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Risk factors for in-hospital mortality among patients hospitalized with COVID-19 and end-stage kidney disease. COVID-19 和终末期肾病住院患者院内死亡的风险因素。
IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Pub Date : 2024-10-01 DOI: 10.5414/CN111323
Jackson Heilbronn, Amir Abdipour, Giv Heidari-Bateni, Mohammad Sharif, Sahib Grewal, Sergio Infante, Kwame Agyeman, Zohreh Gholizadeh Ghozloujeh, Lida Gharibvand, Sayna Norouzi

Aims: The mortality rate for -COVID-19 infection varies significantly depending on age and comorbidities but remains high in hospitalized patients overall. Several retrospective studies have identified patients with end-stage kidney disease (ESKD) to be at increased risk. The objective of this study was to study in-hospital outcomes of ESKD patients at an academic medical center and identify characteristics that place them at a higher risk for in-hospital mortality.

Materials and methods: A retrospective chart review was conducted including adult patients (≥ 18 years old) admitted to Loma Linda Medical Center for COVID-19 infection with a previous diagnosis of ESKD. Patients with prior kidney transplants were excluded. The main outcome of this study was the rate of in-hospital mortality.

Results: 21 of the 91 patients died with a mortality rate of 23%. Age, D-dimer > 0.4 µg/mL, ejection fraction less than 50%, and ferritin > 300 ng/mL were predictors for mortality in unadjusted univariate analysis. Adjusted multivariable analysis demonstrated that only an ejection fraction of less than 50% was associated with increased mortality risk.

Conclusion: Cardiovascular disease is the leading cause of mortality for ESKD patients and also places them at increased risk of mortality in the setting of severe COVID-19 infection.

目的:COVID-19 感染的死亡率因年龄和并发症的不同而有很大差异,但在住院患者中的总体死亡率仍然很高。一些回顾性研究发现,终末期肾病(ESKD)患者的风险更高。本研究的目的是研究一家学术医疗中心的终末期肾病(ESKD)患者的院内预后,并确定导致他们院内死亡风险较高的特征:研究人员对洛马琳达医疗中心因感染 COVID-19、既往诊断为 ESKD 而入院的成年患者(≥ 18 岁)进行了回顾性病历审查。既往接受过肾移植的患者除外。研究的主要结果是院内死亡率。结果:91 名患者中有 21 人死亡,死亡率为 23%。在未经调整的单变量分析中,年龄、D-二聚体> 0.4 µg/mL、射血分数低于50%和铁蛋白> 300 ng/mL是死亡率的预测因素。调整后的多变量分析表明,只有射血分数小于 50%与死亡风险增加有关:结论:心血管疾病是 ESKD 患者死亡的主要原因,同时也增加了他们在 COVID-19 严重感染情况下的死亡风险。
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引用次数: 0
Efficacy and safety analysis of sodium zirconium cyclosilicate and calcium polystyrene sulfonate on rapid reduction of potassium in moderate to severe hyperkalemia patients with chronic kidney disease without dialysis. 环硅酸锆钠和聚苯乙烯磺酸钙对中重度高钾血症患者快速降钾的有效性和安全性分析。
IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Pub Date : 2024-10-01 DOI: 10.5414/CN111226
XiaoJuan Fu, Sen Zhang, Fang Gao, Nan Mao

Background: Hyperkalemia is a common complication of chronic kidney disease (CKD). This study aims to investigate the efficacy and safety of sodium zirconium cyclosilicate and calcium polystyrene sulfonate in reducing potassium in patients with acute and severe hyperkalemia in CKD who are not undergoing dialysis.

Materials and methods: A retrospective real-world study was conducted among 73 patients with non-dialysis chronic kidney disease who were hospitalized in the First Affiliated Hospital of Chengdu Medical College from June 2020 to June 2022. 33 patients treated with sodium zirconium cyclosilicate were categorized as SZC group, and the other 40 patients treated with calcium polystyrene sulfonate were categorized as CPS group. Serum potassium, serum sodium, magnesium, calcium, and phosphorus levels were examined. Adverse reactions were recorded during medication.

