Clinical features of 30 cases of amyloidosis / 中华肝脏病杂志

ABSTRACT

<p><b>OBJECTIVE</b>Clinical features of 30 cases of amyloidosis, a rare disease in China, were analyzed in order to improve the recognition of the disease here.</p><p><b>METHODS</b>30 cases of biopsy-proven amyloidosis, admitted to Beijing Friendship Hospital from July 1980 to December 2003 were retrospectively reviewed.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSION</b>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.</p>