Abstract 40: Adrenal insufficiency in patients with HbE/Beta thalassemia

Abstract

Background: Adrenal insufficiency (AI) in HbE/Beta thalassemia is not well documented.Aims and Objectives: Prevalence of AI in HbE/Beta thalassemia. Difference in prevalence of AI according to severity of HbE/Beta thalassemia and dependence on transfusion.Results: In this observational, cross sectional study, one hundred four patients with HbE/Beta thalassemia were evaluated. Amongst them 57 and 47 were transfusion dependent (TD) and non-transfusion dependent (NTD) thalassemia. According to Mahidol criteria, patients were classified into mild (n=39), moderate (n=39) and severe (n=26) disease. Early morning (8 AM) serum cortisol, plasma ACTH, DHEAS, androstenidione and plasma aldosterone, renin were measured. Patients with baseline cortisol of 5-18 μg/dl underwent both 1 μg and 250 μg SST (Short Synacthen Test). Overt adrenal insufficiency was found in 41% (n= 43). Amongst them 72% (n= 31) had primary AI and 27% (n= 12) had secondary AI. Thirty three patients (31%) with normal or elevated ACTH, with low or normal aldostreone with high renin were diagnosed as subclinical adrenal insufficiency. There was no significant difference in prevalence of AI between TD and NTD (p = 0.56) nor was there was any difference in prevalence of AI according to disease severity (p=0.52).Conclusion: Adrenal insufficiency is common in HbE/Beta thalassemia and is independent of transfusion dependency and disease severity.