Study on Primary Adrenal Insufficiency (PAI): Experience in a Major Teaching Hospital, Riyadh, Saudi Arabia

N. Al-Jurayyan
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引用次数: 2

Abstract

Background: Primary adrenal insufficiency (PAI) in children is an uncommon, but potentially fatal. The current symptoms include weakness, fatigue, anorexia, abdominal pain, weight loss, orthostatic hypotension, salt craving and characterized by hyperpigmentation. Aims: The objective of this study was therefore, to define the etiology of PAI in a large referral, teaching hospital in Riyadh, Saudi Arabia. Materials and Methods: This is a retrospective, hospital based-study, conducted at King Khalid University Hospital (KKUH), during the period January 1989 and December 2014. Review of medical record of patient diagnosed with pri- mary adrenal insufficiency. The diagnosis was based on medical history, physical examination and low levels of glucocor- ticoids and raised adrenocorticotropic hormone (ACTH). Appropriate laboratory and radiological investigations were also reviewed. Results: During the period under review, January 1989 and December 2014, a total of 125 patients with the diagnosis of primary adrenal insufficiency were seen. Inherited disorders like congenital adrenal hyperplasia and hypoplasia were com- mon, 85.5%. However, variable autoimmune mediated etiologic diagnosis accounted for, 13%, were also seen. The appro- priate various laboratory and radiological investigations should be planned. Conclusion: Although, congenital adrenal hyperplasia was the commonest etiology, however, congenital adrenal hypoplasia should not be over looked. The diagnosis of PAI can be challenging in some patients, and therefore appropriate serological.
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原发性肾上腺功能不全(PAI)的研究:沙特阿拉伯利雅得一家大型教学医院的经验
背景:原发性肾上腺功能不全(PAI)在儿童中并不常见,但可能致命。目前的症状包括虚弱、疲劳、厌食、腹痛、体重减轻、体位性低血压、嗜盐,并以色素沉着为特征。目的:因此,本研究的目的是确定沙特阿拉伯利雅得一家大型转诊教学医院PAI的病因。材料和方法:这是一项回顾性的、基于医院的研究,于1989年1月至2014年12月在哈立德国王大学医院(KKUH)进行。原发性肾上腺功能不全患者病历回顾。诊断是基于病史,体格检查和低水平的糖皮质激素和升高的促肾上腺皮质激素(ACTH)。还审查了适当的实验室和放射学调查。结果:1989年1月至2014年12月,共收治125例原发性肾上腺功能不全患者。遗传性疾病如先天性肾上腺增生和发育不全最为常见,占85.5%。然而,可变自身免疫介导的病因诊断也占13%。应计划适当的各种实验室和放射检查。结论:虽然先天性肾上腺皮质增生是最常见的病因,但先天性肾上腺皮质增生不应被忽视。PAI的诊断在一些患者中可能具有挑战性,因此需要适当的血清学检查。
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