Pediatric Nephrology最新文献_第4页

A new era in the treatment of kidney diseases: NLRP3 inflammasome and cytokine-targeted therapies. 肾脏疾病治疗的新时代：NLRP3炎性体和细胞因子靶向疗法。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2024-11-01 DOI: 10.1007/s00467-024-06578-0

Emre Leventoğlu, Sevcan A Bakkaloğlu

{"title":"A new era in the treatment of kidney diseases: NLRP3 inflammasome and cytokine-targeted therapies.","authors":"Emre Leventoğlu, Sevcan A Bakkaloğlu","doi":"10.1007/s00467-024-06578-0","DOIUrl":"10.1007/s00467-024-06578-0","url":null,"abstract":"The kidneys are crucial for filtering blood, managing overall body water, electrolyte, and acid-base balance, and regulating blood pressure. They remove metabolic waste products, toxins, and drugs. In addition, they limit inflammation by clearing cytokines and reduce immune cell activation by removing bacterial components. Dendritic cells (DCs) in the kidney maintain peripheral tolerance. About 85% of filtered water is reabsorbed by the proximal tubule, exposing distal nephron cells to high concentrations of low molecular weight antigens. These antigens are captured by DCs, helping to inactivate potentially autoreactive T cells and maintain tolerance to circulating antigens. In kidney failure, immune function is severely compromised due to the retention of toxins and cytokines, which activate immune cells and increase systemic inflammation. The kidneys are also vulnerable to immune-mediated diseases. Loss of immune homeostasis, characterized by over- or under-activity of the immune response, can adversely affect kidney function. With advances in immunology and cellular biology, biologic therapies targeting various pathways involved in the pathophysiology of kidney diseases are being developed. In this review, the immunologic aspects of kidney diseases and focus on cytokine-based therapies that may hold promise for the treatment of kidney diseases in the future will be presented.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1515-1521"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142562953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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

Prolonged remission after cyclophosphamide or tacrolimus treatment in childhood nephrotic syndrome: a cohort study. 儿童肾病综合征患者接受环磷酰胺或他克莫司治疗后病情长期缓解：一项队列研究。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2024-11-22 DOI: 10.1007/s00467-024-06605-0

Cal H Robinson, Nowrin Aman, Tonny Banh, Josefina Brooke, Rahul Chanchlani, Vaneet Dhillon, Valerie Langlois, Leo Levin, Christoph Licht, Ashlene McKay, Damien Noone, Alisha Parikh, Rachel Pearl, Seetha Radhakrishnan, Veronique Rowley, Chia Wei Teoh, Jovanka H Vasilevska-Ristovska, Rulan S Parekh

{"title":"Prolonged remission after cyclophosphamide or tacrolimus treatment in childhood nephrotic syndrome: a cohort study.","authors":"Cal H Robinson, Nowrin Aman, Tonny Banh, Josefina Brooke, Rahul Chanchlani, Vaneet Dhillon, Valerie Langlois, Leo Levin, Christoph Licht, Ashlene McKay, Damien Noone, Alisha Parikh, Rachel Pearl, Seetha Radhakrishnan, Veronique Rowley, Chia Wei Teoh, Jovanka H Vasilevska-Ristovska, Rulan S Parekh","doi":"10.1007/s00467-024-06605-0","DOIUrl":"10.1007/s00467-024-06605-0","url":null,"abstract":"Background: Steroid-sparing immunosuppression is used in 50% of children with nephrotic syndrome, to prevent relapses and steroid-related toxicity. However, rates and predictors of prolonged remission after cyclophosphamide and tacrolimus are uncertain.Methods: Retrospective analysis of children (1-18 years) enrolled in a longitudinal cohort. We included children diagnosed with steroid-sensitive nephrotic syndrome between 1996-2019 from Toronto, Canada. The exposure was cyclophosphamide or tacrolimus initiation. The primary outcome was prolonged remission (no further relapse or steroid-sparing immunosuppression). We evaluated predictors of prolonged remission and calcineurin inhibitor nephrotoxicity by logistic regression.Results: Of 578 children with steroid-sensitive nephrotic syndrome, 252 received cyclophosphamide and 120 received tacrolimus. Over median 5.4-year (IQR 2.4-9.1) follow-up, prolonged remission occurred in 72 (28.6%) after cyclophosphamide and 17 (14.2%) after tacrolimus. Relapse frequency decreased after initiation of either medication. Lower prior relapse rate, more recent treatment era, and female sex were predictive of prolonged remission after cyclophosphamide treatment. Use of tacrolimus as the first steroid-sparing medication was the only factor predictive of calcineurin inhibitor nephrotoxicity.Conclusions: Less than one-third of children achieve prolonged remission after initiating cyclophosphamide or tacrolimus, although both reduce short-term relapse rates. Few factors predict prolonged remission after cyclophosphamide or tacrolimus use, or calcineurin inhibitor nephrotoxicity.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1625-1634"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142688408","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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

