Pediatric Nephrology最新文献

{"title":"IPNA clinical practice recommendations on care of pediatric patients with pre-existing kidney disease during seasonal outbreak of COVID-19.","authors":"Khalid A Alhasan, Rupesh Raina, Olivia Boyer, Jean Koh, Melvin Bonilla-Felix, Sidharth K Sethi, Yasser S Amer, Paula Coccia, Mohamad-Hani Temsah, Judith Exantus, Samina A Khan, Xuhui Zhong, Vera Koch, Ali Duzova, Anil Vasudevan, Mignon McCulloch, Upton Allen, Guido Filler, Giovanni Montini","doi":"10.1007/s00467-024-06565-5","DOIUrl":"10.1007/s00467-024-06565-5","url":null,"abstract":"<p><p>The coronavirus disease 2019 (COVID-19) pandemic, instigated by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has profoundly impacted healthcare infrastructures around the globe. While children are usually asymptomatic or have mild symptoms, children with pre-existing kidney conditions require specialized attention. This pivotal report, championed by the International Pediatric Nephrology Association (IPNA), delivers precise and actionable recommendations tailored for pediatric patients with kidney ailments in this pandemic landscape. Central to our findings are rigorous infection control protocols. These are particularly stringent in high-risk zones, emphasizing telehealth's indispensable role, the significance of curtailing in-person consultations, and the imperative of following rigorous guidelines in regions with heightened COVID-19 prevalence. Additionally, the report delves into vaccination approaches for children with kidney issues, highlighting that the choice of vaccine is often governed by regional accessibility and policy frameworks, rather than a universal preference. A notable observation is the potential correlation between COVID-19 vaccines and specific kidney disorders. However, establishing a direct causal link remains elusive. In summary, our research accentuates the critical need for specialized pediatric kidney care during global health crises and reaffirms the continuous research imperative, especially regarding vaccination ramifications.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1795-1815"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11946955/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142903462","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":"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":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","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}

{"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":"<p><strong>Background: </strong>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).</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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; OR_adj 6.09, 95% CI 1.43-25.82, p = 0.014). The diagnostic yield of paired ultrasound and MRI was superior to ultrasound alone.</p><p><strong>Conclusions: </strong>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.</p>","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}

