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30例淀粉样变性病的临床特征

[Clinical features of 30 cases of amyloidosis].

作者信息

Zhao Xin-Yan, Jia Ji-Dong, Wang Bao-En, Ou Xiao-Juan, Qian Lin-Xue, Zhang Fu-Kui, Wang Yu, Cui Yan

机构信息

Liver Research Center, Beijing Friendship Hospital, Capital University of Medical Sciences, Beijing 100050, China.

出版信息

Zhonghua Gan Zang Bing Za Zhi. 2005 Jan;13(1):42-4.

Abstract

OBJECTIVE

Clinical features of 30 cases of amyloidosis, a rare disease in China, were analyzed in order to improve the recognition of the disease here.

METHODS

30 cases of biopsy-proven amyloidosis, admitted to Beijing Friendship Hospital from July 1980 to December 2003 were retrospectively reviewed.

RESULTS

12 of the 30 cases were systemic amyloidosis. Among them 9 were primary amyloidosis, 1 secondary amyloidosis and 2 familial amyloid polyneuropathy. The other 18 cases were localized amyloidosis. Males (17) were more than females (13). In the 12 primary amyloidosis patients, kidney (75.00%), liver (58.33%), peripheral nervous system (58.33%) and heart (50.00%) were most commonly involved. Nonspecific symptoms such as fatigue, weight loss, hepatomegaly, limb numbness, edema and heavy albuminuria were the most common clinical manifestations. Localized amyloidosis involved only one organ, such as skin, alimentary tract and nasopharynx without evidences of a systemic disease. Excision of the localized amyloid deposits was performed in 13 cases.

CONCLUSION

Systemic amyloidosis usually involves multiple organs and systems, leading to highly variable clinical manifestations. An increase in the vigilance of the awareness of this disease among clinicians will improve the possibilities for its diagnosis.

摘要

目的

分析30例在中国较为罕见的淀粉样变性病的临床特征,以提高国内对此病的认识。

方法

回顾性分析1980年7月至2003年12月在北京友谊医院住院的30例经活检证实的淀粉样变性病患者。

结果

30例患者中12例为系统性淀粉样变性病。其中9例为原发性淀粉样变性病,1例为继发性淀粉样变性病,2例为家族性淀粉样多神经病。另外18例为局限性淀粉样变性病。男性(17例)多于女性(13例)。12例原发性淀粉样变性病患者中,最常受累的部位是肾脏(75.00%)、肝脏(58.33%)、周围神经系统(58.33%)和心脏(50.00%)。最常见的临床表现为疲劳、体重减轻、肝肿大、肢体麻木、水肿和大量蛋白尿等非特异性症状。局限性淀粉样变性病仅累及一个器官,如皮肤、消化道和鼻咽部,无全身疾病证据。13例患者进行了局限性淀粉样沉积物切除术。

结论

系统性淀粉样变性病通常累及多个器官和系统,导致临床表现高度多变。提高临床医生对此病的警惕性将提高其诊断的可能性。

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