, sputum production, tachypnea, dyspnea, or hypoxia) in setting of SCD

                                          IMAGING

                                          • General Features

                                            • Radiographic Findings

                                              • CT Findings

                                                • Nuclear Medicine Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        CLINICAL ISSUES

                                                        • Presentation

                                                          • Demographics

                                                            • Natural History & Prognosis

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Consider

                                                                  Selected References

                                                                  1. Knight-Madden J et al: Acute pulmonary complications of sickle cell disease. Paediatr Respir Rev. 15(1):13-6, 2014
                                                                  2. Abbas HA et al: A review of acute chest syndrome in pediatric sickle cell disease. Pediatr Ann. 42(3):115-20, 2013
                                                                  3. Desai PC et al: The acute chest syndrome of sickle cell disease. Expert Opin Pharmacother. 14(8):991-9, 2013
                                                                  4. Miller AC et al: Pulmonary complications of sickle cell disease. Am J Respir Crit Care Med. 185(11):1154-65, 2012
                                                                  5. Mekontso Dessap A et al: Pulmonary artery thrombosis during acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 184(9):1022-9, 2011
                                                                  6. Cohen RT et al: Smoking is associated with an increased risk of acute chest syndrome and pain among adults with sickle cell disease. Blood. 115(18):3852-4, 2010
                                                                  7. Laurie GA: Acute chest syndrome in sickle cell disease. Intern Med J. 40(5):372-6, 2010
                                                                  8. Sobota A et al: Corticosteroids for acute chest syndrome in children with sickle cell disease: variation in use and association with length of stay and readmission. Am J Hematol. 2010 Jan;85(1):24-8. Erratum in: Am J Hematol. 85(5):399, 2010
                                                                  9. Kokoska ER et al: Risk factors for acute chest syndrome in children with sickle cell disease undergoing abdominal surgery. J Pediatr Surg. 39(6):848-50, 2004
                                                                  10. Vichinsky EP et al: Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 342(25):1855-65, 2000
                                                                  11. Vichinsky EP et al: Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease. Blood. 89(5):1787-92, 1997