{"title":"Gut-derived metabolites as treatment targets in chronic kidney disease-an avenue toward personalized medicine.","authors":"Johannes Holle, Hendrik Bartolomaeus","doi":"10.1007/s00467-024-06609-w","DOIUrl":"10.1007/s00467-024-06609-w","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1505-1510"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11947041/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143009356","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":"Investigation of a targeted panel of gut microbiome-derived toxins in children with chronic kidney disease.","authors":"Mina Ebrahimi, Stephen R Hooper, Mark M Mitsnefes, Ramachandran S Vasan, Paul L Kimmel, Bradley A Warady, Susan L Furth, Erum A Hartung, Michelle R Denburg, Arthur M Lee","doi":"10.1007/s00467-024-06580-6","DOIUrl":"10.1007/s00467-024-06580-6","url":null,"abstract":"<p><strong>Background: </strong>The gut-kidney axis is implicated in chronic kidney disease (CKD) morbidity. We describe how a panel of gut microbiome-derived toxins relates to kidney function and neurocognitive outcomes in children with CKD, consisting of indoleacetate, 3-indoxylsulfate, p-cresol glucuronide, p-cresol sulfate, and phenylacetylglutamine.</p><p><strong>Methods: </strong>The Chronic Kidney Disease in Children (CKiD) cohort is a North American multicenter prospective cohort that enrolled children aged 6 months to 16 years with estimated glomerular filtration rate (eGFR) 30-89 ml/min/1.73 m². Data from the 2-year study visit were used for this analysis. Toxin quantification (Metabolon Inc., Durham, NC) was performed with ultra-high performance liquid chromatography/tandem mass spectrometry. Executive function and echocardiograms were assessed. Regression analysis examined the association of toxin levels with eGFR, CKD etiology, and neurocognitive and cardiac assessments (adjusted for age, sex, and urine protein:creatinine [UPCR]).</p><p><strong>Results: </strong>There were 150 CKiD participants included in this study. All toxins levels were significantly inversely correlated with eGFR (Spearman's rho - 0.45 to - 0.69). Children with non-glomerular CKD had significantly higher levels of 3-indoxylsulfate, phenylacetylglutamine, and p-cresol glucuronide. The toxin levels did not associate with neurocognitive outcomes. P-cresol glucuronide and phenylacetylglutamine negatively associated with left ventricular mass index z score, but did not associate with left ventricular hypertrophy.</p><p><strong>Conclusions: </strong>Children with CKD have high levels of circulating gut microbiome-derived toxins. The levels of these toxins are strongly correlated with eGFR. There appear to be differences in toxin level based on glomerular versus non-glomerular etiology, even when accounting for the differences in eGFR between these two subgroups. In this sample, we did not detect any associations between these toxin levels and neurocognitive or cardiac outcomes.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1759-1770"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143009358","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":"In vivo assessment of pediatric kidney function using multi-parametric and multi-nuclear functional magnetic resonance imaging: challenges, perspectives, and clinical applications.","authors":"Aurélie De Mul, Maxime Schleef, Guido Filler, Christopher McIntyre, Sandrine Lemoine","doi":"10.1007/s00467-024-06560-w","DOIUrl":"10.1007/s00467-024-06560-w","url":null,"abstract":"<p><p>The conventional methods for assessing kidney function, such as glomerular filtration rate and microalbuminuria, provide only partial insight into kidney function. Multi-parametric and multi-nuclear functional resonance magnetic imaging (MRI) techniques are innovative approaches to unraveling kidney physiology. Multi-parametric MRI includes various sequences to evaluate kidney perfusion, tissue oxygenation, and microstructure characterization, including fibrosis-a key pathological event in acute and chronic kidney disease and in transplant patients-without the need for invasive kidney biopsy. Multi-nuclear MRI detects nuclei other than protons. ²³Na MRI enables visualization of the corticomedullary gradient and assessment of tissue sodium storage, which can be particularly relevant for personalized medicine in salt-wasting tubular disorders. Meanwhile, ³¹P-MRS measures intracellular phosphate and ATP variations, providing insights into oxidative metabolism in the muscle during exercise and recovery. This technique can be useful for detecting subclinical ischemia in chronic kidney disease and in tubulopathies with kidney phosphate wasting. These techniques are non-invasive and do not involve radiation exposure, making them especially suitable for longitudinal and serial assessments. They enable in vivo evaluation of kidney function on a whole-organ basis within a short acquisition time and with the ability to distinguish between medullary and cortical compartments. Therefore, they offer considerable potential for pediatric patients. In this review, we provide a brief overview of the main imaging techniques, summarize available literature data on both adult and pediatric populations, and examine the perspectives and challenges associated with multi-parametric and multi-nuclear MRI.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1539-1548"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11946951/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142648540","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":"Chyloperitoneum in a toddler on peritoneal dialysis.","authors":"Veronica Lavelle Bell, Alyssa A Schlotman, Daniel J Benedetti, Tracy E Hunley","doi":"10.1007/s00467-024-06597-x","DOIUrl":"10.1007/s00467-024-06597-x","url":null,"abstract":"<p><p>Chyloperitoneum is an uncommon diagnosis in peritoneal dialysis (PD) patients. While admitted for emesis and feeding intolerance, a 16-month-old male on PD developed milky-colored dialysate with increased triglycerides, indicating chyloperitoneum. In adult PD patients, chyloperitoneum can indicate potentially life-threatening pathologies including malignancies and liver or heart disease. By contrast, pediatric patients on PD with chyloperitoneum had recently undergone PD catheter or gastrostomy tube placement with presumed disruption of abdominal lymphatics. Slowing lymph flow through dietary manipulation and rarely, temporary withholding of PD, resolved chyloperitoneum. We report a toddler on PD with chyloperitoneum in whom abdominal investigation showed multifocal hepatoblastoma. Chemotherapy and a medium chain triglycerides (MCTs)-based diet led to prompt resolution of chyloperitoneum. Intrabdominal malignancy in this patient illustrates the importance of a prompt, thorough evaluation of chyloperitoneum to allow definitive therapy if required.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"1599-1602"},"PeriodicalIF":2.6,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11947007/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142771182","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 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":"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":"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}