[Steroid profiling characteristics in pediatrc adrenal diseases].

Q4 Medicine
E A Yanar, N/ V Makazan, V A Ioutsi, M A Kareva, O B Bezlepkina, V A Peterkova
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引用次数: 0

Abstract

Background: Adrenocortical adenomas are often followed with steroid hormones hyperproduction, and therefore determination of their concentration plays an important role in the differential diagnosis of adrenal diseases. Steroid profiling by tandem mass spectrometry is one of the main diagnostic methods in steroidogenesis characterization. Currently plasma and urinary steroid profiling is of particular interest in differential diagnosis and subtyping patients with adrenocortical adenomas.

Aim: Steroid profiling of pediatric patients with adrenal diseases (incidentalomas, ACTH-secreting pituitary adenoma, ACTH-independent Cushing syndrome, premature adrenarche).

Materials and methods: We conducted a retrospective analysis of steroid profile of 41 pediatric patients with adrenal diseases who were observed between 2005 and 2020 at the Endocrinology Research Centre.

Results: All patients were divided into groups due to diagnosis: with ACTH-secreting pituitary adenoma [n=7], ACTH-independent Cushing syndrome (autonomous cortisol secretion by an adrenal adenoma) [n=4], with incidentaloma [n=7] and premature adrenarche [n=23]. In group of patients with ACTH-independent Cushing syndrome identified statistically significant higher levels of 11-deoxycortisol (р=0, 0035) and significant lower levels of 17-hydroxypregnenolone (р=0, 0026) and DHEA (р=0, 0047) compared to other groups. Statistically significant differences in steroid profiles between other groups were not identified.

Conclusion: Results of our study steroid profiling can be used as additional differential diagnosis method in patients with adrenocortical adenomas with or without hormonal hyperproduction (ACTH-independent Cushing syndrome and incidentaloma). Further studies are needed to identify steroid markers for subtyping pediatric adrenal diseases.

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[儿科肾上腺疾病的类固醇特征分析]。
背景:肾上腺皮质腺瘤常伴有类固醇激素分泌过多,因此测定其浓度在肾上腺疾病的鉴别诊断中起着重要作用。串联质谱分析是类固醇形成的主要诊断方法之一。目前,血浆和尿类固醇谱分析在肾上腺皮质腺瘤患者的鉴别诊断和分型中具有特别的意义。目的:小儿肾上腺疾病(偶发瘤、垂体促肾上腺皮质激素分泌腺瘤、非促肾上腺皮质激素依赖型库欣综合征、肾上腺早衰)患者的类固醇特征分析。材料和方法:我们对2005年至2020年在内分泌学研究中心观察的41名患有肾上腺疾病的儿科患者的类固醇谱进行了回顾性分析。结果:所有患者根据诊断分为:垂体促肾上腺皮质激素腺瘤组[n=7]、非促肾上腺皮质激素库欣综合征(肾上腺腺瘤自主分泌皮质醇)组[n=4]、偶发瘤组[n=7]和肾上腺早衰组[n=23]。与其他组相比,acth非依赖性库欣综合征患者组11-脱氧皮质醇水平显著升高(0.0035),17-羟基孕烯醇酮(0.0026)和脱氢表雄酮(0.0047)水平显著降低。其他组之间的类固醇谱没有统计学上的显著差异。结论:本研究的类固醇谱分析结果可作为肾上腺皮质腺瘤伴或不伴激素分泌过多(acth非依赖性库欣综合征和偶发瘤)患者的额外鉴别诊断方法。需要进一步的研究来确定小儿肾上腺疾病亚型的类固醇标记物。
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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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