Pediatric Nephrology最新文献_第8页

Long-term kidney outcomes in patients with Kabuki syndrome. 歌舞伎综合征患者的长期肾脏预后。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-05-28 DOI: 10.1007/s00467-025-06815-0

Seongjae Han, Hyeonju Lee, Peong Gang Park, Naye Choi, Yo Han Ahn, Jung Min Ko, Hee Gyung Kang

{"title":"Long-term kidney outcomes in patients with Kabuki syndrome.","authors":"Seongjae Han, Hyeonju Lee, Peong Gang Park, Naye Choi, Yo Han Ahn, Jung Min Ko, Hee Gyung Kang","doi":"10.1007/s00467-025-06815-0","DOIUrl":"10.1007/s00467-025-06815-0","url":null,"abstract":"Background: This study assessed the clinical features, prevalence of kidney and urinary manifestations, and progression of chronic kidney disease (CKD) in patients with Kabuki syndrome (KS).Methods: This retrospective cohort study enrolled patients with KS who visited a single tertiary center from 2003 to 2023.Results: Sixty-five patients (28 boys) were diagnosed with KS at a median age of 2.7 years (interquartile range [IQR] = 1.0-9.3) and followed until a median age of 9.4 years (IQR = 5.5-14.3). Genetic analysis identified KMT2D and KDM6A mutations in 59 and 3 patients, respectively. Congenital anomalies of the kidneys and urinary tract (CAKUT) were found in 21 of 62 patients (33.9%), whereas 7 of 62 patients (11.3%) patients had nephrolithiasis and/or nephrocalcinosis. Meanwhile, 19 of 56 patients (33.9%) progressed to CKD. CKD-free survival analysis illustrated that 25% and 50% of these patients progressed to CKD stage G2 at median ages of 5.8 and 24.6 years, respectively. Younger age at diagnosis and the presence of bilateral kidney anomalies were identified as significant predictors of CKD progression. CAKUT and cardiorenal syndrome were the leading causes of CKD.Conclusions: One-third of patients with KS exhibited various kidney or urinary abnormalities, and 34% progressed to CKD. Screening for kidney or urinary issues and regular follow-up of kidney function are essential for KS management.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3101-3109"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12402012/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144174464","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

Neonatal acute kidney injury as a global priority: the need for contextualized advocacy, education, and collaboration. 新生儿急性肾损伤作为全球优先事项：需要背景宣传，教育和合作。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-05-29 DOI: 10.1007/s00467-025-06832-z

Melissa S Zhou, Shina Menon, Jennifer G Jetton

引用次数: 0

The impact of deportation and disparities on pediatric kidney health: a humanitarian call to action. 驱逐出境和差异对儿童肾脏健康的影响：人道主义行动呼吁。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-03-26 DOI: 10.1007/s00467-025-06749-7

Rupesh Raina, Jieji Hu, Kenji Ishikura, Sampson Antwi, Arsheya Ahuja, Erica Bjornstad, Melvin Bonilla-Felix

引用次数: 0

The unique challenges of childhood-onset systemic lupus erythematosus and lupus nephritis patients: a proposed framework for an individualized transitional care plan. 儿童期系统性红斑狼疮和狼疮肾炎患者的独特挑战：提出了一个个性化过渡护理计划的框架。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-03-13 DOI: 10.1007/s00467-024-06654-5

Thomas Renson, Liz Lightstone, Coziana Ciurtin, Claire Gaymer, Stephen D Marks

{"title":"The unique challenges of childhood-onset systemic lupus erythematosus and lupus nephritis patients: a proposed framework for an individualized transitional care plan.","authors":"Thomas Renson, Liz Lightstone, Coziana Ciurtin, Claire Gaymer, Stephen D Marks","doi":"10.1007/s00467-024-06654-5","DOIUrl":"10.1007/s00467-024-06654-5","url":null,"abstract":"Childhood-onset systemic lupus erythematosus (cSLE) is a severe lifelong and life-threatening autoimmune disease with multi-organ involvement. Compared to those with adult-onset disease, cSLE patients have more aggressive disease with a higher prevalence of early lupus nephritis (LN) causing worse kidney and patient outcomes. The transfer of adolescent patients to adult healthcare poses several major challenges, from a disease as well as a psychosocial perspective. Transitional care even in tertiary centers can be heterogenous, suboptimal, and often even non-existent. In this comprehensive review of the literature, we synthesize the obstacles adolescents and young adults (AYA) with systemic lupus erythematosus (SLE) and LN face and how these challenges impact the transfer to adult health care. Finally, we propose a framework for a structured and individually modifiable transitional care plan, tailored to the unique needs of this population and taking into account their social and cultural background. This framework includes suggestions for the timing of the preparatory phase and the transfer itself, the composition of the transitional care team, increasing transition readiness and treatment adherence, and establishing a supportive network of peers. Efficient transitional care will optimize long-term patient outcomes.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3045-3053"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12401761/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143625873","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

