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儿童及青少年库欣综合征/库欣病

Cushing Syndrome/Disease in Children and Adolescents

作者信息

Tatsi Christina

机构信息

Assistant Clinical Investigator, Unit on Hypothalamic and Pituitary Disorders, Eunice Kennedy Shriver National Institute of Child Health, and Human Development, National Institutes of Health, Bethesda, MD, 20814, USA

Abstract

Endogenous Cushing syndrome (CS) is a rare pediatric endocrine condition commonly caused by pituitary corticotroph tumors or less often by adrenal or ectopic sources. The typical presentation of the child with CS includes weight gain with height deceleration, characteristic skin findings, and hormonal and biochemical findings indicative of excessive glucocorticoid production. The diagnostic evaluation of the patient with suspected hypercortisolemia initially involves the confirmation of cortisol excess in blood and/or urine, and then the identification of source. The first line of management usually requires surgical treatment of a pituitary or adrenal lesion. In persistent or recurrent disease, re-operation, medical treatment, or radiation should be considered. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text, WWW.ENDOTEXT.ORG.

摘要

内源性库欣综合征(CS)是一种罕见的儿科内分泌疾病,通常由垂体促肾上腺皮质激素瘤引起,较少由肾上腺或异位来源引起。患有CS的儿童的典型表现包括体重增加伴身高增长减速、特征性皮肤表现以及提示糖皮质激素分泌过多的激素和生化表现。对疑似高皮质醇血症患者的诊断评估首先包括确认血液和/或尿液中皮质醇过量,然后确定来源。一线治疗通常需要对垂体或肾上腺病变进行手术治疗。对于持续性或复发性疾病,应考虑再次手术、药物治疗或放疗。欲全面涵盖内分泌学的所有相关领域,请访问我们的在线免费网络文本,网址为WWW.ENDOTEXT.ORG。

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