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腰椎管狭窄症中的转甲状腺素淀粉样沉积及全身性淀粉样变性的征象评估。

Transthyretin amyloid deposits in lumbar spinal stenosis and assessment of signs of systemic amyloidosis.

机构信息

From the, Department of Medicine, Karolinska Institute, Karolinska University Hospital, Solna, Sweden.

Stockholm Spine Centre, Löwenströmska Hospital, Upplands Väsby, Sweden.

出版信息

J Intern Med. 2021 Jun;289(6):895-905. doi: 10.1111/joim.13222. Epub 2021 Jan 7.

Abstract

BACKGROUND

Wild-type transthyretin (ATTRwt) amyloidosis is the most common systemic amyloidosis in Western countries and manifests mainly as progressive restrictive cardiomyopathy.

OBJECTIVE

To study the prevalence of ATTR deposits in ligament tissue in patients undergoing surgery for lumbar spinal stenosis and to assess whether these deposits are associated with cardiac amyloidosis.

MATERIALS AND METHODS

A total of 250 patients, aged 50-89 (57% women), none with known cardiovascular disease, were included. Ligaments were investigated microscopically for amyloid. ATTR type was determined by immunohistochemistry and fibril type by Western blot. The amount of amyloid was graded 0-4. All patients with grade 3-4 ATTR deposits were offered cardiac investigation including ECG, cardiac ultrasound, plasma NT-proBNP and cardiac magnetic resonance (CMR), including modern tissue characterization.

RESULTS

Amyloid was identified in 221 of the samples (88.4%). ATTR appeared in 93 samples (37%) of whom 42 (17 women and 25 men) were graded 3-4; all had fibril type A (mixture of full-length TTR and fragmented TTR). Twenty-nine of 42 patients with grade 3-4 ATTR deposits accepted cardiovascular investigations; none of them had definite signs of cardiac amyloidosis, but five men had a history of carpal tunnel syndrome.

CONCLUSIONS

The prevalence of ATTR deposits in ligamentum flavum in patients with lumbar spinal stenosis was high but not associated with manifest ATTR cardiac amyloidosis. However, the findings of fibril type A, the prevalence of previous carpal tunnel syndrome and ATTR amyloid in surrounding adipose and vascular tissue indicate that amyloid deposits in ligamentum flavum may be an early manifestation of systemic ATTR disease.

摘要

背景

野生型转甲状腺素蛋白(ATTRwt)淀粉样变是西方国家最常见的系统性淀粉样变,主要表现为进行性限制型心肌病。

目的

研究在因腰椎椎管狭窄症接受手术的患者的韧带组织中 ATTR 沉积的患病率,并评估这些沉积是否与心脏淀粉样变性有关。

材料和方法

共纳入 250 名年龄在 50-89 岁(57%为女性)、无已知心血管疾病的患者。显微镜下检查韧带是否有淀粉样物。通过免疫组化确定 ATTR 类型,通过 Western blot 确定纤维类型。淀粉样物的量分级为 0-4 级。所有 3-4 级 ATTR 沉积的患者均接受心脏检查,包括心电图、心脏超声、血浆 NT-proBNP 和心脏磁共振(CMR),包括现代组织特征。

结果

221 个样本中(88.4%)发现了淀粉样物。93 个样本(37%)出现 ATTR,其中 42 个(17 名女性和 25 名男性)为 3-4 级;所有患者均为纤维类型 A(全长 TTR 和片段化 TTR 的混合物)。42 例 3-4 级 ATTR 沉积患者中有 29 例接受了心血管检查;他们均无明确的心脏淀粉样变性迹象,但 5 名男性有腕管综合征病史。

结论

腰椎椎管狭窄症患者黄韧带中的 ATTR 沉积患病率较高,但与明显的 ATTR 心脏淀粉样变性无关。然而,纤维类型 A 的发现、先前腕管综合征的患病率以及周围脂肪和血管组织中的 ATTR 淀粉样变性表明,黄韧带中的淀粉样物沉积可能是全身 ATTR 疾病的早期表现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/141e/8248398/129962f192e8/JOIM-289-895-g006.jpg

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