Background:Steroid resistance (SR) and a family history of kidney disease (FH) are key indicators of genetic focal segmental glomerulosclerosis (FSGS) in adults. The diagnostic yield and prognostic implications of genetic testing in these patients remain unclear. Methods:The study was designed in two parts to address specific research objectives. Part 1 aimed to explore the diagnostic yield in adult FSGS patients with SR and/or FH. Part 2 aimed to investigate the clinical significance of different gene mutation types in patients with genetic FSGS. Part 1 included FSGS patients who received next-generation sequencing (NGS) because of SR and/or an FH (n = 32). According to the results of NGS, the patients were divided into the mutation group (n = 18) and the non-mutation group (n = 14). Part 2 involved patients with genetic FSGS (n = 19), who were divided into two groups: the podocyte-related gene mutation group (PO group) and the type IV collagen gene mutation group (IV-col group). The clinical, pathological, and prognostic characteristics of patients in different groups were analyzed separately. Results:In part 1, the mutation carriers showed higher FH prevalence (p = 0.027), reduced acute kidney injury (p = 0.01), milder tubulointerstitial lesions (p = 0.03), and more interstitial foam cells (p = 0.01), with foam cells emerging as a strong genetic FSGS predictor. The mutation group demonstrated superior 2-year kidney survival (p = 0.03), but no advantages in long-term kidney outcome. Part 2 revealed IV-col patients showed later onset (p = 0.03), higher serum creatinine (p = 0.01), heavier proteinuria (p = 0.02), and less hematuria (p = 0.04) vs. the PO group, though kidney outcomes showed no statistical differences. Conclusion:A diagnostic yield of 56.3% diagnostic yield supports routine genetic testing in FSGS patients with SR/FH, and the gene mutations were associated with a better short-term kidney prognosis but no differences in long-term kidney prognosis. Foam cell infiltration strongly predicts the genetic etiology of FSGS. IV-col mutations associate with insidious nephropathy onset of FSGS but similar prognosis to the podocyte mutations.

BackgroundCardiovascular disease (CVD) is a major complication in peritoneal dialysis (PD) patients. Previous studies have demonstrated that platelet distribution width (PDW) is associated with cardiovascular events in hemodialysis (HD) patients. In this study, we hypothesized that elevated PDW can predict all-cause and cardiovascular mortality in PD patients.MethodsWe recruited PD patients for a single-center retrospective cohort study from 1 January 2007, to 30 June 2020. Receiver-operating characteristic (ROC) curves were made to determine the PDW cutoff value for predicting all-cause mortality. The propensity score matching (PSM) method was used to improve the equilibrium between groups. The relation of PDW with all-cause and cardiovascular mortality was analyzed by Cox proportional hazards models. Restricted cubic spline (RCS) models were used to determine whether there was a linear relationship between PDW and all-cause and cardiovascular mortality.ResultsA total of 720 PD patients were screened, and 426 PD patients were enrolled after PSM. After adjusting for confounders, Cox proportional hazards models showed that the PDW value was positively correlated with the risk of all-cause and cardiovascular mortality (HR = 1.162, 95% CI 1.057-1.278, p = 0.002 and HR = 1.200, 95% CI 1.041-1.382, p = 0.012). The adjusted RCS analysis further showed that the relationship of PDW with all-cause and cardiovascular mortality was linear (p for nonlinearly = 0.143 and 0.062).ConclusionElevated PDW is independently associated with all-cause and cardiovascular mortality in PD patients.

