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Demand Analysis of Self-Management Mobile Health Applications for Middle-Aged and Older Patients with Chronic Kidney Disease Based on the Kano Model. 基于卡诺模型的中老年慢性肾病患者自我管理移动医疗应用需求分析。
IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Pub Date : 2024-10-11 DOI: 10.1159/000541729
Yu Yan, Min Liu, Di-Fei Duan, Lin-Jia Yan, Ling Li, Deng-Yan Ma

Introduction: Middle-aged and older individuals often face significant challenges in adopting digital health solutions, leading to a digital divide that hinders their ability to benefit from mobile health (mHealth) interventions. This study aimed to investigate the specific requirements of middle-aged and older patients with chronic kidney disease (CKD) for self-management through mobile health applications (mHealth apps), using the Kano model.

Methods: A multicenter cross-sectional survey was conducted from April to September 2023 in five hospitals across Sichuan, Shandong, Guangdong, and Shaanxi provinces in China. The Kano model was employed to analyze participants' preferences regarding mHealth apps for self-management.

Results: Out of 359 participants (57.1% men, predominantly aged 45-54), the study identified essential and desirable features for mHealth apps. Essential attributes include comprehensive CKD information and robust privacy protection. Key to enhancing user satisfaction is features like symptom and medication management, access to medical insurance information, and app interface simplicity. Additional attractive features for increasing app appeal include diet management, exercise guidance, and customizable text size.

Conclusion: This study identifies critical mHealth app features for self-management in middle-aged and older CKD patients, emphasizing the importance of user-centric design. The findings provide valuable insights for app developers to create tailored solutions that cater to the specific needs of this demographic, potentially enhancing their self-management capabilities.

简介中老年人在采用数字健康解决方案时往往面临巨大挑战,这导致了数字鸿沟,阻碍了他们从移动健康(mHealth)干预措施中获益的能力。本研究旨在采用卡诺模型,调查中老年慢性肾病(CKD)患者通过移动医疗应用程序(mHealth apps)进行自我管理的具体要求:方法:2023 年 4 月至 9 月,在中国四川、山东、广东和陕西四省的五家医院开展了一项多中心横断面调查。采用卡诺模型分析参与者对用于自我管理的移动医疗应用程序的偏好:在 359 名参与者(57.1% 为男性,年龄主要在 45-54 岁之间)中,研究确定了移动医疗应用程序的基本功能和理想功能。基本特性包括全面的慢性肾脏病信息和强大的隐私保护。提高用户满意度的关键在于症状和药物管理、医疗保险信息访问以及应用界面简洁性等功能。其他可增加应用程序吸引力的功能包括饮食管理、运动指导和可定制的文字大小:本研究确定了中老年慢性肾脏病患者进行自我管理的关键移动医疗应用程序功能,强调了以用户为中心的设计的重要性。研究结果为应用程序开发人员提供了宝贵的见解,使他们能够针对这一人群的特殊需求量身定制解决方案,从而提高他们的自我管理能力。
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引用次数: 0
NF-κB/miR-455-5p/SOCS3 Axis Aggravates Sepsis-Induced Acute Kidney Injury through Promoting Renal Inflammation. NF-κB/miR-455-5p/SOCS3轴通过促进肾脏炎症加重脓毒症诱发的急性肾损伤。
IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Pub Date : 2024-10-08 DOI: 10.1159/000541727
Mingjuan Yan, Ni Zhang, Li Quan, Wei Bin, Jing Xi, Caoshuai Dou, Zhiwen Liu, Yongfeng Gui, Liang-Hong Yin

Introduction: Sepsis is the leading contributor to acute kidney injury (AKI), responsible for 45-70% of AKI occurrences. Despite this, septic AKI is a highly multifactorial and complex condition, and our grasp of its pathogenesis is still not fully developed. Consequently, there remains a significant gap in effective diagnostic and therapeutic strategies for septic AKI.

Methods: In the in vitro experiments, BUMPT cells were exposed to lipopolysaccharides (LPS). In vivo experiments involved inducing sepsis in mice through administration of LPS injections. Additionally, in certain experiments, either a miR-455-5p mimic or an anti-miR-455-5p LAN was administered to the mice via injections into the tail vein. The mice were then sacrificed 24 h following LPS administration for subsequent analysis.

Results: We observed a significant elevation in miR-455-5p levels within renal tubular cells following LPS-induced septic AKI. Our investigation revealed that NF-κB plays a crucial role in the upregulation of miR-455-5p. Inhibition of NF-κB using TPCA-1 prevented the rise in miR-455-5p levels in BUMPT cells (mouse proximal tubular cells from Boston University) cultured in vitro. Chromatin immunoprecipitation assays confirmed that NF-κB directly interacts with the promoter region of the miR-455-5p gene in response to LPS treatment. Functionally, introducing miR-455-5p mimics intensified cell apoptosis, kidney damage, and the production of inflammatory cytokines, while silencing miR-455-5p had protective effects in septic mice. Notably, administering anti-miR-455-5p enhanced SOCS3 expression, whereas miR-455-5p mimics reduced SOCS3 levels following LPS exposure. Furthermore, our luciferase reporter assays demonstrated that SOCS3 is a direct target of miR-455-5p.

