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      Changes seen on computed tomography of the chest in mildly symptomatic adult patients with sickle cell disease* Translated title: Alterações na tomografia computadorizada do tórax em pacientes adultos oligossintomáticos com doença falciforme

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          Abstract

          Objective

          To describe and quantify the main changes seen on computed tomography of the chest in mildly symptomatic adult patients with sickle cell disease, as well as to evaluate the radiologist accuracy in determining the type of hemoglobinopathy.

          Materials and Methods

          A prospective study involving 44 adult patients with sickle cell disease who underwent inspiration and expiration computed tomography of the chest. The frequency of tomography findings and the extent of involvement are reported. We also calculated radiologist accuracy in determining the type of hemoglobinopathy by analyzing the pulmonary alterations and morphology of the spleen.

          Results

          The changes found on computed tomography scans, in descending order of frequency, were as follows: fibrotic opacities (81.8%); mosaic attenuation (56.8%); architectural distortion (31.8%); cardiomegaly (25.0%); lobar volume reduction (18.2%); and increased caliber of peripheral pulmonary arteries (9.1%). For most of the findings, the involvement was considered mild, five or fewer lung segments being affected. The accuracy in determining the type of hemoglobinopathy (HbSS group versus not HbSS group) was 72.7%.

          Conclusion

          In adult patients with sickle cell disease, the main tomography findings reflect fibrotic changes. In addition, computed tomography can be helpful in differentiating among hemoglobinopathies.

          Translated abstract

          Objetivo

          Descrever e quantificar as principais alterações na tomografia computadorizada do tórax em pacientes adultos oligossintomáticos com doença falciforme e, secundariamente, avaliar o índice de acerto do radiologista quanto ao tipo de hemoglobinopatia.

          Materiais e Métodos

          Estudo prospectivo em que 44 pacientes adultos com doença falciforme foram submetidos a tomografia computadorizada do tórax tanto em inspiração como em expiração. Foram descritos a frequência dos achados tomográficos e os graus de acometimento. Por meio da análise das alterações pulmonares e do padrão morfológico do baço, foi calculado o índice de acerto do radiologista quanto ao tipo de hemoglobinopatia.

          Resultados

          As alterações encontradas nos exames de tomografia computadorizada, em ordem decrescente de frequência, foram: opacidades reticulares (81,8%), padrão de atenuação em mosaico (56,8%), distorção arquitetural (31,8%), cardiomegalia (25%), redução volumétrica lobar (18,2%) e aumento do calibre de ramos periféricos das artérias pulmonares (9,1%). Na maioria dos achados o grau de acometimento foi considerado leve, com até cinco segmentos pulmonares acometidos. O índice de acerto quanto ao tipo de hemoglobinopatia (grupo HbSS versus grupo não HbSS) foi 72,7%.

          Conclusão

          Em pacientes adultos com doença falciforme os principais achados tomográficos refletem alterações fibróticas. Além do mais, a tomografia computadorizada pode ser útil na diferenciação do tipo de hemoglobinopatia.

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          Most cited references68

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          Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes.

          Hemolysis, long discounted as a critical measure of sickle cell disease severity when compared with sickle vaso-occlusion, may be the proximate cause of some disease complications. New mechanistic information about hemolysis and its effects on nitric oxide (NO) biology and further examination of the subphenotypes of disease requires a reappraisal and deconstruction of the clinical features of sickle cell disease. The biology underlying clinical phenotypes linked to hemolysis may increase our understanding of the pathogenesis of other chronic hemolytic diseases while providing new insights into treating sickle cell disease. The pathophysiological roles of dysregulated NO homeostasis and sickle reticulocyte adherence have linked hemolysis and hemolytic rate to sickle vasculopathy. Nitric oxide binds soluble guanylate cyclase which converts GTP to cGMP, relaxing vascular smooth muscle and causing vasodilatation. When plasma hemoglobin liberated from intravascularly hemolyzed sickle erythrocytes consumes NO, the normal balance of vasoconstriction:vasodilation is skewed toward vasoconstriction. Pulmonary hypertension, priapism, leg ulceration and stroke, all subphenotypes of sickle cell disease, can be linked to the intensity of hemolysis. Hemolysis plays less of a role in the vaso-occlusive-viscosity complications of disease like the acute painful episode, osteonecrosis of bone and the acute chest syndrome. Agents that decrease hemolysis or restore NO bioavailability or responsiveness may have potential to reduce the incidence and severity of the hemolytic subphenotypes of sickle cell disease. Some of these drugs are now being studied in clinical trials.
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            Manual de diagnóstico e tratamento de doenças falciformes

