一中年妇女孤立ACTH缺乏与短暂性生长激素缺乏相关。

José Hernán Martínez, Michelle M Mangual Garcia, Madeleine Gutiérrez Acevedo, Alfredo Sánchez Cruz, Ivan Laboy, Carmen Rivera, Paola Mansilla, Coromoto Palermo Garofalo, María Lourdes Miranda, Oberto Torres Rafael
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引用次数: 0

摘要

孤立性促肾上腺皮质激素缺乏症(IAD)是一种罕见的疾病,其特征是继发性肾上腺肾功能不全,血清皮质醇水平低,促肾上腺皮质激素分泌减少,其他垂体激素分泌充足,放射成像显示垂体结构正常。fiad作为继发性肾上腺功能不全原因的患病率尚未确定。生长激素(GH)分泌障碍已在20%至30%的IAD患者中发现,在糖皮质激素替代后恢复正常。我们报告一例50岁女性的症状和实验室结果提示肾上腺功能不全。胰岛素耐量试验证实ACTH和生长激素缺乏。其余垂体前叶激素均正常。颅脑MRI未见明显变化。开始糖皮质激素替代治疗,8个月后胰高血糖素刺激试验显示持续ACTH缺乏,但生长激素分泌正常。IAD可表现为非特异性症状,在急性应激期可能具有潜在的致命性。及时认知对降低发病率和死亡率至关重要。
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A middle aged woman with isolated ACTH deficiency associated with transient growth hormone deficiency.

Isolated ACTH deficiency (IAD) is a rare entity characterized by secondary adrenal insufficiency with low levels of serum cortisol, decreased production of ACTH, adequate secretion of other pituitary hormones and normal pituitary structure on radioimaging. The prevalence of IAD as a cause of secondary adrenal insufficiency has not been determined. Impairment of growth hormone (GH) secretion has been noted in 20 to 30% of patients with IAD which is normalized after glucocorticoid replacement. We report the case of a 50 years-old female with symptoms and laboratory results suggestive of adrenal insufficiency. Insulin tolerance test confirmed ACTH and growth hormone deficiency. The rest of the anterior pituitary hormones were normal. A pituitary MRI was unremarkable. Glucocorticoid replacement therapy started and eight months afterwards glucagon stimulation test revealed persistent ACTH deficiency but nor- mal growth hormone secretion. IAD can present with nonspecific symptoms and could be potentially fatal in an acute stressful period. Prompt recognition is essential to decrease morbidity and mortality.

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