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原发性空蝶鞍患者的多中心队列研究:长期随访中的激素和神经影像学特征。

A Multicenter Cohort Study in Patients With Primary Empty Sella: Hormonal and Neuroradiological Features Over a Long Follow-Up.

机构信息

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Endocrinology Unit, Milan, Italy.

Sapienza University of Rome, Department of Experimental Medicine, Rome, Italy.

出版信息

Front Endocrinol (Lausanne). 2022 Jun 23;13:925378. doi: 10.3389/fendo.2022.925378. eCollection 2022.

Abstract

OBJECTIVE

primary empty sella (PES) represents a frequent finding, but data on hormonal alterations are heterogeneous, and its natural history is still unclear. Our aim was to evaluate the pituitary function of patients with PES over a long follow-up.

DESIGN

multicenter retrospective cohort study enrolling patients referred between 1984-2020 to five Pituitary Units, with neuroradiological confirmed PES and a complete hormonal assessment.

METHODS

we analyzed hormonal (including basal and dynamic evaluations), clinical and neuroradiological data collected at diagnosis and at the last visit (at least 6 months of follow-up).

RESULTS

we recruited 402 patients (females=63%, mean age=51.5 ± 16 years) with PES (partial, total, undefined in 66%, 13% and 21%, respectively). Hypopituitarism was present in 40.5% (hypogonadism=20.4%, hypoadrenalism=14.7%, growth hormone deficiency=14.7%, hypothyroidism=10.2%, diabetes insipidus=1.5%; multiple deficiencies=11.4%) and hypeprolactinemia in 6.5%. Interestingly, hormonal alterations were diagnosed in 29% of incidental PES. Hypopituitarism was associated with male sex (=0.02), suspected endocrinopathy (<0.001), traumatic brain injury (=0.003) and not with age, BMI, number of pregnancies and neuroradiological grade. A longitudinal assessment was possible in 166/402 (median follow-up=58 months). In 5/166 (3%), new deficiencies occurred, whereas 14/166 (8.4%) showed a hormonal recovery. A progression from partial to total PES, which was found in 6/98 patients assessed with a second imaging, was the only parameter significantly related to the hormonal deterioration (=0.006).

CONCLUSIONS

this is the largest cohort of patients with PES reported. Hypopituitarism is frequent (40%) but hormonal deterioration seems uncommon (3%). Patients need to be carefully evaluated at diagnosis, even if PES is incidentally discovered.

摘要

目的

原发性空蝶鞍(PES)是一种常见的发现,但关于激素改变的数据存在异质性,其自然史尚不清楚。我们的目的是在长期随访中评估 PES 患者的垂体功能。

设计

这项多中心回顾性队列研究纳入了 1984 年至 2020 年间向五个垂体单位转诊的、经神经放射学证实的 PES 且有完整激素评估的患者。

方法

我们分析了在诊断时和末次随访(至少 6 个月的随访)时收集的激素(包括基础和动态评估)、临床和神经放射学数据。

结果

我们招募了 402 名 PES 患者(女性占 63%,平均年龄为 51.5±16 岁)(部分、完全、未定义分别占 66%、13%和 21%)。40.5%的患者存在垂体功能减退(性腺功能减退症 20.4%、肾上腺功能减退症 14.7%、生长激素缺乏症 14.7%、甲状腺功能减退症 10.2%、尿崩症 1.5%;多种缺乏症 11.4%)和 6.5%的高泌乳素血症。有趣的是,29%的偶然发现的 PES 患者被诊断为激素改变。垂体功能减退与男性性别(=0.02)、疑似内分泌疾病(<0.001)、创伤性脑损伤(=0.003)相关,与年龄、BMI、妊娠次数和神经放射学分级无关。在 402 名患者中,有 166 名(中位数随访 58 个月)可进行纵向评估。在 5/166(3%)名患者中出现了新的缺乏,而在 14/166(8.4%)名患者中出现了激素恢复。在接受第二次影像学检查的 98 名患者中,6 名患者的 PES 从部分发展为完全,这是唯一与激素恶化显著相关的参数(=0.006)。

结论

这是报道的最大的 PES 患者队列。垂体功能减退很常见(40%),但激素恶化似乎不常见(3%)。即使 PES 是偶然发现的,患者也需要在诊断时进行仔细评估。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6630/9259926/056054984e87/fendo-13-925378-g001.jpg

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