Pediatric Nephrology最新文献_第4页

Role of INM004 Shiga-toxin antibodies in treatment of STEC-HUS.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-02-06 DOI: 10.1007/s00467-025-06704-6

Sharon Andreoli, Leo Monnens

引用次数: 0

Assessment of obesity in pediatric kidney transplant recipients: an ongoing challenge.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-02-06 DOI: 10.1007/s00467-025-06706-4

Athanasia Chainoglou, Kleo Evripidou, Stella Stabouli

引用次数: 0

A 15-year experience highlighting the spectrum of Alport kidney disease in the pediatric population and novel genetic variants in COL4A3-5. 15 年的经验突显了阿尔波特肾病在儿童群体中的发病范围以及 COL4A3-5 的新型基因变异。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-02-05 DOI: 10.1007/s00467-025-06683-8

Nastja Andrejašič, Anja Blejc Novak, Mirjam Močnik, Nataša Marčun Varda, Špela Stangler Herodež, Danijela Krgović, Andrej Zupan, Anamarija Meglič

{"title":"A 15-year experience highlighting the spectrum of Alport kidney disease in the pediatric population and novel genetic variants in COL4A3-5.","authors":"Nastja Andrejašič, Anja Blejc Novak, Mirjam Močnik, Nataša Marčun Varda, Špela Stangler Herodež, Danijela Krgović, Andrej Zupan, Anamarija Meglič","doi":"10.1007/s00467-025-06683-8","DOIUrl":"https://doi.org/10.1007/s00467-025-06683-8","url":null,"abstract":"Background: Alport kidney disease (AKD) presents one of the most prevalent genetic kidney disorders, characterized by a complex genetic background and diverse clinical manifestations. This study aimed to review the clinical and genetic features of pediatric patients with COL4A3-5 variants and identify novel genetic variants.Methods: Data were collected retrospectively at a national level from pediatric patients up to 19 years old, who underwent genetic testing between 2008 and 2023. Patients with pathogenic and likely pathogenic COL4A3-5 variants were included. Their clinical, laboratory, and genetic characteristics were presented.Results: Over 15 years, 85 children and adolescents tested positive for pathogenic or likely pathogenic COL4A3-5 variants. Increasing incidence was noted as genetic testing became more prevalent. One patient (1.2%) progressed to kidney failure and six (7%) had extrarenal involvement. Pathogenic or likely pathogenic variants in COL4A3, COL4A4, and COL4A5 genes were found in 14 (16.4%), 34 (40.0%), and 37 (43.6%) patients, respectively. Patients were diagnosed with autosomal, X-linked, and digenic AKD in 55.2%, 43.6%, and 1.2%, respectively. Eight novel variants were recorded, and their associated phenotype presented.Conclusions: This study expands the genetic and clinical background of pediatric patients with AKD, presenting on a spectrum from mild hematuria to progressive chronic kidney disease. Genetic confirmation and risk stratification in the pediatric population are critical to ensure timely care and potentially slow down the progression of kidney disease.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143190041","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

Making a start - WE-ROCK registry and the aim of global equity in pediatric CKRT.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-02-05 DOI: 10.1007/s00467-025-06687-4

Shina Menon, Mignon McCulloch, Katja M Gist

引用次数: 0

Challenges in blood pressure measurement in children with obesity: focus on the cuff. 肥胖儿童血压测量的挑战：关注袖带。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-02-05 DOI: 10.1007/s00467-025-06678-5

Kleo Evripidou, Athanasia Chainoglou, Vasilios Kotsis, Stella Stabouli

{"title":"Challenges in blood pressure measurement in children with obesity: focus on the cuff.","authors":"Kleo Evripidou, Athanasia Chainoglou, Vasilios Kotsis, Stella Stabouli","doi":"10.1007/s00467-025-06678-5","DOIUrl":"https://doi.org/10.1007/s00467-025-06678-5","url":null,"abstract":"Childhood obesity is a well-known risk factor for primary hypertension (HTN), especially during puberty. Validated automated oscillometric devices based on universal protocols using proper cuffs according to arm sizes must be selected for blood pressure (BP) measurement in clinical practice. Recent recommendations have addressed the importance of accurate BP measurement in patients with obesity. The American Heart Association (AHA) suggests using a cone-shaped cuff, while the European Society of Hypertension (ESH) emphasizes the significance of cuff characteristics based on arm size. The applicability of cone-shaped cuffs over cylindrical cuffs has been studied in adults with obesity. Most studies focused on the upper arm's shape and provided evidence that the use of cylindrical cuffs may overestimate BP in individuals with obesity, while the conical cuff was proposed as a proper alternative. However, current validation studies for BP measurement devices have not included the arm's size as a recruiting criterion, which may compromise the accuracy of devices in individuals with obesity. This review discusses the role of arm characteristics in cuff selection and the potential role of conical cuffs as an alternative to commonly used cylindrical ones in BP measurement in pediatric patients with obesity.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143190053","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

