Chronic osteo-articular changes in patients with sickle cell disease

Autor: Ana Paula Teixeira dos Santos, Gilberto de Araújo Pereira, Taciana Fernandes Araújo Ferreira, Alexandra Silva Leal, Helio Moraes-Souza, Sheila Soares Silva
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Advances in Rheumatology, Volume: 61, Article number: 11, Published: 01 MAR 2021
Advances in Rheumatology, Vol 61, Iss 1, Pp 1-5 (2021)
Popis: Background Sickle cell disease (SCD) is an autosomal recessive genetic disease in which a mutation occurs in the β-globin chain gene, resulting in abnormal hemoglobin levels. In an environment with reduced oxygen concentration, red blood cells change their conformation, resulting in chronic hemolysis and consequent anemia and vaso-occlusive crises with injuries to several organs, with a significant impairment of the osteoarticular system. This study aimed to verify the chronic osteoarticular alterations and their association with clinical and laboratory characteristics of patients with SCD with a more severe phenotype (SS and Sβ0), on a steady-state fasis. Methods Fifty-five patients were referred to a medical consultation with a specialized assessment of the locomotor system, followed by laboratory tests and radiographic examinations. Results In total, 74.5% patients had hemoglobinopathy SS; 67.3% were female; and 78.2% were non-whites. The mean patient age was 30.5 years. Most patients (61.8%) reported up to three crises per year, with a predominance of high-intensity pain (65.5%). Radiographic alterations were present in 80% patients. A total of 140 lesions were identified, most which were located in the spine, femur, and shoulders. Most lesions were osteonecrosis and osteoarthritis and were statistically associated with the non-use of hydroxyurea. Conclusions There was a high prevalence of chronic osteoarticular alterations, which was statistically associated only with the non-regular use of hydroxyurea.
Databáze: OpenAIRE