Diagnostic and management challenges in paediatric Cushing's syndrome

IF 3 3区医学 Q2 ENDOCRINOLOGY & METABOLISM

Clinical Endocrinology Pub Date : 2024-08-15 DOI:10.1111/cen.15096

Kriti Joshi, Anna Taliou, Constantine A. Stratakis

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引用次数: 0

Abstract

Objective

Cushing syndrome (CS) is the result of chronic exposure to glucocorticoid excess. CS in children is most often caused by the administration of exogenous steroids. Endogenous CS is rare in the paediatric population and is caused mainly by tumours of the pituitary and adrenal glands, with ectopic sources being extraordinarily rare before the age of 18 years. In addition, children and young adults with CS present with different epidemiology, management issues, prognosis and outcomes than older adult patients. This complex disorder needs early diagnosis and management to avoid the significant morbidity and even mortality that can result from chronic untreated CS.

Methods

In this review, we present the complex case of a 7-year-old boy with CS that highlights the diagnostic and management challenges of paediatric CS patients, including the considerations for genetic predisposition and life-long consequences of CS in children and young adults.

Results

The diagnostic protocols for the evaluation of CS have been devised for adults and tested predominantly on adults. In this review, we discuss necessary modifications so that the testing can be adjusted for use in children. Additionally, pituitary adenomas in children are generally smaller and thus more difficult to recognize on pituitary imaging.

Conclusions

The management of the case and its complexities underline the need for children with CS to be managed in a centre with experienced paediatric endocrinologists and skilled neurosurgeons both for their initial diagnosis and treatment as well as for their long-term follow-up and management.

Abstract Image

查看原文本刊更多论文

小儿库欣综合征的诊断和管理难题。

目的：库欣综合征（CS库欣综合征（CS）是糖皮质激素长期过量的结果。儿童库欣综合征多由外源性类固醇引起。内源性 CS 在儿科人群中较为罕见，主要由垂体和肾上腺肿瘤引起，异位来源的 CS 在 18 岁之前极为罕见。此外，儿童和青少年 CS 患者的流行病学、管理问题、预后和疗效都与老年患者不同。这种复杂的疾病需要早期诊断和治疗，以避免因长期不治疗 CS 而导致严重的发病甚至死亡：在这篇综述中，我们介绍了一名患有 CS 的 7 岁男孩的复杂病例，突出强调了儿科 CS 患者在诊断和管理方面的挑战，包括考虑遗传易感性以及 CS 对儿童和青少年的终身影响：评估 CS 的诊断方案是为成人设计的，主要在成人身上进行测试。在这篇综述中，我们讨论了必要的修改，以便将测试调整用于儿童。此外，儿童的垂体腺瘤通常较小，因此在垂体成像中更难识别：该病例的处理及其复杂性突出表明，CS患儿需要在有经验丰富的儿科内分泌专家和技术精湛的神经外科医生的中心进行管理，以便对其进行初步诊断和治疗，以及长期随访和管理。

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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来源期刊

Clinical Endocrinology 医学-内分泌学与代谢

CiteScore

6.40

自引率

3.10%

发文量

192

审稿时长

1 months

期刊介绍： Clinical Endocrinology publishes papers and reviews which focus on the clinical aspects of endocrinology, including the clinical application of molecular endocrinology. It does not publish papers relating directly to diabetes care and clinical management. It features reviews, original papers, commentaries, correspondence and Clinical Questions. Clinical Endocrinology is essential reading not only for those engaged in endocrinological research but also for those involved primarily in clinical practice.