Sickle Cell Disease in Children

Abstract

To identify clinical and laboratory features predictive of osteomyelitis in children with sickle cell disease and bony pain. Patients in the case group and participants in the control group were randomized in a 1:3 ratio. The Hospital for Sick Children, Toronto, Ontario, Canada. Patients with sickle cell disease and osteomyelitis (case patients) and patients with sickle cell disease and bony, vaso-occlusive crisis (control patients), 18 years or younger. Five characteristics (number of painful sites, white blood cell count, swelling of the affected limb[s], and duration of pain and fever before presentation) at the time of presentation to hospital. Data were analyzed for 31 cases and 93 controls. Compared with controls, cases had more days of pain (5 vs 2 days; odds ratio [OR], 1.2; 95% confidence interval [CI], 1.1-1.4 days) and fever (1 vs 0 day; 1.7; 1.2-2.4 days) before presentation. Cases were also more likely to have swelling of the affected limb(s) (71% vs 17%; OR, 11.8; 95% CI, 4.6%-30.0%) and fewer painful sites (1 vs 2; 0.7; 0.5-1.0). On laboratory evaluation, cases had higher white blood cell counts (18.6 vs 15.6/microL; OR, 1.1; 95% CI, 1.0-1.1/microL). Multivariate logistic regression showed that the significant predictors of osteomyelitis were duration of fever (OR, 1.8; 95% CI, 1.2-2.6) and pain (1.2; 1.0-1.4) before presentation and swelling of the affected limb (8.4; 3.5-20.0). The risk of osteomyelitis was decreased if more than 1 painful site was present (OR, 0.7; 95% CI, 0.5-1.0). In the clinical scenario of a child with sickle cell disease presenting with bony pain and swelling affecting a single site, with prolonged fever and pain, the physician should consider closer monitoring and investigations to exclude a diagnosis of osteomyelitis.