Conclusion: This study indicates an NF-κB/miR-455-5p/SOCS3 axis which can exacerbate kidney damage by enhancing renal inflammation. This process highlights potential therapeutic targets for managing septic AKI.

导言:脓毒症是导致急性肾损伤(AKI)的主要因素,占 AKI 发生率的 45% 至 70%。尽管如此,脓毒症急性肾损伤是一种高度多因素和复杂的疾病,我们对其发病机制的掌握仍不完全。因此,在脓毒性 AKI 的有效诊断和治疗策略方面仍存在巨大差距:在体外实验中,BUMPT 细胞暴露于脂多糖(LPS)。体内实验包括通过注射 LPS 诱导小鼠败血症。此外,在某些实验中,小鼠尾静脉注射 miR-455-5p 模拟物或抗 miR-455-5p LAN。小鼠在注射 LPS 24 小时后被处死,以便进行后续分析:结果:我们观察到,LPS 诱导脓毒性 AKI 后,肾小管细胞内 miR-455-5p 水平明显升高。我们的研究发现,NF-κB 在 miR-455-5p 的上调过程中起着至关重要的作用。使用 TPCA-1 抑制 NF-κB 可以阻止体外培养的 BUMPT 细胞(波士顿大学的小鼠近端肾小管细胞)中 miR-455-5p 水平的升高。ChIP 分析证实,NF-κB 会直接与 miR-455-5p 基因的启动子区域相互作用,以应对 LPS 处理。从功能上讲,引入 miR-455-5p 模拟物会加剧细胞凋亡、肾脏损伤和炎症细胞因子的产生,而沉默 miR-455-5p 则对败血症小鼠有保护作用。值得注意的是,给予抗 miR-455-5p 会增强 SOCS3 的表达,而 miR-455-5p 模拟物会降低 LPS 暴露后的 SOCS3 水平。此外,我们的荧光素酶报告实验证明,SOCS3 是 miR-455-5p 的直接靶标:本研究表明,NF-κB/miR-455-5p/SOCS3 轴可通过增强肾脏炎症加剧肾脏损伤。这一过程凸显了治疗脓毒症 AKI 的潜在治疗靶点。
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引用次数: 0
An Outbreak of Shiga Toxin-Positive Enteroaggregative Escherichia coli O104:H4 Related Hemolytic Uremic Syndrome in Turkey: A Multicenter Study. 土耳其爆发志贺毒素阳性肠聚集性大肠杆菌 0104:H4 相关溶血性尿毒症:一项多中心研究。
IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Pub Date : 2024-10-08 DOI: 10.1159/000541687
Merve Havan, Anar Gurbanov, Ersin Özkan, Hacer Uçmak, Fevzi Kahveci, Zeynelabidin Öztürk, Evrim Kargın Çakıcı, Emel Uyar, Serhat Emeksiz, Özlem Temel, Gürkan Bozan, Hüsne Tuba Halıcıoğlu, Hasan Fatih Çakmaklı, Songül Yılmaz, Belkis Levent, Halil Özdemir, Zeynep Ceren Karahan, Zeynep Birsin Özçakar, Tanıl Kendirli

Introduction: Serious outbreaks of Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC-HUS) have been reported globally. In 2011, Germany experienced a significant outbreak of HUS caused by enteroaggregative E. coli (EAEC) O104:H4 strain. Since then, no other outbreaks of this strain have been reported. This study aims to evaluate pediatric patients affected by the second documented worldwide outbreak of STEC-HUS (EAEC O104:H4 serotype) contaminating local drinking water.

Methods: Medical records of patients hospitalized in five pediatric intensive care units (PICUs) diagnosed with STEC-HUS between July and September 2022 were evaluated retrospectively.

Results: Eighteen patients (14 girls and 4 boys) were enrolled in the study. The median age was 7.4 (Interquartile range [IQR] 1.3-17) years. Abdominal pain was the most common symptom (100%). The mean duration between symptom onset and development of STEC-HUS was 3 days (IQ 1-9). EAEC O104:H4 serotype was detected in the stool samples of 8 patients. Neurological involvement was observed in 3 patients, cardiac involvement in 2 patients, and both in 1 patient. Two patients required respiratory support and dialysis was performed in 16 (88.8%) patients. Plasmapheresis was administered to 2 patients, and eculizumab was given to four. No mortality was reported during follow-up; the mean durations of PICU and hospital stays were 11.3 and 31.6 days, respectively.

Conclusion: Outbreaks of HUS can have serious impacts on both mortality and morbidity. However, timely diagnosis and implementation of appropriate supportive care, including dialysis, respiratory support, and medical treatment for eligible patients, can lead to favorable outcomes.

