Pediatric Nephrology最新文献_第7页

Continuous increase in podocyte numbers in the first 36 months of life-insights from forensic autopsies in Japanese children. 在日本儿童的法医尸检中发现，在生命的前36个月里足细胞数量持续增加。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2025-01-10 DOI: 10.1007/s00467-024-06644-7

Kohei Takashima, Masahito Hitosugi, Akari Uno, Naoko Taniura, Ken-Ich Mukaisho, Yoshihiro Maruo

{"title":"Continuous increase in podocyte numbers in the first 36 months of life-insights from forensic autopsies in Japanese children.","authors":"Kohei Takashima, Masahito Hitosugi, Akari Uno, Naoko Taniura, Ken-Ich Mukaisho, Yoshihiro Maruo","doi":"10.1007/s00467-024-06644-7","DOIUrl":"10.1007/s00467-024-06644-7","url":null,"abstract":"Background: Podocyte depletion is a critical factor in glomerulosclerosis development. While podocyte numbers per glomerulus typically decline with age in adults, they are hypothesized to increase during childhood. However, studies on podocyte number progression in childhood have been limited.Methods: This retrospective analysis examined forensic autopsy cases of Japanese children without kidney disease, aged under 192 months, between April 2010 and March 2023. Podocytes were identified using immunostaining with an anti-transducin-like enhancer of split 4 antibody and p57. Podometric parameters were estimated using the correction factor method, allowing estimation from a single histologic section.Results: This study included 68 cases with a median age of 9 months (interquartile range [IQR], 4-78). All podometric parameters correlated with age. Children younger than 36 months displayed significantly fewer podocyte numbers per glomerulus (median, 517; IQR, 483-546) compared to those aged 36 months and older (median, 616; IQR, 595-649; p < 0.001). Regression analysis revealed a significant age-related increase in podocyte numbers per glomerulus in children under 36 months (slope, 3.76; p < 0.001; 95% confidence interval [CI], 2.34-5.19), but not in those aged 36 months and older (slope, 0.25; p = 0.16; 95% CI, - 0.10-0.61). Additionally, the change in the slope at 36 months was significant (p < 0.001; 95% CI, 1.02-2.49); however, this increase did not appear linked to podocyte division.Conclusions: Podocyte numbers per glomerulus increased from birth until 36 months and then stabilized. These findings could facilitate the development of novel treatments for chronic kidney disease caused by glomerulosclerosis and contribute to pediatric kidney health research.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1613-1624"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142952153","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

Acquired cystic kidney disease in children with kidney failure. 肾衰竭儿童的获得性囊性肾病

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2025-01-04 DOI: 10.1007/s00467-024-06628-7

Justin Ming-Yin Ma, Kin-Fen Kevin Fung, Pak-Chiu Tong, Wai-Ming Lai, Alison Lap-Tak Ma, Eugene Yu-Hin Chan

{"title":"Acquired cystic kidney disease in children with kidney failure.","authors":"Justin Ming-Yin Ma, Kin-Fen Kevin Fung, Pak-Chiu Tong, Wai-Ming Lai, Alison Lap-Tak Ma, Eugene Yu-Hin Chan","doi":"10.1007/s00467-024-06628-7","DOIUrl":"10.1007/s00467-024-06628-7","url":null,"abstract":"Background: This study aimed to evaluate the incidence, contributing factors, and clinical outcomes of acquired cystic kidney disease (ACKD) in children undergoing kidney replacement therapy (KRT).Methods: We conducted a cross-sectional, territory-wide study at the designated pediatric nephrology center in Hong Kong. ACKD was defined as the presence of ≥ 3 cysts in the native kidneys, excluding congenital or hereditary cystic diseases. Between June to December 2023, all paediatric patients receiving KRT in Hong Kong underwent ultrasonography, non-contrast magnetic resonance imaging (MRI), or both. Contrast-enhanced computed tomography was performed for patients with complex cysts.Results: Forty-three children (56% female; median age 14.7 years; IQR, 11.7-18.7) were included in the analysis. ACKD was detected in 18 children (42%). Nine subjects had complex cysts (grade 2, n = 5; grade 2F, n = 2; grade 3, n = 2). Most patients with ACKD (89%) were asymptomatic. One patient (5.5%) developed back pain and gross haematuria 72 months after initiation of KRT. Another patient (5.5%) developed infected cyst with back pain and clinical sepsis 60 months following KRT initiation. A dialysis duration of ≥ 28 months was the only significant factor associated with ACKD development (77.8% vs. 40%; p = 0.028; ORadj 6.09, 95% CI 1.43-25.82, p = 0.014). The diagnostic yield of paired ultrasound and MRI was superior to ultrasound alone.Conclusions: ACKD is prevalent among children and adolescents with kidney failure, with most cases being asymptomatic, however serious complications may arise. Longer duration of dialysis is significantly associated with ACKD development. Therefore, early transplantation and active ACKD surveillance are crucial for children receiving KRT.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1741-1750"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11947055/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142927538","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

