Nephrosis

肾病
  • 文章类型: Journal Article
    Secondary nephrosis is a series of chronic kidney diseases secondary to other underlying diseases, mainly manifesting as structural and functional abnormalities of the kidneys and metabolic disorders. It is one of the important causes of end-stage renal disease, with high morbidity and significant harm. Iron is an essential metal element in human cells, and ferroptosis is a non-traditional form of iron-dependent cell death, and its main mechanisms include iron accumulation, lipid metabolism disorders, abnormal amino acid metabolism, and damage to the antioxidant system. Recently studies have found that ferroptosis is involved in the occurrence and progression of secondary nephrosis, and the mechanism of ferroptosis in different secondary nephrosis vary. Therefore, an in-depth and systematic understanding of the association between ferroptosis and secondary nephrosis, as well as their specific regulatory mechanisms, can provide a theoretical basis for the diagnosis, prevention, treatment, and prognosis assessment of secondary nephrosis, laying the foundation for exploring new clinical therapeutic targets for secondary nephrosis.
    继发性肾脏病是继发于其他基础疾病的一系列慢性肾脏病,主要表现为肾结构和功能异常、代谢紊乱,是导致终末期肾病的重要原因之一,具有发病率高、危害大等特点。铁是人体细胞中必需的金属元素,铁死亡是一种非传统形式的、铁依赖性的细胞死亡,其主要机制包括铁蓄积、脂质代谢紊乱、氨基酸代谢异常和抗氧化系统损伤。近年研究发现铁死亡参与继发性肾脏病的发生、发展,不同继发性肾脏病中铁死亡的发生机制不同。深入、系统地了解铁死亡与继发性肾脏病之间的关联及其特异性调控机制,可为继发性肾脏病的诊疗、防治及预后评估提供理论依据,为探索继发性肾脏病新的临床治疗靶点奠定基础。.
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  • 文章类型: Meta-Analysis
    尽管流行病学研究已经评估了环境空气污染与慢性肾脏疾病(CKD)之间的关系,结果喜忧参半。为了澄清协会的性质,我们进行了全面的系统评价和荟萃分析,以评估空气污染与CKD之间的全球关系.WebofScience,PubMed,系统搜索了Embase和Cochrane图书馆数据库,以查找截至2023年7月发表的研究,其中包括32项符合特定标准的研究。随机效应模型用于得出每种污染物的总体风险估计。对于PM2.5每增加10μg/m3,CKD风险的荟萃分析估计比值比(ORs)为1.42(95%置信区间[CI]:1.31-1.54);PM10每增加10μg/m3,则为1.20(95%CI:1.14-1.26);NO2每增加10μg/m3,1.07(95%CI:1.05-1.09)增加1.03:每1.07%1.亚组分析表明,这种影响因性别比例而异,年龄,研究设计,暴露评估方法,和收入水平。此外,PM2.5,PM10,即使在世界卫生组织推荐的可接受浓度范围内,NO2也对CKD产生负面影响。我们的结果进一步证实了空气污染对CKD风险的不利影响。这些发现有助于提高公共卫生官员和决策者对减少空气污染重要性的认识。
    Although epidemiological studies have evaluated the association between ambient air pollution and chronic kidney disease (CKD), the results remain mixed. To clarify the nature of the association, we conducted a comprehensive systematic review and meta-analysis to assess the global relationship between air pollution and CKD. The Web of Science, PubMed, Embase and Cochrane Library databases systematically were searched for studies published up to July 2023 and included 32 studies that met specific criteria. The random effects model was used to derive overall risk estimates for each pollutant. The meta-analysis estimated odds ratio (ORs) of risk for CKD were 1.42 (95% confidence interval [CI]: 1.31-1.54) for each 10 μg/m3 increase in PM2.5 ; 1.20 (95% CI: 1.14-1.26) for each 10 μg/m3 increase in PM10 ; 1.07 (95% CI: 1.05-1.09) for each 10 μg/m3 increase in NO2 ; 1.03 (95% CI: 1.02-1.03) for each 10 μg/m3 increase in NOX ; 1.07 (95% CI: 1.01-1.12) for each 1 ppb increase in SO2 ; 1.03 (95% CI: 1.00-1.05) for each 0.1 ppm increase in CO. Subgroup analysis showed that this effect varied by gender ratio, age, study design, exposure assessment method, and income level. Furthermore, PM2.5 , PM10 , and NO2 had negative effects on CKD even within the World Health Organization-recommended acceptable concentrations. Our results further confirmed the adverse effect of air pollution on the risk of CKD. These findings can contribute to enhance the awareness of the importance of reducing air pollution among public health officials and policymakers.
