Diagnostic imaging in the evaluation of patients with Sickle Cell Anemia in Acute Thoracic Syndrome
Sickle cell anemia is a genetic disease first described in 1910 and is an anomaly caused by a mutation on chromosome 11 that results in the substitution of a glutamic acid for valine at position 6 of the N-terminal end of the globin chain, which gives rise to hemoglobin S, resulting in sickle-shaped red blood cells that do not circulate as they should in the microcirculation, resulting in obstruction of capillary blood flow. The pathophysiological mechanism of sickle cell disease leads to serious clinical manifestations, including Acute Thoracic Syndrome, which is responsible for 25% of deaths. This literature review aims to assess how imaging methods can help in the diagnosis of Acute Thoracic Syndrome in patients with Sickle Cell Anemia, and which would be the best imaging test for the etiological identification of Acute Thoracic Syndrome.
Diagnostic imaging in the evaluation of patients with Sickle Cell Anemia in Acute Thoracic Syndrome
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DOI: https://doi.org/10.22533/at.ed.1594892401108
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Palavras-chave: Sickle Cell Anemia, Imaging Diagnosis, Acute Thoracic Syndrome.
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Keywords: Sickle Cell Anemia, Imaging Diagnosis, Acute Thoracic Syndrome.
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Abstract:
Sickle cell anemia is a genetic disease first described in 1910 and is an anomaly caused by a mutation on chromosome 11 that results in the substitution of a glutamic acid for valine at position 6 of the N-terminal end of the globin chain, which gives rise to hemoglobin S, resulting in sickle-shaped red blood cells that do not circulate as they should in the microcirculation, resulting in obstruction of capillary blood flow. The pathophysiological mechanism of sickle cell disease leads to serious clinical manifestations, including Acute Thoracic Syndrome, which is responsible for 25% of deaths. This literature review aims to assess how imaging methods can help in the diagnosis of Acute Thoracic Syndrome in patients with Sickle Cell Anemia, and which would be the best imaging test for the etiological identification of Acute Thoracic Syndrome.
- Eduardo Hadad Cherulli
- Flávia Coimbra Campos
- Daniel Nava
- Bruna Almeida Silva
- Victor Henrique Ienny Guidini
- Felipe Andreto Mariano
- Henrique Lima Rufeisen
- Victor Garcia Oliveira
- Renato de Castro Rodrigues
- Rodrigo Bonfanti Machado de Campos
- Rayssa Rocha Neves
- Lívia da Costa Franco