Post-transplant diarrhea in pediatric kidney transplant recipients.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-02-05 DOI: 10.1007/s00467-024-06572-6

Machi Kaneko McBee, Lavjay Butani

引用次数: 0

Urinary Dickkopf-related protein 3 as a novel biomarker for kidney function decline in children with Alport syndrome.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-02-04 DOI: 10.1007/s00467-025-06696-3

Jan Boeckhaus, Burkhard Tönshoff, Lutz T Weber, Lars Pape, Kay Latta, Henry Fehrenbach, Baerbel Lange-Sperandio, Matthias Kettwig, Sabine König, Ulrike John-Kroegel, Jutta Gellermann, Matthias Galiano, Sima Jami, Dennis Pieper, Gry Helene Dihazi, Angelika Hafke, Stefan Kohl, Max C Liebau, Jens König, Dieter Haffner, Oliver Gross, Manuel Wallbach

{"title":"Urinary Dickkopf-related protein 3 as a novel biomarker for kidney function decline in children with Alport syndrome.","authors":"Jan Boeckhaus, Burkhard Tönshoff, Lutz T Weber, Lars Pape, Kay Latta, Henry Fehrenbach, Baerbel Lange-Sperandio, Matthias Kettwig, Sabine König, Ulrike John-Kroegel, Jutta Gellermann, Matthias Galiano, Sima Jami, Dennis Pieper, Gry Helene Dihazi, Angelika Hafke, Stefan Kohl, Max C Liebau, Jens König, Dieter Haffner, Oliver Gross, Manuel Wallbach","doi":"10.1007/s00467-025-06696-3","DOIUrl":"https://doi.org/10.1007/s00467-025-06696-3","url":null,"abstract":"Background: Chronic kidney disease (CKD) seriously affects the well-being and shortens the life expectancy of children and adolescents, but its progression is challenging to predict. Therefore, there is an urgent need for biomarkers that can identify children at risk of faster CKD progression. Alport syndrome (AS) is the most common monogenetic glomerular kidney disease. Urinary Dickkopf-related protein 3 (DKK3) is associated with a decline in estimated glomerular filtration rate (eGFR) in adults and children with advanced CKD. However, its potential for early detection of CKD and its prognostic value in children with AS remain unknown.Methods: Urine samples from 49 children enrolled in the EARLY PRO-TECT Alport trial were analyzed to evaluate whether DKK3 could identify children with AS to be at risk for faster CKD progression.Results: DKK3 levels in patients with AS were higher than those of healthy individuals reported in the literature. DKK3 levels were more elevated in patients with later stages of AS. Furthermore, children who were not treated with renin angiotensin system inhibitors (RASi) had higher DKK3 levels than treated children. Children with above-average DKK3 levels were more likely to have increased albuminuria after 2 years of follow-up than children with below-average DKK3 levels.Conclusion: Urinary DKK3 is significantly elevated in children at early stages of AS. There was a potential association between higher DKK3 levels, worsening albuminuria, and a decline in kidney function. These findings suggest that DKK3 may be a prognostic marker for predicting the risk of kidney damage in children with AS.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143190020","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

Disaster preparedness and kidney health in children.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-02-04 DOI: 10.1007/s00467-025-06693-6

Melvin Bonilla-Felix, Rupesh Raina, Ali Düzova, Rajiv Sinha, Sampson Antwi, Erica C Bjornstad, Kenji Ishikura

引用次数: 0

Reference ranges for 24-h urinary protein/creatinine ratio and urinary microalbumin/creatinine ratio in Chinese children. 中国儿童 24 小时尿蛋白/肌酐比值和尿微量白蛋白/肌酐比值的参考范围。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-02-04 DOI: 10.1007/s00467-025-06681-w

Lele Ding, Ying Ding, Shanshan Xu, Shanshan Han, Xuejun Li, Bo Zhang, Xia Zhang, Cancan Li, Mingjie Wei, Xiaofeng Mei, Yuqin Chen

