Pediatric Nephrology最新文献

{"title":"Now what: navigating care of maternal/fetal dyads with bilateral renal agenesis after RAFT. A physician and parent point of view.","authors":"Raj Munshi, Kirsten Turner, Alfredo Berrettini, Elliott Mark Weiss","doi":"10.1007/s00467-024-06460-z","DOIUrl":"10.1007/s00467-024-06460-z","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"291-295"},"PeriodicalIF":2.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141752334","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":"Tubulointerstitial nephritis in children and adolescents.","authors":"Evgenia Gurevich, Daniel Landau","doi":"10.1007/s00467-024-06526-y","DOIUrl":"10.1007/s00467-024-06526-y","url":null,"abstract":"<p><p>The tubulointerstitial compartment comprises most of the kidney parenchyma. Inflammation in this compartment (tubulointerstitial nephritis-TIN) can be acute and resolves if the offending factor is withdrawn or may enter a chronic process leading to irreversible kidney damage. Etiologic factors differ, including different exposures, infections, and autoimmune and genetic tendency, and the initial damage can be acute, recurrent, or permanent, determining whether the acute inflammatory process will lead to complete healing or to a chronic course of inflammation leading to fibrosis. Clinical and laboratory findings of TIN are often nonspecific, which may lead to delayed diagnosis and a poorer clinical outcome. We provide a general review of TIN, with special mention of the molecular pathophysiological mechanisms of the associated kidney damage.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"319-328"},"PeriodicalIF":2.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142351372","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":"Outcome of rituximab treatment in children with non-dialysis-dependent anti-GBM disease.","authors":"Richard Klaus, Nele Kanzelmeyer, Dieter Haffner, Bärbel Lange-Sperandio","doi":"10.1007/s00467-024-06512-4","DOIUrl":"10.1007/s00467-024-06512-4","url":null,"abstract":"<p><strong>Background: </strong>Anti-GBM disease is a rare vasculitis mediated by pathogenic antibodies against collagen IV. Anti-GBM disease presents with rapid progressive glomerulonephritis and leads to kidney failure if untreated. KDIGO recommends plasma exchanges (PEX) for antibody elimination and steroids plus cyclophosphamide (CTX) to suppress antibody production. CTX is associated with severe side effects including gonadal toxicity. Rituximab (RTX) and mycophenolate mofetil (MMF) might be a less toxic but equally efficient alternative to CTX. Studies in pediatric anti-GBM disease patients receiving RTX and MMF instead of CTX are lacking.</p><p><strong>Methods: </strong>A retrospective survey in 8 tertiary German centers was performed. The clinical data of patients diagnosed between 2014 and 2022 were collected and analyzed.</p><p><strong>Results: </strong>Five adolescent patients treated with PEX and RTX and/or MMF due to anti-GBM disease were analyzed. All patients had anti-GBM antibodies, hematuria, glomerular proteinuria, and pulmonary hemorrhage. eGFR was 124 ml/min/1.73 m² (range 47-162), and all patients were non-dialysis-dependent but with relevant histological kidney affection (mean crescents on kidney biopsy 77%). Antibody clearance was achieved after 13 PEX cycles (range 6-31). Four out of 5 patients received methylprednisolone pulses. All patients received oral prednisolone and MMF, and four patients received a median of 4 RTX doses (range 2-4). After a mean follow-up of 27 months, 4/5 patients had conserved or improved kidney function, while one patient (20%) developed kidney failure.</p><p><strong>Conclusions: </strong>In this small series of pediatric non-dialysis-dependent anti-GBM disease patients, first-line treatment with RTX and MMF showed a favorable kidney outcome in 4/5 cases and had an acceptable side effect profile.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"423-430"},"PeriodicalIF":2.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142351468","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":"Seroprevalence of SARS-CoV-2 antibodies in children with nephrotic syndrome and chronic kidney disease: a cross-sectional study from India.","authors":"Richa Singh, Mukta Mantan, Akanksha Mahajan, Vernika Tyagi, Binita Goswami","doi":"10.1007/s00467-024-06534-y","DOIUrl":"10.1007/s00467-024-06534-y","url":null,"abstract":"<p><strong>Background: </strong>There is a paucity of literature on the seroprevalence of SARS-CoV-2 antibodies among pediatric patients with underlying kidney disorders; few serosurveys among healthy children have shown seropositivity of 20-65% after different waves of infections.