Results: Significantly decreased serum potassium was observed in both groups, whereas the potassium reduction was higher in the SZC group than in the CPS group at 2, 4, 24, and 48 hours after medication while there was no statistically significant difference in the serum potassium level between the two groups at 72 hours. For those people whose initial potassium exceeded 6 mmol/L, the potassium reduction was more obvious in the SZC group than in the CPS group at 2 and 4 hours after medication. The control rate of hyperkalemia in the SZC group was significantly higher than in the CPS group at 4, 24, and 48 hours. No distinct change was observed in serum sodium, calcium, magnesium, and phosphorus before and 72 hours after medication. No severe adverse reactions occurred.

Conclusion: Sodium zirconium cyclosilicate has a more obvious effect on reducing potassium particularly for those patients with moderate to severe hyperkalemia who need rapid potassium reduction.

背景:高钾血症是慢性肾脏病(CKD)的常见并发症。本研究旨在探讨环硅酸锆钠和聚苯乙烯磺酸钙对未进行透析的 CKD 急性严重高钾血症患者降钾的有效性和安全性:对2020年6月至2022年6月在成都医学院第一附属医院住院治疗的73例非透析慢性肾脏病患者进行了回顾性真实世界研究。33 名接受环硅酸锆钠治疗的患者被归为 SZC 组,另外 40 名接受聚苯乙烯磺酸钙治疗的患者被归为 CPS 组。检查血钾、血钠、血镁、血钙和血磷水平。记录用药期间的不良反应:结果:两组患者的血清钾均显著下降,但在用药后 2、4、24 和 48 小时,SZC 组的血钾降幅高于 CPS 组,而在 72 小时,两组患者的血清钾水平差异无统计学意义。对于初始血钾超过 6 毫摩尔/升的患者,在用药后 2 小时和 4 小时,SZC 组的血钾降低比 CPS 组更为明显。在用药 4、24 和 48 小时后,深海碳酸钙组的高钾血症控制率明显高于 CPS 组。用药前和用药后 72 小时,血清钠、钙、镁和磷没有明显变化。无严重不良反应发生:结论:环硅酸锆钠具有更明显的降钾效果,尤其适用于需要快速降钾的中重度高钾血症患者。
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引用次数: 0
Kidney complications of Wilson disease and its treatments: A case report and literature review. 威尔逊病的肾脏并发症及其治疗方法:病例报告和文献综述。
IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Pub Date : 2024-10-01 DOI: 10.5414/CN111402
Maria Salman, Ayub Akbari, Gregory L Hundemer

Wilson disease is a rare autosomal recessive genetic disorder of copper metabolism that leads to copper accumulation and subsequent organ dysfunction. While classically considered a condition that primarily affects the liver and nervous system, Wilson disease and its treatments can also result in a wide range of kidney complications as well. We present the case of a 31-year-old female with a longstanding (> 10 year) history of Wilson disease who developed acute-onset nephrotic syndrome including heavy proteinuria, hypoalbuminemia, and edema after being transitioned from zinc to D-penicillamine for copper chelation therapy. Following simple cessation of D-penicillamine (and without any immunosuppressive therapies including corticosteroids), the nephrotic syndrome showed remarkable improvement including complete remission within several months. This review comprehensively summarizes the kidney complications associated with Wilson disease and its treatments.

威尔逊病是一种罕见的常染色体隐性遗传性铜代谢紊乱病,会导致铜蓄积,进而引起器官功能障碍。虽然威尔逊病通常被认为是一种主要影响肝脏和神经系统的疾病,但威尔逊病及其治疗也可导致多种肾脏并发症。我们介绍了一例 31 岁的女性病例,她患有威尔逊病已有 10 年以上的病史,在从锌过渡到 D-青霉胺进行铜螯合治疗后,出现了急性肾病综合征,包括大量蛋白尿、低白蛋白血症和水肿。在简单停用 D-青霉胺(未使用任何免疫抑制剂,包括皮质类固醇)后,肾病综合征得到了显著改善,包括在数月内完全缓解。本综述全面总结了与威尔逊氏病有关的肾脏并发症及其治疗方法。
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引用次数: 0
Efficacy of urokinase in maintaining patency of hemodialysis catheters: A meta-analysis. 尿激酶保持血液透析导管通畅的功效:荟萃分析。
IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Pub Date : 2024-10-01 DOI: 10.5414/CN111239
Jiaxiu Deng, Weixiang Luo, Weiwei Zhang, Shiyi Xiong, Liping Wang

Background: Catheter malfunctions are associated with reduced blood flow and interrupted dialysis during hemodialysis.

