Abstract
BackgroundSickle cell anemia (SCA) is a common genetic disease in Nigeria. Past studies from West Africa focused on isolated aspects of its medical and surgical presentations. To the best of our knowledge, the musculo-skeletal presentations amongst Nigerians with SCA have not been documented in a single all encompassing study. This work aims to prospectively document the musculo-skeletal disease burden among SCA patients.MethodsIn a prospective study of 318 consecutive patients with genotype-confirmed SCA at the Lagos University Teaching Hospital (LUTH), the musculo-skeletal pathologies, anatomic sites, grade of disease, age at presentation and management outcome were recorded over a one-year period. Data obtained were analyzed using Epi-Info software version 6.0. Data are presented as frequencies (%) and mean values (SD) as appropriate.ResultsThe HbSS genotype occurred in 296 (93.0%), while 22 (7.0%) were HbSC. 100 (31.4%) patients with average presenting haemoglobin concentration of 8.2 g/100 ml in the study group, presented with 131 musculo-skeletal pathologies in 118 anatomic sites. Osteomyelitis 31 (31%) and septic arthritis 19 (19%) were most commonly observed in children less than 10 years. Skin ulcers and avascular necrosis (AVN) occurred predominantly in the older age groups, with frequencies of 13 (13.0%) and 26 (26.0%) respectively. 20 (71.5%) of diagnosed cases of AVN presented with radiological grade 4 disease. The lower limbs were involved in 84 (71.1%) of sites affected. Lesions involving the spine were rare 11 (0.9%). Multiple presentations occurred in 89 (28.0%) of patients; 62 (69.7%) of which were children below 10 years.ConclusionsMusculo-skeletal complications are common features of sickle cell anaemia seen in 31.4%. Infectious aetiologies predominate with long bones and joints of lower limbs more commonly affected by osteomyelitis and septic arthritis. Healthcare providers managing SCA should be aware of the potential morbidity and mortality of these conditions to ensure early diagnosis and adequate management.
Highlights
Sickle cell anemia (SCA) is a common genetic disease in Nigeria
Pauling et al [3] discovered the presence of abnormal haemoglobin in patients with sickle cell disease in 1949
Osteomyelitis accounted for 49 (37.4%), followed by avascular necrosis (AVN) 28 (21.4%) and septic arthritis 20 (15.3%). 25 (51.0%) of osteomyelitis were caused by staphylococcus species, 16 (32.7%) by salmonella, 4 (8.2%) by haemophilus and 3 (6.1%) by streptococcus
Summary
Sickle cell anemia (SCA) is a common genetic disease in Nigeria. Past studies from West Africa focused on isolated aspects of its medical and surgical presentations. Sickle cell disease (SCD) is a group of inherited haemoglobinopathies occurring mainly in Negroid populations in and out of Africa, characterized by a predominance of haemoglobin S (HbS) in the erythrocytes [1]. It was first recognized by James B. SCD is the most frequent haemoglobinopathy in the world [4,5] and Sickle cell anaemia (SCA) occurs when there is homozygote HbSS or composite heterozygote HbSC [1]. Most studies of musculo-skeletal presentations of SCA in Nigeria have focused on selected disease conditions [8,10,16,17,18,19]
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