扁桃体摘除可否阻止IgA肾病的进展

IgA肾病的免疫组织学改变是在肾小球基膜有IgA和补体C_3颗粒沉着。本病为免疫复合病之一。IgA抗体的产生与口腔粘膜局部的感染有关。病程中常由于扁桃体炎而反复出现肉眼血尿。故认为IgA肾病的发病、发展、加重均与上呼吸道感染有关。口腔扁桃体摘除是IgA肾病的治疗方法之一,它以除去感染源、改善免疫状态为目的。作者调查了IgA肾病扁桃体摘除和经治的2例,其结果术后蛋白尿、血尿改善者占25～100%,11个报告中有6个尿检改善占100%,同时血清IgA水平降低,也有肾功能改善的病例。由上所述,扁桃体摘除后,扁桃体炎及咽炎发病机     

扁桃体摘除对IgA肾病血清MMP-2的影响

目的：通过检测IgA肾病（IgAN）患者扁桃体摘除前后血清基质金属蛋白酶-2（MMP-2）水平,并结合尿检异常的变化,探讨扁桃体摘除对IgAN的影响及可能的机制。方法：采用明胶酶谱法（SDS-PAGE enzymography）检测8例正常人和33例IgAN患者治疗前后血清MMP-2酶原及活酶的水平,其中包括扁桃体摘除组15例、非手术对照组18例。结果：血清MMP-2活酶在IgAN患者较正常对照组明显升高（P〈0.01）,经过治疗下调,其中以扁桃体摘除组下调更显著,同非手术对照组比较有统计学差异（P〈0.01）。血清MMP-2酶原水平在各组间未发现差异。结论：扁桃体摘除联合传统治疗较单纯传统治疗者血清MMP-2活酶下调更为显著,可能是扁桃体炎症影响IgAN的机制之一;测定血清MMP-2的水平一定程度上可以了解IgAN的病情,评价治疗效果。     

扁桃体与IgA肾病的发病和治疗

自从1968年法国学者Berger首先报道IgA肾病以来,30多年的研究表明,以肾小球系膜区IgA沉积为特征的IgA肾病是最常见的、慢性进展的原发性肾小球疾病。因其发病机制不明,至今仍缺乏行之有效的治疗[1]。由于IgA肾病患者扁桃体感染后常常出现肉眼血尿或尿检异常加重,因此IgA肾病与扁桃体的关系一直受到人们的关注,文章也很多,但系统阐述扁桃体与IgA肾病关系的专论或综述很少。现根据我们最近在国际肾脏病杂志上发表的2篇有关IgA肾病与扁桃体的论著[2]和综述[3],对其二者之间关系进一步深入阐述,从而加深大家对IgA肾病发病机制和治疗的进…     

IgA肾病及非肾炎患者腭扁桃体摘除前后尿和血清IgA1水平比较

IgA肾病(IgAN)占我国原发性肾小球疾病20%～47%[1].机械刺激、化学刺激或腭扁桃体摘除术后,部分IgAN患者血尿和(或)蛋白尿加重[2,3].术后短时间内其血清IgA1有否变化,目前尚未检索到有关报道.我们比较IgAN及非肾炎患者腭扁桃体摘除术后血尿、蛋白尿、血清IgA、IgA1和C3水平变化,探讨扁桃体与IgAN间关系.     

3例多囊肾病合并原发性IgA肾病病例分析

目的常染色体显性遗传多囊肾病（autosomal dominant polycystic kidney disease,AD-PKD）发病率为1／1000-1／400,是主要由PKD1或PKD2基因突变而引起的遗传性肾病。ADPKD合并IgA肾病（IgAnephropathy,IgAN）的病例临床上较为少见,可伴有肾病综合征。本研究旨在探讨ADPKD合并原发性IgAN的病理特点和治疗方案。方法对3例ADPKD并IgAN患者的临床表现、ADPKD家族史、实验室检查、病理诊断及预后进行回顾性分析。结果3例患者发病年龄31-53岁,均以少尿、水肿、大量蛋白尿为主要症状,肾穿刺活检术后诊断为1例HassII型IgAN和2例HassI型IgAN。病例1给予泼尼松联合环磷酰胺治疗,病例2给予泼尼松联合吗替麦考酚酯治疗,病例3单用泼尼松治疗。经过免疫抑制治疗后,患者大量蛋白尿和血尿均得到缓解。虽然患者随访时总肾脏体积仍出现增长,但长期肾功能保持良好。结论ADPKD伴大量蛋白尿根据囊泡位置尽可能开展肾活检。ADPKD并IgAN的患者应根据分型给予循证支持的免疫抑制治疗,可以减少蛋白尿,有助于预防肾衰竭的发生。     

