摘要: |
目的总结垂体柄中断综合征(pituitary stalk interruption syndrome,PSIS)患者的临床特征。方法回顾性分析复旦大学附属中山医院内分泌科收治的17例垂体柄中断综合征患者的一般特征、实验室检查和影像学数据。结果 17例患者中,女性3例(17.6%),男性14例(82.4%),发病年龄8.12±3.31岁,病程18.59±6.87年。性腺功能减退、生长激素缺乏、肾上腺皮质功能减退、甲状腺功能减退的比例分别为100%、94.12%、82.35%、100%,高泌乳素血症的比例为29.41%、中枢性尿崩症的比例为5.88%。所有患者均合并至少3种垂体前叶激素异常,既往曾使用性激素、生长激素、糖皮质激素、甲状腺激素替代治疗的例数分别为10例、7例、3例、7例。结论对于生长发育障碍及性腺发育异常患者常规行垂体影像学检查可减少误诊、漏诊。PSIS患者应定期随访,评估垂体前叶、后叶功能,有助于及时诊断、治疗。 |
关键词: 垂体柄中断综合征 垂体功能减退 磁共振成像 |
DOI:10.12025/j.issn.1008-6358.2021.20201568 |
分类号:R335+.1 |
基金项目: |
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Clinical Features of Pituitary Stalk Interruption Syndrome in 17 Cases |
Yao fei1, zhang linshan2, Lu zhiqiang2, Li xiaoying2, Li xiaomu2
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1.Department of Endocrinology, Fuzhou Hospital of Traditional Chinese Medicine;2.Department of Endocrinology,Zhongshan Hospital,Fudan University
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Abstract: |
Objective To summarize the clinical features of patients withpituitary stalk interruption syndrome (PSIS). Methods Clinical data including general characteristics, laboratory tests, and imaging findings about 17 patients with PSIS admitted tothe Department of Endocrinology, Zhongshan Hospital, Fudan University were retrospectively analyzed. Results Of the 17 patients, 3cases(17.6%) were female, and 14 cases(82.4%) were male.The age of onsetwas 8.12±3.31 years,and the course of disease was 18.59±6.87 years.The proportions of hypogonadism, growth hormone deficiency, adrenocortical dysfunction, and hypothyroidism were 100%, 94.12%, 82.35% and 100%, respectively. The proportion of hyperprolactinemia was 29.41%, and the rate of central diabetes insipiduswas5.88%.All patients had at least three hormone abnormalities.The number of patients who hadsex hormone replacement, growth hormone replacement, glucocorticoid replacement and thyroid hormone replacement respectively were 10, 7, 3 and 7respectively. Conclusion For patients with growth and development disorders and gonadal dysplasia, routine pituitary imaging examination can reduce misdiagnosis and missed diagnosis.. Patients withPSIS should be followed up regularly to assess the functions of the anterior and posterior pituitary, which is helpful fortimely diagnosis and treatment. |
Key words: Pituitary stalk interruption syndrome(PSIS) Hypopituitarism Magnetic resonance imaging(MRI) |