导言:据报道,全球爆发了严重的产志贺毒素大肠埃希菌相关溶血性尿毒症(STEC-HUS)疫情。2011 年,德国爆发了由肠道聚集性大肠埃希菌 (EAEC) O104:H4 菌株引起的严重溶血性尿毒症。此后,再未报告过该菌株引起的其他疫情。本研究旨在评估因当地饮用水污染而导致 STEC-HUS(EAEC O104:H4 血清型)在全球范围内第二次暴发而受到影响的儿科患者:方法:回顾性评估2022年7月至9月期间在5个儿科重症监护病房(PICU)住院的被诊断为STEC-HUS的患者的医疗记录:结果:18 名患者(14 名女孩和 4 名男孩)参与了研究。中位年龄为 7.4 [四分位内范围(IQ)1.3-17]岁。腹痛是最常见的症状(100%)。从症状出现到发生 STEC-HUS 的平均时间为 3 天(IQ 1-9)。8 名患者的粪便样本中检测到 EAEC O104:H4 血清型。三名患者出现神经系统受累,两名患者出现心脏受累,一名患者同时出现神经系统和心脏受累。两名患者需要呼吸支持,16 名患者(88.8%)进行了透析。两名患者接受了血浆置换术,四名患者接受了依库珠单抗治疗。随访期间无死亡报告;PICU和住院的平均时间分别为11.3天和31.6天:结论:HUS 爆发会对死亡率和发病率造成严重影响。结论:HUS 爆发会对死亡率和发病率造成严重影响,但及时诊断并实施适当的支持性治疗,包括透析、呼吸支持和对符合条件的患者进行适当的药物治疗,可带来良好的结果。
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引用次数: 0
Brenner's Unforgettable Legacy: Transforming Medicine and Renal Science for Generations. 布伦纳难忘的遗产:改变医学和肾脏科学的世代传承。
IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Pub Date : 2024-10-05 DOI: 10.1159/000541866
Giuseppe Remuzzi, David Warnock
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引用次数: 0
Urinary Complement C3 and Vitamin D-Binding Protein Predict Adverse Outcomes in Patients with Acute Kidney Injury after Cardiac Surgery. 尿补体 C3 和维生素 D 结合蛋白可预测心脏手术后急性肾损伤患者的不良预后。
IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Pub Date : 2024-09-30 DOI: 10.1159/000540664
Joseph Hunter Holthoff, Joseph L Alge, John M Arthur, Fatima Ayub, Wadhah Bin Homam, Michael G Janech, Sreelakshmi Ravula, Nithin Karakala

Introduction: Acute kidney injury (AKI) is associated with adverse outcomes, including death and dialysis. The goal of this study was to identify prognostic biomarkers of AKI that could be used across multiple phenotypes of AKI and across different species.

Methods: Liquid chromatography/tandem mass spectrometry analysis of urine samples from three species (human, rat, and mouse) and four etiologies of AKI identified five potential biomarkers, of which two were validated, complement C3 and vitamin D-binding protein, in a cohort of 157 patients that developed AKI following cardiothoracic surgery. We studied the relationship between the biomarker's concentration in the urine and the development of a composite primary endpoint (stage 3 AKI within 10 days or death within 30 days).

Results: Of the 153 patients who developed AKI following cardiovascular surgery, 17 met the combined primary outcome. The median concentration of urine complement C3 adjusted to urine creatinine had the best predictive value and was significantly higher in the primary outcome group than in the controls. Similarly, the median concentration of vitamin D-binding protein was higher in the primary outcome group.

Conclusions: The studies provide proof in principle that cross-species discovery analyses could be a valuable tool for identifying novel prognostic biomarkers in AKI. Urine complement C3 and vitamin D-binding protein could be promising early predictors of adverse outcomes in patients who develop AKI after cardiac surgery.

导言急性肾损伤与不良后果有关,包括死亡和透析。本研究的目的是确定急性肾损伤(AKI)的预后生物标志物,这些标志物可用于多种表型的 AKI 和不同物种:对三种物种(人、大鼠、小鼠)和四种急性肾损伤病因的尿液样本进行液相色谱/串联质谱分析,确定了五种潜在的生物标志物;其中补体C3和维生素D结合蛋白这两种生物标志物已在心胸外科手术后发生AKI的157名患者队列中得到验证。我们研究了尿液中生物标志物浓度与综合主要终点(10 天内急性肾损伤 3 期或 30 天内死亡)之间的关系:结果:在心血管手术后出现急性肾损伤的 153 名患者中,有 17 人达到了综合主要终点。根据尿肌酐调整后的尿补体 C3 中位浓度具有最佳预测价值,且主要结果组明显高于对照组。同样,初选结果组的维生素 D 结合蛋白浓度中位数也更高:这些研究从原则上证明了跨物种发现分析可以成为鉴定 AKI 中新型预后生物标志物的重要工具。尿补体C3和维生素D结合蛋白有望成为心脏手术后发生AKI患者不良预后的早期预测指标。
{"title":"Urinary Complement C3 and Vitamin D-Binding Protein Predict Adverse Outcomes in Patients with Acute Kidney Injury after Cardiac Surgery.","authors":"Joseph Hunter Holthoff, Joseph L Alge, John M Arthur, Fatima Ayub, Wadhah Bin Homam, Michael G Janech, Sreelakshmi Ravula, Nithin Karakala","doi":"10.1159/000540664","DOIUrl":"10.1159/000540664","url":null,"abstract":"<p><strong>Introduction: </strong>Acute kidney injury (AKI) is associated with adverse outcomes, including death and dialysis. The goal of this study was to identify prognostic biomarkers of AKI that could be used across multiple phenotypes of AKI and across different species.</p><p><strong>Methods: </strong>Liquid chromatography/tandem mass spectrometry analysis of urine samples from three species (human, rat, and mouse) and four etiologies of AKI identified five potential biomarkers, of which two were validated, complement C3 and vitamin D-binding protein, in a cohort of 157 patients that developed AKI following cardiothoracic surgery. We studied the relationship between the biomarker's concentration in the urine and the development of a composite primary endpoint (stage 3 AKI within 10 days or death within 30 days).</p><p><strong>Results: </strong>Of the 153 patients who developed AKI following cardiovascular surgery, 17 met the combined primary outcome. The median concentration of urine complement C3 adjusted to urine creatinine had the best predictive value and was significantly higher in the primary outcome group than in the controls. Similarly, the median concentration of vitamin D-binding protein was higher in the primary outcome group.</p><p><strong>Conclusions: </strong>The studies provide proof in principle that cross-species discovery analyses could be a valuable tool for identifying novel prognostic biomarkers in AKI. Urine complement C3 and vitamin D-binding protein could be promising early predictors of adverse outcomes in patients who develop AKI after cardiac surgery.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-11"},"PeriodicalIF":2.3,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350544","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
Adoption of CKD-EPI (2021) for Glomerular Filtration Rate Estimation: Implications for UK Practice. 采用 CKD-EPI (2021) 估算 GFR - 对英国实践的影响。
IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Pub Date : 2024-09-28 DOI: 10.1159/000541689
Reuben Roy, Maharajan Raman, Paul M Dark, Philip A Kalra, Darren Green

