不同流行地区的转甲状腺素相关的玻璃体淀粉样变性。

Transthyretin-related vitreous amyloidosis in different endemic areas.

机构信息

Department of Ophthalmology and Visual Science, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan.

出版信息

Amyloid. 2010 Sep;17(3-4):105-8. doi: 10.3109/13506129.2010.527068.

DOI:10.3109/13506129.2010.527068

PMID:21077797

Abstract

BACKGROUND

to investigate the vitreous opacity in patients with familial amyloidotic polyneuropathy (FAP) in two major endemic areas, Japan and Sweden.

METHODS

we obtained clinical data for 90 patients with vitreous opacity that was associated with FAP amyloidogenic transthyretin (ATTR) Val30Met; 18 Japanese patients and 72 Swedish patients. We reviewed medical records at Kumamoto University Hospital in Japan and Umeå University Hospital in Sweden. We evaluated the characteristics of the patients, systemic and ocular histories, clinical findings and treatment.

RESULTS

swedish patients were significantly older at the onset of vitreous opacity (mean age 67.8 years) than were Japanese patients (47.6 years). A similar age difference was found for the onset of polyneuropathy. In addition, Swedish patients without polyneuropathy were significantly older (74.1 years) at the onset of vitreous opacity than those with polyneuropathy (64.6 years). A significant difference in the occurrence of vitreous opacity as the only manifestation of FAP was seen for Swedish patients (35%) compared with Japanese patients (6%).

CONCLUSIONS

swedish FAP ATTR Val30Met patients appeared to develop vitreous opacity later and more frequently compared with Japanese patients.

摘要

背景

研究两个主要流行地区（日本和瑞典）家族性淀粉样多神经病（FAP）患者的玻璃体混浊。

方法

我们获得了与 FAP 淀粉样转甲状腺素（ATTR）Val30Met相关的 90 例玻璃体混浊患者的临床资料；其中包括 18 例日本患者和 72 例瑞典患者。我们复习了日本熊本大学医院和瑞典于默奥大学医院的病历。我们评估了患者的特征、系统和眼部病史、临床发现和治疗。

结果

瑞典患者玻璃体混浊发病年龄（平均 67.8 岁）明显大于日本患者（47.6 岁）。多发性神经病发病年龄也存在类似的差异。此外，无多发性神经病的瑞典患者玻璃体混浊发病年龄（74.1 岁）明显大于有多发性神经病的患者（64.6 岁）。瑞典患者玻璃体混浊作为唯一 FAP 表现的发生率（35%）与日本患者（6%）相比有显著差异。