UTI in infants: less is more, together is better. 婴儿尿毒症：少则多，多则好。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2024-11-11 DOI: 10.1007/s00467-024-06588-y

Marco Pennesi, Davide Atti, Egidio Barbi

引用次数: 0

Care of children with posterior urethral valves after initial endoscopic incision/ablation: what a nephrologist needs to know. 首次内窥镜切口/消融术后后尿道瓣膜患儿的护理：肾科医师须知。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2024-11-06 DOI: 10.1007/s00467-024-06553-9

Jyoti Sharma, Poonam Guha Vaze, Konstantinos Kamperis, Aniruddh V Deshpande

{"title":"Care of children with posterior urethral valves after initial endoscopic incision/ablation: what a nephrologist needs to know.","authors":"Jyoti Sharma, Poonam Guha Vaze, Konstantinos Kamperis, Aniruddh V Deshpande","doi":"10.1007/s00467-024-06553-9","DOIUrl":"10.1007/s00467-024-06553-9","url":null,"abstract":"Posterior urethral valves (PUV) are the most common cause of congenital urethral obstruction and are unique in the challenges they pose in management. Endoscopic ablation/incision of the valves is usually offered as the primary treatment of choice. Following this, a range of different clinical patterns are observed, each with varying bladder dysfunction and continence issues and associated with different grades of chronic kidney disease. This review outlines a systematic approach that could help pediatric nephrologists, pediatricians, and pediatric urologists, as well as nursing and allied health specialists, assess these children and develop well-informed management plans. The need for surveillance for bladder dysfunction (incidence approximately 55%), identification of red flags for progression to kidney failure (incidence approximately 20%), and multidisciplinary approach to care are presented, with a focus on reducing long-term morbidity in patients and difficulties for the families. Where possible, an alternative in resource-constrained situations is suggested. We also briefly outline the role of pharmacotherapy, assisted bladder emptying/drainage, and other interventions that have a role in the medium- to long-term management of these patients.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1549-1564"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142580949","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

A hypertensive adolescent with aripiprazole-induced hyperaldosteronism. 阿立哌唑致高血压青少年高醛固酮增多症1例。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2024-12-02 DOI: 10.1007/s00467-024-06613-0

Pelin Abdal Yıldırım, Eren Soyaltın, Cemaliye Başaran, Seçil Arslansoyu Çamlar, Belde Kasap Demir

{"title":"A hypertensive adolescent with aripiprazole-induced hyperaldosteronism.","authors":"Pelin Abdal Yıldırım, Eren Soyaltın, Cemaliye Başaran, Seçil Arslansoyu Çamlar, Belde Kasap Demir","doi":"10.1007/s00467-024-06613-0","DOIUrl":"10.1007/s00467-024-06613-0","url":null,"abstract":"Background: Aripiprazole-associated hypertension is a previously described side effect, but its mechanism has not been clearly elucidated. Here we present a 16-year-old male patient who developed aripiprazole-associated hypertension, and we discuss the mechanisms that may cause hypertension.Case diagnosis: Antipsychotic drugs are beneficial for the control of mental health conditions. In this report, we present a case of a patient who developed hypertension after using aripiprazole. A 16-year-old male patient was admitted with headache and high blood pressure. In the examinations conducted for etiology, the aldosterone/plasma renin activity (PRA) was found to be high, but in follow-up, hypertension regressed and aldosterone/PRA were detected normal. The patient was questioned again in terms of medical history. It was learned that the patient was initiated on aripiprazole, and hypertension developed after the drug. The aldosterone/PRA was studied 15 days after he stopped the drug. In this patient, hypertension and hyperaldosteronism were associated with aripiprazole.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1595-1597"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142771181","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