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    文章类型: Journal Article
    目的:探讨腹腔镜肾盂成形术联合肾镜超声碎石术治疗肾盂输尿管连接部梗阻(UPJO)合并肾结石的疗效和安全性。
    方法:2016年6月至2022年1月,北京大学人民医院8例患者,男5例,女3例,经19.5F(1F≈0.33mm)肾镜行腹腔镜肾盂成形术联合超声碎石术。年龄为23-51岁(平均:40.5岁),体重指数(BMI)为18.8-32.4kg/m2(平均27.0kg/m2)。8例患者的病变均位于左侧。两名患者患有孤立肾,一名患者患有马蹄肾。一名患者出现孤石,其他七名患者出现多处结石,两名患者患有鹿角结石。结石最大直径0.6~2.5cm(平均1.5cm)。CT或超声检查显示,5例患者出现中度肾病,3例患者出现重度肾病。手术期间,肾盂和输尿管近端暴露后,在肾盂前壁做了一个1.5厘米的小切口,通过腹腔镜套管针和肾盂切口将19.5F肾镜引入肾盂。用通过肾镜放置的3.3mm超声探头将石头碎裂并吸出。石头被移除后,行改良腹腔镜肾盂成形术。
    结果:所有8例患者均顺利完成手术,未转为开放手术。手术时间160~254min,平均213min,肾镜处理时间25~40min,平均33min。血红蛋白减少3-21g/L(平均:10.3g/L)。结石清除率为75%(6/8例),由于肾内结构异常,两名患者的结石未完全清除。1例患者术后发生改良Clavien分类系统(MCCS)ⅢA级并发症,这是肾内出血导致的肾病,并进行了肾造口术。平均随访30个月(2-68个月),所有病人都没有梗阻的迹象,1例患者接受经皮肾镜取石术治疗残余结石。
    结论:腹腔镜肾盂成形术联合19.5F肾镜超声碎石术是安全可行的,并可能是治疗UPJO和肾结石的补充方法。
    OBJECTIVE: To investigate the efficacy and safety of laparoscopic pyeloplasty combined with ultrasonic lithotripsy via nephroscope in the treatment of ureteropelvic junction obstruction (UPJO) with renal calculi.
    METHODS: From June 2016 to January 2022, eight patients including five males and three females underwent laparoscopic pyeloplasty combined with ultrasonic lithotripsy via 19.5F(1F≈0.33 mm) nephroscope in Peking University People\' s Hospital. The age ranged from 23-51 years (mean: 40.5 years) and the body mass index (BMI) ranged from 18.8-32.4 kg/m2 (mean 27.0 kg/m2). The lesion located on the left side in all of the eight patients. Two patients had solitary kidney and one patient had horseshoe kidney. Solitary stone was seen in one patient and the other seven patients suffered multiple stones, with two patients had staghorn stones. The largest diameter of stones ranged from 0.6-2.5 cm (mean: 1.5 cm). CT or ultrasound showed that moderate nephrosis was seen in five patients and severe nephrosis was seen in three patients. During surgery, after exposure of renal pelvis and proximal ureter, a small incision of 1.5 cm was performed in the anterior wall of the renal pelvis, and a 19.5F nephroscope was introduced into renal pelvis through laparoscopic trocar and renal pelvis incision. Stones were fragmented and sucked out by 3.3 mm ultrasonic probe placed through nephroscope. After stones were removed, modified laparoscopic pyeloplasty was performed.
    RESULTS: Surgery was successfully completed in all of the eight patients without conversion to open surgery. The operation time ranged from 160-254 min (mean 213 min) and the time of nephroscopic management time was 25-40 min (mean: 33 min). The hemoglobin was decreased by 3-21 g/L (mean: 10.3 g/L). The stone-free rate was 75% (6/8 cases), stones were incompletely removed in two patients due to abnormal intrarenal structure. The modified Clavien classification system (MCCS) grade ⅢA complication occurred in one patient postoperatively, which was nephrosis due to intrarenal bleeding, and nephrostomy was performed. With the mean follow-up of 30 months (ranged from 2-68 months), there was no evidence of obstruction in all the patients, and one patient underwent percutaneous nephrolithotomy to treat residual calculi.
    CONCLUSIONS: Laparoscopic pyeloplasty combined with ultrasonic lithotripsy via 19.5F nephroscope is feasible and safe, and could be a complementary method to treat UPJO and renal calculi.
