Pediatric Nephrology最新文献

{"title":"Health-related quality of life, mental health and caregiver burden in children with autosomal recessive polycystic kidney disease.","authors":"Charlotte Gimpel, Susanne Schaefer, Franz Schaefer","doi":"10.1007/s00467-025-06795-1","DOIUrl":"10.1007/s00467-025-06795-1","url":null,"abstract":"<p><strong>Background: </strong>Pediatric chronic kidney disease (CKD) causes significantly impaired health-related quality of life (hrQOL) and caregiver burden, but no studies focus specifically on autosomal recessive polycystic kidney disease (ARPKD).</p><p><strong>Methods: </strong>This prospective case-control study assessed hrQOL (using PedsQL®ESRD) and screened for psychosocial problems (strength and difficulties questionnaire (SDQ)) in 43 children with ARPKD. Fifty-eight caregivers reported on the disease's impact on family (FaBel) and their own QOL (Ulm inventory of parental caregiver QOL (ULQIE)). As controls, we questioned 36 matched healthy children and 57 parents under similar pandemic restrictions and used published historical controls (healthy and with advanced CKD).</p><p><strong>Results: </strong>Patients were aged 9.0 ± 4.8 years with CKD stage G1-4 (45%), on dialysis (14%) or after kidney transplantation (26%). Nine patients had developmental delay secondary to medical complications. PedsQL®ESRD total scores correlated significantly to kidney function, but could not capture liver-specific symptoms. All 4 measures showed significant differences between treatment modalities with best scores in patients during CKD stages G1-4 and worst on dialysis, except SDQ, which was worst after transplantation. The most significant extra-renal risk factor for all 4 scores was developmental delay of the child. SDQ scores were elevated in contemporary vs. historical controls, but even further in ARPKD especially for peer relationship problems.</p><p><strong>Conclusion: </strong>In summary, ARPKD causes significantly impaired hrQOL, psychosocial problems and caregiver burden, which were equal to, if not greater than, that of controls with more advanced kidney failure. Treatment modality and developmental delay were the most important risk factors.</p><p><strong>Trial registration: </strong>Trial registered 06/2020 DRKS S00021059.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"135-150"},"PeriodicalIF":2.6,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12686003/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145081216","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":"Incidence and risk factors associated with acute kidney injury in newborns receiving therapeutic hypothermia.","authors":"Mariateresa Sinelli, Emanuela Zannin, Gaia Alessandra Kullmann, Ilaria Lombardo, Elena Malpezzi, Alessandro Rinaldi, Anna Riva, Maria Luisa Ventura","doi":"10.1007/s00467-025-06926-8","DOIUrl":"10.1007/s00467-025-06926-8","url":null,"abstract":"<p><strong>Background: </strong>To evaluate the incidence and potential predisposing factors for the development of acute kidney injury (AKI) in asphyxiated neonates undergoing hypothermic treatment.</p><p><strong>Methods: </strong>This retrospective study was conducted at the Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy. All neonates above 34 weeks of gestation diagnosed with hypoxic-ischemic encephalopathy (HIE) and treated with hypothermia, weighing more than 1800 g, admitted from January 1, 2013 to December 31, 2022, were included. AKI was defined according to the neonatal KDIGO classification.</p><p><strong>Results: </strong>A total of 75 neonates were enrolled, including 13 (17%) with severe HIE. The incidence of AKI was 35%, with 62% of cases identified as a reduction in diuresis, 27% as changes in creatinine and oliguria, and only 11% as isolated creatinine elevation. The rate of AKI was significantly higher in infants with severe HIE (p < 0.001). The development of AKI added significantly (aOR = 41.2, p = 0.007) to the probability of death, even after adjusting for severe HIE. Infants who developed AKI required more inotropes, had higher rates of hyponatremia (serum sodium < 125 mEq/L), and were less likely to normalize lactate levels within 24 h of birth.</p><p><strong>Conclusions: </strong>In asphyxiated newborns, reduced kidney perfusion can cause kidney impairment in nearly 40% of those undergoing treatment. Enhancing the detection of AKI is crucial for improving patient outcomes. We recommend proactive monitoring of lactate trends, urinary output, and serum sodium levels to enable early interventions that protect kidney function and improve outcomes for these vulnerable infants.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"217-223"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144874429","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":"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":"10.1007/s00467-025-06984-y","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"119-123"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","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}