Vanadium exposure and kidney markers in a pediatric population: a cross-sectional study. 钒暴露和儿科人群的肾脏标志物：一项横断面研究。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2024-12-07 DOI: 10.1007/s00467-024-06561-9

Elodia Rojas-Lima, Manolo Ortega-Romero, Octavio Gamaliel Aztatzi-Aguilar, Juan Carlos Rubio-Gutiérrez, Juana Narváez-Morales, Mariela Esparza-García, Pablo Méndez-Hernández, Mara Medeiros, Olivier Christophe Barbier

{"title":"Vanadium exposure and kidney markers in a pediatric population: a cross-sectional study.","authors":"Elodia Rojas-Lima, Manolo Ortega-Romero, Octavio Gamaliel Aztatzi-Aguilar, Juan Carlos Rubio-Gutiérrez, Juana Narváez-Morales, Mariela Esparza-García, Pablo Méndez-Hernández, Mara Medeiros, Olivier Christophe Barbier","doi":"10.1007/s00467-024-06561-9","DOIUrl":"10.1007/s00467-024-06561-9","url":null,"abstract":"Background: Anthropogenic vanadium (V) emissions and exposure in the general population have recently increased. Experimental studies have shown that V is a nephrotoxic agent, but little is known about its effects on human kidney health. This work evaluated the association between urinary V concentrations with early kidney damage biomarkers and function in a pediatric population without any disease diagnosed.Methods: A cross-sectional study was carried out and included 914 healthy subjects and determined urinary V concentrations, glomerular filtration rate (eGFR), albumin-creatinine ratio (ACR), and the presence of kidney injury molecule 1 (KIM-1) and neutrophil gelatinase-associated lipocalin (NGAL) in urine. We evaluated the V effect using linear and logistic regression models adjusted by confounders.Results: Subjects found in the second and third tertiles of V showed an increase in urinary log-NGAL levels (βT2 vs. T1 = 0.39; 95% CI 0.14, 0.64, and βT3 vs. T1 = 1.04; 95% CI 0.75, 1.34) and log-KIM-1(βT2 vs. T1 = 0.25; 95% CI 0.04, 0.45 and βT3 vs. T1 = 0.39; 95% CI 0.15, 0.63); in addition, subjects in the third tertile had a positive and significant association with ACR (ORT3 vs. T1 = 1.96; 95% CI 1.29, 2.97) and increased in eGFR (βT3 vs. T1 = 3.98, 95% CI 0.39, 7.58), compared with subjects in the first tertile.Conclusions: Our study reports the effect of V on kidney markers in a healthy pediatric population. It could be related to tubulointerstitial lesions and function abnormalities.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1689-1700"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11946968/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142792168","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

A nonsense mutation in the Tripartite motif containing 8 (TRIM8) gene, mimicking collagenopathy. 含有8 （TRIM8）基因的三方基序无义突变，模拟胶原病。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2024-12-21 DOI: 10.1007/s00467-024-06636-7

Rehna K Rahman, Harisankar T, Smilu Mohanlal, Divya Pachat, Shalini Kuruvilla, Shephali Sharma

引用次数: 0

Detect early, protect kidney health: World Kidney Day 2025. 早发现，保护肾脏健康：2025年世界肾脏日。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2025-02-17 DOI: 10.1007/s00467-025-06714-4

Florencio McCarthy Waith, Nilzete Liberato Bresolin, Sampson Antwi

引用次数: 0

Evaluation of renal elasticity by shear wave elastography in children with Familial Mediterranean Fever. 用横波弹性成像评价家族性地中海热患儿肾脏弹性。

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2025-01-09 DOI: 10.1007/s00467-024-06637-6

