苯扎贝特治疗儿童ApoE Tokyo(Arg25Cys)突变型脂蛋白肾病1例

脂蛋白肾病(Lipoprotein glomerulopathy,LPG),1989年首次由日本学者Saito报道,LPG主要累及肾脏,且以肾小球病变为主[1]。几乎所有患者均有不同程度的蛋白尿,多数表现为肾病综合征,少数表现为轻微蛋白尿和镜下血尿,部分患者伴有不同程度的贫血及高血压,血脂异常易被忽略为肾病综合征的低蛋白血症所致。载脂蛋白E(apolipoprotein E,ApoE)增高是LPG血脂改变的主要特点[2-3]。LPG为一种与脂质代谢紊乱密切相关的肾脏疾病,目前世界范围内有报道的病例不足200例,儿童报道仅10余例[2]。本病进展缓慢,临床常误诊为原发性肾病综合征[4]。因此,为增强对LPG的认识,提高诊治水平,现分析1例确诊的儿童LPG临床资料,总结LPG的临床特点、诊断、治疗及预后。     

Retinal disease in the C3 glomerulopathies and the risk of impaired vision

Background: Dense deposit disease and atypical hemolytic uremic syndrome are often caused by Complement Factor H (CFH) mutations. This study describes the retinal abnormalities in dense deposit disease and, for the first time, atypical haemolytic uremic syndrome. It also reviews our understanding of drusen pathogenesis and their relevance for glomerular disease. Methods: Six individuals with dense deposit disease and one with atypical haemolytic uremic syndrome were studied from 2 to 40 years after presentation. Five had renal transplants. All four who had genetic testing had CFH mutations. Individuals underwent ophthalmological review and retinal photography, and in some cases, optical coherence tomography, and further tests of retinal function. Results: All subjects with dense deposit disease had impaired night vision and retinal drusen or whitish-yellow deposits. Retinal atrophy, pigmentation, and hemorrhage were common. In late disease, peripheral vision was restricted, central vision was distorted, and there were scotoma from sub-retinal choroidal neovascular membranes and atypical serous retinopathy. Drusen were present but less prominent in the young person with atypical uremic syndrome due to a heterozygous CFH mutation. Conclusions: Drusen are common in forms of C3 glomerulopathy caused by compound heterozygous or heterozygous CFH mutations. They are useful diagnostically but also impair vision. Drusen have an identical composition to glomerular deposits. They are also identical to the drusen of age-related macular degeneration, and may respond to the same treatments. Individuals with a C3 glomerulopathy should be assessed ophthalmologically at diagnosis, and monitored regularly for vision-threatening complications.     

Antibody Response Against the Glomerular Basement Membrane Protein Agrin in Patients with Transplant Glomerulopathy

Chronic allograft nephropathy (CAN) of renal allografts is still the most important cause of graft loss. A subset of these patients have transplant glomerulopathy (TGP), characterized by glomerular basement membrane (GBM) duplications, but of unknown etiology. Recently, a role for the immune system in the pathogenesis of TGP has been suggested. In 11 of 16 patients with TGP and in 3 of 16 controls with CAN in the absence of TGP we demonstrate circulating antibodies reactive with GBM isolates. The presence of anti-GBM antibodies was associated with the number of rejection episodes prior to diagnosis of TGP. Sera from the TGP patients also reacted with highly purified GBM heparan sulphate proteoglycans (HSPG). Indirect immunofluorescence with patient IgG showed a GBM-like staining pattern and colocalization with the HSPGs perlecan and especially agrin. Using patient IgG, we affinity purified the antigen and identified it as agrin. Reactivity with agrin was found in 7 of 16 (44%) of patients with TGP and in 7 of 11 (64%) patients with anti-GBM reactivity. In conclusion, we have identified a humoral response against the GBM-HSPG agrin in patients with TGP, which may play a role in the pathogenesis of TGP.     