Abstract Objective This study aimed to explore the role of glomerular lesions in patients who suffered from acute kidney injury (AKI) during hemorrhagic fever with renal syndrome (HFRS). Methods The study comprised 66 patients with AKI during HFRS treated at the National Clinical Research Center of Kidney Diseases of China, Jinling Hospital, from January 2014 to December 2018. According to the kidney pathological findings, the 66 patients were divided into two groups: the tubulointerstitial injury group (HFRS-TI group, n = 43) and the tubulointerstitial injury with glomerular lesions group (HFRS-GL group, n = 23). The clinical and pathological characteristics of the 66 patients were analyzed. Results There were 9 cases of IgA nephropathy, 1 case of membranous nephropathy, 2 cases of diabetic nephropathy, and 11 cases of mesangial proliferative glomerulonephritis in the HFRS-GL group. There were more males in the HFRS-GL group than in the HFRS-TI group (92.3% vs. 69.8%, p < .05). A higher proportion of interstitial fibrosis (56.5% vs. 27.9%, p < .05) and more immunoglobulin and complement depositions (p < .001) were observed in the HFRS-GL group than in the HFRS-TI group. Rates of remission of AKI were lower in the HFRS-GL group than in the HFRS-TI group (73.9% vs. 95.3%, p < .05). The presence of glomerular lesions (HR = 5.636, 95% CI = 1.121–28.329, p = .036) and moderate tubulointerstitial injury (HR = 3.598, 95% CI = 1.278–10.125, p = .015) were found to be independent risk factors for kidney prognosis. Conclusions Patients with AKI during HFRS can have glomerular lesions or glomerulonephritis. Patients with AKI during HFRS who have glomerular lesions or moderate renal tubulointerstitial injury proven by kidney biopsy have a relatively poor kidney prognosis. A kidney biopsy can help determine long-term prognosis in patients with AKI during HFRS.

目的:回顾性分析慢性肾脏病(CKD)合并新型冠状病毒肺炎(NCP)患者的临床特征及预后.方法:纳入2022年12月至2023年6月国家肾脏疾病临床医学研究中心收治的CKD合并NCP患者,分析新型冠状病毒感染(COVID-19)前、后的临床特征及随访情况.结果:共纳入72例患者,男性39例(54.17％),COVID-19时中位年龄64(39,76)岁,CKD中位病程36(7,120)月.导致CKD的病因,主要为糖尿病肾病(22.22％)、狼疮性肾炎(12.50％)、膜性肾病(9.72％)和抗中性粒细胞胞质抗体相关肾炎(9.72％),新冠病毒疫苗接种率为18.06％.NCP中型占51.39％、重型20.83％、危重型27.78％.14例(19.44％)患者院内死亡,死亡组患者治疗期间炎症指标、真菌感染、心力衰竭、肝功能受损、气胸的比例高于存活组,淋巴细胞亚群均低于存活组(P＜0.05).多因素COX模型发现,年龄、乳酸脱氢酶、CD4+T细胞、C反应蛋白是CKD合并NCP患者死亡的独立危险因素.结论:CKD合并NCP患者,疫苗接种率低,伴发疾病多.高龄、缺氧、免疫功能低下、炎症反应强的患者预后差.治疗过程中易出现混合或重叠感染和多器官功能衰竭.

BACKGROUND:Polycythemia vera (PV) is a myeloproliferative neoplasm which is characterized by excessive production of erythrocytes as well as myeloid and megakaryocytic proliferation. PV associated with IgA nephropathy (IgAN) has rarely been reported in the literature. The long-term renal prognosis of these patients is unknown. METHODS:Clinical and pathological characteristics of 7 patients with renal biopsy-proven IgAN associated with PV were retrospectively analyzed. RESULTS:The 7 patients were all males, with a mean age of 49.1 ± 18.8 years when admitted to our hospital. Systemic symptoms include: hypertension in case 2, 3, 5, and 6, splenomegaly in case 2, 4, and 5, and multiple lacunar infarction in case 6. Bone marrow biopsy test revealed relative erythroid hyperplasia and atypical megakaryocyte proliferation which consistent with a chronic myeloproliferative neoplasm. All patients had JAK2V617F and BCR-ABL tested, and JAK2V617F positive in 2 patients. Mild mesangial proliferation was observed in 5 patients and moderate/severe mesangial proliferation in 2patients. Immunofluorescence mainly showed diffuse granular deposition of dominant IgA in mesangium. After follow-up of 56.7 ± 44.0 months, hemoglobin level was 144 ± 29 g/L and hematocrit lever was 0.470 ± 0.03, compared with 187 ± 29 g/L and 0.563 ± 0.087 respectively when admitted to our hospital. The urine protein was 0.85 ± 0.64 g/24 h compared with 3.97 ± 4.68 g/24 h. Case 3 progressed to end stage renal disease and had received hemodialysis for 5 years before renal transplantation. CONCLUSIONS:The results of this study showed that PV associated with IgAN mainly occurs in males and is often accompanied by hematuria and mild-to-moderate renal insufficiency. The long-term prognosis was good for most patients, and few progressed relatively quickly to end stage renal disease.

