Plasma cortisol in sickle cell disease.

The plasma cortisol levels of 108 children aged 1-16 years with various Hb genotypes (AA, AS and SS) were measured. The mean plasma cortisol levels of both the AS and SS groups (during steady state) fell within the normal range but the mean of the SS group is significantly lower than the AA group. In fact, 9 of the sickle cell children had plasma cortisol levels below the normal range while no child in the HbAS and HbAA groups had plasma cortisol below the normal range. These results indicate that in sickle cell disease, there is low cortisol production while the secretion is increased during painful crisis.