Diagnostic evaluation and treatment of osteoarticular lesions in patients having sickle cell anemia: Literature review
DOI:
https://doi.org/10.33448/rsd-v13i4.45489Keywords:
Sickle cell anemia; Osteoarticular lesions; Imaging diagnostic methods; Complications; Treatment.Abstract
Sickle cell anemia is a genetic disorder in which the structure of hemoglobin undergoes a significant alteration through a point mutation in the β-globin gene. When the gene is altered in sickle cell disease, hemoglobin S is expressed, causing the red blood cell to acquire the characteristic sickle shape. Thus, in the presence of this mutation, especially with changes in oxygen concentration and pH, hemoglobin S tends to polymerize, resulting in sickling of the red blood cells, leading to a shortened lifespan of red blood cells, vascular occlusion phenomena, and episodes of pain and organ damage. Among the complications caused by sickle cell disease, stroke, recurrent infections, osteoarticular disease, among others, can be mentioned. One of the complications that generates the greatest morbidity is osteoarticular lesions, which can present with symptoms such as pain, swelling, warmth, fever, reduced vascularization, and even necrosis. Therefore, this study aimed to evaluate the diagnostic methods used in osteoarticular lesions in patients with sickle cell anemia. Among the most commonly used diagnostic methods in patients with sickle cell disease suspected of osteoarticular lesions, we can emphasize imaging diagnostic methods such as X-rays, magnetic resonance imaging (MRI), scintigraphy, and PET-CT, which can be used to differentiate between different types of lesions and suspected infectious processes, such as osteomyelitis. Therefore, imaging diagnostic methods are essential in identifying the lesion and the best treatment, with total hip arthroplasty being the most invasive method, although it is often the initial choice due to the severity of the lesion, or it is used after unpromising results from other less invasive procedures, such as femoral head decompression, grafting, and subchondroplasty.
References
Adams, R. J., McKie, V. C., Carl, E. M., Nichols, F. T., Perry, R., & Brock, K. (1998). Long-term stroke risk in children with sickle cell disease screened with transcranial Doppler. Annals of Neurology, 42(5), 699-704. https://doi.org/10.1002/ana.410420504
Alencar, S. S. D., Santos, D. E., et al. (2015). Most prevalent clinical complications in patients with sickle cell disease from a medium-sized town in Minas Gerais, Brazil. Revista Médica de Minas Gerais, 25(2), 162-169. GN1 Genesis Network. http://dx.doi.org/10.5935/2238-3182.20150032
Al-Salem, A. H., Qaisruddin, S., Nasserullah, Z., Al-Jam’a, A., & Bhamidimarri, K. R. (1992). Osteomyelitis in patients with sickle cell disease: early diagnosis with contrast-enhanced MRI. Journal of Pediatric Orthopaedics, 12(6), 786-789.
Anvisa (2002). Manual de Diagnóstico Laboratorial da Malária. Agência Nacional de Vigilância Sanitária. http://www.anvisa.gov.br/servicosaude/manuais/malaria.pdf
Ballas, S. K., Dover, G. J., Charache, S., & Usman, M. (1996). Rheologic predictors of the severity of the painful sickle cell crisis. Blood, 87(7), 1777-1780. https://doi.org/10.1182/blood.V87.7.1777.bloodjournal8771777
Bennett, I. L., Beaudoin, G., & Klainer, A. S. (1990). Ischemic necrosis of the femoral head in sickle cell anemia. Clinical Orthopaedics and Related Research, 261, 66-73. https://doi.org/10.1097/00003086-199009000-00009
Bishop, P. D., Skaggs, D. L., Kim, H. J., Laurent, B. F., Hunter, J. B., & Alman, B. A. (1998). Cytokine response to osteomyelitis in a murine model. The Journal of Pediatric Orthopaedics, 18(3), 348-352. https://doi.org/10.1097/00004694-199805000-00018
Brasil, Agência Nacional de Vigilância Sanitária. (2002). Manual de Diagnóstico e Tratamento de Doença Falciformes. Brasília: Anvisa.