目的:观察罗沙司他和重组人促红细胞生成素(rHuEPO)对腹膜透析(PD)肾性贫血患者血压、心血管指标和心脑血管并发症的影响.方法:本文回顾性分析2019年6月至2020年4月国家肾脏疾病临床医学研究中心服用罗沙司他的PD患者50例,按照1∶1的比例随机选取皮下注射rHuEPO的PD患者50例,比较两组患者在治疗前后的血红蛋白(Hb)、血压、心血管指标、心脑血管并发症.所有患者至少随访18个月.结果:两组基线临床资料及实验室检查指标均无统计学差异;随访18个月后两组Hb较基线值均显著升高,两组间Hb无统计学差异.罗沙司他组治疗前后收缩压、舒张压及夜间高血压发生率无明显变化,rHuEPO组治疗后收缩压、舒张压均显著升高,夜间高血压发生率增多(P＜0.05).罗沙司他组治疗前后心血管指标无明显差异.rHuEPO组治疗后氨基末端脑钠肽(NT-proBNP)、肌钙蛋白T(TnT)、肌钙蛋白Ⅰ(TnⅠ)、心胸比升高,左心室射血分数(LVEF)下降,心脑血管并发症增多(P＜0.05);与罗沙司他组相比,rHuEPO组夜间高血压发生率高,NT-proBNP、TnT、TnⅠ、心胸比高,LVEF低,心脑血管并发症多(P＜0.05).多因素COX回归分析发现基线空腹血糖及使用rHuEPO是PD患者发生心脑血管并发症的独立危险因素,罗沙司他治疗对心脑血管具有独立保护作用(HR 0.208,95％CI 0.057～0.761,P=0.018).结论:PD患者使用罗沙司他与rHuEPO相比,对血压及心血管指标影响较小,降低了血压升高及发生心脑血管并发症的风险,在PD肾性贫血患者中使用具有心脑血管保护优势.

目的:探讨贝利尤单抗联合标准方案治疗2例重型活动性狼疮肾炎(LN)的近期临床疗效和安全性.方法:前瞻性观察国家肾脏疾病临床医学研究中心诊治的2例重型活动性LN患者,在LN标准基础治疗上联合使用贝利尤单抗治疗,评估24周疗程中2例患者疾病转归和临床用药情况.结果:2例均为初发初治的女性LN患者,年龄分别为22岁和27岁;系统性红斑狼疮疾病活动度评分(SLE-DAI)分别为15分和17分;LN病理类型分别为Ⅳ-G(A)[活动指数(AI)13,慢性化指数(CI)0)和Ⅳ-G(AI 12,CI 0).2例均在甲泼尼龙冲击(MP)治疗后口服泼尼松(起始45 mg/d、30 mg/d),并接受多靶点疗法(MT)或吗替麦考酚酯(MMF)诱导治疗.均在MP后2周内启动贝利尤单抗治疗,用药方案为10 mg/kg静脉输注,1次/2周×3次,1次/4周×5次,共8次.治疗满24周后评估疗效:1例获得肾脏完全缓解,SLE-DAI下降至2分(补体C3 0.593 g/L),1例获得肾脏部分缓解,SLE-DAI评分降至8分(蛋白尿0.78 g/24h、尿沉渣红细胞计数15.8个/HPF).泼尼松减量至15 mg/d和10 mg/d,均无感染和肾功能恶化.结论:贝利尤单抗联合标准化方案治疗成人重型活动性LN,近期疗效好,SLE-DAI和肾脏损害指标缓解明显,激素可撤减至较低剂量维持.

