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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. Dako F et al: Dual-energy CT evidence of pulmonary microvascular occlusion in patients with sickle cell disease experiencing acute chest syndrome. Clin Imaging. 78:94-7, 2021
                                  2. Dolatkhah R et al: Blood transfusions for treating acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev. 1:CD007843, 2020
                                  3. El-Gohary Y et al: Acute chest syndrome after splenectomy in children with sickle cell disease. J Surg Res. 242:336-41, 2019
                                  4. Monus T et al: Current and emerging treatments for sickle cell disease. JAAPA. 32(9):1-5, 2019
                                  5. Rincón-López EM et al: Low-risk factors for severe bacterial infection and acute chest syndrome in children with sickle cell disease. Pediatr Blood Cancer. 66(6):e27667, 2019
                                  6. Bou-Maroun LM et al: An analysis of inpatient pediatric sickle cell disease: Incidence, costs, and outcomes. Pediatr Blood Cancer. 65(1), 2018
                                  7. Takahashi T et al: Acute chest syndrome among children hospitalized with vaso-occlusive crisis: a nationwide study in the United States. Pediatr Blood Cancer. 65(3), 2018
                                  8. Takahashi T et al: Factors associated with mechanical ventilation use in children with sickle cell disease and acute chest syndrome. Pediatr Crit Care Med. 19(9):801-9, 2018
                                  9. Subramaniam S et al: Managing acute complications of sickle cell disease in pediatric patients [digest]. Pediatr Emerg Med Pract. 13(11 Suppl Points & Pearls):S1-S2, 2016
                                  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. Dako F et al: Dual-energy CT evidence of pulmonary microvascular occlusion in patients with sickle cell disease experiencing acute chest syndrome. Clin Imaging. 78:94-7, 2021
                                                                  2. Dolatkhah R et al: Blood transfusions for treating acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev. 1:CD007843, 2020
                                                                  3. El-Gohary Y et al: Acute chest syndrome after splenectomy in children with sickle cell disease. J Surg Res. 242:336-41, 2019
                                                                  4. Monus T et al: Current and emerging treatments for sickle cell disease. JAAPA. 32(9):1-5, 2019
                                                                  5. Rincón-López EM et al: Low-risk factors for severe bacterial infection and acute chest syndrome in children with sickle cell disease. Pediatr Blood Cancer. 66(6):e27667, 2019
                                                                  6. Bou-Maroun LM et al: An analysis of inpatient pediatric sickle cell disease: Incidence, costs, and outcomes. Pediatr Blood Cancer. 65(1), 2018
                                                                  7. Takahashi T et al: Acute chest syndrome among children hospitalized with vaso-occlusive crisis: a nationwide study in the United States. Pediatr Blood Cancer. 65(3), 2018
                                                                  8. Takahashi T et al: Factors associated with mechanical ventilation use in children with sickle cell disease and acute chest syndrome. Pediatr Crit Care Med. 19(9):801-9, 2018
                                                                  9. Subramaniam S et al: Managing acute complications of sickle cell disease in pediatric patients [digest]. Pediatr Emerg Med Pract. 13(11 Suppl Points & Pearls):S1-S2, 2016