</p><p><strong>Methods: </strong>The study had a cross-sectional design and was conducted between January 2023 and July 2023; 163 children and adolescents (1-18 years) with nephrotic syndrome and chronic kidney disease (CKD) were screened for Anti-Spike SARS-COV-2 IgG antibodies as detected by a quantitative chemiluminescence immunoassay. Children with nephrotic syndrome, both steroid sensitive (SSNS) and steroid resistant (SRNS) were enrolled during disease remission. Correlation of SARS-CoV-2 seropositivity status was done with age, gender, disease type, treatment duration, immunosuppressants, previous SARS-CoV-2 infection, and immunization status.</p><p><strong>Results: </strong>Of 163 children (63.8% boys) with median age of 9 years; 101 (62%) had underlying nephrotic syndrome (61 SSNS and 40 SRNS), and 62 (38%) children had CKD. Seroprotective titers for SARS-COV2 antibodies were present in 100 (61.3%) children. The median titers for all patients were 37.1 BAU/mL; for nephrotic syndrome they were 27.1 BAU/mL and for CKD they were 76.7 BAU/mL (p = 0.0033). A total of 43 (26.4%) children had high positive antibody levels (> 200 BAU/ml). Among those with nephrotic syndrome 60.7% with SSNS and 43.5% SRNS had seropositive titers. Only 4 (2.5%) children had a history of previous COVID infection and 6 (3.7%) were vaccinated.</p><p><strong>Conclusions: </strong>In a largely unvaccinated population of children with nephrotic syndrome and CKD, 61.3% were seropositive for SARS-CoV-2 IgG antibody indicating a past asymptomatic infection; titers were significantly higher in CKD compared to nephrotic syndrome.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"441-447"},"PeriodicalIF":2.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142351469","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":"Steroid pulse therapy in idiopathic nephrotic syndrome in the era of modern immunosuppressive treatment-still up to date?","authors":"Cyrielle Parmentier, Solene Victor, Claire Dossier, Jean Daniel Delbet, Julien Hogan, Antoine Mouche, Olivia Boyer, Tim Ulinski","doi":"10.1007/s00467-024-06535-x","DOIUrl":"10.1007/s00467-024-06535-x","url":null,"abstract":"<p><strong>Background: </strong>Intravenous steroid pulses (SP) are successfully used for the treatment of patients with idiopathic nephrotic syndrome (INS) resistant to oral prednisone.</p><p><strong>Methods: </strong>We performed a retrospective analysis of all patients in the three pediatric nephrology centers of the Paris region from 2002 to 2022 who were resistant to a 30-day course of oral prednisone and who received SP for their first INS flare and analyzed their disease course over 4 years.</p><p><strong>Results: </strong>Forty-seven patients (17 girls), median age 3.4 years, were analyzed. Of them, 68% reached remission within 7 days of SP. No significant short-term side effects were noted. Half of the patients started immunosuppressive treatment immediately after their first remission and 62% of them relapsed at least once, whereas all the patients who did not receive immunosuppressive treatment since their first remission relapsed. Among the SP-sensitive patients, 75% needed calcineurin inhibitor (CNI) or B-cell depletion during their disease course to achieve stable remission. Forty-two percent of the whole cohort received B-cell-depleting agents. Among the 15 SP-resistant patients, all received CNI. Twelve/fifteen patients reached remission. After 4 years, 68% among the SP-sensitive patients and 87% of SP-resistant patients still had an active disease.</p><p><strong>Conclusions: </strong>SP are helpful to obtain rapid remission in pediatric INS patients resistant to oral steroids. However, as most SP-sensitive patients need immunosuppressive drugs, mainly CNI and B-cell-depleting agents it could be interesting to discuss the possibility to start CNI directly after the 30-day course of prednisone instead of SP.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"417-422"},"PeriodicalIF":2.