It is difficult to treat cardiorenal syndrome (CRS) in clinical practice, which is the common reason for the death of patients. This report aimed to describe the effects of sacubitril/valsartan treatment on cardiac and renal functions of a patient with cardiorenal syndrome type 4 (CRS4) after more than 3 years of follow-up. A 77-year-old Chinese woman was admitted to our hospital because of CRS4 and stage 5 chronic kidney disease (CKD), who had a history of long-term proteinuria and renal failure. The patient's cardiothoracic ratio (CTR) measured by chest X-ray was 0.6. Cardiac ultrasonography showed that the left ventricular ejection fraction (LVEF) was 0.40. The patient had been treated for heart failure (HF) for 5 months, but there was no improvement in clinical manifestations, and the renal function gradually deteriorated. In our hospital, she received sacubitril/valsartan treatment for at least 40 months. The symptoms of HF relieved, and the indices of cardiac function improved. In addition, the patient's renal function was stable. During the treatment, the dosage of sacubitril/valsartan needed to be adjusted to achieve the optimal therapeutic effect. Follow-up results showed that she achieved cardiac function of New York Heart Association (NYHA) class II with an ejection fraction of 0.60 and E/A > 1 indicated by echocardiogram, and did not develop hyperkalemia. In summary, the improvement of cardiac and renal functions of the CRS4 patient was associated with the long-term sacubitril/valsartan treatment.

目的 分析沙库巴曲缬沙坦在心肾综合征(CRS)患者中的临床应用,评估患者的早期临床疗效及安全性.方法 回顾性分析2017年11月至2020年12月期间在东部战区总医院国家肾脏疾病临床医学研究中心就诊的CRS并接受沙库巴曲缬沙坦治疗3个月以上的25例患者,分析其临床疗效与安全性.结果 患者服药时间为8(4.5～14)个月,随访时间为16(8～29.5)个月.与治疗前相比,治疗3个月后氨基末端脑钠肽(NT?proBNP)下降[1429(506.4～3057)pmol/L vs 225(105.4～869.3)pmol/L,P<0.01)、平均动脉压(MAP)下降[(109.4±10.7)mmHg vs(94.7±12.3)mmHg,P<0.05)]、心率下降(P<0.01)、心功能NYHA分级改善,患者在治疗后估算的肾小球滤过率(eGFR)、血钾与治疗前相比差异无统计学意义(P>0.05).所有患者经治疗后不良反应发生较少,头晕、周围性水肿、低血压、高钾血症、血肌酐上升各1例.结论 沙库巴曲缬沙坦可在早期有效改善CRS患者的心功能指标、血压、心率及血白蛋白水平,患者肾功能及血钾水平稳定,不良反应发生率较低,预后好,故具有一定的有效性及安全性.

Minimal change disease（MCD）is one of the most common types of pathology for primary nephrotic syndrome, especially in children and adolescents.Patients with nephrotic syndrome are prone to a series of complications, such as infection, venous thromboembolism and acute kidney injury（AKI）,etc.In recent years, the incidence of nephrotic syndrome complicated with AKI has spiked markedly.And MCD complicated with AKI is the most common.MCD with AKI shall aggravate patient conditions, compound the difficulty of clinical treatment and bring a worse clinical prognosis.The mechanism of MCD associated with AKI involves a variety of different pathways, including a reduction of glomerular filtration rate（GFR）due to a lower effective circulating blood volume, nephronic ischemia resulting from renal vascular lesions and a direct toxic effect of proteinuria on renal tubules, etc.This review summarized clinical and basic researches in the field of MCD combined with AKI in recent years.It also described its incidence, pathophysiological mechanism, early diagnostic indicators and prevention and treatment principles for a better management of MCD combined with AKI.

