孤立性ACTH缺乏症3例并文献复习

目的以3例孤立性ACTH缺乏症(IAD)患者为例,探讨IAD的诊治原则。方法回顾分析3例IAD的临床资料,并进行文献复习。结果 3例IAD患者临床表现为进行性乏力、食欲不振及体质量减轻,可伴性欲减退,女性患者产后无阴毛、腋毛增长,2例伴TSH增高,1例伴临床甲状腺功能减退。应激时出现恶心、呕吐诱发低钠血症、低血压及低血糖倾向等肾上腺皮质功能低减危象,通过血ACTH、皮质醇水平测定及ACTH兴奋试验或胰岛素低血糖试验可明确诊断。结论对临床上以进行性乏力、食欲不振及体质量减轻,或以低钠血症或低血糖为表现的急症患者应考虑到IAD的可能,尽早给予糖皮质激素治疗。

Three cases report of isolated adrenocorticotropin deficiency

Objective To explore the principle of diagnosis and treatment of isolated adrenocorticotropin deficiency （IAD） by the example of 3 cases presenting with hyponatremia and hypoglycemia.Methods Clinical materials of 3 IAD cases were analyzed retrospectively and literature reviews were given.Results 3 cases of IAD patients had progressive fatigue,anorexia and unintentional weight loss,accompanied by loss of libido and no growth of pubic and auxiliary hair after delivery for a woman,two cases had mildly elevated TSH,and one case accompanied by clinical hypothyroidism.Some stressful events triggered an acute adrenal crisis presenting with vomiting and hypotension and tendency towards hypoglycemia.The morning plasma Cortisol value and ACTH level in association with ACTH stimulation test or insulin tolerance test is considered as the standard for diagnosis of IAD.Conclusion The patients presenting fatigue,anorexia and weight loss,vomiting and hypotension and tendency towards hypoglycemia in acute state may lead to the diagnosis of IAD,the main treatment of glucocorticoid should be given as soon as possible.