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垂体柄阻断综合征6例临床分析并文献复习

魏伟平 全会标 李云倩 方团育 刘海蔚 陈道雄 陈开宁

疑难病杂志2018,Vol.17Issue(5):507-510,4.
疑难病杂志2018,Vol.17Issue(5):507-510,4.DOI:10.3969/j.issn.1671-6450.2018.05.018

垂体柄阻断综合征6例临床分析并文献复习

Clinical analyses of 6 cases with pituitary stalk interruption syndrome and literature review

魏伟平 1全会标 1李云倩 1方团育 1刘海蔚 1陈道雄 1陈开宁1

作者信息

  • 1. 570311海口,海南省人民医院内分泌科
  • 折叠

摘要

Abstract

Objective To improve the clinical diagnosis for patients with pituitary stalk interruption syndrome (PSIS).Methods Six patients (4 males and 2 females) with PSIS were retrospectively analyzed with respects to the clinical features,endocrine status and image characteristics.Results The clinical manifestations included growth retardation and delayed puberty.All of them were deficient in growth hormone and pituitary gonadotropin secretion.Secondary hypothyroidism and hypocortisolism occurred in 5 patients.The typical imaging finding is a thin or absent pituitary stalk,often associated with an ectopic posterior pituitary and a hypoplastic anterior pituitary.Replacement of multiple anterior pituitary hormones is the key of the treatment.Conclusion PSIS is rarely seen and the clinical characteristics of patients with PSIS are growth retardation and delayed puberty,partial or complete adenohypophyseal dysfunction.The detection of hypothalamic pituitary region by MR is an important diagnostic tool.The evaluation of anterior pituitary function is necessary.Doctors should have a complete understanding of the disease and give patients effective treatment and a better prognosis would be obtained.

关键词

垂体柄阻断综合征/临床特征/诊断/治疗

Key words

Pituitary stalk interruption syndrome/Clinical feature/Diagnosis/Treatment

引用本文复制引用

魏伟平,全会标,李云倩,方团育,刘海蔚,陈道雄,陈开宁..垂体柄阻断综合征6例临床分析并文献复习[J].疑难病杂志,2018,17(5):507-510,4.

疑难病杂志

OACSTPCD

1671-6450

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