Introduction: Recommendations to move to a race-free estimating equation for glomerular filtration rate (GFR) have gained increasing prominence since 2021. We wished to determine the impact of any future adoption upon the chronic kidney disease (CKD) patient population of a large teaching hospital, with a population breakdown largely similar to that of England as a whole.

Methods: We compared four estimating equations (Modification of Diet in Renal Disease [MDRD], CKD-EPI [2009], CKD-EPI [2021], and European Kidney Function Consortium [EKFC]) using the Bland-Altman method. Bias and precision were calculated (in both figures and percentages) for all patients with CKD and specific subgroups determined by age, ethnic group, CKD stage, and sex. CKD stage was assessed using all four equations.

Results: All equations studied had a positive bias in South Asian patients and a negative bias in black patients compared to CKD-EPI (2021). Similarly, there was a positive bias in white patients across all equations studied. Comparing CKD-EPI (2009) and EKFC, this positive bias increased as patients aged; the opposite was seen with MDRD. Between 10% and 28% of patients in our dataset changed their CKD staging depending upon the estimating equation used.

Discussion: Our work confirms previous findings that the MDRD equation overestimates estimated GFR (eGFR) in South Asians and underestimates eGFR in blacks. The alternative equations also demonstrated similar bias. This may, in part, explain the health inequalities seen in ethnic minority patients in the UK. Applying our findings to the UK CKD population as a whole would result in anywhere from 260,000 to 730,000 patients having their CKD stage reclassified, which in turn will impact secondary care services.

导言:自 2021 年以来,关于改用无种族限制的肾小球滤过率估算方程的建议日益受到重视。我们希望确定未来采用这种方法对一家大型教学医院的 CKD 患者群体的影响,该医院的人口分布与英格兰全国人口分布基本相似:我们使用 Bland-Altman 方法比较了四种估计方程(MDRD、CKD-EPI(2009)、CKD-EPI(2021)和 EKFC)。计算了所有 CKD 患者以及根据年龄、种族、CKD 分期和性别确定的特定亚组的偏差和精确度(数字和百分比)。使用所有四个公式对 CKD 阶段进行了评估:结果:与 CKD-EPI(2021)相比,所有研究方程在南亚病人中都存在正偏差,在黑人病人中则存在负偏差。同样,在所研究的所有公式中,白人患者的偏倚率均为正。比较 CKD-EPI (2009) 和 EKFC,随着患者年龄的增长,这种正偏倚会增加,而 MDRD 则相反。在我们的数据集中,10% 到 28% 的患者改变了他们的 CKD 分期,这取决于所使用的估计方程:讨论:我们的研究证实了之前的发现,即 MDRD 方程高估了南亚人的 eGFR,低估了黑人的 eGFR。替代方程也显示出类似的偏差。这可能在一定程度上解释了英国少数民族患者健康不平等的原因。如果将我们的研究结果应用于英国所有的 CKD 患者,将会有 26 万至 73 万患者的 CKD 分期被重新划分,这反过来又会影响二级护理服务。
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引用次数: 0
Immediate-Release versus Extended-Release Tacrolimus: Comparing Blood Pressure Control in Kidney Transplant Recipients - A Retrospective Cohort Study. 速释与缓释他克莫司:比较肾移植受者的血压控制 - 一项回顾性队列研究。
IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Pub Date : 2024-09-22 DOI: 10.1159/000541334
Chien-Wen Yang, Juan Carlos Q Velez, Debbie L Cohen

Background: Hypertension (HTN) is a common side effect of tacrolimus (Tac), the first-line antirejection medication for kidney transplant recipients. The impact of immediate-release tacrolimus (Tac IR) dosed twice daily versus extended-release tacrolimus (Tac ER) dosed once daily on long-term blood pressure control in kidney transplant recipients remains understudied. This study aims to compare the use of Tac IR versus Tac ER in kidney transplant recipients and evaluate the effects of the different formulations on systolic blood pressure (SBP), diastolic blood pressure (DBP), and HTN crisis.

