Abstract
Rheological modifications observed in sickle cell anemia are associated with ischemic complications that can cause target organ functional impairment. The objective was to investigate adrenal function of adult patients with sickle cell disease. In this cross-sectional study conducted in a tertiary referral hospital of the capital city of Cameroon, we enrolled ten crisis-free adult patients with sickle cell disease (SCD) and ten age- and sex-matched healthy individuals. We assessed adrenal function by testing basal cortisol levels and 60 min after tetracosactide (Synacthen®) injection using immuno-chemiluminescence method. Post-stimulatory cortisol was defined as primary endpoint and secondary endpoints include basal cortisol levels, post-stimulatory cortisol increments and the fold increase of cortisol one hour after stimulation. Sickle cell patients had an impairment of adrenal function despite no significant difference between patients’ and controls’ for basal or post-stimulatory cortisol levels. In fact, one patient in two failed to achieve a two-fold increase in cortisol levels after stimulation (5/10) as opposed to 1 in 10 in the control population (1/10), P = 0.070. The percent increment of cortisol after stimulation was lower in patients versus controls (133 vs 207, P = 0.047). Relative adrenal insufficiency is frequent in sub-Saharan adult patients with sickle cell disease despite normal basal cortisol levels. Our results suggest that adrenal function require further investigation during SCD crises as these represent an important stress and may worsen the prognosis.
Highlights
Sickle cell disease (SCD) is an autosomal recessive disease that results from a point mutation on the beta-globin molecule leading to erythrocyte rigidity and increased episodes of vaso-occlusive crisis [1]
Our findings suggest that relative adrenal insufficiency is not a rare event in crisis-free adult patients with SCD
We believe that this study may pave the way to further investigations of adrenal function during vaso-occlusive crisis and to subsequent studies aiming at unraveling the interplay between iron-overload, acute low-grade inflammation, oxidative stress and adrenal insufficiency in SCD patients in the presence or not of vaso-occlusive events
Summary
Sickle cell disease (SCD) is an autosomal recessive disease that results from a point mutation on the beta-globin molecule leading to erythrocyte rigidity and increased episodes of vaso-occlusive crisis [1]. Under precipitating conditions, red blood cell sequestration and hemolysis give rise to the clinical presentation of the disease mainly marked by anemia, pain and multi-organ ischemic damage [2]. Recurrence of vaso-occlusive episodes has been associated to cerebro-vascular ischemic events, renal damage, pulmonary ischemia and alteration of some endocrine functions in SCD [3,4]. Beside red blood cell sequestration and the resulting ischemia, the pathophysiology entails iron overload which has been associated to oxidative stress and multi-organ failure [5,6].
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