Aim: This meta-analysis aimed to determine whether the use of urokinase to lock hemodialysis catheters can maintain their patency and prevent catheter-related bloodstream infections (CRBSIs).

Materials and methods: The PubMed, Cochrane Library, Web of Science, Embase, and Chinese medical databases were searched for controlled trials of hemodialysis catheter locking using urokinase from database inception until July 15, 2021. The primary outcome was catheter malfunction, and the secondary outcomes were the peak catheter blood flow rate (Qb) and CRBSIs.

Results: Across 16 trials, 1,041 patients were randomized to receive either urokinase/urokinase mixture (treated) or heparin (control) locks once or thrice a week. Locking with urokinase alone or in combination with another substance significantly prevented catheter malfunction. The effect on Qb was significant, with that in the treated group being better than in the control group. Similarly, the incidence of CRBSIs in the treated group was lower.

Conclusion: Urokinase locking maintains catheter patency more effectively than heparin. Prophylactic locking with urokinase or urokinase mixtures reduces incidences of catheter malfunction, which ensures the smooth progression of hemodialysis and reduces patient medical costs. The results of this study have important clinical implications and will provide guidance to medical practitioners globally.

背景:目的:本荟萃分析旨在确定使用尿激酶锁定血液透析导管是否能保持导管通畅并预防导管相关血流感染(CRBSIs):在 PubMed、Cochrane Library、Web of Science、Embase 和中文医学数据库中检索了自数据库建立起至 2021 年 7 月 15 日使用尿激酶锁定血液透析导管的对照试验。主要结果为导管故障,次要结果为导管峰值血流量(Qb)和CRBSIs:在16项试验中,1041名患者随机接受了每周一次或三次的尿激酶/尿激酶混合物(治疗)或肝素(对照)锁定。单独使用尿激酶或与其他物质联合使用可显著预防导管故障。对 Qb 的影响非常明显,治疗组的 Qb 优于对照组。同样,治疗组的 CRBSI 发生率也较低:结论:与肝素相比,尿激酶锁定能更有效地保持导管通畅。使用尿激酶或尿激酶混合物进行预防性锁定可降低导管故障的发生率,从而确保血液透析的顺利进行并降低患者的医疗费用。这项研究的结果具有重要的临床意义,将为全球医疗从业人员提供指导。
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引用次数: 0
Denosumab for immobilization-induced hypercalcemia in patients with end-stage renal disease. 地诺单抗治疗终末期肾病患者因固定诱发的高钙血症。
IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Pub Date : 2024-10-01 DOI: 10.5414/CN111361
Jugal Thaker, Abdallah S Geara
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引用次数: 0
Nutritional and psychological sequelae of COVID-19 infection in maintenance hemodialysis patients. 维持性血液透析患者感染 COVID-19 后的营养和心理后遗症。
IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Pub Date : 2024-09-24 DOI: 10.5414/CN111369
Sara T Ibrahim, Basant M Talaat, Iman E ElGohary, Gihane I Khalil, Dalia A Maharem

Aim: The risk of infection with COVID-19 in hemodialysis (HD) patients is higher compared to the general population. Additionally, HD patients are at higher risk of developing post-COVID-19 infection sequelae. However, this has not been thoroughly investigated. Therefore, we aimed to study the impact of COVID-19 on nutritional status and psychological health in HD patients 6 months following recovery.

Materials and methods: We recruited HD patients who were proven to be infected with COVID-19 and received treatment at two HD units in two institutions between April 2022 and December 2022. Additionally, we enrolled a group of age- and sex-matched HD patients who had not previously been infected with COVID-19 or received vaccination. Nutritional status was assessed using malnutrition inflammation score (MIS), while psychological health was assessed using online questionnaires. The Patient Health Questionnaire 9 (PHQ 9) was employed to assess symptoms of depression, the Generalized Anxiety Disorder 7 (GAD 7) was used to identify anxiety disorders, the Patient Health Questionnaire 15 (PHQ 15) was utilized to measure somatic symptoms, and the Insomnia Severity Index (ISI) was used to measure insomnia.