{"title":"Critical appraisal of \"Thiamin deficiency in children with chronic kidney disease on peritoneal dialysis and its association with dialysis duration and transport peritoneal membrane status\".","authors":"Noaman Khan, Sameed Ahmad, Shahkar Ahmad","doi":"10.1007/s00467-025-06902-2","DOIUrl":"10.1007/s00467-025-06902-2","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"253-254"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144619725","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":"Urothelial protein expression in pediatric patients with ureteropelvic junction obstruction.","authors":"Ashley R Jackson, Sudipti Gupta, Macie Kercsmar, John W Froehlich, Richard S Lee, Brian Becknell, Christina B Ching","doi":"10.1007/s00467-026-07466-5","DOIUrl":"10.1007/s00467-026-07466-5","url":null,"abstract":"<p><strong>Background: </strong>Pediatric ureteropelvic junction obstruction (UPJO) is the leading cause of congenital obstructive uropathy. Experimental models have identified local urothelial remodeling in UPJO and implicated this remodeling as a protective adaptation to preserve kidney structural integrity and function. We investigated the expression of urothelial proteins in human urine and resected tissue specimens from children with UPJO in a pilot study.</p><p><strong>Methods: </strong>Children undergoing pyeloplasty for clinically significant UPJO and healthy age- and sex-matched controls were included. Bladder urine samples were evaluated by ELISA for urothelial proteins (KRT5, KRT14, KRT20, UPK2, UPK3A) and normalized to creatinine (Cr). When available, kidney urine collected at pyeloplasty was compared to bladder urine from the same subject. Urothelial proteins were localized by immunofluorescence microscopy in surgically resected kidney pelvises proximal to the obstruction.</p><p><strong>Results: </strong>Twenty-three UPJO and 14 control urines were evaluated. KRT14/Cr, KRT20/Cr, and UPK2/Cr levels were significantly higher in UPJO urine compared to controls (p < 0.05; Mann-Whitney U test). Levels of urothelial proteins were as high or higher in urine obtained from the obstructed kidney when compared to paired bladder urine. Immunofluorescence microscopy of obstructed urothelium identified basal KRT14+ cells, pan-urothelial UPK+ cells, and superficial KRT20 + cells, with nuclear localization of two transcription factors implicated in obstructive urothelial remodeling, GRHL3 and PPARγ.</p><p><strong>Conclusions: </strong>We provide evidence of increased urothelial proteins in urine from children with UPJO in this pilot study, along with evidence of their local expression by obstructed urothelium.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4581-4587"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13672525/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148422954","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":"Response to letter: Renal maturation lays the physiological basis for precision aminoglycoside dosing in pediatrics.","authors":"Felix J Meigel, Ana Catalina Alvarez-Elías, Rasha Hussein, Doris H Fuertinger","doi":"10.1007/s00467-026-07506-0","DOIUrl":"10.1007/s00467-026-07506-0","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4735"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148707388","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":"Major adverse kidney events at pediatric intensive care unit discharge in a nationwide Japanese pediatric continuous kidney replacement therapy registry.","authors":"Taiki Haga, Takahiro Matsudo, Ryuta Itakura, Yuji Yamagami, Kenji Sonota, Takuya Miyazawa, Shunsuke Noda, Sho Wada, Hiroshi Sakihama, Takuya Hayashi, Naoki Fujiwara, Yuki Ichisaka, Hiroshi Okada, Kohei Tsukahara, Mai Miyaji, Kazufumi Yaginuma, Akito Horikawa, Taku Koizumi, Shinya Takarada, Norio Omori, Keiichiro Toma, Wataru Sakai, Takafumi Obara, Noriaki Kurita","doi":"10.1007/s00467-026-07411-6","DOIUrl":"10.1007/s00467-026-07411-6","url":null,"abstract":"<p><strong>Background: </strong>Major adverse kidney events (MAKE)-a composite of death, new dialysis dependence, and persistent kidney dysfunction-are key outcomes in pediatric patients receiving continuous kidney replacement therapy (CKRT). Due to limited Asian pediatric population data, we determined MAKE incidence at pediatric intensive care unit (PICU) discharge and explored associated factors using the first nationwide Japanese pediatric continuous renal replacement therapy (jpCRRT) registry.