Table of Contents

HK J Paediatr (New Series)
Vol 20. No. 3, 2015

HK J Paediatr (New Series) 2015;20:137-144

Original Article

Adrenal Insufficiency in Paediatric Transfusion Dependent Thalassaemia Major in Hong Kong: A Pilot Study

GSW Pang, CY Lee, ASC Ling, WK Leung, HC Yau


Abstract

Endocrinopathies including hypogonadism, diabetes mellitus and hypothyroidism have been well described in transfusion dependent thalassaemic patients. Adrenal insufficiency, on the other hand, was thought to be rare. Recent studies conducted in India, Thailand and Italy have confirmed that primary and secondary adrenal insufficiency occur too, as a result of iron overloading. In this prospective study, local transfusion dependent thalassaemic patients were recruited for adrenal function testing. Out of 28 patients, 10 (36%) were found to have adrenal insufficiency. Four of them have primary adrenal insufficiency and 6 have secondary (pituitary/hypothalamic) adrenal insufficiency. The prevalence of adrenal insufficiency is statistically related to age and years of blood transfusion, but does not correlate with the serum ferritin levels. These patients are advised to have hydrocortisone stress cover during illnesses and surgery to prevent crises.

Keyword : Adrenal insufficiency; Iron; Thalassaemia major


Abstract in Chinese

 
 

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