目的:观察罗沙司他治疗腹膜透析(PD)肾性贫血患者的有效性及安全性.方法:回顾性分析2019年6月至2020年2月国家肾脏疾病临床医学研究中心口服罗沙司他的PD患者为罗沙司他组,按照1:1比例随机选取皮下注射重组人促红细胞生成素(rHuEPO)的PD患者为对照组,分析两组患者的一般资料、血红蛋白(Hb)、铁代谢指标、炎症指标、脂代谢指标、不良反应等,所有患者至少完成随访时间1年.结果:共收集罗沙司他组34例,对照组34例,两组患者基线临床资料及实验室指标无统计学差异,罗沙司他组基线平均Hb(85.3±11.4)g/L.两组患者经治疗后使Hb维持在110～120 g/L,1年后Hb较基线值均显著升高,两组患者Hb无明显差异(P=0.081),但与对照组相比,罗沙司他组患者的铁代谢指标、脂代谢指标、炎症指标、平均动脉压(MAP)、空腹血糖优于对照组(P＜0.05).与基线相比,罗沙司他治疗1年后血清铁、转铁蛋白饱和度(TSAT)和铁蛋白降低,总铁结合力升高(P＜0.05).罗沙司他组患者不同炎症状态下Hb无明显变化,而对照组炎症指标高的患者Hb更低(P＜0.05).罗沙司他组血压升高发生率低于对照组(0vs23.5％,P=0.005).结论:罗沙司他治疗PD肾性贫血疗效不低于rHuEPO,其疗效不受炎症状态影响,且可改善脂代谢、糖代谢和炎症指标,对血压影响较小,故具有较好的疗效及安全性.

关于IgA肾病的2点新认识 早年认为,IgA肾病主要临床表现为血尿,伴或不伴少量蛋白尿,但病程进展缓慢,预后良好.经过40多年的临床实践,对IgA肾病的认识已有了深刻的变化,主要体现在下面2个方面. 一是原发性IgA肾病的共性是肾小球系膜区有弥漫性的IgA沉积,但临床表现五花八门,从单纯的镜下血尿,镜下血尿伴或不伴蛋白尿、肉眼血尿,到高血压、水肿、大量蛋白尿、肾功能不全等,几乎所有肾小球疾病的临床症状和表现均可以出现.显微镜下肾小球病理形态改变也多种多样.

INTRODUCTION:Cell-mediated autoimmunity, especially the autoreactivity of T cells, is known to underlie the initiation of anti-glomerular basement membrane disease. However, the T lymphocyte subsets that determine the disease activity, renal fibrosis, and prognosis of anti-GBM disease have not been clearly elucidated. METHODS:The T lymphocyte subsets (CD4+ and CD8+) were examined on peripheral blood and renal biopsy tissues from 65 patients with biopsy proven anti-GBM disease. Patients were divided into the high ratio group and low ratio group according to the cutoff values in the receiver operating characteristic curve analysis. The correlations of T lymphocyte subsets with clinical, pathological data, and renal outcome were analyzed. RESULTS:By the end of follow-up, 45 patients (69.2%) developed end-stage renal disease (ESRD). In peripheral blood, the CD4+/CD8+ ratio showed a predictive ability with a sensitivity and specificity of 91.3% and 52.9%, respectively, which gave rise to a cutoff value of 0.89. There was a significant difference in the activity index between these two groups (3.91 ± 1.38 vs. 2.89 ± 1.13, p = 0.007). In the renal tissues, the CD4+/CD8+ ratio had the optimal cutoff point of 0.82 with a sensitivity of 57.8% and specificity of 85%. The renal activity index was higher for the renal tissues with high CD4+/CD8+ ratios than that of tissues with low CD4+/CD8+ ratios (4.32 ± 1.55 vs. 3.37 ± 1.41, p = 0.016). Peripheral blood CD4+/CD8+ ratios of ≥0.89 or renal tissue CD4+/CD8+ ratios of < 0.82 positively correlated with poor renal prognosis in patients with anti-GBM nephritis. CONCLUSIONS:The CD4+/CD8+ ratio was associated with renal activity index both in peripheral blood and renal tissue and predicts the renal prognosis of patients with anti-GBM nephritis.

环境污染是影响公众健康和死亡的重要原因之一,肾脏的血液供应占心输出量的20％,血液经过肾小球滤过后,在肾小管上皮细胞重吸收,环境毒素可以浓集,因而肾脏容易受到环境污染的损害.环境污染是造成慢性肾脏病(CKD)疾病谱变迁和不同地区疾病谱不同的重要的因素之一.近年,针对环境污染对肾脏损害的因素进行了大量的临床与基础研究,由于环境污染因素众多,对肾脏损害的机制和临床表现多种多样,环境污染对肾脏的损害没有得到应有重视和深刻的认识.

