Gildasio Daltro1, Bruno Adelmo Franco1, Thiago Batista Faleiro1, Davi Araujo Veiga Rosário1, Paula Braga Daltro2, Vitor Fortuna3. 1. Prof. Edgar Santos Hospital Complex, Federal University of Bahia, R. Doutor Augusto Viana, s/n, Canela, Salvador, BA, 40110-060, Brazil. 2. Health Science Institute, Federal University of Bahia, Av. Reitor Miguel Calmon, s/n, Vale do Canela, Salvador, BA, 40110-100, Brazil. 3. Health Science Institute, Federal University of Bahia, Av. Reitor Miguel Calmon, s/n, Vale do Canela, Salvador, BA, 40110-100, Brazil. vfort@ufba.br.
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.
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 SCDpatients 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, SCDpatients should have radiological examination of their joints more often in order to prevent severe functional disability and increase patient's life quality.
Authors: Clinton Baldwin; Vikki G Nolan; Diego F Wyszynski; Qian-Li Ma; Paola Sebastiani; Stephen H Embury; Alice Bisbee; John Farrell; Lindsay Farrer; Martin H Steinberg Journal: Blood Date: 2005-03-22 Impact factor: 22.113
Authors: P F Milner; A P Kraus; J I Sebes; L A Sleeper; K A Dukes; S H Embury; R Bellevue; M Koshy; J W Moohr; J Smith Journal: N Engl J Med Date: 1991-11-21 Impact factor: 91.245
Authors: M Mukisi-Mukaza; A Elbaz; Y Samuel-Leborgne; L Kéclard; C Le Turdu-Chicot; E Christophe-Duchange; G Mérault Journal: Orthopedics Date: 2000-04 Impact factor: 1.390
Authors: Philippe Hernigou; Benjamin Thiebaut; Victor Housset; Claire Bastard; Yasuhiro Homma; Younes Chaib; Charles Henri Flouzat Lachaniette Journal: Int Orthop Date: 2018-04-26 Impact factor: 3.075