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Published in: International Orthopaedics 7/2018

01-07-2018 | Original Paper

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

Authors: Gildasio Daltro, Bruno Adelmo Franco, Thiago Batista Faleiro, Davi Araujo Veiga Rosário, Paula Braga Daltro, Vitor Fortuna

Published in: International Orthopaedics | Issue 7/2018

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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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Metadata
Title
Osteonecrosis in sickle cell disease patients from Bahia, Brazil: a cross-sectional study
Authors
Gildasio Daltro
Bruno Adelmo Franco
Thiago Batista Faleiro
Davi Araujo Veiga Rosário
Paula Braga Daltro
Vitor Fortuna
Publication date
01-07-2018
Publisher
Springer Berlin Heidelberg
Published in
International Orthopaedics / Issue 7/2018
Print ISSN: 0341-2695
Electronic ISSN: 1432-5195
DOI
https://doi.org/10.1007/s00264-018-3905-z

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