尿激酶联合苯那普利治疗IgA肾病的随访对照研究

目的 观察联合应用尿激酶(UK)和血管紧张素转换酶抑制剂(ACEI)苯那普利治疗IgA肾病(IgAN)的效果。方法 将71例Lee分级≥Ⅲ级的IgAN患者随机分为两组:UK+ACEI组及ACEI组,随访观察两组的疗效。结果 (1)12个疗程后,UK+ACEI组24h尿蛋白定量明显下降(P<0.01),血白蛋白(AIb)水平升高(P<0.05),疗效优于ACEI组。(2)治疗前应用Katafuchi IgA肾病积分系统进行IgAN的病理评分,在肾小球积分≥7分的患者中,治疗至12个疗程时,UK+ACEI治疗效果优于ACEI组(P<0.05)。(3)UK+ACEI组中有10例患者进行了重复肾活检,经治疗后多数患者病理改变保持稳定。结论 UK联合ACEI治疗中重度IgAN安全有效,疗效优于单用ACEI者。肾小球硬化及间质炎细胞浸润的程度可作为估计UK治疗IgAN效果的指标。     

IgA肾病的治疗

Ｂｅｒｇｅｒ在只有血尿的正常人中，观察到 ＩｇＡ沉积于肾脏的系膜，ＩｇＡ肾病由此而来。这些病人表现为反复发作的上感后血尿及单纯性血尿、蛋白尿。ＩｇＡ肾病是终未期肾功能衰竭的常见原因之一，但总的预后可能比文献中提到要好！因一些单纯性血尿的病人往往没有进行肾活检，且许多发表的文章很少涉及这类病人。本文章对 １９７６年以来成人 ＩｇＡ肾病治疗的研究进行总结、分析，并提出 ＩｇＡ肾病合理的治疗方案。若病人每日尿蛋白大于３ｇ，肾小球改变轻微，肌酐清除率大于７０ｍｌ／ｍｉｎ，建议应用强的松。类固醇激素可减少尿蛋…     

IgA肾病的治疗

IgA肾病,是以IgA为主的免疫球蛋白在肾小球系膜区弥漫沉积所致的肾小球损害。不难看出,IgA肾病系免疫病理学诊断名词,即肾活检组织经免疫荧光染色,在肾小球系膜区可见IgA为主的免疫球蛋白颗粒状沉积,常伴有C3沉积,而少见补体的前期产物如C1q和C4的沉积。     

IgA肾病患者腭扁桃体单个核细胞IgA类别转换基因表达变化

目的 研究脂多糖(LPS)或溶血性链球菌(HS)刺激IgA肾病和非肾脏疾病慢性扁桃体炎患者腭扁桃体单个核细胞Iα-Cα胚系转录本、激活诱导的胞嘧啶脱氨酶(AID)mRNA和蛋白的表达,以探讨IgA肾病腭扁桃体单个核细胞IgA及IgA1产生异常的分子机制.方法 入组2009年1月到2010年2月在我院住院的IgA肾病患者27例,非肾脏疾病慢性扁桃体炎患者27例作为对照.通过单个核细胞分离液和密度梯度离心法分离出腭扁桃体单个核细胞.IgA肾病组及非肾脏疾病慢性扁桃体炎组腭扁桃体单个核细胞分别分为3组:LPS刺激组,HS刺激组和未刺激组.ELISA法检测培养上清中IgA和IgA1的浓度.实时PCR检测Iα-Cα胚系转录本和AID mRNA的表达;Western印迹检测AID蛋白的表达.结果 IgA肾病组腭扁桃体单个核细胞IgA和IgA1的分泌,特别是IgA1/IgA较慢性扁桃体炎组显著增加(P＜0.05),Iα-Cα和AID mRNA和AID蛋白的表达较慢性扁桃体炎组显著增加(均P＜0.05).IgA肾病组腭扁桃体单个核细胞IgA和IgA1的水平在刺激后明显增加(P＜0.05);Iα-Cα和AID mRNA的表达明显上调(均P＜0.05);AID蛋白表达明显增加(LPS刺激组P＜0.05,HS刺激组P＜0.01).结论 LPS和HS均能够诱导IgA肾病患者腭扁桃体单个核细胞IgA和IgA1的分泌、AID和Iα-Cα的表达增加,提示IgA肾病患者腭扁桃体IgA和IgA1的分泌增加可能与IgA类别转换相关基因AID和Iα-Cα高表达有关.     