Nadide Melike Sav, Hasan Baki Altinsoy, Betül Türen, Ayşe Gökçe

{"title":"Evaluation of renal elasticity by shear wave elastography in children with Familial Mediterranean Fever.","authors":"Nadide Melike Sav, Hasan Baki Altinsoy, Betül Türen, Ayşe Gökçe","doi":"10.1007/s00467-024-06637-6","DOIUrl":"10.1007/s00467-024-06637-6","url":null,"abstract":"Background: Familial Mediterranean Fever (FMF) is a genetic disorder that can cause kidney damage. Shear wave elastography (SWE), a non-invasive method, was used to evaluate the decrease in renal tissue elasticity as a predictive parameter for amyloidosis. This study aimed to examine the changes in renal elasticity in patients with FMF using the renal SWE measurement method.Methods: The present study included 50 pediatric patients diagnosed with FMF. The median SWE values of both kidneys were compared between the groups. Acute phase reactants were also evaluated.Results: The SWE measurements (for the left kidney p = 0.007, for the right kidney p = 0.06) and proteinuria levels (p < 0.001) of the patient group were found to be higher than those of the control group. No correlation was observed between the disease activity score and the SWE measurements. Erythrocyte sedimentation rate (p < 0.001), C-reactive protein (p < 0.001) and urine protein/creatinine ratio (p < 0.001) were significantly higher in the remission period compared to the control group, whereas estimated glomerular filtration rate was found to be low in the patient group (p < 0.001), which was considered as an indicator that subclinical inflammation continued in the course of the disease.Conclusions: The acute phase reactants were elevated in patients with FMF even in the remission period which indicates that the disease is constantly active and have the potential to cause damage in all organs and tissues. It is thought that this subclinical inflammation may also contribute to increased tissue stiffness, which may serve as a predictor for the development of amyloidosis.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1701-1709"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142952659","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

Single-cell transcriptomics in a child with coenzyme Q10 nephropathy: potential of single-cell RNA sequencing in pediatric kidney disease. 辅酶Q10肾病儿童的单细胞转录组学：单细胞RNA测序在儿童肾脏疾病中的潜力

IF 2.6 3区医学

Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2025-01-14 DOI: 10.1007/s00467-024-06611-2

Peong Gang Park, Sowon Choi, Yo Han Ahn, Seong Heon Kim, Chaeyoon Kim, Hyun Je Kim, Hee Gyung Kang

{"title":"Single-cell transcriptomics in a child with coenzyme Q10 nephropathy: potential of single-cell RNA sequencing in pediatric kidney disease.","authors":"Peong Gang Park, Sowon Choi, Yo Han Ahn, Seong Heon Kim, Chaeyoon Kim, Hyun Je Kim, Hee Gyung Kang","doi":"10.1007/s00467-024-06611-2","DOIUrl":"10.1007/s00467-024-06611-2","url":null,"abstract":"Background: Coenzyme Q10 (CoQ10) nephropathy is a well-known cause of hereditary steroid-resistant nephrotic syndrome, primarily impacting podocytes. This study aimed to elucidate variations in individual cell-level gene expression in CoQ10 nephropathy using single-cell transcriptomics.Methods: We conducted single-cell sequencing of a kidney biopsy specimen from a 5-year-old boy diagnosed with a CoQ10 nephropathy caused by a compound heterozygous COQ2 mutation complicated with immune complex-mediated glomerulonephritis. The analysis focused on the proportion of cell types, differentially expressed genes in each cell type, and changes in gene expression related to mitochondrial function and oxidative phosphorylation (OXPHOS).Results: Our findings revealed a uniform downregulation of mitochondrial gene expression across various cell types in the context of these mutations. Notably, there was a specific decrease in mitochondrial gene expression across all cell types. The study also highlighted an altered immune cell population proportion attributed to the COQ2 gene mutation. Pathway analysis indicated a downregulation in OXPHOS and an upregulation of various synthesis pathways, particularly in podocytes.Conclusions: This study improves our understanding of CoQ10 nephropathy's pathogenesis and highlights the potential applications of single-cell sequencing in pediatric hereditary kidney diseases.","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1653-1662"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11946986/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142979537","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