Methods: This retrospective cohort study at a single institution collected baseline characteristics, time-varying exposure to Tac IR versus Tac ER, SBP, DBP, HTN crisis, and confounders at each posttransplant visit. A marginal structural linear mixed-effects model was employed to analyze the longitudinal blood pressure control in kidney transplant recipients receiving Tac IR and Tac ER.

Results: The final analysis included 654 patients, with mean ages of 52.0 years for Tac IR and 50.3 years for Tac ER. Males constituted 56.7% in Tac IR and 55.0% in Tac ER. Notably, the black population had 2.44 times higher odds of receiving Tac ER after adjusting for the rest of the baseline characteristics. No difference was found between longitudinal SBP (p = 0.386, 95% CI: -1.00, 2.57) or DBP (p = 0.797, 95% CI: -1.38, 1.06).

Conclusion: Our study indicates that posttransplant patients taking Tac ER exhibit no difference in chronic SBP and DBP controls compared to Tac IR.

背景高血压(HTN)是肾移植受者一线抗排斥药物他克莫司(Tac)的常见副作用。在肾移植受者中,每天服用两次的速释他克莫司(Tac IR)与每天服用一次的缓释他克莫司(Tac ER)对长期血压控制的影响仍未得到充分研究。本研究旨在比较肾移植受者使用 Tac IR 和 Tac ER 的情况,并评估不同制剂对收缩压 (SBP)、舒张压 (DBP) 和高血压危机的影响。方法 该回顾性队列研究在一家机构进行,收集了移植后每次就诊时的基线特征、Tac IR 与 Tac ER 的时变暴露、SBP、DBP、HTN 危机和混杂因素。采用边际结构线性混合效应模型分析了接受 Tac IR 和 Tac ER 治疗的肾移植受者的纵向血压控制情况。结果 最终分析包括 654 名患者,Tac IR 患者的平均年龄为 52.0 岁,Tac ER 患者的平均年龄为 50.3 岁。男性在 Tac IR 中占 56.7%,在 Tac ER 中占 55.0%。值得注意的是,在调整了其他基线特征后,黑人接受 Tac ER 的几率要高出 2.44 倍。纵向 SBP(p=0.386,95% CI:-1.00,2.57)或 DBP(p=0.797,95% CI:-1.38,1.06)之间未发现差异。结论 我们的研究表明,与 Tac IR 相比,服用 Tac ER 的移植后患者在慢性 SBP 和 DBP 控制方面没有差异。
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引用次数: 0
Lysinuric Protein Intolerance: Not Only a Disorder for Pediatric Nephrologists - Case Report. 赖氨酸尿蛋白不耐受症:不仅仅是儿科肾病专家的疾病。病例报告。
IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Pub Date : 2024-09-18 DOI: 10.1159/000541363
Miriam Rigoldi, Caterina Mele, Matteo Breno, Marina Noris, Amantia Imeraj, Sara Gamba, Arrigo Schieppati, Erica Daina

Introduction: Lysinuric protein intolerance (LPI) is a multisystemic inborn error of metabolism with a variable clinical expressivity that usually begins in childhood with growth failure and gastroenterological/neurological problems related to the altered urea cycle and, later, with complications involving the renal, pulmonary, and immunohematological systems.

Case report: We present the case of a 40-year-old woman suffering from chronic kidney disease in the context of a LPI, whose diagnosis was challenging because the signs of the disease were always blurred and the patient never manifested critical episodes typical of this multisystemic disease. In addition to renal disease, splenomegaly, thrombocytopenia, elevated lactate dehydrogenase (LDH), hyperferritinemia, and hypertriglyceridemia were also present. A thorough investigation of the patient's food preferences revealed her spontaneous aversion to protein-containing foods and excessive drowsiness during the occurrence of infectious episodes or on the rare occasions of excessive protein intake, although without ever coming to medical attention. These nuanced signs led us to suspect an impairment of the urea cycle and ultimately allowed us to narrow down the diagnosis to LPI through biochemical and genetic investigations.

Conclusion: Nephrologists should consider LPI in the differential diagnosis, whenever a patient presents with mixed proteinuria, tubular dysfunction, and/or chronic kidney disease of unknown origin. In these circumstances, we suggest looking for other signs such as growth failure, signs and symptoms ascribed to urea-cycle impairment, pulmonary involvement, hepatosplenomegaly, and laboratory alterations such as pancytopenia, hyperferritinemia, lipid abnormalities, and elevated LDH.

