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多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白血症和皮肤改变综合征患者的眼部表现。

Ocular findings in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome.

机构信息

Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA.

出版信息

Ophthalmology. 2011 Apr;118(4):778-82. doi: 10.1016/j.ophtha.2010.08.013. Epub 2010 Oct 29.

DOI:10.1016/j.ophtha.2010.08.013
PMID:21035860
Abstract

OBJECTIVE

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare disorder. This study investigated the types of ocular signs and symptoms in patients with POEMS and any systemic factors that may be associated with development of such ocular findings.

DESIGN

Case series from tertiary referral center.

PARTICIPANTS

A total of 33 patients with POEMS syndrome underwent at least 1 ophthalmologic examination and were included in the study.

METHODS

A 10-year retrospective chart review of patients diagnosed with POEMS syndrome was performed.

MAIN OUTCOME MEASURES

Visual symptoms, visual acuity, presence of optic disc edema (ODE), and levels of systemic factors (including plasma vascular endothelial growth factor [VEGF], plasma interlukin-6 [IL-6], and raised intracranial pressure) and their relationship to ODE.

RESULTS

Five of the patients (15%) reported diplopia, 15 patients (45%) had blurred vision, and 3 patients (9%) had ocular pain. The most common ocular finding was bilateral ODE in 17 patients (52%). Of the patients with ODE, 5 (29%) were asymptomatic at the first ocular examination. Among patients with ODE, there was a significant difference (P = 0.03) between the mean plasma VEGF level at the time of diagnosis of the ODE compared with when the ODE resolved. There was no difference in plasma IL-6 levels between people with and without ODE. Patients with ODE had a higher mean lumbar puncture opening pressure (276±14 mm H(2)O; normal range, 100-250 mm H(2)0) than patients without ODE, although the difference was not statistically significant (P = 0.08).

CONCLUSIONS

Optic disc edema is a common finding in patients with POEMS. Because patients can be asymptomatic, eye examinations should be performed in all patients with POEMS. There may be an association between elevated VEGF and intracranial pressure and ODE; further studies are required.

摘要

目的

多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变(POEMS)综合征是一种罕见的疾病。本研究调查了 POEMS 患者的眼部体征和症状类型,以及可能与这些眼部发现相关的任何系统性因素。

设计

来自三级转诊中心的病例系列研究。

参与者

共有 33 名 POEMS 综合征患者接受了至少 1 次眼科检查,并被纳入研究。

方法

对诊断为 POEMS 综合征的患者进行了为期 10 年的回顾性病历审查。

主要观察指标

视觉症状、视力、视盘水肿(ODE)的存在以及系统性因素(包括血浆血管内皮生长因子[VEGF]、血浆白细胞介素-6[IL-6]和颅内压升高)的水平及其与 ODE 的关系。

结果

5 名患者(15%)报告有复视,15 名患者(45%)有视力模糊,3 名患者(9%)有眼部疼痛。最常见的眼部表现是 17 名患者(52%)双侧 ODE。在有 ODE 的患者中,有 5 名(29%)在首次眼部检查时无症状。在有 ODE 的患者中,在 ODE 诊断时与 ODE 消退时的平均血浆 VEGF 水平之间存在显著差异(P = 0.03)。有 ODE 和无 ODE 的患者的血浆 IL-6 水平无差异。有 ODE 的患者的平均腰椎穿刺开放压(276±14mmHg;正常范围 100-250mmHg)高于无 ODE 的患者,尽管差异无统计学意义(P = 0.08)。

结论

视盘水肿是 POEMS 患者的常见表现。由于患者可能无症状,因此应在所有 POEMS 患者中进行眼部检查。VEGF 和颅内压升高与 ODE 之间可能存在关联;需要进一步的研究。

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