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142308285","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":"Tracking of blood pressure levels from childhood.","authors":"Carissa M Baker-Smith","doi":"10.1007/s00467-024-06485-4","DOIUrl":"10.1007/s00467-024-06485-4","url":null,"abstract":"<p><p>The overall goal of this review is to summarize what is currently known regarding the tracking of blood pressure levels from early childhood into later adulthood and to describe the factors contributing to increased hypertension prevalence across the lifespan. This review describes 4 theoretical constructs of blood pressure trajectory across the lifespan: forward development, forward tracking, forward reversal, and maintenance of normal blood pressure levels.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"367-376"},"PeriodicalIF":2.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142081218","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 and urodynamic findings in children and adolescents with neurogenic bladder undergoing augmentation cystoplasty: a systematic review.","authors":"Otávio Augusto Fonseca Reis, Hilton Naoto Ito, Juliana de Oliveira Otávio, Diniz José de Oliveira Filho, Eleonora Moreira Lima, José de Bessa, Paula Larissa Lebron da Silva, Mônica Maria de Almeida Vasconcelos, Flávia Cristina de Carvalho Mrad","doi":"10.1007/s00467-024-06499-y","DOIUrl":"10.1007/s00467-024-06499-y","url":null,"abstract":"<p><strong>Background: </strong>Augmentation cystoplasty (AC) is a procedure to improve the clinical and urodynamic parameters of neurogenic bladder (NB) in children and adolescents refractory to other treatments. We performed a systematic review to investigate these parameters in children and adolescents with NB undergoing AC.</p><p><strong>Methods: </strong>We followed PRISMA guidelines and searched electronic databases until March 2024 for studies involving patients aged three to 19 years diagnosed with NB undergoing AC. We assessed clinical and urodynamic parameters before and after surgery, focusing on improvements in urinary incontinence, vesicoureteral reflux (VUR), bladder capacity, compliance, and end filling detrusor pressure (EFP).</p><p><strong>Results: </strong>A total of 212 NB patients underwent AC and were evaluated for urinary incontinence before and after surgery. Two studies showed a 76.5% to 78.9% improvement in incontinence without bladder outlet procedures (BOP). Another study found no significant difference in incontinence improvement rates between AC with and without BOP. The VUR resolution rate assessed in three studies ranged from 12.5 to 64%. Three studies showed a variation in bladder capacity from 52.8 to 70% of the expected bladder capacity pre-AC to 95.9 to 119%, post-AC. A fourth study showed a variation in bladder capacity from 87 ml pre-AC to 370 ml post-AC. Two studies showed a variation from 3.2 to 4.6 ml/cm H₂O pre-AC to 13.7 to 41.3 ml/cm H₂O post-AC in bladder compliance. The EFP in three studies varied from 37.2 to 47.6 cm H₂O pre-AC to 11 to 17.4 cm H₂O post-AC.</p><p><strong>Conclusion: </strong>After AC, urinary incontinence, bladder capacity, EFP, and bladder compliance improved in children and adolescents with NB.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"355-365"},"PeriodicalIF":2.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142154683","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":"Bilateral renal agenesis: fetal intervention and outcomes.","authors":"Katherine Jones, Amaris M Keiser, Jena L Miller, Meredith A Atkinson","doi":"10.1007/s00467-024-06449-8","DOIUrl":"10.1007/s00467-024-06449-8","url":null,"abstract":"<p><p>Bilateral renal agenesis (BRA) is a fetal anomaly which leads to anhydramnios and resultant pulmonary hypoplasia. Historically, this anomaly was universally fatal early in the neonatal period due to the severity of the associated lung disease. Over the last 30 years, innovations in fetal therapies-specifically, serial amnioinfusions-have led to instances of infant pulmonary survival and initiation of postnatal dialysis, raising the possibility that early neonatal death may not be inevitable. Amnioinfusions are not without risk, and maternal complications can include prelabor rupture of membranes, preterm labor, infection, and bleeding. The data detailing neonatal outcomes are still limited and actively