Brasil. Ministério da Saúde. Secretaria de Atenção à Saúde. Departamento de Atenção Hospitalar e de Urgência. (2014). Doença falciforme: atenção e cuidado: a experiência brasileira: 2005-2010. Brasília: Ministério da Saúde.
Cissé, R., Wandaogo, A., Tapsoba, T. L., Chateil, J. F., Ouiminga, R. M., & Diard, F. (1999). Apport de l'imagerie médicale dans les manifestations ostéo-articulaires de la drépanocytose chez l'enfant [Contribution of medical imaging in osteoarticular manifestations of sickle cell anemia in the child]. Dakar Med, 44(1), 40-4.
Clarke, N. M. P., Woods, C. G., & Broughton, N. S. (1989). Scoliosis in children with sickle cell disease. Journal of Bone and Joint Surgery-British, 71(1), 63-66. https://doi.org/10.1302/0301-620X.71B1.2912782
Dong, R., McClain, K. L., Cole, K. B., & Nance, S. (1992). Sickle cell anemia with osteomyelitis and discitis: case report and review. Pediatric Infectious Disease Journal, 11(5), 413-416. https://doi.org/10.1097/00006454-199205000-00021
Farook, M. Z., Awogbade, M., Somasundaram, K., Reichert, I. L. H., & Li, P. L. S. (2019). Total hip arthroplasty in osteonecrosis secondary to sickle cell disease. International orthopaedics, 43(2), 293–298. https://doi.org/10.1007/s00264-018-4001-0
Ferreira, T. F. A., dos Santos, A. P. T., Leal, A. S., de Araújo Pereira, G., Silva, S. S., & Moraes-Souza, H. (2021). Chronic osteo-articular changes in patients with sickle cell disease. Advances in Rheumatology, 61(1), 11. https://doi.org/10.1186/s42358-021-00169-5
Gladvin, M. T., Vichinsky, E. P., Neumayr, L. D., Chait, P. G., Lowenstern, L. J., Koral, K., ... & Styles, L. A. (2004). Prevalence of priapism in children and adolescents with sickle cell anemia. Journal of Pediatric Hematology/Oncology, 26(8), 518-521. https://doi.org/10.1097/01.mph.0000134896.01355.28
Gravitz, S. L., & Pincock, A. (2014). Sickle cell disease in childhood: Part I. Nursing Clinics, 49(2), 255-264. https://doi.org/10.1016/j.cnur.2014.02.002
Hernigou, P., Habibi, A., Bachir, D., Galacteros, F., (2008). The natural history of asymptomatic osteonecrosis of the femoral head in adults with sickle cell disease. Journal of Bone and Joint Surgery, 90(2), 262-269. https://doi.org/10.2106/JBJS.F.01271
Jain, S. K., & Nagpal, S. (2008). Evaluation of clinical and laboratory parameters in patients of sickle cell disease and its correlation with the severity of the disease.
Johnny Rayes, Ivan Wong. (2021). Arthroscopic Approach to Preservation of the Hip with Avascular Necrosis. Arthroscopy Techniques, 10(10).
Kan, Y. M., & Dozy, A. M. (1978). Polymorphism of DNA sequence adjacent to human β-globin structural gene: relationship to sickle mutation. Proceedings of the National Academy of Sciences, 75, 5631-5635. https://doi.org/10.1073/pnas.75.11.5631
Kato, G. J., Hsieh, M., Machado, R., et al. (2006). Cerebrovascular disease associated with sickle cell pulmonary hypertension. American Journal of Hematology, 81, 503–510. https://doi.org/10.1002/ajh.20581
Khoury, R. A., Musallam, K. M., Mroueh, S., & Abboud, M. R. (2011). Pulmonary complications of sickle cell disease. Hemoglobin, 35(5-6), 625-635. https://doi.org/10.3109/03630269.2011.621149
Lacaille, F., Allali, S., & De Montalembert, M. (2021). The Liver in Sickle Cell Disease. Journal of Pediatric Gastroenterology and Nutrition, 72(1), 5-10. https://doi.org/10.1097/MPG.0000000000002886
Lardé, D., Galacteros, F., Benameur, C., Djédjé, A., & Ferrané, J. (1980). Manifestations radiologiques osseuses des syndromes drépanocytaires majeurs chez l'adulte [Radiological appearance of bone manifestations in acute drepanocytic syndromes in adults]. Journal de Radiologie, 61(6-7), 429-435.
