Pediatric Nephrology最新文献

{"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":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","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}

{"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":"<p><strong>Background: </strong>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.</p><p><strong>Case diagnosis: </strong>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.</p>","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}

{"title":"A nonsense mutation in the Tripartite motif containing 8 (TRIM8) gene, mimicking collagenopathy.","authors":"Rehna K Rahman, Harisankar T, Smilu Mohanlal, Divya Pachat, Shalini Kuruvilla, Shephali Sharma","doi":"10.1007/s00467-024-06636-7","DOIUrl":"10.1007/s00467-024-06636-7","url":null,"abstract":"<p><p>Tripartite motif-containing 8 (TRIM8) gene mutations are associated with autosomal dominantly inherited neurorenal syndrome. The kidney manifestations range from nephrotic range proteinuria to nephrotic syndrome and kidney failure. The histopathology has been focal segmental glomerulosclerosis (FSGS) in all reported cases. We now report a nonsense mutation in TRIM8 in a 1-year-old boy, mimicking collagenopathy in kidney biopsy.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1579-1581"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142872732","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}

{"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":"<p><p>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.</p>","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}

{"title":"UTI in infants: less is more, together is better.","authors":"Marco Pennesi, Davide Atti, Egidio Barbi","doi":"10.1007/s00467-024-06588-y","DOIUrl":"10.1007/s00467-024-06588-y","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1819"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142625665","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}

{"title":"Detect early, protect kidney health: World Kidney Day 2025.","authors":"Florencio McCarthy Waith, Nilzete Liberato Bresolin, Sampson Antwi","doi":"10.1007/s00467-025-06714-4","DOIUrl":"10.1007/s00467-025-06714-4","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1511-1514"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143441691","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}

{"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":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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).</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>This study improves our understanding of CoQ10 nephropathy's pathogenesis and highlights the potential applications of single-cell sequencing in pediatric hereditary kidney diseases.</p>","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}

{"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":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","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}

{"title":"Resolution of chyluria due to congenital malformation with a minimally invasive procedure.","authors":"Paola Zayas-Borges, Reema Panjwani, Xiaoyan Wu, Jay Shah, Larry A Greenbaum","doi":"10.1007/s00467-024-06489-0","DOIUrl":"10.1007/s00467-024-06489-0","url":null,"abstract":"<p><p>Milky white urine is most commonly due to chyluria secondary to filariasis, though other causes of milky white urine and other etiologies of chyluria must be considered. Evaluation of a 3-year-old girl with milky white urine demonstrated chyluria, but testing for filariasis was negative despite a history of travel to an endemic region. Magnetic resonance lymphangiography demonstrated a congenital lymphatic malformation, which was repaired following this minimally invasive imaging technique.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1591-1593"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142801873","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}

{"title":"How dialysis frequency and duration impact uremic toxin and fluid removal: a pediatric perspective.","authors":"Pauline Van Wesemael, Sunny Eloot, Ann Raes, Rukshana Shroff, Evelien Snauwaert","doi":"10.1007/s00467-024-06598-w","DOIUrl":"10.1007/s00467-024-06598-w","url":null,"abstract":"<p><p>Three-weekly 4-h hemodialysis/hemodiafiltration (HD/HDF) per week has become the \"standard HD/HDF\" regimen in children across the globe, although increasingly criticized, since crucial determinants such as residual kidney function and patient preferences are not considered. As a consequence, several children fail to achieve adequate dialysis while on a \"standard HD/HDF.\" In these circumstances, an extended dialysis prescription such as short daily (2-3 h/session, 5-7 days a week) or nocturnal HD/HDF (6-9 h/session, 3-5 days a week), either at home or in a dialysis center, may be considered. The purpose of this educational review is to summarize the impact of dialysis duration and frequency on uremic toxin and fluid removal. Moreover, we aim to summarize the existing literature on HD/HDF strategies with extended dialysis duration and/or increased frequency (> 12 h dialysis time per week) in pediatrics. Dialysis duration and frequency plays a crucial role in uremic toxin removal, in particular for uremic toxins with retarded transport in patients, such as phosphate, β₂-microglobulin (β₂m), and protein-bound uremic toxins. Also, increasing dialysis duration and/or frequency decreases the gap between plasma refilling and ultrafiltration volume), thereby decreasing the need for a high ultrafiltration rate. Observational studies in children demonstrate a beneficial effect of extended dialysis regimens (i.e., more frequent or longer duration) on blood pressure control, left ventricular hypertrophy, growth, and quality of life. PTH levels tend to decrease in the majority of studies, while hypocalcemia or suppressed PTH levels were also reported. Dietary restrictions were decreased or stopped, along with tapering of phosphate binders and potassium chelators. Extended HD/HDF regimens are beneficial in a particular group of children. Pediatric-specific international guidelines are needed to support pediatric nephrologists in determining for which children extended HD regimens are beneficial, along with increasing efforts to decrease the financial, organizational, and psychosocial barriers that are present in extended HD/HDF.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1565-1578"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142786392","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}