目的 感染是移植术后最常见的非心血管疾病死亡原因.文中分析和探讨肾移植受者围手术期感染的流行病学特征、危险因素以及预防性使用抗菌药物的合理性.方法 回顾性分析2015年1月至2019年3月于东部战区总医院接受肾移植术的303例受者围手术期感染的发生率、类型、来源、病原菌分布情况及危险因素,并比较不同级别抗菌药物的预防效果.结果 肾移植受者围手术期发生感染30例(9.90％),29例为医院感染(96.67％).其中最常见的感染为肺部感染(12/30,40.00％),感染病原体主要为G-菌26株(66.67％).心脏死亡器官捐献(DCD)肾移植受者围手术期感染发生率显著高于活体肾移植受者(12.78％vs 5.69％,χ2=4.114,P<0.05).DCD肾移植围术期使用不同级别的抗菌药物预防,感染发生率差异无统计学意义(10.47％vs 14.89％,χ2=0.790,P=0.374).Logistic回归分析表明,术中输血(OR=3.973,95％CI 1.092～14.455,P=0.036)、术后运用霉酚酸酯联合环孢素及激素的免疫抑制方案(OR=6.404,95％CI 1.216～33.717,P=0.028)是肾移植受者围手术期感染的独立危险因素.结论 肾移植受者围手术期易并发术后感染,且多为医院感染.DCD肾移植受者比活体肾移植受者更易发生围手术期感染,且预防性使用高级别的抗菌药物不能有效降低围手术期感染发生率,需针对危险因素采取有效措施来降低感染发生率.

目的:回顾性分析体外徒手腹膜透析(PD)导管拔除术在PD患者中的临床应用,评估手术效果及并发症.方法:回顾2018年9月至2019年11月国家肾脏疾病临床医学研究中心运用体外徒手方法拔除PD导管的患者,分析患者的一般资料、拔管方法、术中术后特点及术后并发症,并随访至少1年.结果:共纳入45例患者,男性21例、女性24例,平均年龄43.7± 14.5岁,PD导管使用时长62(11.5～94)个月,拔管时长23.6±3.8s.45例患者均成功拔除PD导管,其中41例患者拔管后出口愈合良好;3例患者出口处持续少量渗液,出现愈合较慢,其中2例患者经换药加压包扎后逐渐愈合,1例患者予缝合导管出口处后皮肤逐渐愈合;1例患者出现浅涤纶套处感染,在隧道出口处分离组织与浅涤纶套,感染消失,愈合较好.所有患者均未出现新发腹膜炎、渗血、PD导管断裂、黏连性肠梗阻、腹膜疝等并发症.术后3个月、半年及1年时随访患者无任何拔管相关并发症.结论:体外徒手PD导管拔除术相对快速、简单、有效、微创且并发症少,可作为临床中PD导管拔除的常用方法.

目的 分析硫普罗宁相关肾损害的临床病理特征,提高对硫普罗宁相关肾损害诊断的治疗水平.方法 报道东部战区总医院收治的1例硫普罗宁导致膜性肾病的临床表现、病理改变及治疗预后,并收集自1979年至2020年2月所有硫普罗宁相关肾损害文献报道,分析其肾损害的临床病理特点及预后.结果 此例患者临床表现为中等量蛋白尿(1.02～1.3 g/24 h),肾活检为肾小球膜性病变,停药后1个月尿蛋白部分缓解,8个月后完全缓解.包括文中病例在内,文献报道硫普罗宁致肾病综合征/蛋白尿共55例,其中12例患者表现为非综合征范围蛋白尿,43例表现为肾病综合征,有4例合并急性肾损伤.17例接受肾活检,其中膜性肾病9例,微小病变肾病5例,肾小球系膜增生性病变1例,局灶节段性肾小球硬化1例,膜增生性肾小球肾炎1例.33例预后数据不详,余22例数据齐全的患者均于停药后完全缓解.结论 硫普罗宁可导致不同程度的蛋白尿,病理改变以肾小球足细胞病变为主,以膜性肾病为最常见表现,停药可缓解.及时诊断,可避免不必要的免疫抑制剂治疗.