Impact of urinary extravasation in posterior urethral valves: clinical implications of urinomas and ascites on kidney function. 尿外渗对后尿道瓣膜的影响：尿瘤和腹水对肾功能的临床意义。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-09-30 DOI: 10.1007/s00467-025-06984-y

Adree Khondker, Joana Dos Santos, Samer Maher, Michael Chua, Armando J Lorenzo, Mandy Rickard

{"title":"Impact of urinary extravasation in posterior urethral valves: clinical implications of urinomas and ascites on kidney function.","authors":"Adree Khondker, Joana Dos Santos, Samer Maher, Michael Chua, Armando J Lorenzo, Mandy Rickard","doi":"10.1007/s00467-025-06984-y","DOIUrl":"https://doi.org/10.1007/s00467-025-06984-y","url":null,"abstract":"Background: Urinary extravasation (UE) in patients with posterior urethral valves (PUV), in the form of urinomas or urinary ascites, is thought to represent a pop-off mechanism. Previous literature has proposed a kidney protective mechanism, although this remains controversial. Here, we performed a matched comparison to assess the effect of UE on kidney outcomes.Methods: We retrospectively reviewed our PUV database, including all patients diagnosed < 3 months of age with at least 1-year follow-up. We collected demographics, management, vesicoureteral reflux (VUR) status, and kidney function parameters. UE was defined as postnatal urinoma or urinary ascites. We performed both unadjusted and propensity-matched comparisons of patients with and without UE. Matching was used to balance age, diversion status, urinary tract infection history, presence of VUR, and nadir creatinine. The primary outcomes were 1-, 3-, and 5-year kidney outcomes.Results: Of the 138 patients meeting inclusion criteria, 27 (20%) had UE (23 urinoma, 4 urinary ascites). The median age at presentation was 5 days. Six patients (26%) required percutaneous drainage, and the median time to resolution was 21 days. Patients with UE had significantly higher initial creatinine levels but no difference in nadir values. Of those with available data, 18 (18%) and 10 (14%) had chronic kidney disease (CKD) at 3 and 5 years. Overall, there was no difference in 3- and 5-year rates of CKD between patients with and without a history of UE.Conclusions: In a matched comparison, UE was not associated with long-term adverse or beneficial effects on kidney function. This study provides further evidence that the presence of UE may not be a relevant prognostic factor in children with PUV.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145200642","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

Urinary leukotriene E4 for predicting steroid sensitivity in children with nephrotic syndrome: an observational cohort study. 尿白三烯E4预测儿童肾病综合征类固醇敏感性：一项观察性队列研究

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-09-30 DOI: 10.1007/s00467-025-06952-6

Nehal Saad, Amal Osman, Mostafa Mansour, Ashraf M Bakr

{"title":"Urinary leukotriene E4 for predicting steroid sensitivity in children with nephrotic syndrome: an observational cohort study.","authors":"Nehal Saad, Amal Osman, Mostafa Mansour, Ashraf M Bakr","doi":"10.1007/s00467-025-06952-6","DOIUrl":"https://doi.org/10.1007/s00467-025-06952-6","url":null,"abstract":"Background: Nephrotic syndrome (NS) is a common pediatric kidney disorder characterized by proteinuria, hypoalbuminemia, and edema. Leukotrienes (LTs), as inflammatory mediators, may contribute to NS pathogenesis and influence treatment response. This study aimed to assess urinary leukotriene E4 (LTE4) levels in children with an initial onset of NS and evaluate their potential as biomarkers for steroid responsiveness.Methods: In this observational cohort study, 41 children with a first episode of NS and 41 age- and sex-matched healthy controls were enrolled. Patients were classified into steroid-sensitive NS (SSNS; n = 29) and steroid-resistant NS (SRNS; n = 12) groups following initial steroid therapy. Urinary LTE4 levels were measured prior to treatment, using enzyme-linked immunosorbent assay (ELISA).Results: Urinary LTE4 levels were significantly elevated in children with NS compared to controls (p = 0.001). Although urinary LTE4 to urinary creatinine (U cr) ratios were also higher in patients, the difference did not reach statistical significance (p = 0.09). No significant correlations were observed between urinary LTE4 levels and urinary protein excretion or serum albumin. Furthermore, urinary LTE4 levels did not significantly differ between SSNS and SRNS groups. A receiver operating characteristic (ROC) curve analysis showed poor predictive value of urinary LTE4 for steroid responsiveness, with area-under-the-curve (AUC) values near 0.5.Conclusions: While urinary LTE4 levels are elevated in children with NS, they failed to reliably differentiate between SSNS and SRNS. These findings suggest a limited role for urinary LTE4 as a predictive biomarker of steroid responsiveness in pediatric NS. However, future large-scale studies incorporating both plasma and urinary leukotriene profiles are warranted to validate its role in disease pathogenesis and treatment response.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145200602","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

