Joradol N, et al. – This descriptive study was carried out to evaluate the prevalence of adrenal insufficiency (AI) in patients with non–transfusion–dependent (NTD) alpha ( α)–thalassemia. Results revealed similar rates of biochemical AI in α–thalassemia patients, as reported in the previous studies. Annual screening for AI in α–thalassemia patients was recommended, and glucocorticoid replacement should be considered in NTD α–thalassemia patients with AI during critical illness.

Methods

• This study included NTD α–thalassemic children at 3 referral hospitals in Thailand during the 2015 to 2016 study period.
• With 1 mcg ACTH stimulation test, preliminary screening for AI was performed.
• By insulin tolerance test (ITT), suspected AI was confirmed.
• AI was defined as peak cortisol <18 mcg/dL.
• AI was categorized as either primary or secondary AI using peak ACTH level.

Results

• This study enrolled 30 patients with NTD α–thalassemia.
• Ten of 25 (40%) patients had abnormal initial screening.
• Eight of 9 (88.9%) patients who underwent ITT were confirmed as having AI.
• No patients diagnosed with AI had any clinical symptoms of AI.
• Findings revealed that the percentage of primary and secondary AI (n=8) was 25% and 75%, respectively.
• Mean age and mean hemoglobin level showed a trend toward being associated with AI (p=0.98).