Abstract
Background: Sickle cell anemia is the most common genetic disease in sub-Saharan Africa. It is an inherited autosomal recessive disorder characterized by chronic hemolysis secondary to falciformation of red blood cells, also responsible of ischemia, bone infarction and accompanied by serious infections and organic lesions.Normal for weight at birth, Sickle cell anemia subjects have low pre puberty growth compared to normal children and also have compromised bone remodeling balance which results in decrease of bone mass and increase of bone fragility. Several studies have established that 37% to 50% of SCA patients were osteopenic or osteoporotic. This study aims to confirm the existence of bone remodeling disorders with osteoporotic translation and to compare the values found in Congolese sickle cell adults subjects to the general population.Methods: Spine and hip DXA were conducted on 270 SS homozygotes aged 18 to 50 years (121 men and 149 women) and 359 AA homozygotes as controls (138 men and 221 women), aged from 18 to 50 years old, who agreed to participate in the study, considered as a control group. AS heterozygotes were not included in the study.Results: AA subjects shows higher density (BMD) and Bone mineral content (BMC) values. Both SCA and AA controls showed the characteristic curve with peak bone mass at the fourth decade of life, followed by a decay with age. The difference in BMD and BMC with the control population ranged from 7.94% to 26.34% (average of 16.02%) which means -0.8 to -2.7 standard deviations, whereas, compared to the T -score in the Congolese population, was 11.6% to 22.15% less (average of -17.5%) equivalent of -0.9 to -2 standard deviations.The overall decrease in bone mass rate for -2.5 DS of the T-score was: -28.4% and 33.2% for -2 DS of T-score.Conclusion: SCA subjects shows high rate of osteopenia and osteoporosis and are more likely at risk for fractures.
Highlights
Sickle cell anemia (SCA), the most common genetic disease in sub-Saharan Africa, is an inherited autosomal recessive disorder (Rees et al, 2010)
Acute attacks as well as chronic complications of this disease are the result of falciformation of red blood cells and chronic hemolysis, which are responsible for ischemia, bone infarction and hyperplasia of the red marrow with a reduction in the trabecular component
Blood levels of vitamin D, serum calcium and cholesterol were significantly higher in SCA than in the control, (p = 0.032 to p < 0.001) whereas serum iron was significantly lower in SCA (p < 0.001))
Summary
Sickle cell anemia (SCA), the most common genetic disease in sub-Saharan Africa, is an inherited autosomal recessive disorder (Rees et al, 2010). Several studies have reported that 37 to 50% of SCA patients were osteopenic or osteoporotic which is associated with the foci of osteomyelitis and bone infarction and leads to an increased risk of fracture in SCA patients (Arlet et al, 2013; Bahebeck et al, 2002; Bennet et al, 1990; Meeuwes et al, 2013; Emodi et al, 2001; Demirbas et al, 2004; Finkelstien et al, 1996; Ganesh et al, 2001; Lal et al, 2006) The majority of these studies were conducted out of sub-Saharan Africa, and, despite a relatively different biological, genotypic and phenotypic profile, the Congolese SCA clinical profile reflects an equivalent impact of this hemoglobinopathy on the bone system, suggesting the presence of remodeling abnormalities in the setting of low bone mass (Kabeya et al, 2017; Kazadi et al, 2017; Mikobi et al, 2017; Mikobi et al, 2017; Tshilolo et al, 2012; Platt et al, 1984).
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
