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儿童库欣综合征:最新进展。

Cushing's Syndrome in Pediatrics: An Update.

机构信息

Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), NIH-Clinical Research Center, 10 Center Drive, Building 10, Room 1E-3330, MSC1103, Bethesda, MD 20892, USA.

Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), NIH-Clinical Research Center, 10 Center Drive, Building 10, Room 1E-3330, MSC1103, Bethesda, MD 20892, USA.

出版信息

Endocrinol Metab Clin North Am. 2018 Jun;47(2):451-462. doi: 10.1016/j.ecl.2018.02.008.

Abstract

Cushing syndrome (CS) is a multisystem disorder resulting from the prolonged exposure to excess glucocorticoids. In children, CS most commonly results from the exogenous administration of steroids and the typical presentation is height deceleration concomitant with weight gain. Endogenous and ectopic causes are rare. CS in children may be associated with distinct germline and somatic mutations. Clinical practice guidelines are available assist clinicians. Patients should be referred to multidisciplinary centers of excellence with experience in endocrinology and surgery. Early detection and treatment is essential to reduce associated acute and long-term morbidity and potential death.

摘要

库欣综合征(CS)是一种多系统疾病,由长期暴露于过量糖皮质激素引起。在儿童中,CS 最常见于外源性给予类固醇,典型表现为身高减速伴体重增加。内源性和异位性病因罕见。儿童 CS 可能与独特的种系和体细胞突变有关。临床实践指南可协助临床医生。患者应转至具有内分泌和外科经验的多学科卓越中心。早期发现和治疗对于降低相关的急性和长期发病率以及潜在的死亡风险至关重要。

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