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Osteonecrosis in sickle cell disease patients from Bahia, Brazil: a cross-sectional study

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Abstract

Purpose

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.

Methods

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.

Results

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.

Conclusions

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.

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Abbreviations

HbS:

haemoglobin S

MRI:

magnetic resonance imaging

ON:

osteonecrosis

SCD:

sickle cell disease

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Funding

This work was supported by the Brazilian National Research Council (CNPq) and the Research Support Foundation of the State of Bahia (FAPESB).

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Authors and Affiliations

  1. Prof. Edgar Santos Hospital Complex, Federal University of Bahia, R. Doutor Augusto Viana, s/n, Canela, Salvador, BA, 40110-060, Brazil

    Gildasio Daltro, Bruno Adelmo Franco, Thiago Batista Faleiro & Davi Araujo Veiga Rosário

  2. Health Science Institute, Federal University of Bahia, Av. Reitor Miguel Calmon, s/n, Vale do Canela, Salvador, BA, 40110-100, Brazil

    Paula Braga Daltro & Vitor Fortuna

Authors
  1. Gildasio Daltro
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  2. Bruno Adelmo Franco
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  3. Thiago Batista Faleiro
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  4. Davi Araujo Veiga Rosário
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  5. Paula Braga Daltro
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  6. Vitor Fortuna
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Contributions

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.

Corresponding author

Correspondence to Vitor Fortuna.

Ethics declarations

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.

Competing interests

The authors declare that they have no competing interests.

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Daltro, G., Franco, B.A., Faleiro, T.B. et al. Osteonecrosis in sickle cell disease patients from Bahia, Brazil: a cross-sectional study. International Orthopaedics (SICOT) 42, 1527–1534 (2018). https://doi.org/10.1007/s00264-018-3905-z

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  • DOI: https://doi.org/10.1007/s00264-018-3905-z

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