Left atrial mechanics in youth with chronic kidney disease and similarly aged healthy controls. 青年慢性肾病患者左心房力学及相似年龄健康对照

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2025-01-14 DOI: 10.1007/s00467-024-06600-5

Alexander J Kula, Jeanne Frisby-Zedan, Jarrett R Linder, Amanda Hauck

{"title":"Left atrial mechanics in youth with chronic kidney disease and similarly aged healthy controls.","authors":"Alexander J Kula, Jeanne Frisby-Zedan, Jarrett R Linder, Amanda Hauck","doi":"10.1007/s00467-024-06600-5","DOIUrl":"10.1007/s00467-024-06600-5","url":null,"abstract":"Background: In adults with chronic kidney disease (CKD), abnormal left atrial reservoir strain (LASr) is an early, yet clinically significant, indicator of myocardial dysfunction. However, left atrial mechanics are understudied in youth with CKD. The objective of this study was to assess left atrial strain function in youth with CKD and similarly aged, healthy controls.Methods: We performed a single-center, retrospective, observational study of persons aged 12-21 years with stage 3-4 CKD and healthy controls. Exclusion criteria included a history of a kidney or other solid organ transplant, congenital heart disease, and/or dialysis requirement < 3 months prior. We measured LAS (LASr, conduit, and contractile), E/e', E/A, left ventricular mass index (LVMI), and ejection fraction. Pearson correlations were performed between echocardiographic measures.Results: This study included 37 patients with CKD and 19 controls. Mean age was similar between groups, and male sex was over-represented in both groups (CKD 62%; healthy 63%). Mean ± standard deviation (SD) eGFR in the CKD group was 32 ± 14 mL/min/1.73 m2. Mean absolute LASr was lower in those with CKD (43.0 ± 8.5%) compared to healthy controls (47.4 ± 6.1%) p = 0.050. Patients with CKD had significantly higher LVMI and lower E/A and E' compared to controls. There was poor correlation between LASr with E/A, E/e', and LVMI.Conclusions: As observed in adults with CKD, LASr trended lower in youth with CKD compared to healthy controls. Moreover, LASr poorly correlated with traditional measures of diastolic dysfunction such as E/e' and E/A.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1731-1739"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142979484","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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

Long-term kidney outcomes in survivors of Wilms tumor: a single-center retrospective cohort study. 肾母细胞瘤幸存者的长期肾脏预后：一项单中心回顾性队列研究

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2025-01-09 DOI: 10.1007/s00467-024-06624-x

Shannon Reinert, Stefanie W Benoit, Rajaram Nagarajan

{"title":"Long-term kidney outcomes in survivors of Wilms tumor: a single-center retrospective cohort study.","authors":"Shannon Reinert, Stefanie W Benoit, Rajaram Nagarajan","doi":"10.1007/s00467-024-06624-x","DOIUrl":"10.1007/s00467-024-06624-x","url":null,"abstract":"Background: Several studies have investigated long-term kidney outcomes in survivors of Wilms tumor (WT). However, many have small sample sizes, and there is a wide variation in reported outcomes. The aim of this study is to investigate the long-term kidney outcomes in survivors of WT (S-WT), including those patients considered to be at high risk for poor kidney outcomes, and using updated estimated glomerular filtration rate (eGFR) equations.Methods: This was a retrospective chart review of 64 patients treated for WT at a single pediatric center. Patients were off treatment for 5 years or more at the time of analysis and were evaluated for decreased kidney function, hypertension, proteinuria, and compensatory hypertrophy of the contralateral kidney.Results: At a median follow-up time of 11.3 years off treatment (range 5-22.6) and average age of 16.7 years (range 6.5-30), 35 patients had a decreased eGFR (< 90 mL/min/1.73 m2), and 2 patients had progressed to chronic kidney disease stage 5. Compensatory hypertrophy was observed in 67% of cases. 41% of patients had elevated clinic blood pressures, with 2 patients on an anti-hypertensive medication. Three of 9 patients had evidence of hypertension on ambulatory blood pressure monitoring. Eight of 37 patients (22%) had proteinuria.Conclusions: Kidney dysfunction is common in S-WT at a young age. This population should be carefully monitored for the development of decreased eGFR, hypertension, and proteinuria as part of their routine survivorship care. This is particularly true for modifiable risk factors of chronic kidney disease progression, such as hypertension and proteinuria.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1603-1611"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11947031/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142952575","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Is body composition important in the context of renal function in pediatric neurogenic bladder? 身体成分对小儿神经源性膀胱的肾功能是否重要？

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2024-10-17 DOI: 10.1007/s00467-024-06557-5

