Pediatric Nephrology最新文献_第9页

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

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

Gut microbiota-targeted therapies in pediatric chronic kidney disease: gaps and opportunities. 儿童慢性肾病的肠道微生物群靶向治疗：差距和机遇。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 DOI: 10.1007/s00467-025-06789-z

Arpana Iyengar, Balamurugan Ramadass, Shruthi Venkatesh, Robert H Mak

{"title":"Gut microbiota-targeted therapies in pediatric chronic kidney disease: gaps and opportunities.","authors":"Arpana Iyengar, Balamurugan Ramadass, Shruthi Venkatesh, Robert H Mak","doi":"10.1007/s00467-025-06789-z","DOIUrl":"https://doi.org/10.1007/s00467-025-06789-z","url":null,"abstract":"Given the complex relationship between the gut microbiome and chronic kidney disease (CKD), exploring the potential role and scope of microbiota-targeted therapies in pediatric CKD is highly relevant. We aim to provide an overview of gut-targeted therapeutic strategies, including nutritional interventions (fiber, phytochemicals, fermented foods, and traditional Chinese medicines), probiotics, synbiotics, oral absorbents, and fecal microbial transplantation. Enhancing physical activity and preventing constipation are additional strategies that may promote gut microbiome health. In a uremic environment, gut microbiota-targeted therapies could potentially rebalance the gut microbiota, improve gut barrier function, decrease uremic toxin concentrations, enhance the production of short-chain fatty acids (SCFA), and reduce inflammation. While research in adult CKD patients has provided insights into these approaches, there are limited data in children with CKD. This review aims to summarize potential targeted therapies for restoring a balanced gut microbiota, emphasizing the need for studies that evaluate their effects on clinical outcomes in pediatric CKD.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144041019","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

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

Anti-CD20 monoclonal antibodies for idiopathic nephrotic syndrome: Advances, challenges, and future directions. 抗cd20单克隆抗体治疗特发性肾病综合征：进展、挑战和未来方向

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 DOI: 10.1007/s00467-025-06738-w

Andrea Angeletti, Gian Marco Ghiggeri

引用次数: 0

Acute kidney injury with cast nephropathy following creatine loading in a 17-year-old. 17岁患者肌酸负荷后急性肾损伤伴铸型肾病。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-04-30 DOI: 10.1007/s00467-025-06784-4

Guido Filler, Eugene Maung, Maria Esther Díaz González de Ferris, Nancy Gain Chan, Ajay Parkash Sharma

引用次数: 0

Clinical differences between female monozygotic twins with X-linked Alport syndrome with somatic mosaicism. x -连锁Alport综合征女性同卵双胞胎体细胞嵌合体的临床差异。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-04-29 DOI: 10.1007/s00467-025-06772-8

Naoaki Mikami, Hideaki Kitakado, Naoki Kimura, Nana Sakakibara, Kandai Nozu, Riku Hamada

{"title":"Clinical differences between female monozygotic twins with X-linked Alport syndrome with somatic mosaicism.","authors":"Naoaki Mikami, Hideaki Kitakado, Naoki Kimura, Nana Sakakibara, Kandai Nozu, Riku Hamada","doi":"10.1007/s00467-025-06772-8","DOIUrl":"https://doi.org/10.1007/s00467-025-06772-8","url":null,"abstract":"Although female patients with X-linked Alport syndrome (XLAS) have a generally milder prognosis than male counterparts, some female patients show poor prognosis. The clinically observed variation in the severity of female XLAS is thought to be due mainly to X chromosome inactivation (XCI) or modifier gene variants. We describe herein a 4-year-old female patient with persistent hematuria and proteinuria whose monozygotic, twin sister had no apparent abnormality on urinalysis. A kidney biopsy of the patient found the typical pattern of female XLAS. Genetic testing revealed a de novo pathogenic variant of COL4 A5 with somatic mosaicism. However, additional tests revealed that the asymptomatic twin sister had the same COL4 A5 variant with somatic mosaicism, and that the twins had the same ratio of somatic mosaicism and XCI. Moreover, targeted exome sequencing found no other modifier gene variants, suggesting that the severity of female XLAS depends on distinct but unknown factors in addition to the XCI and modifier gene variants. The findings of the present report emphasize the need to identify the hidden factors affecting the severity of Alport syndrome.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144038952","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

Re: Renal vascular lesions in childhood-onset lupus nephritis. 儿童期狼疮性肾炎的肾血管病变。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-04-29 DOI: 10.1007/s00467-025-06786-2

Sonia, Riya Rani Lohana, Rakhi Kesrani

引用次数: 0

Erythropoietin resistance among pediatric patients on chronic hemodialysis: A cross-sectional study. 儿童慢性血液透析患者的促红细胞生成素抵抗：一项横断面研究。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-04-28 DOI: 10.1007/s00467-025-06776-4

Rasha Helmy, Fatina I Fadel, Rasha E Galal, Amira M Mohammed, Shaimaa Sayed

{"title":"Erythropoietin resistance among pediatric patients on chronic hemodialysis: A cross-sectional study.","authors":"Rasha Helmy, Fatina I Fadel, Rasha E Galal, Amira M Mohammed, Shaimaa Sayed","doi":"10.1007/s00467-025-06776-4","DOIUrl":"https://doi.org/10.1007/s00467-025-06776-4","url":null,"abstract":"Background: The erythropoietin resistance index (ERI) is an accurate indicator of erythropoietin (EPO) resistance and is related to a worse prognosis in patients on hemodialysis (HD). ERI is simple, cheap and could be calculated easily in children receiving HD. We aimed to assess the EPO resistance in children with kidney failure on regular HD.Methods: An analytical cross-sectional study was conducted on 80 children with kidney failure on regular HD. They were assessed by history taking and laboratory investigations including complete blood count, C- reactive protein (CRP), iron, ferritin, parathyroid hormone and serum electrolytes. ERI was calculated.Results: The study included 80 patients; 41 (51.2%) were male. The mean age of the study group was 8.86 ± 2.76 years. Sixty-three patients (78.8%) were on iron therapy. Mean ERI was 28.87 ± 10.62. The ERI was significantly positively correlated with age (r = 0.242; P = 0.031), EPO dose (r = 0.290; P = 0.001) and CRP (r = 0.219; P = 0.049). The ERI had a significantly negative correlation with KT/V (r = - 0.262; P = 0.019), hemoglobin level (r = - 0.265; P = 0.001) and platelet count (r = - 0.254; P = 0.023).Conclusions: Erythropoietin resistance is associated with many risk factors, including high CRP and low KT/V. Inadequate HD is the most important risk factor for EPO resistance in children on chronic HD. Adequate HD is considered as a protective measure against EPO resistance.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144035122","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