Emphysematous pyelonephritis and infection-related calculi. 肺气性肾盂肾炎和感染相关性结石。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-09-30 DOI: 10.1007/s00467-025-06918-8

Shimrit Tzvi-Behr, Yaacov Frishberg, Ruth Cytter-Kuint, Boris Chertin, Ilan Z Kafka, Efrat Ben-Shalom

引用次数: 0

Is home best when it comes to pediatric hemodialysis? 儿童血液透析在家进行最好吗？

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-09-30 DOI: 10.1007/s00467-025-06982-0

Sarah J Swartz, Nicholas G Larkins

引用次数: 0

Letter to the Editor: Long-term kidney outcomes in patients with Kabuki syndrome. 致编辑的信：歌舞伎综合征患者的长期肾脏预后。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-09-28 DOI: 10.1007/s00467-025-06976-y

Zoha Mirza, Laiba Fiaz, Muhammad Irfan

引用次数: 0

Urinary periostin as a novel non-invasive biomarker for kidney scarring in pediatric vesicoureteral reflux patients. 尿骨膜蛋白作为儿科膀胱输尿管反流患者肾脏瘢痕形成的一种新的无创生物标志物。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-09-27 DOI: 10.1007/s00467-025-06953-5

Reza Mohammadi Farsani, Amirreza Shamshirgaran, Seyyed Mohammad Ghahestani, Hossein Amirzargar, Fateme Guitynavard, Hossein Chivaee, Fardin Asgari, Mohammad Hadi Samadi, Parsa Nikoofar, Seyed Mohammad Kazem Aghamir

{"title":"Urinary periostin as a novel non-invasive biomarker for kidney scarring in pediatric vesicoureteral reflux patients.","authors":"Reza Mohammadi Farsani, Amirreza Shamshirgaran, Seyyed Mohammad Ghahestani, Hossein Amirzargar, Fateme Guitynavard, Hossein Chivaee, Fardin Asgari, Mohammad Hadi Samadi, Parsa Nikoofar, Seyed Mohammad Kazem Aghamir","doi":"10.1007/s00467-025-06953-5","DOIUrl":"https://doi.org/10.1007/s00467-025-06953-5","url":null,"abstract":"Background: Vesicoureteral reflux (VUR) is a common pediatric urological condition associated with kidney scarring, which can lead to hypertension, proteinuria, and chronic kidney disease. Current diagnostic methods, such as 99mTc-dimercaptosuccinic acid (DMSA) scans, are costly, involve radiation, and fail to detect early fibrosis. Urinary periostin, an extracellular matrix protein upregulated in kidney fibrosis, holds promise as a non-invasive biomarker for kidney scarring in VUR patients. This study aimed to evaluate urinary periostin as a biomarker for kidney scarring in children with VUR and compare its diagnostic performance with DMSA scans.Methods: This prospective case-control study enrolled 60 children (35 males and 25 females) aged between 1 and 140 months (with mean (SD) of 43.7 (34.45)) with VUR (30 with kidney scarring [Scar+] and 30 without [Scar-]). Urinary periostin levels were measured via ELISA and corrected with urine creatinine. DMSA scans confirmed scarring. Receiver operating characteristic (ROC) analysis assessed periostin's diagnostic accuracy, and logistic regression identified predictors of scarring.Results: Urinary periostin levels were significantly higher in Scar+ patients (27.4 ± 6.64 ng/mL) than in Scar- patients (18.1 ± 4.63 ng/mL, p < 0.001). ROC analysis yielded an area under the curve of 0.869, with a 21.5 ng/mL cutoff showing 80.0% sensitivity and 76.6% specificity. Periostin (OR 1.44, p < 0.001) was the only independent predictor of scarring. Periostin levels correlated with VUR grade (r = 0.63, p < 0.001).Conclusions: Urinary periostin is a highly sensitive and specific non-invasive biomarker for detecting kidney scarring in VUR, potentially reducing reliance on DMSA scans.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145177139","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