Paydas S
Cukurova University Faculty of Medicine, Department of Internal Medicine, Adana, Turkey.
Int Urol Nephrol. 1999;31(5):619-31. doi: 10.1023/a:1007152320216.
We studied a group of 59 patients with renal amyloidosis. Mean age (45 male, 14 female) was 33.05+/-13.04 years. All of the cases had secondary amyloidosis. The causes of secondary amyloidosis were as follows: familial Mediterranean fever (FMF) 18 (30.5%), pulmonary tuberculosis 12 (20.33%), chronic oseomyelitis 8 (13.55%), bronchiectasia 9 (15.25%), rheumatic diseases 4 (6.4%), Castleman's disease 1 (1.6%), unknown aetiology 7 (11.86%). Hypertension was detected in 15.3% of the cases. In patients with less than 20 ml/min creatinine clearance (Ccr) hypertension was found in 20%. Hypotension was detected in 6 patients and all of these cases had severe hypoalbuminaemia (<2.1 g/dl). Nephrotic range proteinuria (>3.5 g/day) was found in 75% of cases. Daily proteinuria was correlated with serum levels of albumin, total lipid and cholesterol, haematocrit and duration of disease. The mean Ccr was 51.03+/-40.60 ml/min. Twenty-nine per cent of patients had Ccr less than 20 ml/min. Renal, subcutaneous fat and rectal biopsies demonstrated amyloid in 100%, 20% and 57.6%, respectively, of patients tested. Patients with secondary amyloidosis were treated with colchicine in addition to the therapy of primary disease (in 6 patients). Nine patients died, and end-stage renal disease developed in 12 patients during four years of follow-up. Proteinuria disappeared or decreased in patients with secondary amyloidosis except secondary to collagen tissue disease, without advanced renal failure. Colchicine did not affect amyloid deposition in 2 patients with normal renal function and negative proteinuria, who were rebiopsied. It can be questioned that "Colchicine may have effect(s) for decrement on proteinuria". At least colchicine can be of use in secondary amyloidosis.
我们研究了一组59例肾淀粉样变性患者。平均年龄（45例男性，14例女性）为33.05±13.04岁。所有病例均为继发性淀粉样变性。继发性淀粉样变性的病因如下：家族性地中海热（FMF）18例（30.5%），肺结核12例（20.33%），慢性骨髓炎8例（13.55%），支气管扩张9例（15.25%），风湿性疾病4例（6.4%），Castleman病1例（1.6%），病因不明7例（11.86%）。15.3%的病例检测到高血压。肌酐清除率（Ccr）低于20 ml/min的患者中，20%发现有高血压。6例患者检测到低血压，所有这些病例均有严重低白蛋白血症（<2.1 g/dl）。75%的病例发现有肾病范围蛋白尿（>3.5 g/天）。每日蛋白尿与血清白蛋白水平、总脂质和胆固醇、血细胞比容及病程相关。平均Ccr为51.03±40.60 ml/min。29%的患者Ccr低于20 ml/min。肾活检、皮下脂肪活检和直肠活检显示，分别有100%、20%和57.6%接受检测的患者存在淀粉样物质。除6例患者外，继发性淀粉样变性患者在治疗原发性疾病的基础上加用秋水仙碱进行治疗。在四年的随访期间，9例患者死亡，12例患者发展为终末期肾病。除胶原组织病继发的继发性淀粉样变性且无晚期肾衰竭外，继发性淀粉样变性患者的蛋白尿消失或减少。秋水仙碱对2例肾功能正常且蛋白尿阴性的患者再次进行活检时，未影响淀粉样物质沉积。“秋水仙碱可能对减少蛋白尿有作用”这一点值得怀疑。至少秋水仙碱可用于继发性淀粉样变性。
