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库欣综合征患者的病因及临床特征:单中心经验

Etiology and clinical profile of patients with Cushing's syndrome: A single center experience.

作者信息

Ammini Ariacherry C, Tandon Nikhil, Gupta Nandita, Bhalla Ashu Seith, Devasenaspathy Kandaswamy, Kumar Guresh, Sahoo Jaiprakash P, Chittawar Sachin, Philip Jim, Baruah Manas P, Dwarakanath C S, Tripathi Sudhir

机构信息

Department of Endocrinology and Metabolism, AIIMS, New Delhi, India.

Department of Radiology, AIIMS, New Delhi, India.

出版信息

Indian J Endocrinol Metab. 2014 Jan;18(1):99-105. doi: 10.4103/2230-8210.126586.

Abstract

BACKGROUND

There is little published literature on the profile of patients with Cushing's syndrome (CS) from India. The aim of this study was to compile data of CS patients treated at this hospital.

MATERIALS AND METHODS

Patients referred to the endocrine services of this hospital for diagnosis/treatment of CS from January 1985 to July 2012 were the subjects for this study. All patients had detailed medical history, physical examination and biochemical and hormonal assays (which changed with availability of tests and changing views). Assays for plasma adrenocorticotropic hormone (ACTH) (late 90s), salivary cortisol estimation, IJV sampling for ACTH and corticotrophin releasing hormone stimulation tests were added on later. Imaging included computed tomography (CT), magnetic resonance imaging (since the late 80's) and (68)Ga DOTA-TOC/FDG PET-CT (2008).

RESULTS

Three hundred sixty-four patients (250 females, 114 males, age 6 months to 65 years, mean 28 years + 12 years) were diagnosed to have CS during this period. Two hundred and ninety-three patients (80.5%) were ACTH dependent (CD 215, ectopic ACTH syndrome 22, occult ACTH source 56) while 71 (19.5%) were ACTH independent (adrenal carcinoma 36, adenoma 30, primary pigmented nodular adrenal disease 4, AIMAH 1). Pituitary macro adenoma was seen in 14% of the CD cases. The most common presenting complaints were hypertension and diabetes mellitus. A total of 63% patients complained of weight gain while 15% had lost weight. Myopathy, infections, skeletal fractures and psychiatric problems were the other common observations in our patients.

CONCLUSION

The clinical spectrum was broad. CD was the most common cause for CS.

摘要

背景

关于印度库欣综合征(CS)患者的情况,发表的文献较少。本研究的目的是汇编在本院接受治疗的CS患者的数据。

材料与方法

1985年1月至2012年7月转诊至本院内分泌科进行CS诊断/治疗的患者为本研究对象。所有患者均有详细的病史、体格检查以及生化和激素检测(检测项目随检测方法的可获得性和观念的改变而变化)。后来增加了血浆促肾上腺皮质激素(ACTH)检测(90年代后期)、唾液皮质醇测定、经颈内静脉取血检测ACTH以及促肾上腺皮质激素释放激素刺激试验。影像学检查包括计算机断层扫描(CT)、磁共振成像(自80年代后期起)以及(68)镓-多胺基多乙酸-奥曲肽/氟代脱氧葡萄糖正电子发射断层扫描-CT(2008年)。

结果

在此期间,共诊断出364例CS患者(女性250例,男性114例,年龄6个月至65岁,平均28岁±12岁)。293例患者(80.5%)为促肾上腺皮质激素依赖性(库欣病215例,异位ACTH综合征22例,隐匿性ACTH来源56例),而71例(19.5%)为促肾上腺皮质激素非依赖性(肾上腺皮质癌36例,腺瘤30例,原发性色素沉着性结节性肾上腺病4例,原发性醛固酮增多症伴ACTH非依赖性皮质醇增多症1例)。在促肾上腺皮质激素依赖性病例中,14%可见垂体大腺瘤。最常见的主诉是高血压和糖尿病。共有63%的患者主诉体重增加,而15%的患者体重减轻。肌病、感染、骨折和精神问题是我们患者中的其他常见表现。

结论

临床谱广泛。促肾上腺皮质激素依赖性是CS最常见的病因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d4c/3968742/847a1ad4aca4/IJEM-18-99-g001.jpg

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