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  • 文章类型: Journal Article
    研究居住在中国广东省人口众多且城市化迅速的成年人中抑郁和焦虑症状的患病率和健康状况。
    广东省睡眠和心身健康调查于2019年9月至11月进行,这是一项基于人群的研究,对18-85岁的成年人进行了代表性样本。采用多阶段分层整群抽样。总共纳入了13768名居民,并使用标准化评估工具进行了面对面的采访。使用患者健康问卷(PHQ-9)和广义焦虑障碍量表(GAD-7)评估抑郁和焦虑症状,分别。
    抑郁和焦虑症状的加权患病率分别为8.6%(95%置信区间[CI]:8.0%-9.3%)和6.0%(95CI,5.4%-6.5%)。从未结过婚的人,不规则的饮食节奏,几乎不锻炼,睡眠不好,酒精消费,慢性疾病以及失业和退休更容易出现抑郁和焦虑症状。此外,抑郁症状的患病率在患有心血管疾病的人群中明显更高(赔率[OR]:3.89,95CI:1.77-8.54),慢性肾病(OR:3.89,95CI:1.52-9.94),高脂血症(OR:2.68,95CI:1.57-4.59),贫血(OR:1.96,95CI:1.33-2.89)和关节炎(OR:1.93,95CI:1.17-3.16)。在心血管疾病患者中,焦虑症状的患病率明显更高(OR:3.15,95CI:1.39-7.14),慢性肾病(OR:2.89,95CI:1.22-6.83),高脂血症(OR:2.27,95CI:1.20-4.29)和糖尿病(OR:1.99,95CI:1.15-3.44)。
    抑郁和焦虑症状常见于慢性病患者。鉴于这些对健康的不利结果,卫生专业人员应在高危人群的医疗保健中更加关注抑郁和焦虑问题。
    To examine the prevalence and health correlates of depressive and anxiety symptoms among adults living in Guangdong province of China with a large population and rapid urbanization.
    The Guangdong Sleep and Psychosomatic Health Survey was conducted from September to November in 2019, which was a population-based study with a representative sample of adults aged 18-85 years. Multistage stratified cluster sampling was used. A total of 13,768 inhabitants were included and face-to-face interviewed using standardized assessment tools. Depressive and anxiety symptoms were evaluated using the Patient Health Questionnaire (PHQ-9) and Generalized Anxiety Disorder Scale (GAD-7), respectively.
    The weighted prevalence rates of depressive and anxiety symptoms were 8.6% (95% confidence interval [CI]: 8.0%-9.3%) and 6.0% (95%CI, 5.4%-6.5%) respectively. People who have never married, irregular diet rhythm, hardly exercise, poorer sleep, alcohol consumption, chronic diseases and being unemployment and retirement were more likely to suffer depressive and anxiety symptoms. Moreover, the prevalence of depressive symptoms was significantly higher in those suffering from cardiovascular disease (Odds ratio[OR]:3.89, 95%CI: 1.77-8.54), chronic nephrosis (OR:3.89, 95%CI: 1.52-9.94), hyperlipidemia (OR:2.68, 95%CI: 1.57-4.59), anemia (OR:1.96, 95%CI: 1.33-2.89) and arthritis (OR:1.93, 95%CI: 1.17-3.16). The prevalence of anxiety symptoms was evidently greater within patients with cardiovascular disease (OR:3.15, 95%CI: 1.39-7.14), chronic nephrosis (OR: 2.89, 95%CI: 1.22-6.83), hyperlipidemia (OR:2.27, 95%CI: 1.20-4.29) and diabetes (OR:1.99, 95%CI: 1.15-3.44).
    Depressive and anxiety symptoms are commonly found in patients with chronic diseases. Given these adverse outcomes on health, health professionals should pay more attention to depressive and anxiety problems in health care for high-risk population.
