Premium
Adrenal insufficiency in non‐transfusion‐dependent α‐thalassemia
Author(s) -
Joradol Nipapan,
Supornsilchai Vichit,
Tongmeesee Somlak,
Tanyong Benjamas,
Aphikulchatkit Yodkwan,
Sosothikul Darintr
Publication year - 2017
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/ped.13387
Subject(s) - medicine , thalassemia , pediatrics , adrenal insufficiency , insulin tolerance test , transfusion therapy , blood transfusion , insulin , insulin resistance , insulin sensitivity
Background Very few studies about adrenal insufficiency ( AI ) have been published with regard to non‐transfusion‐dependent ( NTD ) thalassemia, and none of those studies involved α‐thalassemia patients. The aim of this study was therefore to determine the prevalence of AI in patients with NTD α‐thalassemia, and to identify factors that predict the development of AI in this thalassemia subpopulation. Methods This cross‐sectional descriptive study was conducted in NTD α‐thalassemic children at three referral hospitals in Thailand in 2015–2016. Preliminary screening for AI was done using the 1 μg adrenocorticotropic hormone ( ACTH ) stimulation test. Suspected AI was then confirmed on insulin tolerance test ( ITT ). AI was defined as peak cortisol <18 μg/dL. AI was categorized as either primary or secondary AI according to peak ACTH . Results Thirty patients with NTD α‐thalassemia were included in this study. Ten of 25 patients (40%) had abnormal initial screening. Eight of nine (88.9%) who underwent ITT were confirmed as having AI . No patients diagnosed with AI had any clinical symptoms of AI . 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). Conclusion The prevalence of biochemical AI in α‐thalassemia patients was similar to rates previously reported for NTD β‐thalassemia. Annual screening for AI in α‐thalassemia patients is recommended, and glucocorticoid replacement should be considered in NTD α‐thalassemia patients with AI during critical illness.