Pediatric Nephrology最新文献

{"title":"Healthcare access barriers for Hispanic pediatric nephrology patients: a KICK study.","authors":"Debora Matossian, Carlos C Becerril Romero, Priya S Verghese","doi":"10.1007/s00467-025-06881-4","DOIUrl":"10.1007/s00467-025-06881-4","url":null,"abstract":"<p><strong>Background: </strong>Hispanics, the fastest growing minority in the USA, suffer from high chronic kidney disease (CKD) burden. Barriers to accessing medical care increase disparities and quality of care for Hispanic children with CKD.</p><p><strong>Methods: </strong>As a part of the grant-funded \"Kidney Initiative in Community Kids\" (KICK), the voluntary \"Barriers to Care Questionnaire\" was distributed to self-identified Hispanic young adult patients (> 18 years of age) or parents (if patient < 18 years of age), between January 2021 to June 2021, in a single urban pediatric nephrology ambulatory clinic site. Survey was available in English and Spanish. Sociodemographic and health data was obtained from the medical record and Area Deprivation Index (ADI) was collected as a surrogate marker for socioeconomic disadvantage.</p><p><strong>Results: </strong>Of the 179 completed surveys (49% Spanish, 51% English), skills (strategies necessary to navigate the healthcare system) and pragmatic (logistical issues that might delay/prevent utilization) barriers were identified with mean (± standard deviation) scores of 74 (± 26) and 75 (± 21), respectively (100 = no barriers). Spanish-speaking preference families had higher skills barriers (p < 0.001). The mean ADI was 55 ± 20: higher ADI score (more disadvantaged population) correlated with higher total barriers (p = 0.04) and was exacerbated by preferred language (p = 0.003).</p><p><strong>Conclusions: </strong>Hispanic pediatric patients with kidney disease have significant barriers which may impact their interaction with healthcare. Spanish-speaking families are particularly vulnerable. Prospective studies are necessary to advocate for program and policy changes to reduce racial and ethnic disparities in access to pediatric nephrology care.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3477-3483"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12484322/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144619726","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 rare case of BK cystitis in an immunocompetent patient.","authors":"Jordy Salcedo-Giraldo, Yi Shi","doi":"10.1007/s00467-025-06824-z","DOIUrl":"10.1007/s00467-025-06824-z","url":null,"abstract":"<p><p>Hemorrhagic cystitis secondary to BK virus is often seen in immunocompromised patients, but it is a rare cause of hematuria or hemorrhagic cystitis in immunocompetent children. We report a case of an otherwise healthy 4-year-old female who presented to urgent care for urinary frequency, dysuria, and gross hematuria. The initial hematuria workup, including complements, anti-nuclear antibody, anti-streptolysin O titer, and urine culture, was all negative. However, hematuria persisted for 14 days and further work up showed viral PCRs that were positive for BK virus both in blood and urine.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3395-3397"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144143223","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":"Parental perspectives on the effectiveness and safety of rituximab in treating pediatric nephrotic syndrome: a nationwide multi-center survey study.","authors":"Ning Lan, Chengguang Zhao, Li Wang, Hanyun Tang, Weiran Zhou, Hongtao Zhu, Xiaoyan Li, Yuhong Li, Ximing Xu, Gaofu Zhang","doi":"10.1007/s00467-025-06862-7","DOIUrl":"10.1007/s00467-025-06862-7","url":null,"abstract":"<p><strong>Background: </strong>Rituximab is increasingly used for steroid-dependent/frequently relapsing nephrotic syndrome, yet parental perspectives on its real-world impact remain understudied. This study evaluated parental perceptions of rituximab's effectiveness, safety, and socioeconomic effects.</p><p><strong>Methods: </strong>A nationwide cross-sectional survey was conducted across eight pediatric centers in China (Feb 2025). Validated questionnaires were administered to 570 parents of children with nephrotic syndrome, assessing rituximab efficacy, safety, financial burden, caregiver quality of life (SF-36), and treatment barriers. Multivariable regression models analyzed predictors of treatment satisfaction and parental well-being.