Results: A total of 60 subjects (30 patients and 30 controls) were assessed in the study. We found statistically significant differences between patients and controls regarding the MIS (median score (interquartile range (IQR)); 11 (9 - 12) and 5.5 (5 - 7), respectively), PHQ 15 (median score (IQR); 17.5 (15 - 19) and 9 (6 - 11), respectively), PHQ 9 (median score (IQR); 17 (13 - 19) and 5 (7 - 8), respectively), GAD 7 (median score (IQR); 14 (11 - 16) and 6 (4 - 8), respectively), and ISI (median score (IQR); 20 (15 - 22) and 8 (7 - 11), respectively), with p < 0.001 for all scores.

Conclusion: COVID-19 has long-term effects on the psychosocial health of HD patients and may lead to a higher incidence of malnutrition 6 months post recovery.

目的:与普通人群相比,血液透析(HD)患者感染 COVID-19 的风险更高。此外,血液透析患者发生 COVID-19 感染后遗症的风险更高。然而,这一点尚未得到深入研究。因此,我们旨在研究 COVID-19 对 HD 患者康复后 6 个月的营养状况和心理健康的影响:我们招募了 2022 年 4 月至 2022 年 12 月期间被证实感染 COVID-19 并在两家机构的两家 HD 单位接受治疗的 HD 患者。此外,我们还招募了一组年龄和性别匹配的 HD 患者,他们之前未感染 COVID-19,也未接种疫苗。营养状况通过营养不良炎症评分(MIS)进行评估,心理健康则通过在线问卷进行评估。患者健康问卷9(PHQ 9)用于评估抑郁症状,广泛性焦虑症7(GAD 7)用于识别焦虑症,患者健康问卷15(PHQ 15)用于测量躯体症状,失眠严重程度指数(ISI)用于测量失眠:研究共评估了 60 名受试者(30 名患者和 30 名对照组)。我们发现,患者和对照组在失眠症严重程度指数(MIS)(中位数分数(四分位距);分别为 11 (9 - 12) 和 5.5 (5 - 7))、PHQ 15(中位数分数(四分位距);分别为 17.5(15 - 19)和 9(6 - 11))、PHQ 9(中位数分数(IQR);分别为 17(13 - 19)和 5(7 - 8))、GAD 7(中位数分数(IQR);分别为 14(11 - 16)和 6(4 - 8))和 ISI(中位数分数(IQR);分别为 20(15 - 22)和 8(7 - 11)),P 结论:COVID-19 对 HD 患者的社会心理健康有长期影响,并可能导致康复后 6 个月营养不良的发生率升高。
{"title":"Nutritional and psychological sequelae of COVID-19 infection in maintenance hemodialysis patients.","authors":"Sara T Ibrahim, Basant M Talaat, Iman E ElGohary, Gihane I Khalil, Dalia A Maharem","doi":"10.5414/CN111369","DOIUrl":"10.5414/CN111369","url":null,"abstract":"<p><strong>Aim: </strong>The risk of infection with COVID-19 in hemodialysis (HD) patients is higher compared to the general population. Additionally, HD patients are at higher risk of developing post-COVID-19 infection sequelae. However, this has not been thoroughly investigated. Therefore, we aimed to study the impact of COVID-19 on nutritional status and psychological health in HD patients 6 months following recovery.</p><p><strong>Materials and methods: </strong>We recruited HD patients who were proven to be infected with COVID-19 and received treatment at two HD units in two institutions between April 2022 and December 2022. Additionally, we enrolled a group of age- and sex-matched HD patients who had not previously been infected with COVID-19 or received vaccination. Nutritional status was assessed using malnutrition inflammation score (MIS), while psychological health was assessed using online questionnaires. The Patient Health Questionnaire 9 (PHQ 9) was employed to assess symptoms of depression, the Generalized Anxiety Disorder 7 (GAD 7) was used to identify anxiety disorders, the Patient Health Questionnaire 15 (PHQ 15) was utilized to measure somatic symptoms, and the Insomnia Severity Index (ISI) was used to measure insomnia.</p><p><strong>Results: </strong>A total of 60 subjects (30 patients and 30 controls) were assessed in the study. We found statistically significant differences between patients and controls regarding the MIS (median score (interquartile range (IQR)); 11 (9 - 12) and 5.5 (5 - 7), respectively), PHQ 15 (median score (IQR); 17.5 (15 - 19) and 9 (6 - 11), respectively), PHQ 9 (median score (IQR); 17 (13 - 19) and 5 (7 - 8), respectively), GAD 7 (median score (IQR); 14 (11 - 16) and 6 (4 - 8), respectively), and ISI (median score (IQR); 20 (15 - 22) and 8 (7 - 11), respectively), with p < 0.001 for all scores.</p><p><strong>Conclusion: </strong>COVID-19 has long-term effects on the psychosocial health of HD patients and may lead to a higher incidence of malnutrition 6 months post recovery.</p>","PeriodicalId":10396,"journal":{"name":"Clinical nephrology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142307252","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Treating primary membranous nephropathy with extremely high titer of anti-phospholipase A2 receptor antibodies: A case of failed treatment with very high-dose rituximab. 治疗抗磷脂酶A2受体抗体滴度极高的原发性膜性肾病:一个使用超大剂量利妥昔单抗治疗失败的病例。
IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Pub Date : 2024-09-18 DOI: 10.5414/CN111228
Jingshu Sun, Shengqin Wu, Fang Yin, Kunying Zhang, Jianying Wang