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  • 文章类型: Journal Article
    主要目的是确定中国住院儿科患者CKD的基本流行病学数据。
    从HQMS数据库的电子记录中提取了2013年6月1日至2017年5月31日住院的CKD儿科住院患者的数据,其中包括超过1400万住院患者。使用国际疾病分类(ICD-10)第十次修订版的代码来搜索数据库。
    本研究共纳入524例CKD原发疾病。总之,有278231名CKD儿科住院患者,在HQMS数据库中注册的14250594名儿科住院患者中,占1.95%。2013年CKD儿科住院患者为67498人,2014年为76810人,2015年为81665人,2016年为82649人,占1.93%,1.93%,1.99和2.09%,分别,儿科住院患者的总人口。CKD的病因为继发性肾病占37.95%,排名第一,其次是CAKUT,比例为24.61%。肾小球疾病和囊性肾病分别占21.18%和5.07%,分别。在所有278231名患者中,6581(2.37%)的主要出院诊断为CKD。CKD肾脏病理显示IgA占51.17%。
    本研究对儿科CKD患者的住院人群进行了描述性分析。我们的研究提供了重要的,政策制定和立法的基本数据,注册表实施和诊断,中国CKD的治疗和预防。
    The main purpose was to determine basic epidemiological data on CKD among hospitalized pediatric patients in China.
    Data from pediatric inpatients with CKD hospitalized from June 1, 2013 to May 31, 2017 were extracted from the electronic records of HQMS database, which includes over 14 million inpatients. Codes from the 10th revision of the International Classification of Diseases (ICD-10) were used to search the database.
    A total of 524 primary diseases of CKD were included in this study. In all, there were 278 231 pediatric inpatients with CKD, which accounted for 1.95 % of the 14 250 594 pediatric inpatients registered in the HQMS database. The number of pediatric inpatients with CKD was 67 498 in 2013, 76 810 in 2014, 81 665 in 2015 and 82 649 in 2016, which accounted for 1.93 %, 1.93 %, 1.99 and 2.09 %, respectively, of the total population of pediatric inpatients. The etiology of CKD was secondary nephrosis in 37.95 % of cases, which ranked first and followed by CAKUT with a percentage of 24.61 %. Glomerular diseases and cystic kidney disease accounted for 21.18 and 5.07 %, respectively. Among all 278 231 patients, 6 581 (2.37 %) had a primary discharge diagnosis of CKD. The renal pathology findings of CKD showed that IgA accounted for 51.17 %.
    This study provides a descriptive analysis of the hospitalized population of pediatric CKD patients. Our study provides important, fundamental data for policy making and legislation, registry implementation and the diagnosis, treatment and prevention of CKD in China.
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  • 文章类型: Journal Article
    This study is aim to analyze pathological characteristics of kidneys in cirrhotic patients with renal disease. Fifty-six cirrhotic patients with various renal diseases at Peking University First Hospital who underwent percutaneous renal biopsy from January 2010 to September 2019 were evaluated retrospectively. Immunoglobulin A nephropathy (IgAN) was the most common type of kidney biopsy (23 cases, 41.1%). Different pathologic types were often overlapping. More than 60% patients were treated with steroids and/or immunosuppressants, and 1 patient with anti-CD20 monoclonal antibody. Percutaneous renal biopsy is important for the diagnosis and treatment in cirrhotic patients with renal disease.
    为了解肝硬化伴肾损害患者的肾脏病理类型,本研究回顾性分析2010年1月至2019年9月北京大学第一医院肾内科收治的56例肝硬化伴肾病患者的肾脏病理类型。56例患者的肾脏病理类型具有多样性及复杂性,多种病理类型可在同一患者中存在,其中以IgA肾病最常见(41.1%,23例)。39例患者在肾脏病理结果指导下调整了治疗方案,其中36例患者行糖皮质激素和/或免疫抑制剂治疗,1例患者行利妥昔单抗治疗,1例患者血液透析后肾功能恢复。肾组织活检对肝硬化伴肾病患者临床诊断及治疗有重要指导作用,肝硬化并非肾组织活检的禁忌证。.
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  • 文章类型: Case Reports
    背景:肾脏是原发性干燥综合征(pSS)的常见颗粒外部位之一,以慢性肾小管间质性肾炎(TIN)最常见的病理类型。pSS的肾脏受累通常表现为慢性TIN并伴有1型或2型肾小管酸中毒(RTA)。很少有报道将肾脏受累描述为pSS中具有III型RTA的急性TIN。
    一名37岁女子因口干而入院,干眼,进行性肌肉无力持续了17个月.入院前两个月,患者的血钾水平为1.7mmol/L。
    方法:进一步的测试证实了pSS和III型RTA。肾活检显示急性TIN和薄基底膜肾病(TBMN)。
    方法:应用全剂量皮质类固醇(1mg/kg/天)和环磷酰胺(100mg/天)。
    结果:患者的肌酐水平在3个月的随访期间下降了0.28mg/dL(1.18-0.90mg/dL)。
    结论:我们报道了一例pSS相关肾损伤患者,表现为急性TIN,3型RTA和TBMN。这种情况增加了对pSS相关肾损伤的罕见表现的认识。在pSS相关的急性TIN中,环磷酰胺联合全剂量糖皮质激素可能取得良好的疗效.