导言赖氨酸尿蛋白不耐受症(LPI)是一种多系统的先天性代谢异常,临床表现不一,通常在儿童期开始出现生长发育障碍和与尿素循环改变有关的胃肠道/神经系统问题,随后出现涉及肾脏、肺部和免疫血液系统的并发症:我们介绍了一例 40 岁女性慢性肾脏病患者的病例,该患者患有 LPI,其诊断具有挑战性,因为该疾病的体征总是很模糊,而且患者从未出现过这种多系统疾病的典型危重发作。除肾脏疾病外,患者还出现脾肿大、血小板减少、乳酸脱氢酶(LDH)升高、高铁蛋白血症和高甘油三酯血症。对患者饮食偏好的深入调查显示,她自发地厌恶含蛋白质的食物,在感染发作期间或在极少数摄入过量蛋白质的情况下过度嗜睡,但从未就医。这些细微的迹象让我们怀疑尿素循环出现了障碍,并最终让我们通过生化和基因检查将诊断范围缩小到 LPI:结论:只要患者出现混合性蛋白尿、肾小管功能障碍和/或不明原因的慢性肾衰竭,肾病学家就应在鉴别诊断中考虑 LPI。在这种情况下,我们建议寻找其他体征,如生长发育衰竭、尿素周期损伤的体征和症状、肺部受累、肝脾肿大和实验室改变,如泛发性、高铁蛋白血症、血脂异常、LDH 升高。
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引用次数: 0
The Impact of Age and Body Composition on the Agreement between Estimated and Measured GFR in Heart Transplant Recipients. 年龄和身体成分对心脏移植受者估计和测量的 GFR 之间一致性的影响。
IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Pub Date : 2024-09-14 DOI: 10.1159/000540530
Mads Hornum, Morten Buus Jørgensen, Lærke Marie Sidenius Nelson, Bo Feldt-Rasmussen, Kasper Rossing, Esteban Porrini, Peter Oturai, Finn Gustafsson

Background: Estimated GFR (eGFR) has shown poor agreement with measured GFR (mGFR) in several populations. We investigated the impact of age and body composition on the accuracy and precision of eGFR in heart transplant (HTx) recipients.

Methods: In a longitudinal, observational, retrospective study design, patients receiving first-time HTx with at least one registered mGFR value within 15 months after HTx and a corresponding plasma creatinine were included. GFR was measured by 51Cr-EDTA and eGFR calculated by creatinine-based CKD-EPI formula.

Results: A total of 150 patients with a total of 723 mGFR measurements were included. During the first year after HTx, mean weight increased by 4.2 kg (CI: 3.2 to 5.1) followed by an annual decrease of 0.35 kg/year (Cl: -0.05 to 0.74). mGFR increased by 7.5 mL/min (Cl: 3.2 to 11.8) the first year but was stable hereafter (0.0 mL/min/year; CI: -1.0 to 1.0). The initial weigh gain and increase in mGFR were most pronounced in patients <45 years. Neither eGFR adjusted nor unadjusted for BSA detected the initial increase in mGFR. At 1 year after HTx, limits of agreement on the Bland-Altman plot were -37.2 to 33.1 mL/min with a bias of -2.1 mL/min (Cl: -5.0 to 0.9). In patients <45 years, eGFR significantly overestimated mGFR by 7.1 mL/min (Cl: 1.0 to 13.2) and showed a significant lower precision than patients >45 years. There was no effect of BMI class, weight, BSA, or change in BMI class on the difference between eGFR and mGFR.

Conclusion: eGFR is, on average, accurate but imprecise in HTx patients. The agreement is affected by age but not body composition.