</p><p><strong>Results: </strong>Among 489 parents analyzed, 232 children (47.4%) received rituximab. Most parents (79.3%) reported good disease control, and 90.5% perceived overall improvement, with 10.3% achieving sustained complete remission without relapse after rituximab treatment. Regarding safety, 86.2% of parents observed no increase in respiratory infections, and 98.3% reported improved child quality of life. Financial burden influenced perceptions; parents from households earning < $3000 annually were less satisfied with rituximab efficacy (OR = 0.22, 95% CI: 0.08-0.60, P = 0.004). Higher SF-36 scores were reported by parents of children who discontinued steroids or had significant quality-of-life improvements. Immune suppression concerns (80.6%) and high costs remained major barriers.</p><p><strong>Conclusions: </strong>This study demonstrates favorable parental perceptions of rituximab regarding efficacy, safety, and caregiver quality of life in patients with pediatric nephrotic syndrome. However, financial challenges and safety concerns limit its broader use. Policy interventions should prioritize financial subsidies, expand insurance coverage, and implement multidisciplinary support programs to address caregivers' psychosocial needs. Enhanced pre-treatment counseling and steroid-sparing protocols may further optimize parental satisfaction and treatment outcomes.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3423-3436"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144485370","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":"Incidence, risk factors, and outcomes of nephrocalcinosis in preterm infants: a prospective cohort study.","authors":"Mithun Krishna, Jitendra Meena, Anmol Bhatia, Arushi Yadav, Jogender Kumar, Kanya Mukhopadhyay, Praveen Kumar","doi":"10.1007/s00467-025-06888-x","DOIUrl":"10.1007/s00467-025-06888-x","url":null,"abstract":"<p><strong>Background: </strong>Kidneys in preterm infants are exposed to various exogenous factors that may affect their maturation and development. Hence, they are at a higher risk of developing nephrocalcinosis (NC). We aimed to evaluate the incidence, risk factors, and short-term outcomes of NC in preterm neonates admitted to a tertiary-care neonatal unit.</p><p><strong>Methods: </strong>This prospective observational study included preterm infants born at < 33 weeks gestation. Infants with congenital anomalies of the kidney and urinary tract (CAKUT) or major malformations were excluded. Ultrasound scans were performed at 3-6 weeks of postnatal age to screen for NC using a modified grading system. Clinical data, including demographics, respiratory support, medication exposure, nutrition, and biochemical markers, were collected. Infants with NC were followed up at 3 and 6 months of corrected age to assess the resolution of NC and kidney function.</p><p><strong>Results: </strong>Among the 152 enrolled neonates, 23 (15.1%) had NC. Respiratory distress syndrome (RDS) (adjusted odds ratio [aOR] 3.6; 95% CI: 1.1-12.3; p = 0.04) and total parenteral nutrition (TPN) (aOR-4.5; 95% CI: 1.2-16.8; p = 0.03) were independently associated with NC. At 3 months, 17 of the 23 infants were re-evaluated, and NC resolved in 13 (76.5%). By 6-8 months, all but one infant showed resolution. All the infants had normal kidney function at the last follow-up.</p><p><strong>Conclusion: </strong>Nephrocalcinosis occurred in 15% of preterm neonates and was significantly associated with RDS and TPN use. Most cases resolved spontaneously without adverse outcomes. These findings support targeted NC screening in high-risk neonates.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3469-3476"},"PeriodicalIF":2.6,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144637710","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":"Outcomes and prognostic factors in childhood-onset steroid-resistant nephrotic syndrome.","authors":"Emil Zerkowitz, Verena Klämbt","doi":"10.1007/s00467-025-06977-x","DOIUrl":"https://doi.org/10.1007/s00467-025-06977-x","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145213341","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":"Clinical differences between female monozygotic twins with X-linked Alport syndrome with somatic mosaicism.","authors":"Naoaki Mikami, Hideaki Kitakado, Naoki Kimura, Nana Sakakibara, Kandai Nozu, Riku Hamada","doi":"10.1007/s00467-025-06772-8","DOIUrl":"10.1007/s00467-025-06772-8","url":null,"abstract":"<p><p>Although female patients with X-linked Alport syndrome (XLAS) have a generally milder prognosis than male counterparts, some female patients show poor prognosis. The clinically observed variation in the severity of female XLAS is thought to be due mainly to X chromosome inactivation (XCI) or modifier gene variants. We describe herein a 4-year-old female patient with persistent hematuria and proteinuria whose monozygotic, twin sister had no apparent abnormality on urinalysis. A kidney biopsy of the patient found the typical pattern of female XLAS. Genetic testing revealed a de novo pathogenic variant of COL4 A5 with somatic mosaicism. However, additional tests revealed that the asymptomatic twin sister had the same COL4 A5 variant with somatic mosaicism, and that the twins had the same ratio of somatic mosaicism and XCI. Moreover, targeted exome sequencing found no other modifier gene variants, suggesting that the severity of female XLAS depends on distinct but unknown factors in addition to the XCI and modifier gene variants. The findings of the present report emphasize the need to identify the hidden factors affecting the severity of Alport syndrome.