Dixit M P, Cabansag M R, Piscitelli J, Greifer I, Silverstein D M
Department of Pediatrics, Albert Einstein College of Medicine, Bronx, New York, USA.
Pediatr Nephrol. 1999 Feb;13(2):139-42. doi: 10.1007/s004670050582.
Amyloidosis is a complication of long-term hemodialysis treatment. The major histological feature of hemodialysis-associated amyloidosis (HAA) is the deposition of amyloid fibrils in the affected lesions, due, in part, to elevated serum beta2-microglobulin (beta2M) levels. In vitro studies reveal that serum immunoglobulin light and heavy chains co-deposit with beta2M in tissues affected by HAA. Only one study of HAA has been performed in young dialysis patients. We therefore assessed risk factors for HAA in a group (n=30) of young (18.7+/-0.9 years) patients receiving chronic, uninterrupted hemodialysis using cellulose acetate membranes. All patients initiated dialysis before reaching 18 years of age. The pre-dialysis serum beta2M level was 49.7+/-3.9 mg/l (normal 0-2.4 mg/l). Since serum albumin was normal (4.3+/-0.1 mg/dl) and serum protein/albumin was elevated (1.7+/-0.0, normal 1.2-1.5), indicating increased circulating protein, we assayed immunoglobulins in the same patients. The serum immunoglobulin levels (expressed as a percentage of the total level of serum proteins) were elevated (21.3+/-0.9%, normal 11.1%-21.0%). The Kt/v was 1.37+/-0.03, suggesting that the high levels of serum beta2M and immunoglobulins were not due to inadequate dialysis in these patients. Patients with residual renal function (Kr) did display significantly lower serum levels of beta2M (33.2+/-2.3, P=0.03). Furthermore, improved clearance of beta2M correlated with higher values of Kr (r=0.914). In contrast, serum levels of immunoglobulin (22.6+/-3.7, P=0.5) were unaffected by Kr. In addition, there was no correlation between older age at onset of dialysis and serum levels of either beta2M (r=0.107) or immunoglobulins (r=0.321). Finally, the length of time on dialysis had no effect on serum levels of either beta2M (r=0.105) or immunoglobulins (r=0.092). Taken together, these results indicate that young hemodialysis patients may be at risk for HAA.
淀粉样变性是长期血液透析治疗的一种并发症。血液透析相关淀粉样变性(HAA)的主要组织学特征是淀粉样纤维在受累病变部位沉积,部分原因是血清β2微球蛋白(β2M)水平升高。体外研究表明,血清免疫球蛋白轻链和重链与β2M在受HAA影响的组织中共同沉积。仅对年轻透析患者进行过一项关于HAA的研究。因此,我们评估了一组(n = 30)年轻(18.7±0.9岁)患者发生HAA的危险因素,这些患者使用醋酸纤维素膜进行慢性、不间断血液透析。所有患者在18岁之前开始透析。透析前血清β2M水平为49.7±3.9 mg/l(正常为0 - 2.4 mg/l)。由于血清白蛋白正常(4.3±0.1 mg/dl)且血清蛋白/白蛋白升高(1.7±0.0,正常为1.2 - 1.5),表明循环蛋白增加,我们检测了同一组患者的免疫球蛋白。血清免疫球蛋白水平(以血清蛋白总水平的百分比表示)升高(21.3±0.9%,正常为11.1% - 21.0%)。Kt/v为1.37±0.03,表明这些患者血清β2M和免疫球蛋白水平升高并非由于透析不充分所致。有残余肾功能(Kr)的患者血清β2M水平确实显著较低(33.2±2.3,P = 0.03)。此外,β2M清除率的提高与较高的Kr值相关(r = 0.914)。相比之下,免疫球蛋白血清水平(22.6±3.7,P = 0.5)不受Kr影响。此外,透析开始时年龄较大与血清β2M水平(r = 0.107)或免疫球蛋白水平(r = 0.321)之间均无相关性。最后,透析时间长短对血清β2M水平(r = 0.105)或免疫球蛋白水平(r = 0.092)均无影响。综上所述,这些结果表明年轻血液透析患者可能有发生HAA的风险。