FAMILIAL AMYLOID POLYNEUROPATHY──CLINICAL REPORT OF A FAMILY  

作　　者：李延峰 郭玉璞 池田修一 方定华 

出　　处：《Chinese Medical Sciences Journal》1996年第2期113-116,共4页中国医学科学杂志（英文版）

摘　　要：This paper reports a familial amyloid polyneuropathy (FAP) family in China. This family being investigated had 69 members of five generations. From the third generation, there have been 16 patients. The age of onset was about 3 to 5 decades. The initial symptoms were autonomic nerve symptoms, such as impotence, dyspepsia and diarrhoea, associated with the sensory loss of lower extremities. As the disease progressed, the upper extremities and motor ability were also involved. The duration of disease course was about 8-10 years, most patients died of infection and cachexia. Sural biopsy in 3 patients had showed positive Congo red staining. From the clinical view, this FAP family is similar to FAP I found in Japan. The true classification, however, should be confirmed by further genetic analysis.

关 键 词：familial amyloid polyneuropathy sural biopsy 

分 类 号：R597.2[医药卫生—内科学]