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3077-3080"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144038952","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":"The gut microbiome and metabolome in children with a first febrile urinary tract infection: a pilot study.","authors":"Barbora Piteková, Ivan Hric, Jakub Zieg, Eva Baranovičová, Patrik Konopásek, Jakub Gécz, Paul J Planet, Viktor Bielik","doi":"10.1007/s00467-025-06782-6","DOIUrl":"10.1007/s00467-025-06782-6","url":null,"abstract":"<p><strong>Background: </strong>Urinary tract infection (UTI) is a common bacterial infection in the pediatric population. Febrile urinary tract infection (fUTI) can lead to severe complications such as urosepsis as well as kidney scarring, chronic kidney disease, and systemic hypertension. Recent research supports the hypothesis that dysbiosis of the microbiome may play a role in the pathogenesis and development of fUTI in infants. Our main aim was to compare the shift in gut microbiota composition between children with the first fUTI and controls.</p><p><strong>Methods: </strong>We conducted an observational study with 17 children with the first fUTI compared to 18 healthy controls. We performed analysis of the gastrointestinal microbiome and measurements of metabolites in stool and urine.</p><p><strong>Results: </strong>In the gut microbiome, we found significant differences with lower α-diversity the Shannon index) and significantly lower relative abundance of probiogenic bacteria (short-chain fatty acids (SCFA)) in children with the first episode of fUTI before the start of antibiotic therapy. Furthermore, our findings confirm that the length of breastfeeding has significant influence on gut microbiota composition, reducing pathogenic bacteria and enhancing beneficial taxa. Shannon diversity, duration of breastfeeding, and specific taxa, particularly Faecalibacterium and Escherichia, emerged as strong predictors linked to the development of fUTI.</p><p><strong>Conclusions: </strong>This study demonstrates that gut microbiome changes are associated with the onset of fUTI in children. Machine learning models identified Shannon index, specific bacterial taxa, and breastfeeding as strong predictors of fUTI. The study highlighted the potential role of the gut microbiome in preventing fUTI.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3145-3154"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12402013/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144079194","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":"Moving toward a better understanding of renal lymphatics: challenges and opportunities.","authors":"Jianyong Zhong, Jing Liu, Ashley L Mutchler, Haichun Yang, Annet Kirabo, Elaine L Shelton, Valentina Kon","doi":"10.1007/s00467-025-06692-7","DOIUrl":"10.1007/s00467-025-06692-7","url":null,"abstract":"<p><p>The development of lymphatic-specific markers has enabled detailed visualization of the lymphatic vascular network that has greatly enhanced our ability to explore this often-overlooked system. Lymphatics remove fluid, solutes, macromolecules, and cells from the interstitium and return them to circulation. The kidneys have lymphatics. As in other organs, the kidney lymphatic vessels are highly sensitive to changes in the local microenvironment. The sensitivity to its milieu may be especially relevant in kidneys because they are central in regulating fluid homeostasis and clearance of metabolites delivered into and eliminated from the renal interstitial compartment. Numerous physiologic conditions and diseases modify the renal interstitial volume, pressure, and composition that can, in turn, influence the growth and function of the renal lymphatics. The impact of the renal microenvironment is further heightened by the fact that kidneys are encapsulated. This review considers the development, structure, and function of the renal lymphatic vessels and explores how factors within the kidney interstitial compartment modify their structure and functionality. Moreover, although currently there are no pharmaceutical agents that specifically target the lymphatic network, we highlight several medications currently used in children with kidney disease and hypertension that have significant but underappreciated effects on lymphatics.