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低血糖作为孤立性促肾上腺皮质激素缺乏患者的初始表现

HYPOGLYCEMIA AS INITIAL PRESENTATION IN PATIENT WITH ISOLATED ACTH DEFICIENCY.

作者信息

Alabdrabalnabi Fatimah Mohammed, Al Saeed Zahra Abdulwahed, Elamin Yasir Abuelgasim

出版信息

AACE Clin Case Rep. 2020 Sep 24;6(6):e338-e341. doi: 10.4158/ACCR-2020-0093. eCollection 2020 Nov-Dec.

Abstract

OBJECTIVE

Isolated adrenocorticotropic hormone (ACTH) deficiency is characterized by loss of adrenocorticotropic hormone, resulting in adrenal insufficiency, which can lead to life threatening severe hypoglycemia. We report a case of isolated ACTH deficiency with emphases on presentation, common etiologies, diagnosis, and management.

METHODS

The clinical course in addition to laboratory and imaging results are presented. These include cortisol level, ACTH, other pituitary hormones, insulin tolerance test, pituitary antibodies, and pituitary magnetic resonance imaging.

RESULTS

A 19-year-old male was brought by ambulance to the emergency department with loss of consciousness and a random blood sugar of 30 mg/dL. Our patient had a barely detectable level of plasma ACTH repeatedly and cortisol <1 μg/dL. There was no involvement of other pituitary hormones. Steroid replacement therapy led to resolution of all symptoms and prevented further episodes of hypoglycemia.

CONCLUSION

Isolated ACTH deficiency is a rare condition that is challenging to diagnose and can lead to serious life-threatening problems if unrecognized. Proper management can be achieved with appropriate hydrocortisone supplementation to mimic the normal secretion under both normal conditions and during a state of stress.

摘要

目的

孤立性促肾上腺皮质激素(ACTH)缺乏症的特征是促肾上腺皮质激素缺失,导致肾上腺功能不全,进而可能引发危及生命的严重低血糖。我们报告一例孤立性ACTH缺乏症病例,重点关注其临床表现、常见病因、诊断及治疗。

方法

呈现临床病程以及实验室和影像学检查结果。这些检查包括皮质醇水平、ACTH、其他垂体激素、胰岛素耐量试验、垂体抗体以及垂体磁共振成像。

结果

一名19岁男性被救护车送往急诊科,意识丧失,随机血糖为30mg/dL。我们的患者血浆ACTH水平多次几乎检测不到,皮质醇<1μg/dL。其他垂体激素未受影响。类固醇替代疗法使所有症状得到缓解,并预防了低血糖的进一步发作。

结论

孤立性ACTH缺乏症是一种罕见疾病,诊断具有挑战性,如果未被识别,可能导致严重的危及生命的问题。通过适当补充氢化可的松以模拟正常状态和应激状态下的正常分泌,可实现恰当的治疗。

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