    BACKGROUND: The kidney is one of the common extraglandular sites involved in primary Sjögren syndrome (pSS), with chronic tubulointerstitial nephritis (TIN) the most common pathology type. Renal involvement in pSS often presents as chronic TIN accompanied by type 1 or 2 renal tubular acidosis (RTA). Description of renal involvement as acute TIN with type III RTA in pSS has been rarely reported.
    UNASSIGNED: A 37-year-old woman was admitted with complaints of dry mouth, dry eyes, and progressive muscle weakness for 17 months. Two months before admission, the patient had a blood potassium level of 1.7 mmol/L.
    METHODS: Further tests confirmed pSS and type III RTA. Renal biopsy demonstrated acute TIN and thin basement membrane nephropathy (TBMN).
    METHODS: Full-dose corticosteroid (1 mg/kg/day) and cyclophosphamide (100 mg/day) were applied.
    RESULTS: The creatinine levels of the patient decreased 0.28 mg/dL (1.18-0.90 mg/dL) during 3-month follow-up.
    CONCLUSIONS: We reported a patient with pSS-associated kidney injury, presenting as acute TIN with type 3 RTA and TBMN. This case increases the awareness of a rare manifestation of pSS-associated kidney injury. In pSS-associated acute TIN, cyclophosphamide combined with full-dose corticosteroids may achieve good outcomes.
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  • 文章类型: Journal Article
    Proximal tubule (PT) cells express a single saturable albumin-binding site whose affinity matches the estimated tubular concentration of albumin; however, albumin uptake capacity is greatly increased under nephrotic conditions. Deciphering the individual contributions of megalin and cubilin to the uptake of normal and nephrotic levels of albumin is impossible in vivo, as knockout of megalin in mice globally disrupts PT endocytic uptake. We quantified concentration-dependent albumin uptake in an optimized opossum kidney cell culture model and fit the kinetic profiles to identify albumin-binding affinities and uptake capacities. Mathematical deconvolution fit best to a three-component model that included saturable high- and low-affinity uptake sites for albumin and underlying nonsaturable uptake consistent with passive uptake of albumin in the fluid phase. Knockdown of cubilin or its chaperone amnionless selectively reduced the binding capacity of the high-affinity site, whereas knockdown of megalin impacted the low-affinity site. Knockdown of disabled-2 decreased the capacities of both binding sites. Additionally, knockdown of megalin or disabled-2 profoundly inhibited the uptake of a fluid phase marker, with cubilin knockdown having a more modest effect. We propose a novel model for albumin retrieval along the PT in which cubilin and megalin receptors have different functions in recovering filtered albumin in proximal tubule cells. Cubilin binding to albumin is tuned to capture normally filtered levels of the protein. In contrast, megalin binding to albumin is of lower affinity, and its expression is also essential for enabling the recovery of high concentrations of albumin in the fluid phase.
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  • 文章类型: Case Reports
    Barakat综合征是一种常染色体显性疾病,其特征是甲状旁腺功能减退三联症,感觉神经性耳聋,和肾脏异常,是由GATA3基因突变引起的。SLC34A3是高钙尿症低磷酸盐血症的病因基因,和杂合携带者可能有较轻的临床症状。这项研究的目的是确定最初出现肾衰竭的患者的潜在遗传原因,高钙尿症,肾结石,和双侧感音神经性耳聋。
    研究了一个临床表现复杂的6岁男孩。进行了全面的医学评估,包括听觉功能测试,内分泌功能试验,代谢研究,和影像学检查。通过三全外显子组测序分析分子诊断。
    一个新颖的从头有害变体(c。在患者中鉴定出GATA3基因的324del)。患者可被诊断为Barakat综合征。此外,一种新颖的变体(c。589A>G)的SLC34A3基因被检测到,是从父亲那里继承的。这种杂合变体可以解释患者及其父亲发生的高钙尿症和肾结石。
    这项研究提供了一种特殊情况,即表型驱动的双重诊断,这两个新的变种可以巧妙地解释该患者的复杂临床表现。
    Barakat syndrome is an autosomal dominant disorder characterized by the triad of hypoparathyroidism, sensorineural deafness, and renal anomalies and is caused by mutations in GATA3 gene. SLC34A3 is the cause gene of hypophosphatemic rickets with hypercalciuria, and heterozygous carriers may have milder clinical symptoms. The aim of this study was to identify the underlying genetic cause of a patient who initially presented with renal failure, hypercalciuria, kidney stone, and bilateral sensorineural deafness.