Rituximab (RTX) is the anti-CD20 monoclonal antibody that has been used as the first-line therapy for primary membranous nephropathy (PMN) in recent years. However, the optimal dosing regimen and timing of RTX, or combination with other immunosuppressants, especially in patients with extremely high titers (> 1,000 RU/mL) of anti-PLA2R antibody (aPLA2R), are unclear at present. This report describes the case of a 70-year-old PMN patient with extremely high aPLA2R titer who failed to respond to very high doses of RTX. We also discuss the possible reasons for treatment failure.

利妥昔单抗(RTX)是一种抗 CD20 单克隆抗体,近年来一直被用作原发性膜性肾病(PMN)的一线疗法。然而,目前尚不清楚 RTX 的最佳给药方案和时机,或与其他免疫抑制剂联用的最佳方案和时机,尤其是在抗 PLA2R 抗体(aPLA2R)滴度极高(> 1,000 RU/mL)的患者中。本报告描述了一例 70 岁的 PMN 患者的病例,该患者的 aPLA2R 滴度极高,但对超大剂量 RTX 治疗无效。我们还讨论了治疗失败的可能原因。
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引用次数: 0
Impact of depression on clinical outcomes of peritoneal dialysis: A systematic review and meta-analysis. 抑郁症对腹膜透析临床结果的影响:系统回顾和荟萃分析。
IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Pub Date : 2024-09-06 DOI: 10.5414/CN111454
Meiling Xu, Wei Zhang

Objective: Depression has become a highly prevalent mental disorder around the globe. With a large number of end-stage renal disease patients taking up peritoneal dialysis (PD), a substantial number of PD patients with concomitant depression are expected to be treated in the future. However, the effects of depression on outcomes of PD are unclear. This review systematically examines the effect of depression on mortality, technique survival, or peritonitis in PD patients.

Materials and methods: Studies comparing outcomes of PD patients with and without depression and published on Google Scholar, Embase, Web of Science, and PubMed till February 5, 2024 were included.

Results: Eleven studies were eligible; 5 studies reported data on mortality. Pooled analysis showed that depression was not a significant predictor of mortality in PD patients (HR: 1.22 95% CI: 0.86, 1.72). Only 2 studies reported analyzable data on technique survival and 3 studies on peritonitis. Meta-analysis found no statistically significant effect of depression on technique survival (OR: 1.28 95% CI: 0.38, 4.35) and peritonitis (OR: 1.89 95% CI: 0.82, 4.33). Qualitative analysis of remaining studies also suggested no effect of depression on patient and technique survival.

Conclusion: Depression may not be an independent predictor of patient and technique survival in PD patients. Data on the risk of peritonitis is conflicting and needs to be investigated further.

