Endothelial dysfunction in sickle cell anaemia tied to acute chest syndrome in children
Paediatric sickle cell anaemia (SCA) patients suffer from impaired endothelial function, a new study has found. Moreover, those with lower flow-mediated vasodilation (FMD) values have acute chest syndrome (ACS).
In a study sample of 40 paediatric SCA patients (mean age 12.3±3 years; 60 percent male), average FMD values were significantly lower than in the healthy controls (n=25; mean age 11.4±3 years; 28 percent male). Eighteen participants were on hydroxyurea treatment upon enrolment.
According to receiver operator characteristics (ROC) curve analysis, an FMD value of below 11.5 percent could distinguish SCA patients from controls with a sensitivity of 70 percent and a specificity of 80 percent. This suggests that endothelial dysfunction is a marker of SCA.
Patients with a history of ACS within the past year had significantly lower FMD values compared to those with no prior history. ROC curve analysis also showed that FMD could distinguish between those with and without prior ACS histories with an accuracy of 71 percent and a specificity of up to 79 percent. FMD also showed a significantly negative correlation with ACS (p=0.03).
The chances of having a prior ACS history were also significantly higher in paediatric SCA patients with FMD values lower than 6.65 percent (odds ratio, 1.45; 95 percent CI, 1.02 to 3.45; p=0.023).
Paediatric participants aged 6 to 18 years with haemoglobinopathy were recruited and subjected to Doppler ultrasonography to evaluate their endothelial function. Exclusion criteria included acute infection, obesity, systemic inflammation or dyslipidaemia.