背景 在一些人群中,估算的 GFR(eGFR)与测量的 GFR(mGFR)的一致性较差。我们研究了年龄和身体成分对心脏移植(HTx)受者 eGFR 的准确性和精确性的影响。方法 采用纵向、观察性、回顾性研究设计,纳入首次接受心脏移植的患者,这些患者在接受心脏移植后 15 个月内至少有一次登记的 mGFR 值和相应的血浆肌酐。用 51Cr-EDTA 测量 GFR,用基于肌酐的 CKD-EPI 公式计算 eGFR。结果 150 名患者共进行了 723 次 mGFR 测量。HTx 术后第一年,平均体重增加了 4.2 千克(CI:3.2-5.1),随后每年减少 0.35 千克(Cl:-0.05-0.74),mGFR 在第一年增加了 7.5 毫升/分钟(Cl:3.2-11.8),但此后保持稳定(0.0 毫升/分钟/年,CI:-1.0-1.0)。最初体重增加和 mGFR 增加在 45 岁患者中最为明显。调整或未调整 BSA 的 eGFR 均未检测到 mGFR 的初始增长。在 HTx 一年后,Bland-Altman 图的一致性界限为-37.2-33.1 毫升/分钟,偏差为-2.1 毫升/分钟(Cl:-5.0-0.9)。在 45 岁患者中,eGFR 明显高估了 mGFR 7.1 毫升/分钟(Cl:1.0-13.2),精确度明显低于 45 岁患者。BMI 等级、体重、BSA 或 BMI 等级的变化对 eGFR 和 mGFR 之间的差异没有影响。结论 在高血压患者中,eGFR 平均准确但不精确。一致性受年龄影响,但不受身体成分影响。
{"title":"The Impact of Age and Body Composition on the Agreement between Estimated and Measured GFR in Heart Transplant Recipients.","authors":"Mads Hornum, Morten Buus Jørgensen, Lærke Marie Sidenius Nelson, Bo Feldt-Rasmussen, Kasper Rossing, Esteban Porrini, Peter Oturai, Finn Gustafsson","doi":"10.1159/000540530","DOIUrl":"10.1159/000540530","url":null,"abstract":"<p><strong>Background: </strong>Estimated GFR (eGFR) has shown poor agreement with measured GFR (mGFR) in several populations. We investigated the impact of age and body composition on the accuracy and precision of eGFR in heart transplant (HTx) recipients.</p><p><strong>Methods: </strong>In a longitudinal, observational, retrospective study design, patients receiving first-time HTx with at least one registered mGFR value within 15 months after HTx and a corresponding plasma creatinine were included. GFR was measured by 51Cr-EDTA and eGFR calculated by creatinine-based CKD-EPI formula.</p><p><strong>Results: </strong>A total of 150 patients with a total of 723 mGFR measurements were included. During the first year after HTx, mean weight increased by 4.2 kg (CI: 3.2 to 5.1) followed by an annual decrease of 0.35 kg/year (Cl: -0.05 to 0.74). mGFR increased by 7.5 mL/min (Cl: 3.2 to 11.8) the first year but was stable hereafter (0.0 mL/min/year; CI: -1.0 to 1.0). The initial weigh gain and increase in mGFR were most pronounced in patients &lt;45 years. Neither eGFR adjusted nor unadjusted for BSA detected the initial increase in mGFR. At 1 year after HTx, limits of agreement on the Bland-Altman plot were -37.2 to 33.1 mL/min with a bias of -2.1 mL/min (Cl: -5.0 to 0.9). In patients &lt;45 years, eGFR significantly overestimated mGFR by 7.1 mL/min (Cl: 1.0 to 13.2) and showed a significant lower precision than patients &gt;45 years. There was no effect of BMI class, weight, BSA, or change in BMI class on the difference between eGFR and mGFR.</p><p><strong>Conclusion: </strong>eGFR is, on average, accurate but imprecise in HTx patients. The agreement is affected by age but not body composition.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-11"},"PeriodicalIF":2.3,"publicationDate":"2024-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142291736","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
Efficacy and Safety of Telitacicept in IgA Nephropathy: A Retrospective, Multicenter Study. 泰利他赛治疗 IgA 肾病的疗效和安全性:一项回顾性多中心研究。
IF 2.3 4区 医学 Q2 UROLOGY & NEPHROLOGY Pub Date : 2024-09-09 DOI: 10.1159/000540326
Lijun Liu, Yimeng Liu, Juan Li, Chen Tang, Huiming Wang, Cheng Chen, Haibo Long, Xiaowen Chen, Guolan Xing, Jingru Cheng, Jianbo Liang, Xuan Peng, Liang Wang, Sijia Shao, Yongqiang Lin, Tianmu Chen, Ying Tang, Shizhong Shen, Lingyun Sun, Henglan Wu, Yuan Yu, Xuanyi Du, Hong Liu, Liyu He, Hong Liu, Meixing Ye, Wei Chen, Qiong Wen, Hong Zhang, Hongmin Cao, Jing Yuan, Hong Chen, Ming Wang, Jicheng Lv, Hong Zhang

Introduction: The efficacy of telitacicept treatment in reducing proteinuria in patients with IgA nephropathy (IgAN) was indicated in a phase II clinical trial with small sample size. In this study, we conducted a large multicenter retrospective study to explore the efficacy and safety of telitacicept in patients with IgAN.

Methods: This study recruited patients with IgAN from 19 sites from China who were treated with telitacicept and had been followed up at least once or with side effect reported, since April 1, 2021, to April 1, 2023. The primary outcomes of the study were the changing in proteinuria and eGFR over time.

Results: A cohort of 97 patients with IgAN who were treated with telitacicept were recruited, with a median follow-up duration of 3 months. The median baseline proteinuria was 2.3 [1.3, 3.9] g/day and eGFR was 45.0 [26.8, 73.7] mL/min/1.73 m2. There was a significant reduction of proteinuria at 2, 4, 6 months when compared with baseline (2.3 [1.5, 4.1] vs. 1.5 [0.8, 2.3] g/day; 2.3 [1.1, 3.7] vs. 1.1 [0.6, 1.9] g/day; 2.1 [1.0, 2.7] vs. 0.9 [0.5, 1.7] g/day, all p values <0.01). The level of eGFR were comparable between at the baseline and 2, 4, 6 months of follow-up time (41.5 [29.7, 72.0] vs. 42.5 [28.8, 73.3] mL/min/1.73 m2; 41.0 [26.8, 67.7] vs. 44.7 [31.0, 67.8] mL/min/1.73 m2; 33.7 [24.0, 58.5] vs. 32.6 [27.8, 57.5] mL/min/1.73 m2, all p values >0.26). Telitacicept was well tolerated in the patients.

Conclusions: This study indicates that telitacicept alone or on top of steroids therapy can significantly and safely reduce proteinuria in patients with IgAN. The long-term kidney protection still needs to be confirmed in large phase III trial.