目的:抑郁症已成为全球高发的精神疾病。随着大量终末期肾病患者接受腹膜透析(PD)治疗,预计未来将有大量伴有抑郁症的腹膜透析患者接受治疗。然而,抑郁症对腹膜透析疗效的影响尚不明确。本综述系统研究了抑郁症对腹膜透析患者死亡率、技术存活率或腹膜炎的影响:纳入截至 2024 年 2 月 5 日在 Google Scholar、Embase、Web of Science 和 PubMed 上发表的比较有抑郁症和无抑郁症的腹腔镜手术患者预后的研究:结果:11项研究符合条件;5项研究报告了死亡率数据。汇总分析表明,抑郁症不是预测帕金森病患者死亡率的重要因素(HR:1.22 95% CI:0.86, 1.72)。只有 2 项研究报告了技术存活率方面的可分析数据,3 项研究报告了腹膜炎方面的可分析数据。Meta 分析发现,抑郁对技术存活率(OR:1.28 95% CI:0.38, 4.35)和腹膜炎(OR:1.89 95% CI:0.82, 4.33)没有统计学意义上的显著影响。对其余研究的定性分析也表明,抑郁症对患者和技术的存活率没有影响:结论:抑郁可能不是腹腔镜手术患者存活率和技术存活率的独立预测因素。有关腹膜炎风险的数据相互矛盾,需要进一步研究。
{"title":"Impact of depression on clinical outcomes of peritoneal dialysis: A systematic review and meta-analysis.","authors":"Meiling Xu, Wei Zhang","doi":"10.5414/CN111454","DOIUrl":"10.5414/CN111454","url":null,"abstract":"<p><strong>Objective: </strong>Depression has become a highly prevalent mental disorder around the globe. With a large number of end-stage renal disease patients taking up peritoneal dialysis (PD), a substantial number of PD patients with concomitant depression are expected to be treated in the future. However, the effects of depression on outcomes of PD are unclear. This review systematically examines the effect of depression on mortality, technique survival, or peritonitis in PD patients.</p><p><strong>Materials and methods: </strong>Studies comparing outcomes of PD patients with and without depression and published on Google Scholar, Embase, Web of Science, and PubMed till February 5, 2024 were included.</p><p><strong>Results: </strong>Eleven studies were eligible; 5 studies reported data on mortality. Pooled analysis showed that depression was not a significant predictor of mortality in PD patients (HR: 1.22 95% CI: 0.86, 1.72). Only 2 studies reported analyzable data on technique survival and 3 studies on peritonitis. Meta-analysis found no statistically significant effect of depression on technique survival (OR: 1.28 95% CI: 0.38, 4.35) and peritonitis (OR: 1.89 95% CI: 0.82, 4.33). Qualitative analysis of remaining studies also suggested no effect of depression on patient and technique survival.</p><p><strong>Conclusion: </strong>Depression may not be an independent predictor of patient and technique survival in PD patients. Data on the risk of peritonitis is conflicting and needs to be investigated further.</p>","PeriodicalId":10396,"journal":{"name":"Clinical nephrology","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142139502","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Renal involvement in genetic neurocutaneous syndromes. 遗传性神经皮肤综合征的肾脏受累。
IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Pub Date : 2024-09-06 DOI: 10.5414/CN111425
Hassan Izzedine, Farhana Begum, Simon Kashfi, Morgan Rouprêt, Alina Bridges, Kenar D Jhaveri

Phakomatoses, otherwise known as neurocutaneous syndromes, are a heterogeneous group of rare genetic disorders that predominantly affect structures arising from the embryonic ectoderm, namely the skin, eye globe, retina, and central nervous system. In addition to the common neurocutaneous syndromes (neurofibromatosis, tuberous sclerosis complex, Sturge Weber syndrome, Von Hippel-Lindau syndrome), a large number of relatively uncommon phakomatoses have been described in the literature. Cardiovascular, pulmonary, and musculoskeletal systems involvement in these disorders have been reported. Data on kidney involvement is not well described. This review discusses renal involvement in neurocutaneous syndromes. This includes the association with renal masses (cyst, angiomyolipoma, benign or malignant tumor), known vasculopathy, glomerular or tubular disorders, urinary tract anomalies, hypertension, and chronic kidney disease.

