Osteonecrosis in sickle cell disease patients from Bahia, Brazil: a cross-sectional study
- 37 Downloads
The aim of this study was to describe the clinical features of osteonecrosis (ON) in sickle cell disease (SCD) patients in Bahia, a Northeast state with the highest prevalence of the disease in Brazil.
Between 2006 and 2017, 283 cases of osteonecrosis in SCD patients were enrolled to analyse the age at diagnosis, genotype, gender, pain, distribution of the lesions and disease staging. MRI and radiograph were obtained at the participation.
Of the 283 SCD cases, 120 (42.4%) were haemoglobin SS genotype while 163 (57.6%) were SC genotype. Two hundred and forty-six cases were bilateral and 37 were unilateral, with an average age at diagnosis of 33.7 (range 10–67) years. The most frequent identified ON site not only was the hip (74.6%), but also affected shoulder, knee and ankle. Most cases presented at early stage I (172, 60.8%) disease. No significant differences on the features of osteonecrosis were identified between haemoglobin SS and haemoglobin SC cases.
Given the relatively high prevalence of bilateral osteonecrosis at early stages, painful symptoms and rather late age at diagnosis, SCD patients should have radiological examination of their joints more often in order to prevent severe functional disability and increase patient’s life quality.
KeywordsBone necrosis Sickle cell disease Femoral head Joint MRI diagnosis
magnetic resonance imaging
sickle cell disease
GD, TBF and DAR were the surgeons who performed surgeries in this study. TBF, BAM and DAR assessed most of the clinical outcomes. GD and VF were responsible for conception, design, critical revision and analysis of the results. PBD assisted data collection. BAF drafted part of the manuscript. VF coordinated and made a major contribution to writing the manuscript. All authors read and approved the final manuscript.
Compliance with ethical standards
The Committee of Ethics in Research of the Climério de Oliveira Maternity Hospital (Federal University of Bahia, UFBA) approved this study, and informed consent was obtained in accordance with ethical principles and the Helsinki Declaration of 1975, and by the Brazilian resolution 196/96, of the Ministry of Health, Law 6638/79 and Normative Resolution 04/97.
The authors declare that they have no competing interests.
- 3.Brasil, Ministério da Saúde, Secretaria de Atenção à Saúde (2012) Doença Falciforme: condutas básicas para o tratamento. Brasília: Ministério da Saúde. http://bvsms.saude.gov.br/bvs/publicacoes/doenca_falciforme_condutas_basicas.pdf. Accessed 28 February 2018
- 13.Mont MA, Tomek IM, Hungerford DS (1997) Core decompression for avascular necrosis of the distal femur: long term follow-up. Clin Orthop Relat Res 334:124-130Google Scholar
- 21.Aleluia MM, Fonseca TCC, Souza RQ, Neves FI, da Guarda CC et al (2017) Comparative study of sickle cell anemia and hemoglobin SC disease: clinical characterization, laboratory biomarkers and genetic profiles. BMC Hematol 17:15. https://doi.org/10.1186/s12878-017-0087-7 CrossRefPubMedPubMedCentralGoogle Scholar
- 22.Naessens V, Ward R, Kuo KHM (2017) A proposed treatment algorithm for adults with haemoglobin SC disease. Br J Haematol. https://doi.org/10.1111/bjh.14852
- 26.Hauzeur JP, Malaise M, de Maertelaer V (2016) A prospective cohort study of the clinical presentation of non-traumatic osteonecrosis of the femoral head: spine and knee symptoms as clinical presentation of hip osteonecrosis. Int Orthop 40(7):1347–1351. https://doi.org/10.1007/s00264-015-3079-x CrossRefPubMedGoogle Scholar