{"title":"Reference ranges for 24-h urinary protein/creatinine ratio and urinary microalbumin/creatinine ratio in Chinese children.","authors":"Lele Ding, Ying Ding, Shanshan Xu, Shanshan Han, Xuejun Li, Bo Zhang, Xia Zhang, Cancan Li, Mingjie Wei, Xiaofeng Mei, Yuqin Chen","doi":"10.1007/s00467-025-06681-w","DOIUrl":"https://doi.org/10.1007/s00467-025-06681-w","url":null,"abstract":"Background: The purpose of this study was to investigate the age and gender characteristics of 24-h urinary protein/creatinine ratio (24hUPCR) and urinary microalbumin/creatinine ratio (UMACR) among Chinese children and other related factors, and to establish preliminary reference ranges.Methods: A total of 200 healthy children aged 2-15 years were enrolled. We divided the subjects into twelve groups according to age. Kruskal-Wallis one-way ANOVA test and Spearman correlation analysis were used to compare 24hUPCR and UMACR with other variables and 95% reference range was used to establish the reference range.Results: 24hUPCR and UMACR showed correlation with age, height, weight, and body mass index (BMI) (p < 0.001), and females had higher levels than males among all healthy children (p < 0.01). 6 and 8 years of age were the critical nodes in the change of 24hUPCR and UMACR levels, respectively. The reference ranges established based on the 200 Chinese children in this study are as follows: 24hUPCR: < 24.34 mg/mmol (male) and < 43.04 mg/mmol (female) for ≥ 2-6 years; < 17.60 mg/mmol (male) and < 22.80 mg/mmol (female) for ≥ 6-15 years.Umacr: < 1.53 mg/mmol (male) and < 1.74 mg/mmol (female) for ≥ 2-8 years, < 1.13 mg/mmol (male) and < 3.48 mg/mmol (female) for ≥ 8-15 years.Conclusions: 24hUPCR and UMACR in children were correlated with age, gender, height, weight, BMI, and ethnicity. Establishing the right pediatric cutoff values that account for age, gender, and ethnicity can guarantee the sensitivity and specificity needed for clinical applications.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143190017","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 health outcomes of Shiga toxin-producing Escherichia coli O157 (STEC O157) infection and STEC-associated haemolytic uraemic syndrome (STEC-HUS), Wales, 1990-2020.

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-02-04 DOI: 10.1007/s00467-024-06640-x

Rachel Merrick, Jiao Song, Laia Fina, Clare Sawyer, Claire Jenkins, Grace King, Drew Turner, Daniel Thomas, Christopher Williams

{"title":"Long-term health outcomes of Shiga toxin-producing Escherichia coli O157 (STEC O157) infection and STEC-associated haemolytic uraemic syndrome (STEC-HUS), Wales, 1990-2020.","authors":"Rachel Merrick, Jiao Song, Laia Fina, Clare Sawyer, Claire Jenkins, Grace King, Drew Turner, Daniel Thomas, Christopher Williams","doi":"10.1007/s00467-024-06640-x","DOIUrl":"https://doi.org/10.1007/s00467-024-06640-x","url":null,"abstract":"Background: Information on sequelae of Shiga toxin-producing Escherichia coli (STEC) O157 infection is limited to follow-up of paediatric haemolytic uraemic syndrome (HUS) cases. We investigate recorded long-term health outcomes experienced by individuals exposed to STEC O157 and STEC-HUS up to three decades on.Methods: We compared acute or new onset of chronic outcomes in individuals ≥ 1 year after STEC O157 or STEC-HUS to unexposed general population comparators between 01/01/1990-01/01/2019. The unexposed were their age- and sex-equivalents (4:1 matching ratio) and assigned the same study entry date. Outcomes were identified in primary and secondary care and categorised as kidney, neurological, cardiac, gastrointestinal, respiratory, or endocrine. Hazard ratios (HRs) and 95% confidence intervals (95% CI) were calculated using Cox regression.Results: Of 1,245 individuals with STEC O157, 65 developed HUS (5.2%). Individuals with STEC O157 were more likely to experience kidney (adjusted (a)HR: 1.9, 95% CI: 1.1-3.3), gastrointestinal (aHR: 1.7, 95% CI: 1.1-2.5) and respiratory (aHR: 1.4, 95% CI: 1.2-1.6) outcomes compared to the unexposed, on average between 3.4-11 years after exposure. Gastrointestinal (HR: 7.7, 95% CI: 2.6-23), kidney (HR: 5.5, 95% CI: 1.6-19), cardiac (HR: 5.1, 95% CI: 1.1-23) and respiratory (HR: 1.9, 95% CI: 1.1-3.1) outcomes were more common in the STEC-HUS cohort and occurred sooner, on average after 2.7-4.8 years.Conclusions: Long-term complications were nearly twice as likely in the STEC O157 cohort, and as many as eight times more likely following STEC-HUS. We recommend that those exposed to STEC be monitored for at least five years for late-emerging kidney and extrarenal complications.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143190068","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