角膜病又称神经皮肤综合征,是一组异质性的罕见遗传疾病,主要影响胚胎外胚层的结构,即皮肤、眼球、视网膜和中枢神经系统。除了常见的神经皮肤综合征(神经纤维瘤病、结节性硬化综合征、Sturge Weber 综合征、Von Hippel-Lindau 综合征)外,文献中还描述了大量相对不常见的噬皮综合征。有报道称这些疾病会累及心血管、肺部和肌肉骨骼系统。而有关肾脏受累的数据则不多。本综述将讨论神经皮肤综合征的肾脏受累情况。这包括与肾脏肿块(囊肿、血管脂肪瘤、良性或恶性肿瘤)、已知的血管病变、肾小球或肾小管疾病、尿路异常、高血压和慢性肾病的关联。
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引用次数: 0
Phosphorus: Chronicles of the epistemology of a vital element. 磷:一种重要元素的认识论编年史。
IF 1.1 4区 医学 Q3 UROLOGY & NEPHROLOGY Pub Date : 2024-09-01 DOI: 10.5414/CN111435
Garabed Eknoyan, Eleanor D Lederer

It was in the philosopher's stone quest that the alchemist Hennig Brand isolated chemiluminescent white phosphorus (P), Greek for "light bearer", from urine in 1669. By 1771 phosphorus was isolated from bone, and in 1777 it was identified by Antoine Lavoisier as a highly reactive element that exists predominantly in nature as ionic phosphate (PO43-) and in solution as phosphoric acid (H3PO4). Early 20th century studies revealed phosphorylated biomolecules as essential components of replicative nuclear material (RNA, DNA), a metabolic source of energy (ATP), and structural components of cellular membrane (phospholipid bilayer). Life on earth began as organophosphates of a self-replicating RNA that evolved into DNA and acquired a membrane to form the original eukaryotes, which eventually joined to form multicellular organisms of the deep sea. Tissue mineralization during transition from the ocean to land generated the endoskeleton, the largest phosphorus stores of evolving vertebrates. Subsequent studies of phosphate homeostasis elucidated its complex regulatory system based on the interaction of the kidney, small intestine, bone, and parathyroid glands, orchestrated by hormones (PTH, calcitriol, FGF23, Klotho), and carried out by phosphate-specific transporters (SLC34 and SLC20 families) all to ensure adequate phosphate for survival and health. Paradoxically, kidney replacement therapy in the 1970s, by prolonging the lives of millions of individuals with kidney failure, revealed the hazards of phosphorus excess. "Phosphorus the light bearer" has become in the eyes of many nephrologists "Phosphorus the cardiovascular toxin".

1669 年,炼金术士亨尼格-布兰德从尿液中分离出化学发光的白磷(P),希腊语的意思是 "光的承载者"。1771 年,人们从骨头中分离出了磷,1777 年,安托万-拉瓦锡(Antoine Lavoisier)确认磷是一种高活性元素,在自然界中主要以离子磷酸(PO43-)的形式存在,在溶液中则以磷酸(H3PO4)的形式存在。20 世纪早期的研究发现,磷酸化生物大分子是复制核材料(RNA、DNA)的重要组成部分,是能量(ATP)的代谢来源,也是细胞膜(磷脂双分子层)的结构成分。地球上的生命起源于自我复制的 RNA 的有机磷酸盐,进化成 DNA 并获得细胞膜,形成最初的真核生物,最终联合形成深海的多细胞生物。在从海洋向陆地过渡的过程中,组织矿化产生了内骨骼,这是进化脊椎动物最大的磷储存库。随后对磷酸盐稳态的研究阐明了其复杂的调节系统,该系统以肾脏、小肠、骨骼和甲状旁腺的相互作用为基础,由激素(PTH、钙三醇、FGF23、Klotho)协调,并由磷酸盐特异性转运体(SLC34 和 SLC20 家族)执行,以确保生存和健康所需的充足磷酸盐。矛盾的是,20 世纪 70 年代的肾脏替代疗法延长了数百万肾衰竭患者的生命,揭示了磷过量的危害。在许多肾病学家眼中,"磷是光明的使者 "已经变成了 "磷是心血管毒素"。
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引用次数: 0
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Clinical nephrology
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