being collected. Two case series and one non-randomized clinical trial have supplied most of the known outcome data for infants with BRA after prenatal amnioinfusion. Although there are survivors reported in the literature, mortality remains high, with many deaths in infancy due to dialysis-associated sepsis. In addition, previously unknown morbidities have been documented in these infants, including neurologic injury. These challenges, in addition to the mechanical difficulties of providing dialysis to extremely small infants, can result in significant burdens for patients and their caregivers and moral distress for the health care team. The present review aims to explain the pathophysiology of BRA, detail the historical context and rationale for serial amnioinfusions to treat the pulmonary insufficiency associated with BRA, describe the available data regarding outcomes of infants born following prenatal amnioinfusions, discuss ethical issues surrounding this fetal intervention, and describe critical aspects of prenatal counseling for patients considering the intervention.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"329-338"},"PeriodicalIF":2.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141601194","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":"Genetic insights and early monitoring: optimizing outcomes in pediatric renal cyst detection.","authors":"Liliana Italia De Rosa, Martina Catania, Maria Teresa Sciarrone Alibrandi","doi":"10.1007/s00467-024-06527-x","DOIUrl":"10.1007/s00467-024-06527-x","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"597"},"PeriodicalIF":2.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142366087","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":"Biomarker-based acute kidney injury sub-phenotypes refine risk assessment in children undergoing cardiac surgery.","authors":"Kevin A Pettit, Katherine F Melink, Jeffrey A Alten, Stuart L Goldstein, Nicholas Ollberding, Megan SooHoo, Emily Sullivan, Huaiyu Zang, Natalja L Stanski, Katja M Gist","doi":"10.1007/s00467-024-06541-z","DOIUrl":"10.1007/s00467-024-06541-z","url":null,"abstract":"<p><strong>Background: </strong>Pediatric cardiac surgery-associated acute kidney injury (CS-AKI) is common with variable association with outcomes, possibly because transient serum creatinine (SCr) elevations are unrelated to kidney disease. Sub-phenotypes of CS-AKI with biomarker integration may provide prognostic enrichment. This study aims to determine if combining early postoperative urine neutrophil gelatinase-associated lipocalin (uNGAL) and SCr into sub-phenotypes strengthens associations with AKI and outcomes. We hypothesized that patients with early subclinical (uNGAL + , SCr -) or damage (uNGAL + , SCr +) CS-AKI would have more postoperative day 2-4 KDIGO-defined AKI and worse clinical outcomes than patients with early functional AKI (uNGAL - , SCr +).</p><p><strong>Methods: </strong>Two-center prospective observational study evaluating combinations of early uNGAL (8-12 h from ICU admission, ≥ 150 ng/mL) and early postoperative (≤ 8 h of admission) KDIGO SCr-defined AKI to predict CS-AKI on postoperative days (POD) 2-4. Four CS-AKI phenotypes were derived (uNGAL - /SCr - ; uNGAL + /SCr - ; uNGAL - /SCr + and uNGAL + /SCr +). The primary outcome was POD2-4 KDIGO SCr-defined CS-AKI. Secondary outcomes included ventilator and intensive care unit-free days (maximum 28).</p><p><strong>Results: </strong>Four hundred seventy-six patients (median age 4.8 [IQR 1.4-30.4] months, 39% female) were included. POD2-4 AKI occurred in 44 (9.2%). 27% were uNGAL + /SCr - and 0.4% (n = 2) uNGAL + /SCr + . The adjusted odds of POD2-4 AKI was ninefold higher (aOR: 9.09, 95%CI: 3.84-21.53) in uNGAL + /SCr - when compared to uNGAL - /SCr - . uNGAL + /SCr - was associated with fewer ventilator-free (aOR: 0.30, 95%CI: 0.19-0.48) and ICU-free days (aOR: 0.41, 95%CI: 0.26-0.66) when compared to uNGAL - /SCr - .</p><p><strong>Conclusion: </strong>Early postoperative uNGAL, regardless of SCr elevation, refines risk assessment for pediatric POD2-4 CS-AKI and associated morbidity, enabling earlier AKI identification and prognostics.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"523-531"},"PeriodicalIF":2.6,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142351459","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}