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儿童库欣综合征。

Cushing syndrome in pediatrics.

机构信息

Section on Endocrinology and Genetics, Program in Developmental Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892-1862, USA.

出版信息

Endocrinol Metab Clin North Am. 2012 Dec;41(4):793-803. doi: 10.1016/j.ecl.2012.08.002. Epub 2012 Sep 27.

Abstract

Cushing syndrome is characterized by truncal obesity, growth deceleration, skin changes, muscle weakness, and hypertension. Cushing syndrome in childhood usually results from the exogenous administration of glucocorticoids. This article presents the causes and discusses the treatment of endogenous Cushing syndrome. It also discusses the clinical and molecular genetics of inherited forms of this syndrome. Cushing syndrome needs to be diagnosed and treated properly when first recognized; improper treatment can turn this otherwise completely curable disorder into a chronic ailment. Barriers to optimal care of a pediatric patient with Cushing syndrome are discussed.

摘要

库欣综合征的特征为躯干肥胖、生长迟缓、皮肤改变、肌肉无力和高血压。儿童时期的库欣综合征通常是由于外源性给予糖皮质激素引起的。本文介绍了内源性库欣综合征的病因,并讨论了其治疗方法。还讨论了这种综合征遗传形式的临床和分子遗传学。当首次发现库欣综合征时,需要进行正确的诊断和治疗;不适当的治疗可能会使这种原本完全可治愈的疾病转变为慢性疾病。本文还讨论了库欣综合征患儿接受最佳治疗的障碍。

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