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3023-3036"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12402047/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080721","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":"Non-infectious complications of peritoneal dialysis in children.","authors":"Mattia Parolin, Giovanni Ceschia, Nicola Bertazza Partigiani, Edoardo La Porta, Enrico Verrina, Enrico Vidal","doi":"10.1007/s00467-025-06713-5","DOIUrl":"10.1007/s00467-025-06713-5","url":null,"abstract":"<p><p>Peritoneal dialysis (PD) remains a cornerstone treatment for children with stage 5 chronic kidney disease, offering significant benefits in terms of quality of life and patient autonomy compared to hemodialysis. Recent advances in care protocols, particularly in infection prevention, have led to a substantial reduction in peritonitis and other infectious complications in pediatric patients on PD. Despite these improvements, non-infectious complications continue to pose significant challenges to the long-term efficacy of PD. Mechanical issues, such as catheter malposition and dysfunction, are common and can lead to discomfort and diminished dialysis efficiency. A more serious concern is peritoneal membrane failure, which results from prolonged PD, particularly with glucose-based solutions, and recurrent peritonitis, leading to structural changes in the membrane. Encapsulating peritoneal sclerosis (EPS), although rare, is a severe and often devastating complication that significantly impacts patient morbidity and mortality. Despite its low incidence, EPS underscores the importance of careful monitoring and management of long-term PD patients. Additional complications, including metabolic disturbances, pancreatitis, and hemoperitoneum, further complicate care. Looking ahead, improving catheter management, preserving peritoneal membrane function, and exploring new dialysis solutions are essential to reducing these complications and optimizing outcomes for pediatric patients on PD.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3055-3066"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12402023/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542843","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":"Chronic kidney disease in Syrian children: a single center experience.","authors":"Hala Wannous, Asshaar Albalkhi","doi":"10.1007/s00467-025-06817-y","DOIUrl":"10.1007/s00467-025-06817-y","url":null,"abstract":"<p><strong>Background: </strong>Chronic kidney disease (CKD) is a condition marked by permanent kidney damage, which can eventually advance to kidney failure. Our study aimed to identify CKD causes and investigate the risk factors for CKD progression in Syrian children.</p><p><strong>Methods: </strong>A single-center observational cohort study was conducted at Children's University Hospital in Damascus, included all patients admitted to the department of pediatric nephrology (inpatient unit) from February 2022 to February 2023, with CKD stages 2-5.</p><p><strong>Results: </strong>Our study included 100 patients, with a slight female predominance, and mean age of 4.73 years ± 3.7, ranging from 3 months to 14 years. CKD causes were congenital anomalies of the kidney and urinary tract (CAKUT) in 79%, hereditary nephropathies in 12%, and glomerular diseases in 9%. After one year of follow-up, 38% of them were stable on conservative management and 62% had kidney failure. Gender, age, and family history of CKD were not statistically significant factors in CKD progression. The progression rate to kidney failure was highest in glomerular diseases, with 100% for glomerulonephritis and 80% for steroid-resistant nephrotic syndrome, and the progression rate in kidney dysplasia patients was 78.1%. Proteinuria, metabolic acidosis, hyperparathyroidism, hypertension, anemia, and hyperphosphatemia were significantly associated with CKD progression.</p><p><strong>Conclusions: </strong>In Syrian children, CAKUT are the leading cause of CKD. CKD progression is most rapid in glomerular diseases. Modifiable risk factors, especially hypertension and proteinuria, play a significant role in CKD progression, which highlights the importance of early detection and appropriate management of these factors.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3245-3251"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144143236","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}