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儿童库欣病:诊断和治疗的挑战。

Childhood Cushing disease: a challenge in diagnosis and management.

机构信息

Department of Endocrinology, K.E.M. Hospital, Mumbai, India.

出版信息

Horm Res Paediatr. 2011;76 Suppl 1:65-70. doi: 10.1159/000329173. Epub 2011 Jul 21.

Abstract

BACKGROUND

Cushing syndrome (CS) in childhood is an extremely rare disorder that poses unique diagnostic difficulties. Different etiopathological states account for endogenous hypercortisolism in various age groups. Diagnosis is especially challenging in individuals with adrenocorticotropic hormone-dependent CS. The two most common etiologies are adrenal tumors and pituitary corticotrophin- secreting adenomas (Cushing disease).

CASE REPORT

We present the case of a 9-year-old boy that illustrates some of the challenges of diagnosing and managing patients with CS.

CONCLUSIONS

Diagnosis is usually made by an experienced clinician using a combination of tests to establish a state of endogenous hypercortisolism followed by localization of its source.Management requires an individualized approach and multidisciplinary care. Even after curative therapy, these children require long-term follow-up into adulthood to assess growth and body composition and routine monitoring for metabolic anomalies and/or recurrence of the disease.

摘要

背景

儿童库欣综合征(CS)是一种极其罕见的疾病,其诊断具有独特的难度。不同的病因病理状态导致了不同年龄组的内源性皮质醇增多症。依赖促肾上腺皮质激素的 CS 患者的诊断尤其具有挑战性。两种最常见的病因是肾上腺肿瘤和垂体促肾上腺皮质激素分泌腺瘤(库欣病)。

病例报告

我们报告了一例 9 岁男孩的病例,该病例说明了诊断和管理 CS 患者的一些挑战。

结论

诊断通常由经验丰富的临床医生使用一系列测试来确定内源性皮质醇增多症的状态,然后定位其来源。治疗需要个体化方法和多学科护理。即使在治愈性治疗后,这些儿童也需要长期随访至成年期,以评估生长和身体成分,并常规监测代谢异常和/或疾病复发。

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