Joanna Bagińska-Chyży, Adrianna Błahuszewska, Agata Korzeniecka-Kozerska

{"title":"Is body composition important in the context of renal function in pediatric neurogenic bladder?","authors":"Joanna Bagińska-Chyży, Adrianna Błahuszewska, Agata Korzeniecka-Kozerska","doi":"10.1007/s00467-024-06557-5","DOIUrl":"10.1007/s00467-024-06557-5","url":null,"abstract":"Background: Neurogenic bladder due to myelomeningocele (MMC) is a significant risk factor for chronic kidney disease in children. Cystatin C (CysC) is a more accurate GFR marker than creatinine as it is unaffected by muscle mass but may be influenced by fat mass and BMI. This study evaluates: (1) GFR measurement accuracy using CysC and creatinine in MMC-related neurogenic bladder, (2) the relationship between body composition via bioelectrical impedance analysis (BIA) and renal parameters, and (3) the use of BIA for non-invasive GFR and body composition assessment.Methods: Forty children (median age 9.96 years) underwent serum creatinine, CysC testing, and BIA measurements. We assessed age, sex, spinal lesion level, anthropometric measurements, BMI, and activity using Hoffer's scale. GFR was calculated using five creatinine-based formulas, three CysC-based, and three combining CysC and creatinine, including BIA GFR as an alternative approach.Results: Creatinine-based GFR estimates were significantly higher than CysC-based GFR. Although only 30% of MMC patients met the traditional BMI criteria for overweight/obesity, 62.5% were obese based on BIA-measured body fat percentage. Significant differences were found in CysC and CysC-based GFR equations within BMI and fat mass groups. Positive correlations were observed between CysC and body weight, BMI percentiles, body fat mass and fat-to-muscle ratio. Muscle mass positively correlated with creatinine.Conclusions: BIA-determined fat mass percentage is a more sensitive obesity indicator than BMI in MMC patients. CysC levels and CysC-based GFR equations are influenced by body fat mass, requiring consideration of adiposity to avoid misestimating renal impairment.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1677-1687"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11946935/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142471846","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

PDSS1 mutations-associated steroid-resistant nephrotic syndrome: case report and review of literature. PDSS1突变相关的类固醇抵抗性肾病综合征：病例报告和文献复习。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2024-12-10 DOI: 10.1007/s00467-024-06596-y

Clair Habib, Galit Tal, Karin Weiss, Daniella Magen, Shirley Pollack

引用次数: 0

Voices of Resilience - integrating psychosocial support and music for children with kidney disease.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-04-03 DOI: 10.1007/s00467-025-06766-6

Alison Lap-Tak Ma, Fanny Tsz-Wai Ho, Eugene Yu-Hin Chan

引用次数: 0

A machine learning model to predict intradialytic hypotension in pediatric continuous kidney replacement therapy.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-04-03 DOI: 10.1007/s00467-025-06764-8

Jian-An Wang, Hsiang-Wei Hu, Yuan-Yow Chiou, Kuan-Yu Chen, Chun-Chuan Hsueh, Chih-Chia Chen

{"title":"A machine learning model to predict intradialytic hypotension in pediatric continuous kidney replacement therapy.","authors":"Jian-An Wang, Hsiang-Wei Hu, Yuan-Yow Chiou, Kuan-Yu Chen, Chun-Chuan Hsueh, Chih-Chia Chen","doi":"10.1007/s00467-025-06764-8","DOIUrl":"https://doi.org/10.1007/s00467-025-06764-8","url":null,"abstract":"Background: Intradialytic hypotension (IDH) is associated with mortality in adults undergoing intermittent hemodialysis, but this relationship is unclear in critically ill children receiving continuous kidney replacement therapy (CKRT). We aim to evaluate the relationship between IDH and hospital mortality and if pressure data from dialysis machines could predict IDH.Methods: We conducted a retrospective cohort study in a tertiary pediatric intensive care unit and NICU from December 2019 to July 2022, including 23 patients across 38 admissions (median age 10 years).Results: IDH proportion was significantly associated with mortality (risk ratio [RR]: 4.40, 95% confidence interval [CI]: 1.22-15.90, p = 0.02). Random Forest models using Entropy or Gini criteria demonstrated high sensitivity. The CatBoost model achieved the highest average F1-score and area under the receiver operating characteristic (ROC) curve (AUC) (88.18% and 86.6% with and without dialysis settings, respectively). Local Interpretable Model-agnostic Explanations (LIME) indicated that dialysis machine-derived time-series pressure parameters were critical predictive features for IDH, whereas blood pressure-related variables were not among the top predictors.Conclusions: Dialysis machine-derived pressure parameters may serve as effective predictive markers for IDH, which is associated with increased mortality. These findings support the potential of integrating pressure data in the early detection and management of IDH in pediatric CKRT patients.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143772750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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

Successful initiation of continuous kidney replacement therapy in an extremely premature infant.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-04-03 DOI: 10.1007/s00467-025-06763-9

Ranganatha A Devaranavadagi, Joel Thomas, S Vishwanath, Karthik Nagesh

引用次数: 0

Acute kidney injury after swimming: a pediatric case of McArdle disease.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-04-03 DOI: 10.1007/s00467-025-06758-6

Dorna Derakhshan, Ali Derakhshan, Faizan Bashir

引用次数: 0