循环库欣综合征:诊断、寻找的困难和治疗策略的选择。一个临床病例]。

Q4 Medicine Problemy endokrinologii Pub Date : 2023-02-25 DOI:10.14341/probl13140
L K Dzeranova, A V Dorovskikh, E A Pigarova, A M Lapshina, S Y Vorotnikova, A S Shutova, M A Perepelova, A Yu Grigoriev, V N Azizyan
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引用次数: 0

摘要

周期性库欣综合征是一种病理状态,其特点是皮质醇分泌过量并伴有相应的临床表现和疾病的自然缓解期交替出现。为了诊断周期性库欣综合征,有必要记录至少三次皮质醇分泌过多的发作,交替出现皮质醇分泌正常化的时期。在大多数情况下,这种病理诊断为垂体促肾上腺皮质激素分泌瘤患者,但也有罕见的周期性高皮质,不同部位肿瘤的异位促肾上腺皮质激素分泌,没有病理激素分泌病灶的验证。此外,在与皮质固醇瘤或肾上腺增生相关的acth非依赖性库欣综合征中,也可以观察到皮质醇的循环高生成。目前对周期性高皮质的确切原因和机制的研究还不够充分。由于疾病的非典型病程,新“周期”发生的不可预测性,其持续时间和表现的可变性(不仅在不同的患者中,而且在同一患者中),诊断的验证和治疗策略的确定在专家的日常实践中可能是困难的,并且这种情况的患病率可能被低估。
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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[Cyclic Cushing's syndrome: difficulties of diagnostic search and choice of treatment tactics. A clinical case].

Cyclic Cushing's syndrome is a pathological condition characterized by alternating periods of excessive cortisol secretion with corresponding clinical manifestations and periods of spontaneous remission of the disease.To diagnose Cyclic Cushing's syndrome it is necessary to record at least three episodes of excessive cortisol secretion alternating with periods of normalization of its production.In most cases, this pathology is diagnosed in patients with ACTH-secreting pituitary tumor, however, there are rare cases of cyclic hypercorticism with ectopic ACTH secretion by tumors of different localization and without verification of pathological hormonal secretion focus. In addition, cyclic hyperproduction of cortisol can be also observed in ACTH-independent Cushing's syndrome associated with the presence of corticosteroma or adrenal hyperplasia. The exact causes and mechanisms of the cyclic hypercorticism are currently insufficiently studied.Due to the atypical course of the disease, the unpredictability of the occurrence of a new «cycle», the variability of its duration and manifestations (not only in different patients, but also in the same patient), verification of the diagnosis and determination of treatment tactics may be difficult in the daily practice of specialists, and the prevalence of this condition can be undervalued.

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来源期刊
Problemy endokrinologii
Problemy endokrinologii Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
1.40
自引率
0.00%
发文量
59
期刊介绍: Since 1955 the “Problems of Endocrinology” (or “Problemy Endocrinologii”) Journal publishes timely articles, balancing both clinical and experimental research, case reports, reviews and lectures on pressing problems of endocrinology. The Journal is aimed to the most topical issues of endocrinology: to chemical structure, biosynthesis and metabolism of hormones, the mechanism of their action at cellular and molecular level; pathogenesis and to clinic of the endocrine diseases, new methods of their diagnostics and treatment. The Journal: features original national and foreign research articles, reflecting world endocrinology development; issues thematic editions on specific areas; publishes chronicle of major international congress sessions and workshops on endocrinology, as well as state-of-the-art guidelines; is intended for scientists, endocrinologists diabetologists and specialists of allied trade, general practitioners, family physicians and pediatrics.
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