引言 一项样本量较小的 II 期临床试验显示,泰利昔普能有效减少 IgA 肾病(IgAN)患者的蛋白尿。在本研究中,我们进行了一项大型多中心回顾性研究,以探讨泰利昔普在 IgAN 患者中的疗效和安全性。方法 本研究招募了中国 19 个研究机构的 IgAN 患者,这些患者自 2021 年 4 月 1 日至 2023 年 4 月 1 日接受过泰利肝素治疗,并至少接受过一次随访或有副作用报告。研究的主要结果是蛋白尿和 eGFR 随时间的变化。结果 共招募了97名接受泰利肝素治疗的IgAN患者,中位随访时间为3个月。中位基线蛋白尿为 2.3 [1.3, 3.9] 克/天,eGFR 为 45.0 [26.8, 73.7] 毫升/分钟/1.73 平方米。与基线相比,2、4、6 个月时的蛋白尿明显减少(2.3 [1.5, 4.1] vs. 1.5 [0.8, 2.3] 克/天;2.3 [1.1, 3.7] vs. 1.1 [0.6, 1.9] 克/天;2.1 [1.0, 2.7] vs. 0.9 [0.5, 1.7] 克/天,所有 P 值均为 0.01)。基线和随访 2、4、6 个月时的 eGFR 水平相当(41.5 [29.7, 72.0] vs. 42.5 [28.8, 73.3] ml/min/1.73m2; 41.0 [26.8, 67.7] vs. 44.7 [31.0, 67.8] ml/min/1.73m2; 33.7 [24.0, 58.5] vs. 32.6 [27.8, 57.5] ml/min/1.73m2, 所有 P 值均为 0.26)。患者对泰利他赛的耐受性良好。结论 本研究表明,单独使用泰利肝素或在类固醇治疗基础上使用泰利肝素可显著、安全地减少 IgAN 患者的蛋白尿。对肾脏的长期保护作用仍需在大型 III 期试验中得到证实。
{"title":"Efficacy and Safety of Telitacicept in IgA Nephropathy: A Retrospective, Multicenter Study.","authors":"Lijun Liu, Yimeng Liu, Juan Li, Chen Tang, Huiming Wang, Cheng Chen, Haibo Long, Xiaowen Chen, Guolan Xing, Jingru Cheng, Jianbo Liang, Xuan Peng, Liang Wang, Sijia Shao, Yongqiang Lin, Tianmu Chen, Ying Tang, Shizhong Shen, Lingyun Sun, Henglan Wu, Yuan Yu, Xuanyi Du, Hong Liu, Liyu He, Hong Liu, Meixing Ye, Wei Chen, Qiong Wen, Hong Zhang, Hongmin Cao, Jing Yuan, Hong Chen, Ming Wang, Jicheng Lv, Hong Zhang","doi":"10.1159/000540326","DOIUrl":"10.1159/000540326","url":null,"abstract":"<p><strong>Introduction: </strong>The efficacy of telitacicept treatment in reducing proteinuria in patients with IgA nephropathy (IgAN) was indicated in a phase II clinical trial with small sample size. In this study, we conducted a large multicenter retrospective study to explore the efficacy and safety of telitacicept in patients with IgAN.</p><p><strong>Methods: </strong>This study recruited patients with IgAN from 19 sites from China who were treated with telitacicept and had been followed up at least once or with side effect reported, since April 1, 2021, to April 1, 2023. The primary outcomes of the study were the changing in proteinuria and eGFR over time.</p><p><strong>Results: </strong>A cohort of 97 patients with IgAN who were treated with telitacicept were recruited, with a median follow-up duration of 3 months. The median baseline proteinuria was 2.3 [1.3, 3.9] g/day and eGFR was 45.0 [26.8, 73.7] mL/min/1.73 m2. There was a significant reduction of proteinuria at 2, 4, 6 months when compared with baseline (2.3 [1.5, 4.1] vs. 1.5 [0.8, 2.3] g/day; 2.3 [1.1, 3.7] vs. 1.1 [0.6, 1.9] g/day; 2.1 [1.0, 2.7] vs. 0.9 [0.5, 1.7] g/day, all p values &lt;0.01). The level of eGFR were comparable between at the baseline and 2, 4, 6 months of follow-up time (41.5 [29.7, 72.0] vs. 42.5 [28.8, 73.3] mL/min/1.73 m2; 41.0 [26.8, 67.7] vs. 44.7 [31.0, 67.8] mL/min/1.73 m2; 33.7 [24.0, 58.5] vs. 32.6 [27.8, 57.5] mL/min/1.73 m2, all p values &gt;0.26). Telitacicept was well tolerated in the patients.</p><p><strong>Conclusions: </strong>This study indicates that telitacicept alone or on top of steroids therapy can significantly and safely reduce proteinuria in patients with IgAN. The long-term kidney protection still needs to be confirmed in large phase III trial.</p>","PeriodicalId":18998,"journal":{"name":"Nephron","volume":" ","pages":"1-10"},"PeriodicalIF":2.3,"publicationDate":"2024-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142291732","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
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Nephron
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