IgA肾病：扁桃体黏膜与肠黏膜之争

<正>IgA肾病(IgA Nephropathy, IgAN)是全世界最常见的原发性慢性肾小球疾病^[1],是一组以IgA在肾小球系膜区或者毛细血管袢沉积为特征,通常伴有不同程度的IgG和(或)IgM存在,同时存在补体C3沉积的免疫复合物性肾小球肾炎^[2,3]。其典型临床表现是青年男性在上呼吸道或胃肠道黏膜感染后24～48 h出现无痛性肉眼血尿,这表明黏膜免疫系统在IgAN的进展中起着重要作用^[4,5]。据报道,     

Efficacy of tonsillectomy pulse therapy versus multiple-drug therapy for IgA nephropathy

We evaluated the efficacy of tonsillectomy plus pulse prednisolone, warfarin, and dipyridamole including methylprednisolone pulse (tonsillectomy plus pulse therapy), versus prednisolone, warfarin, and dipyridamole including mizoribine (PWDM) for the treatment diffuse IgA nephropathy (IgAN) in children. The patients were randomly assigned to be treated by tonsillectomy plus pulse therapy for 2 years (Group A, n=16) or PWDM for 2 years (Group B, n=16). The clinical features and pathological findings in both groups were analyzed prospectively. The mean urinary protein excretion after 6 months of treatment in both groups had decreased significantly compared with pre-therapy. The activity index (AI) in both groups was lower at the time of the second biopsy than at the time of the first biopsy. The chronicity index (CI) in Groups A and B did not differ between the first and second biopsy. At the latest follow-up examination none (0%) of the patients in either group had renal insufficiency. None of the patients in Group A, but six patients in Group B experienced an acute exacerbation of IgAN as a result of tonsillitis (P<0.05). In conclusion, although there was no untreated control group in this study, the results suggested that tonsillectomy plus pulse therapy is as effective as PWDM in ameliorating proteinuria and histological severity in IgAN patients and in preventing acute exacerbation of IgAN by tonsillitis.     

Significance of tonsillectomy combined with steroid pulse therapy for IgA nephropathy with mild proteinuria

Background

Medical intervention for patients with IgA nephropathy and mild proteinuria (<1.0 g/day) is controversial, and the effectiveness of tonsillectomy plus steroid pulse therapy (TSP) for such patients remains obscure.

Methods

Among 323 patients in our multicenter cohort study, 79 who had mild proteinuria (0.4–1.0 g/day) at diagnosis were eligible to participate in this study. We compared the clinicopathological findings at diagnosis, a decline in renal function defined as a 50 or 100 % increase in serum creatinine (sCr) and clinical remission (CR) defined as the disappearance of hematuria and proteinuria (<0.3 g/day) among groups given TSP (n = 46), steroid therapy (ST) (n = 9), and non-ST (n = 24). Factors contributing to CR were also evaluated using multivariate analysis.

Results

Background factors at diagnosis including age, ratio (%) of patients with hypertension, sCr, proteinuria, and histological severity did not significantly differ among the groups. Only two patients each in the TSP (4.3 %) and non-ST (8.3 %) groups achieved a 50 % increase in sCr during a mean follow–up period of 4.7 years. At the final observation, 71.7, 44.4, and 41.7 % of patients in the TSP, ST, and non-ST groups, respectively, achieved CR (p = 0.032). Cox proportional hazards models revealed that TSP led to CR more effectively than non-TSP by a factor of about threefold (hazard ratio, 2.74; p = 0.008).

Conclusion

TSP therapy has potential for inducing CR in patients with IgAN and mild proteinuria (<1.0 g/day).