目的:了解华氏巨球蛋白血症(WM)相关肾脏损害的临床和病理特征. 方法:回顾分析2002年2月～2019年9月在国家肾脏疾病临床医学研究中心因肾脏损害入院的WM患者的临床表现、实验室检查及肾活检病理. 结果:(1)一般情况:本研究共纳入16例患者,平均年龄60.9±11.3岁,男性11例,女性5例,WM病程3(1～6.75)个月.(2)临床特征:11例首发肾脏损害表现为肾功能不全;就诊时中位尿蛋白定量1.79g/24h(0.75～2.97 g/24h);血清肌酐183.8 μmol/L(76.9 ～ 359.7 μmol/L).16例均有贫血和血清IgM均升高,12/16例球蛋白升高,11例免疫固定电泳示IgM单克隆免疫球蛋白条带,15/15例血轻链比值异常,4/11例血冷球蛋白阳性,8/14例低补体血症;2/15例尿本周蛋白阳性;3/16例脾肿大.(3)病理特征:直肠淀粉样变性3例(1例合并皮肤淀粉样变性).8例行肾活检,3例肾小球系膜增生病变;肾小球轻微病变、肾小球内皮细胞病变、肾小球膜增生样病变、肾小球毛细血管袢皱缩、κ型轻链沉积病各1例.(4)治疗及预后:11例明确诊断后接受化疗,3例接受免疫吸附.6例死亡,8例存活(其中1例转化为骨髓瘤,4例缓解),2例失访. 结论:WM相关肾损害好发于中老年,男性多见,肾功能不全发生率高,肾脏病理改变多种多样,总体预后不佳.

[作者简介] 王金泉,医学博士,主任医师,主要从事肾小球疾 病诊断、治疗及分子致病机制的研究。E-mail:doc_wjq@163.com [摘要] 免疫球蛋白G4(IgG4)相关疾病是新近认识的系统性自身免疫性疾病,其特征性改变是一个或多个器官局 灶弥散性炎性细胞浸润和(或)脏器肿大,血清IgG4水平升高。典型的组织病理学改变为淋巴浆细胞样细胞浸润、席纹 状间质纤维化和(或)闭塞性静脉炎。本病发生率低,极具异质性,且与其他多种疾病在临床、病理及实验室检查上存 在相似性,因而延迟诊断及误诊十分常见。该文综述了IgG4相关疾病,尤其是IgG4相关肝胆管疾病、IgG4相关皮肤疾 病、米库利奇病及自身免疫性胰腺炎的早期诊断和鉴别诊断思路,以减少误诊的发生。 [关键词] 免疫球蛋白G4相关疾病;诊断;鉴别诊断;病理学 [中图分类号] R593.2 [文献标志码] A [文章编号] 0577-7402(2020)07-0772-09 [DOI] 10.11855/j.issn.0577-7402.2020.07.17

目的:观察超声引导下Seldinger法经皮穿刺腹膜透析(PD)置管术的临床特点,评估近中期患者的并发症、转归及导管技术生存率. 方法:回顾性分析2018年1月至2020年1月间国家肾脏疾病临床医学研究中心在超声引导下行Seldinger法经皮穿刺PD置管术的患者一般资料、术中及术后特点、术后并发症、导管技术生存率,所有患者术后至少随访期半年. 结果:共收集49例患者,男性28例,女性21例,平均年龄44.1±13.3岁.手术时间为23.4±2.4 min,腹部切口长度2.6±0.3 cm,平均术后12h即开始PD治疗.术中腹腔出血1例(约20ml),经PD液冲洗后出血消失;术后出现血性PD液2例,3d后血性PD液均消失;术后3d出现渗漏合并疝气、导管移位、网膜包裹各1例.所有患者均未出现腹膜炎、切口处感染、隧道感染、愈合不良等.术后1个月1例患者因网膜包裹改血液透析治疗,1例患者肾功能恢复拔除PD导管.术后3个月1例患者导管移位经保守治疗成功复位. 结论:超声引导下Seldinger法经皮穿刺PD置管术相对安全、简便、损伤小,并发症少,值得在临床推广.