    A 6-year-old boy with complex clinical presentations was investigated. Comprehensive medical evaluations were performed including auditory function tests, endocrine function tests, metabolic studies, and imaging examinations. Molecular diagnoses were analyzed by trio whole-exome sequencing.
    One novel de novo deleterious variant (c. 324del) of the GATA3 gene was identified in the patient. The patient can be diagnosed with Barakat syndrome. In addition, one novel variant (c. 589A>G) of the SLC34A3 gene was detected, which was inherited from the father. This heterozygous variant can explain the hypercalciuria and kidney stone that occurred in both the patient and his father.
    This study provides a special case which is phenotype-driven dual diagnoses, and the two novel variants can parsimoniously explain the complex clinical presentations of this patient.
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  • 文章类型: Journal Article
    Objective: To investigate the clinicopathological characteristics and prognosis of renal cell carcinoma (RCC) in patients with end-stage renal disease (ESRD). Methods: The clinicopathological data of patients of renal cell carcinoma arising in end-stage renal disease were collected from the Affiliated Hospital of Qingdao University (ten cases) and 971 Hospital of PLA Navy (five cases) from January 2009 to August 2018. Results: Among 15 patients, 14 were male and 1 was female, and the age ranged from 38 to 78 years (mean 51 years, median 49 years). All patients had history of chronic renal failure (7-192 months), including 9 patients treated with hemodialysis for 6 to 132 months. In 12 cases the tumor border was distinct and the tumor size ranged from 1.8 to 11.0 cm. Two cases were multifocal and one case showed extensive renal hemorrhage with an inconspicuous tumor mass. Microscopically, 9 cases were clear cell reanl cell carcinoma including one with sarcomatoid differentiation, 4 were acquired cystic kidney disease-associated(ACKD-RCC) and two were papillary renal cell carcinoma. All patients had a follow-up of 3 to 120 months. Four patients died during a follow-up of 6 to 60 months (mean 30 months) as a result of extensive distant metastases (two cases) and renal failure (two cases), while other eleven patients were alive without tumor recurrence or metastasis (median 40.8 months of follow-up ranging from 3 to 120 months). Conclusions: ESRD-RCC is more often seen in younger male patients. The time intervals from the onset of chronic renal failure to the diagnosis of renal cell carcinoma differ and tumors are frequently incidental findings. The histological types can be sporadic renal cell carcinoma or unique ACKD-RCC. Tumors are often hemorrhagic and necrotic. Routine physical examination and early detection could benefit ESRD-RCC patients. ESRD-RCC may have a favorable prognosis despite of a large tumor size or the presence of sarcomatoid differentiation.
    目的: 探讨终末期肾病合并肾细胞癌(ESRD-RCC)的临床病理学特点及预后。 方法: 收集2009年1月至2018年8月青岛大学附属医院(10例)和海军第九七一医院(5例)ESRD-RCC患者的临床病理资料,分析其临床病理特点及预后,并复习相关文献。 结果: 15例中男14例,女1例,发病年龄38~78岁(平均年龄51岁,中位年龄49岁)。所有患者均有慢性肾功能衰竭病史(7~192个月),其中9例曾行透析治疗(6~132个月)。大体检查:12例肿瘤组织边界清楚,直径1.8~11.0 cm,2例为多灶性,1例肾脏广泛出血、肿瘤不明显。组织学诊断:肾透明细胞癌9例(1例伴肉瘤样分化)、获得性囊性肾病相关性肾细胞癌(ACKD-RCC)4例、乳头状肾细胞癌(Ⅰ型)2例。15例随访时间3~120个月,4例因远处转移(2例,1例伴灶状肉瘤样分化)或肾衰竭(2例)于术后6~60个月(平均30个月)死亡,11例术后生存3~120个月(平均40.8个月),未见复发或远处转移。 结论: ESRD-RCC多见于青年男性,自确诊为慢性肾功能衰竭至诊断为肾细胞癌的时间长短不一,大多因体检发现,组织学类型既可以表现为与散发性肾细胞癌一致的形态学特点,也可以表现为独特的病理组织类型,以肾透明细胞癌和ACKD-RCC最多见,癌组织易出血、坏死。定期体检、早期发现、及时治疗会让患者获得良好的预后,但肿瘤体积较大、伴肉瘤样分化时预后较差。.
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