     

MPO-ANCA-positive IgA nephropathy successfully treated with tonsillectomy

A 20-year-old Japanese woman was admitted to a hospital because of gross hematuria. She was diagnosed with IgA nephropathy with a poor prognosis, based on the formation of many crescents in the glomerulus and monocyte infiltration in the interstitium in a renal biopsy specimen in February 2003. Myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA) was not identified at that time. After treatment with high-dose steroid pulse therapy and heparin/warfarin, her urinary protein improved, to 0.5 g/day. However, 1 year after the steroid pulse therapy, urinary protein was increased to 1.2 g/day, associated with repeated episodes of tonsillitis. A second renal biopsy was performed, and showed an improving tendency, compared to the findings of the previous one, although some crescent formation and adhesions of Bowman's capsule remained. Interestingly, MPO-ANCA was positive in the serological examination done at this time. One month and a half after the second renal biopsy, she had a tonsillectomy, followed by a regimen of 5 mg oral prednisolone daily, in order to prevent the progression of IgA nephropathy. After the tonsillectomy, her urinary protein level was markedly improved, at 0.14 g/day. Her creatinine clearance was ameliorated, at 102 ml/min, and in addition, MPO-ANCA had disappeared. This case suggests that an inflammation such as tonsillitis may be associated not only with the activity of IgA nephropathy but also with the production of MPO-ANCA.     

Comparison between steroid pulse therapy alone and in combination with tonsillectomy for IgA nephropathy

Purpose

To the best of our knowledge, no study has compared intermittent steroid pulse therapy, according to Pozzi’s regimen, with versus without tonsillectomy.

Methods

In this retrospective cohort analysis, we compared clinical findings, histological findings according to the Oxford classification, and complete remission rates (RR), defined in terms of urinary protein excretion (U-Prot <0.3 g/g creatinine) and urinary red blood cell count (U-RBC <5/high-power field), after 1 year of treatment in patients with IgA nephropathy (IgAN), who received tonsillectomy with steroid pulse therapy (TSP group, n = 26) or steroid pulse therapy alone (SP group, n = 15).

Results

The baseline clinical and histological characteristics did not differ between the two groups. The RR for U-Prot analyzed by the Kaplan–Meier method did not differ between the groups (76.9 vs. 53.3 %). However, the RR for U-RBC was significantly higher in the TSP than in the SP group (88.4 vs. 33.3 %, log-rank test; P = 0.0008). The RRs for U-Prot and U-RBC were significantly higher in the TSP group than in the SP group (69.2 vs. 13.3 %, log-rank test; P = 0.0019). Cox’s regression analysis showed that combination therapy was associated with higher RR (odds ratio, 12.5; 95 % confidence interval, 2.91–86.7; P = 0.0002).

Conclusions

Tonsillectomy combined with steroid pulse therapy achieved higher RR after 1 year of treatment, compared with steroid pulse monotherapy in patients with IgAN. The long-term effects on renal survival should be analyzed in further studies.     

Comparison of long-term follow-up outcomes between multiple-drugs combination therapy and tonsillectomy pulse therapy for pediatric IgA nephropathy

Background

To clarify the long-term efficacy of multiple-drugs combination therapy (PWDM) and tonsillectomy pulse therapy (TPT) for pediatric IgA nephropathy (IgAN), we retrospectively evaluated the clinical and laboratory findings as well as the prognosis for IgAN patients treated with each treatment at long-term follow-up.

Methods

We collected data on 61 children who had been diagnosed with severe IgAN. The children were retrospectively divided into two groups. Group 1 consisted of 44 severe IgAN children treated with PWDM, and Group 2 consisted of 17 severe IgAN children treated with TPT. The clinical features, pathological findings, and prognosis were analyzed for both groups.

Results

The mean urinary protein excretion, serum creatinine, IgA levels, MESTCG scores, and percentage of glomeruli showing crescents in both groups at the second renal biopsy were lower than those at the first renal biopsy. At the time of the second biopsy, the IgA level in Group 2 was lower than that in Group 1; however, there were no significant differences in the mean urinary protein excretion, frequency of hematuria, serum albumin, creatinine, or e-GFR between the two groups. At the most recent follow-up, there were no significant differences in prognosis between the groups.

Conclusions

Our study suggested that PWDM and TPT are effective in ameliorating urinary abnormalities and improving the long-term outcome of pediatric IgAN.