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
香港需定期輸血的重型地中海貧血病人腎上腺功能不全的一項前導性研究

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.

在需定期輸血的重型地中海貧血病人中,內分泌疾病如性腺機能減退、糖尿病和甲狀腺功能減退。另一方面,腎上腺功能不全卻比較少見。印度、泰國和義大利最近進行的一項研究,有較多的資料指病童體內鐵質積聚,也會導致原發和繼發性過多腎上腺功能不全。在我們這項前瞻性研究中,招募當地需定期輸血的地中海貧血病人進行腎上腺功能檢測。在共28名病人中,發現其中10名病童(36%)出現腎上腺功能不全。當中4名為原發性腎上腺功能不全,另外6名為繼發性腎上腺功能不全(垂體/下丘腦)。出現腎上腺功能減退跟年齡和輸血時間有明顯關聯,但是同血清鐵蛋白水準卻沒有關係。建議病人在病發或在進行手術時服用氫化可的松,以避免腎上腺功能出現問題。

Keyword : Adrenal insufficiency; Iron; Thalassaemia major

關鍵詞:腎上腺功能不全、鐵、重型地中海貧血

 
 

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