Hofer J F, Wimmer G
Department of Internal Medicine, General Hospital Freistadt, Krankenhausstrasse 1, 4240 Freistadt, Austria.
Z Kardiol. 2003 Jan;92(1):90-5. doi: 10.1007/s00392-003-0875-x.
Primary (AL)amyloidosis is characterized by clonal production of immunoglobulin with subsequent deposition in several organs. We describe the clinical features of a 66-year old female who was referred to our department for congestive heart failure. One year before, she was examined and found to have diastolic dysfunction of the left ventricle. We could evaluate the diagnosis of primary amyloid cardiomyopathy by echocardiography, Doppler echocardiography and laboratory findings and confirmed the diagnosis by biopsy of the rectum mucosa. Clinical outcome was poor, because therapy is poor.
原发性（AL）淀粉样变性的特征是免疫球蛋白的克隆性产生，随后沉积于多个器官。我们描述了一名66岁女性的临床特征，她因充血性心力衰竭转诊至我科。一年前，她接受检查时发现左心室舒张功能障碍。我们通过超声心动图、多普勒超声心动图和实验室检查结果评估原发性淀粉样心肌病的诊断，并通过直肠黏膜活检确诊。由于治疗效果不佳，临床预后较差。
