Osteomyelitis in sickle cell haemoglobinopathy with elevated fetal haemoglobin

Abstract

The prevalence rate and characteristics of osteomyelitis in children below 15 years of age were studied in a population characterized by a high prevalence of sickle cell disease (SCD) with elevated fetal haemoglobin (HbF). All children born in our institution routinely undergo a haemoglobinopathy screening test. Osteomyelitis developed in 25 children (16 boys and nine girls) with a mean age of 5.5 years. Fourteen had sickle cell disease (SCD) and 11 had no haemoglobinopathy. The prevalence of osteomyelitis in children with SCD was 69 times higher than in those without (283 vs 4/10(5)). All 14 children with SCD and osteomyelitis also had elevated HbF. Three children had HbS alpha-thalassaemia, with mean HbF 24% and HbS 68.6%. Eleven children had sickle cell anaemia with elevated HbF, mean HbF 31.7% and HbS 66.7%. There was no significant difference in symptoms, signs, leucocyte, neutrophil and band counts, nor in sedimentation rates. Salmonella organisms were significantly more frequently responsible for osteomyelitis in SCD. The presence of elevated HbF in children with SCD should not lead to complacency as they are still at increased risk of developing osteomyelitis.

Osteomyelitis in Sickle Cell Anemia: Does Age Predict Risk of Salmonella Infection?

“…The remaining 258 abstracts were reviewed for inclusion and exclusion criteria, 30 articles were examined in full and 18 included in quantitative analysis. 12,[17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33] The 12 articles that were excluded after being examined in full did not clearly or failed to differentiate between the homozygous and heterozygous disease or were case reports.…”

“…There were no reported randomized control trials, metaanalyses or prospective studies found in the literature reporting on the rates of osteomyelitis in SCA. There were 14 retrospective studies 17,[19][20][21][22][23][24][25][27][28][29][30][31][32] and 4 case series studies 18,24,26,33 included in analysis. Two studies included analysis of heterozygote or SCT patients, however, distinguished clearly between homozygote and heterozygote patients.…”