Liu Y C, Liu P, Pan J S
Nephrology Center, Beijing Medical University.
Zhonghua Nei Ke Za Zhi. 1990 Oct;29(10):593-6, 636-7.
14 cases were investigated between 1963 to 1988. Age ranging from 25-66 (mean 48.2). 9 cases (64%) were over age 50. Diagnosis was established by renal biopsy or autopsy. 11 cases (79%) were primary. 3 cases (21%) were secondary with chronic infection. Besides renal involvement, primary amyloidosis principally affected the heart, gastrointestinal tract and tongue. Hepatosplenomegaly and anemia were found in secondary cases. Proteinuria was found in 14 cases. Nephrotic Syndrome (NS) found in 8 cases, both renal insufficiency and hypertension were 21% respectively. In 3 cases performed renal venography, 2 cases showed renal vein thrombosis. 9 cases (64%) showed kidneys were enlarged. Congo red test showed positive reaction were 100% in secondary and 50% in primary. By light microscopy stained with congo red there was a specific protein deposited in glomeruli and mesangium, by electron microscopy. There was fine amyloid fibrils. These findings are characteristic and to have diagnostic value.
(1) Renal amyloidosis is not very rare in China. (2) Proteinuria and NS were major clinical manifestations. (3) Renal biopsy and autopsy have diagnostic value, congo red test does not prove the absence of primary amyloidosis.
1963年至1988年间对14例病例进行了调查。年龄在25至66岁之间（平均48.2岁）。9例（64%）年龄超过50岁。通过肾活检或尸检确诊。11例（79%）为原发性。3例（21%）为继发于慢性感染。除肾脏受累外，原发性淀粉样变性主要累及心脏、胃肠道和舌。继发病例中发现肝脾肿大和贫血。14例均有蛋白尿。8例出现肾病综合征（NS），肾功能不全和高血压的发生率均为21%。3例行肾静脉造影，2例显示肾静脉血栓形成。9例（64%）显示肾脏增大。刚果红试验显示继发性病例100%呈阳性反应，原发性病例50%呈阳性反应。刚果红染色光镜检查显示肾小球和系膜中有特异性蛋白沉积，电镜检查显示有细小淀粉样纤维。这些发现具有特征性且有诊断价值。
（1）肾淀粉样变性在中国并非十分罕见。（2）蛋白尿和NS是主要临床表现。（3）肾活检和尸检具有诊断价值，刚果红试验不能排除原发性淀粉样变性。