</p><p><strong>Methods: </strong>This cohort study included children (aged < 16 years) who underwent CKRT for acute conditions between 2023 and 2025 in 17 Japanese PICUs. The primary outcome, MAKE at PICU discharge, was defined as death, new dialysis dependence, or persistent kidney dysfunction (≥ 25% increase in serum creatinine from baseline or ≥ 25% decrease in estimated glomerular filtration rate). Multivariable logistic regression, with variable selection using the least absolute shrinkage and selection operator and adjustment for clinically relevant covariates, was used to identify independent predictors of MAKE.</p><p><strong>Results: </strong>Of 139 patients, 76 (54.7%) developed MAKE, comprising death, new dialysis dependence, and persistent kidney dysfunction in 25 (18.0%), 18 (13.0%), and 33 (23.7%), respectively. Independent MAKE factors were primary admission diagnoses of kidney disease (adjusted odds ratio (AOR), 60.7; 95% confidence interval (CI), 5.8-639), infection/sepsis (AOR, 37.0; 95% CI, 3.7-373), higher vasoactive-inotropic score (per 1-point increase; AOR, 1.04; 95% CI, 1.004-1.08), and hyperkalemia (per 1 mmol/L increase; AOR, 2.0; 95% CI, 1.1-3.6). High vasoactive support and hyperkalemia were associated with mortality, whereas kidney and infectious admission diagnoses were associated with new dialysis dependence or persistent kidney dysfunction.</p><p><strong>Conclusion: </strong>Among children receiving CKRT, more than half experienced MAKE at PICU discharge. Notably, a primary admission diagnosis of kidney disease was associated with adverse short-term outcomes, along with infectious/septic admission diagnoses, higher vasoactive-inotropic scores, and hyperkalemia. Longer-term follow-up is needed to clarify subsequent kidney recovery and post-discharge outcomes.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4617-4626"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148520786","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":"Comparison of Kirpa Kit™ manual hemodialysis device to standard continuous kidney replacement therapy in vitro.","authors":"Giovanni Ceschia, Jolyn Morgan, Amanda Snyder, James E Rose, Ed Plute, Apaara K Chawla, Stuart L Goldstein, Denise C Hasson","doi":"10.1007/s00467-026-07485-2","DOIUrl":"10.1007/s00467-026-07485-2","url":null,"abstract":"<p><strong>Background: </strong>Access to kidney replacement therapy remains limited in many low- and middle-income countries. The Kirpa Kit™ is a low-cost, electricity-free manual hemodialysis device designed for resource-limited settings. Although it has previously shown feasibility in vitro, in vivo, and in first-in-human use, it has never been directly compared with an automated extracorporeal dialysis platform under matched conditions.</p><p><strong>Methods: </strong>In vitro experiments were performed in triplicate comparing the Kirpa Kit™ and the Prismaflex™. A 5-L bag of diluted packed red blood cells enriched with urea was used for each device across all runs. Each system was operated for 30 min per experiment at a blood flow rate of ~ 100 mL/min and a dialysis fluid flow rate of 4 L/h. Blood urea nitrogen (BUN) was measured at baseline and every 10 min. UF target was 0 mL.</p><p><strong>Results: </strong>Median BUN reduction at 10, 20, and 30 min was 7.1%, 13.6%, and 18.0% for the Kirpa Kit™ and 8.2%, 16.4%, and 23.2% for the Prismaflex™, respectively. Final BUN reduction did not differ between devices (p = 0.38). Median achieved cumulative UF volume per experiment with a goal target of 0 mL was 16 mL (IQR 14-28) for the Kirpa Kit™ and 61 mL (IQR 54-62) for the Prismaflex™. Manual operation of the Kirpa Kit™ was feasible throughout the experiments, with only mild operator fatigue.</p><p><strong>Conclusions: </strong>Under controlled experimental conditions, the Kirpa Kit™ achieved BUN reduction comparable to a standard automated CKRT platform, supporting its potential as a low-cost dialysis option for infants, children, adolescents, and adults in resource-limited settings.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4637-4645"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148536583","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":"Ambulatory arterial stiffness index: a window into early vascular ageing in childhood chronic kidney disease.","authors":"Subhankar Sarkar, Girish Chandra Bhatt, Rajiv Sinha","doi":"10.1007/s00467-026-07594-y","DOIUrl":"10.1007/s00467-026-07594-y","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"4253-4257"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149113231","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":"Blood pressure variability and cardiovascular risk in pediatric hypertension.","authors":"Kleo Evripidou, Athanasia Chainoglou, Stella Stabouli","doi":"10.1007/s00467-025-06874-3","DOIUrl":"10.1007/s00467-025-06874-3","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"11-15"},"PeriodicalIF":2.5,"publicationDate":"2026-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144541799","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}