Endogenous Cushing Syndrome- A Spectrum of Cases

B. Santosh, R. Boddula, Ashok Venkatanarasu, Aditya Hegde, Chimutai Chinte, Vidya Tickoo
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Abstract

Introduction Cushing’s syndrome (CS) is a rare disorder characterised by symptoms and signs of chronic excessive tissue exposure to glucocorticoids, and is associated with high morbidity and mortality if untreated. The excess cortisol state can result from a number of etiologies- both exogenous and endogenous. It can present with varied and non-specific clinical features. Hence having a high clinical suspicion is important and it can be challenging to arrive at an exact diagnosis, which is very important to initiate appropriate treatment. Here, we present a spectrum of endogenous Cushing’s syndrome of different etiologies, who presented to us at different age groups and different clinical features. Description of Cases The main aim of this article is to understand the different symptoms and signs with which the patients with Endogenous Cushing’s syndrome present, the knowledge required to suspect it even in a non-typical presentation, the extensive and laborious work-up done at arriving the diagnosis and localizing the cause, and then finally initiating appropriate treatment. We randomly chose 8 cases of Endogenous Cushing’s syndrome of different presentation and etiology, who presented to our Hospital. We had patients whose age ranged from 13 years to 60 years; patients presenting with typical features of Cushing’s syndrome to non-specific features and even being asymptomatic. All the cases underwent appropriate and elaborate work-up to arrive at a diagnosis and also to localize the exact lesion. They then underwent appropriate treatment either in the form of surgery (excision of the lesion responsible for the cortisol excess) or medical therapy. The outcome of the patients was good in 6 of these patients, they achieved remission during follow-up. However, 2 patients, one with Adrenocortical carcinoma and another with Ectopic adreno-cortico tropic hormone (ACTH) syndrome due to Poorly differentiated adenocarcinoma of left lung, died due to the severity of underlying diseases. Conclusion Endogenous Cushing’s syndrome, though rare, is associated with high morbidity and mortality if untreated. A high degree of suspicion and a good clinical examination is required to diagnose Cushing’s syndrome especially in nonflorid cases and those with nonspecific features. A systematic, orderly evaluation is required to establish the correct diagnosis and to localize the cause, which will help in planning of appropriate treatment.
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内源性库欣综合征-一系列病例
库欣综合征(CS)是一种罕见的疾病,其特征是慢性组织过度暴露于糖皮质激素的症状和体征,如果不治疗,发病率和死亡率都会很高。皮质醇水平过高的原因有很多,包括外源性的和内源性的。它可以表现出多种多样的非特异性临床特征。因此,有高度的临床怀疑是很重要的,要做出准确的诊断是具有挑战性的,这对开始适当的治疗是非常重要的。在这里,我们提出了不同病因的内源性库欣综合征的频谱,谁向我们提出了不同的年龄组和不同的临床特征。本文的主要目的是了解内源性库欣综合征患者所表现出的不同症状和体征,即使在非典型的表现中也需要怀疑它的知识,在诊断和定位病因时所做的广泛而艰苦的检查,然后最终开始适当的治疗。我们随机选择8例不同表现和病因的内源性库欣综合征来我院就诊。我们的病人年龄从13岁到60岁不等;患者表现为库欣综合征的典型特征到非特异性特征,甚至无症状。所有病例都进行了适当和详细的检查,以得出诊断并确定病变的确切位置。然后,他们接受了手术(切除导致皮质醇过量的病变)或药物治疗的适当治疗。6例患者预后良好,随访期间均获得缓解。2例左肺低分化腺癌合并肾上腺皮质癌和异位促肾上腺皮质激素综合征,均因基础疾病严重而死亡。结论内源性库欣综合征虽然罕见,但如果不及时治疗,其发病率和死亡率很高。诊断库欣综合征需要高度的怀疑和良好的临床检查,特别是在非典型病例和具有非特异性特征的病例中。需要进行系统有序的评估,以确定正确的诊断并确定病因,这将有助于规划适当的治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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