Machado, A., et al. (2018). Anemia falciforme: aspectos clínicos e epidemiológicos. In: XXIII Seminário internacional de ensino, pesquisa e extensão, 23., 2018, Cruz Alta. Ciência e Diversidade. (pp. 1-11). Cruz Alta: Unicruz.
Meier, E. R. (2018). Treatment Options for Sickle Cell Disease. Pediatric Clinics of North America, 65(3), 427-443. https://doi.org/10.1016/j.pcl.2018.01.005
Moalla, M., Baklouti, S., Rais, H., Hamza, R., Hamza, M. H., Hachicha, A., Lakhoua, H., & Ayed, H. B. (1987). Manifestations ostéoarticulaires de la drépanocytose. Mise au point à propos d'une série de 29 cas [Osteoarticular manifestations of sickle cell anemia. Update apropos of a series of 29 cases]. Journal de Radiologie, 68(10), 609-614.
Mukisi-Mukaza, M., Manicom, O., Alexis, C., Bashoun, K., Donkerwolcke, M., & Burny, F. (2009). Treatment of sickle cell disease's hip necrosis by core decompression: a prospective case-control study. Orthopaedics & Traumatology: Surgery & Research, 95(7), 498-504. https://doi.org/10.1016/j.otsr.2009.07.009
Nuzzo, D. V. P. D., & Fonseca, S. F. (2004, May). Anemia falciforme e infecções. Jornal de Pediatria, 347-354.
Piel, F. B., Steinberg, M. H., & Rees, D. C. (2017, April 20). Sickle Cell Disease. New England Journal of Medicine, 376(16), 1561-1573. https://doi.org/10.1056/nejmra1510865.
Pinto, V. M., et al. (2019). Sickle cell disease: a review for the internist. Internal and Emergency Medicine, 14(7), 1051-1064. https://doi.org/10.1007/s11739-019-02160-x.
Rifai, A., & Nyman, R. (1997). Scintigraphy and ultrasonography in differentiating osteomyelitis from bone infarction in sickle cell disease. Acta Radiologica, 38(1), 139-143. https://doi.org/10.1080/02841859709171258.
Rother, E. T.. (2007). Revisão sistemática X revisão narrativa. Acta Paulista De Enfermagem, 20(2), v–vi. https://doi.org/10.1590/S0103-21002007000200001
Umans, H., Haramati, N., & Flusser, G. (2000). The diagnostic role of gadolinium-enhanced MRI in distinguishing between acute medullary bone infarct and osteomyelitis. Magnetic Resonance Imaging, 18(3), 255-262. https://doi.org/10.1016/s0730-725x(99)00137-x.
Vijay, M. R., Mitchell, D. G., Steiner, R. M., Rifkin, M. D., Burk, D. L., Levy, D., & Ballas, S. K. (1988). Femoral head avascular necrosis in sickle cell anemia: MR characteristics. Magnetic Resonance Imaging, 6(6).
Wang, Q., Li, D., Yang, Z., & Kang, P. (2020). Femoral Head and Neck Fenestration Through a Direct Anterior Approach Combined With Compacted Autograft for the Treatment of Early Stage Nontraumatic Osteonecrosis of the Femoral Head: A Retrospective Study. The Journal of Arthroplasty, 35(3).
Witjes, M. J., Berghuis-Bergsma, N., & Phan, T. T. (2006). Positron emission tomography scans for distinguishing between osteomyelitis and infarction in sickle cell disease. British Journal of Haematology, 133(2), 212-214. https://doi.org/10.1111/j.1365-2141.2006.06035.x.
Yanaguizawa, M., et al. (2008). Diagnóstico por imagem na avaliação da anemia falciforme. Revista Brasileira de Reumatologia, 48(2), 102-105. https://doi.org/10.1590/S0482-50042008000200007.
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