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              Sickle cell anemia.

              Sickle cell anemia (SCA) is a disease caused by production of abnormal hemoglobin, which binds with other abnormal hemoglobin molecules within the red blood cell to cause rigid deformation of the cell. This deformation impairs the ability of the cell to pass through small vascular channels; sludging and congestion of vascular beds may result, followed by tissue ischemia and infarction. Infarction is common throughout the body in the patient with SCA, and it is responsible for the earliest clinical manifestation, the acute pain crisis, which is thought to result from marrow infarction. Over time, such insults result in medullary bone infarcts and epiphyseal osteonecrosis. In the brain, white matter and gray matter infarcts are seen, causing cognitive impairment and functional neurologic deficits. The lungs are also commonly affected, with infarcts, emboli (from marrow infarcts and fat necrosis), and a markedly increased propensity for pneumonia. The liver, spleen, and kidney may experience infarction as well. An unusual but life-threatening complication of SCA is sequestration syndrome, wherein a considerable amount of the intravascular volume is sequestered in an organ (usually the spleen), causing vascular collapse; its pathogenesis is unknown. Finally, because the red blood cells are abnormal, they are removed from the circulation, resulting in a hemolytic anemia. For the patient with SCA, however, the ischemic complications of the disease far outweigh the anemia in clinical importance.
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                Author and article information

                Journal
                Radiol Bras
                Radiol Bras
                rb
                Radiologia Brasileira
                Colégio Brasileiro de Radiologia e Diagnóstico por Imagem
                0100-3984
                1678-7099
                Jul-Aug 2016
                Jul-Aug 2016
                : 49
                : 4
                : 214-219
                Affiliations
                [1 ]MD, Radiologist at the Hospital Universitário Pedro Ernesto (HUPE), Student in the Graduate Program in Medical Sciences at the Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil.
                [2 ]PhD, Adjunct Professor of Pulmonology at the Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil.
                [3 ]PhD, Adjunct Professor of Hematology at the Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil.
                [4 ]PhD, Associate Professor, Head of the Biomedical Instrumentation Laboratory, Head of the Laboratory for Clinical and Experimental Research in Vascular Biology, Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ, Brazil.
                [5 ]PhD, Adjunct Professor of Radiology at the Universidade do Estado do Rio de Janeiro (UERJ), Head of the Radiology Department of the Hospital Universitário Pedro Ernesto (HUPE), Rio de Janeiro, RJ, Brazil.
                Author notes
                Mailing address: Dr. Agnaldo José Lopes. Universidade do Estado do Rio de Janeiro. Rua Araguaia, 1266, Bloco 1/405, Freguesia, Jacarepaguá. Rio de Janeiro, RJ, Brazil, 22745-271. E-mail: agnaldolopes.uerj@ 123456gmail.com .
                Article
                10.1590/0100-3984.2015.0111
                5073386
                27777473
                01337bd6-8081-4177-833b-0f91831bc287
                © Colégio Brasileiro de Radiologia e Diagnóstico por Imagem

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 05 June 2015
                : 24 July 2015
                Funding
                Funded by: Faperj
                Award ID: E-26/110.255/2014
                Categories
                Original Articles

                anemia, sickle cell,lung diseases/etiology,tomography, x-ray computed

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