肥胖相关性肾病诊疗研究进展

肥胖相关性肾病已呈流行发展趋势,已成为终末期肾病的主要原因之一。目前认为其发病机制主要与肥胖所致肾小球血流动力学改变和脂质异位沉积等相关,但其机制仍未完全阐明。减轻体重是治疗肥胖相关性肾病的重要方法,但常因环境、个人执行情况的影响而收效甚微,且有部分患者体重控制后肾病仍持续进展。综合应用有效的肥胖相关性肾病治疗方法,对控制其进展十分重要。该文综述肥胖相关性肾病的致病机制和诊疗进展,探讨中药在肥胖相关性肾病治疗中的价值,旨在为临床提供参考,提高肥胖相关性肾病一体化管理水平。     

Alterations in extracellular matrix components in transplant glomerulopathy

The distribution pattern of extracellular matrix (ECM) components in transplant glomerulopathy was studied in relation to light microscopic features, actin expression of mesangial cells, and intraglomerular inflammatory cells. Nine cases of mild (group I) and nine cases of severe (group II) transplant glomerulopathy were stained with antisera against fibronectin (FN), tenascin (TN), collagen types III and IV, smooth muscle actin, CD45RO, CD68, and Ki-67 antigen. The composition of ECM was similar in the two groups. The expanded mesangium was diffusely stained by type-IV collagen, FN and TN, and focally and weakly stained by type-III collagen and smooth muscle actin. Type-IV collagen was linearly stained along the capillary walls, imparting a double-contour feature, whereas FN and TN showed granular staining along the capillary walls. CD68 positive cells were increased in severe transplant glomerulopathy, but this increase was not related to ECM deposition. These findings suggest that increased glomerular deposition of normal and abnormal ECM components participate in the evolution of transplant glomerulopathy. Received: 5 October 1999 / Accepted: 17 January 2000     

雷公藤多甙片加益肾活血法治疗IgM相关性肾小球疾病

目的探讨IgM相关性肾小球疾病的治疗。方法53例患者分为雷公藤多甙片组(A组)和雷公藤多甙片＋中药益肾活血(B组)治疗,疗程2个月。结果两组治疗前后尿蛋白均下降（P＜0．05）,组间比较则B组降低更明显(P＜0．05)。尿红细胞均有减少,A组P＞0．05,B组P＜0．05;组间比较P＜0．05。结论雷公藤多甙片＋中药益肾活血法治疗IgM相关性肾小球疾病较单用雷公藤多甙片疗效显著提高。认为辨病辨证相结合组方用药,能够提高疗效。     

赵玉庸教授治疗原发性肾小球疾病经验介绍

赵玉庸教授认为，原发性肾小球疾病的病机是湿热蕴结、肾络瘀阻、脾肾亏虚，结合临床分析各病机因素在疾病过程中的作用与影响，拟定清热利湿、化瘀通络、补脾益肾的治疗大法。     

The clinicopathologic spectrum of segmental membranous glomerulopathy

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Correlation analysis between serum α-klotho level and the development of obesity-related glomerulopathy

Objective To detect the level of serum α-klotho in different obese people and to investigate the correlation between serum α-klotho and obesity-related glomerulopathy (ORG). Methods A total of 48 cases of ORG diagnosed by renal biopsy were enrolled in the study. Forty-eight gender-, age- and BMI- matched obese participants, and 48 obese chronic kidney disease (CKD) patients without ORG were included as controls. The clinical manifestations, laboratory examinations of all three groups were collected, and the level of serum α-klotho protein was measured by ELISA. Results The patients with ORG were characterized by decreased serum α-klotho concentration compared with obese patients group and obese CKD patients group [572.66(439.92, 690.58) pg/ml vs 635.85(559.52, 769.20) pg/ml and 690.30(516.15, 828.20) pg/ml, P＜0.01]. Multinomial multiple logistic regression analysis revealed that serum α-klotho (per 100 pg/ml increased) was independently associated with the prevalence of ORG, and the risk of ORG decreased by 35% in the obese participants (OR=0.652, 95% CI: 0.487-0.872) and 38% in CKD patients (OR=0.617, 95% CI: 0.453-0.832) respectively. Conclusions The level of serum α-klotho is significantly decreased in ORG and associated with the prevalence of ORG independently. Serum α-klotho may be a protective factor for ORG.