Osuwannaratana Prapanrat, Nimkarn Saroj, Santiprabhob Jeerunda, Likitmaskul Supawadee, Sawathiparnich Pairunyar
Division of Pediatric Endocrinology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
J Med Assoc Thai. 2008 Oct;91(10):1544-50.
Adrenal insufficiency (AI) is an event caused by an inadequate secretion or action of adrenal hormones. It can be classified as primary (1 degree) and secondary (2 degree). AI may result in severe morbidity and mortality when undiagnosed or ineffectively treated.
To determine the etiologies of AI in Thai children.
Data of children with AI presented to the authors' pediatric endocrine service between 1982 and 2002 (20 years) were retrospectively collected and analyzed.
AI was diagnosed by clinical and laboratory data in 73 children (31 boys and 42 girls). Sixty-two (84.9%) patients had 1degree AI while 11 (15.1%) had 2 degree AI. The majority of patients with 1 degree AI (87.1%) were diagnosed with congenital adrenal hyperplasia (CAH). Other causes of 1 degree AI were uncommon such as ACTH unresponsiveness (4.8%) and no definite diagnosis (8.1%). Most children with 1 degree AI presented with hyperpigmentation. Causes of 2 degree AI were as follows: panhypopituitarism (63.6%), isolated ACTH deficiency (9.1%), and low birth weight (27.3%).
In the present study, CAH was the most common cause of 1 degree AI while panhypopituitarism was the most common cause of 2 degree AI. Other causes of AI were quite uncommon. Definite causes of AI have not yet been identified in some children. Further clinical observation and special tests including molecular studies in these children are warranted for diagnostic and prognostic importance.
肾上腺功能不全（AI）是一种由肾上腺激素分泌不足或作用异常引起的病症。它可分为原发性（1级）和继发性（2级）。若未被诊断或治疗不当，AI可能导致严重的发病率和死亡率。
确定泰国儿童AI的病因。
回顾性收集并分析了1982年至2002年（20年）间到作者所在儿科内分泌科就诊的AI患儿的数据。
通过临床和实验室数据诊断出73例AI患儿（31例男孩和42例女孩）。62例（84.9%）患者为1级AI，11例（15.1%）为2级AI。大多数1级AI患者（87.1%）被诊断为先天性肾上腺增生（CAH）。1级AI的其他病因并不常见，如促肾上腺皮质激素（ACTH）无反应性（4.8%）和未明确诊断（8.1%）。大多数1级AI患儿表现为色素沉着。2级AI的病因如下：全垂体功能减退（63.6%）、孤立性ACTH缺乏（9.1%）和低出生体重（27.3%）。
在本研究中，CAH是1级AI最常见的病因，而全垂体功能减退是2级AI最常见的病因。AI的其他病因相当少见。部分患儿尚未明确AI的确切病因。对这些患儿进行进一步的临床观察以及包括分子研究在内的特殊检查，对于诊断和预后具有重要意义。
