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        <title>Frontiers in Pediatrics | Pediatric Nephrology section | New and Recent Articles</title>
        <link>https://www.frontiersin.org/journals/pediatrics/sections/pediatric-nephrology</link>
        <description>RSS Feed for Pediatric Nephrology section in the Frontiers in Pediatrics journal | New and Recent Articles</description>
        <language>en-us</language>
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        <pubDate>2026-05-14T17:28:04.981+00:00</pubDate>
        <ttl>60</ttl>
        <item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1699921</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1699921</link>
        <title><![CDATA[Retrospective comparison of two distinct dual-induction strategies in pediatric kidney transplantation: three doses vs. a single dose of rabbit antithymocyte globulin, each combined with two basiliximab doses]]></title>
        <pubdate>2026-05-12T00:00:00Z</pubdate>
        <category>Original Research</category>
        <author>Gaetano Ciancio</author><author>Jeffrey J. Gaynor</author><author>Mahmoud Morsi</author><author>Angel Alvarez</author><author>Matthew Gaynor</author><author>Marissa Defreitas</author><author>Jayanthi Chandar</author>
        <description><![CDATA[BackgroundWe retrospectively compared two distinct dual-induction strategies in pediatric kidney transplantation.MethodsPatients transplanted at our center prior to 15 February 2020 were scheduled to receive three doses of rabbit antithymocyte globulin (rATG; 1.0 mg/kg/dose) along with two doses of basiliximab (N = 42), while patients transplanted thereafter were scheduled to receive a single dose of rATG with two doses of basiliximab (N = 50). Both intent-to-treat (ITT) groups were scheduled to receive reduced tacrolimus dosing, mycophenolate acid, and early corticosteroid withdrawal. The two primary endpoints were first biopsy-proven acute rejection (BPAR) and any viral viremia. In allowing for a similar follow-up period, all clinical outcomes were compared during the first 24 months (mo) post-transplant. In addition, multivariable analyses were performed adjusting for the propensity to belong to the single-dose ITT rATG group.ResultsMedian follow-up in both groups was 24 mo post-transplant. In the single-dose ITT rATG group, a higher proportion of patients developed a first BPAR [P = 0.10; 34.0% (17/50) vs. 21.4% (9/42)], while a lower proportion developed any viral viremia [P = 0.08; 28.0% (14/50) vs. 47.6% (20/42)]. Two baseline variables were associated with a significantly higher propensity to belong to the single-dose ITT rATG group: older recipient age (P = 0.02) and longer WIT (P = 0.02). After adjusting for the propensity score in multivariable analyses, the single-dose ITT rATG group remained associated with a higher first BPAR rate (P = 0.06) but was no longer associated with the rate of viral viremia (P = 0.30). Estimated graft survival at 24 mo post-transplant (combined across both groups) was 92.9% ± 2.8%.ConclusionsA lower rate of first BPAR but a somewhat higher rate of viral infection was observed in the three-dose ITT rATG group. These study results support the use of tailored induction protocols to optimize effective immunosuppression while minimizing adverse effects in pediatric kidney transplantation.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1757626</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1757626</link>
        <title><![CDATA[Congenital urinary tract dilation: when is it clinically significant and when should it prompt intervention?]]></title>
        <pubdate>2026-04-30T00:00:00Z</pubdate>
        <category>Original Research</category>
        <author>Gunes Isik</author><author>Cemil Oktay</author>
        <description><![CDATA[BackgroundUrinary tract dilation is the most common urinary tract anomaly detected on prenatal ultrasonography. Postnatal follow-up, the necessity of advanced imaging, and indications for surgical intervention remain confusing and subject to ongoing debate.ObjectivesOur aim is to evaluate the etiology, clinical and radiological characteristics of congenital urinary tract dilation, and assess the timing and outcomes of spontaneous resolution, advanced imaging, surgical interventions, and postoperative follow-up.MethodsThis retrospective study evaluated the etiology, clinical and radiological features, and outcomes of congenital urinary tract dilation in children at Adiyaman University Pediatric Nephrology Clinic between November 2021 and 2023. Urinary tract dilation was classified by ultrasonographic anteroposterior (AP) diameter on postnatal urinary system ultrasonography as normal (<10 mm), mild–moderate (10–15 mm), or severe (>15 mm).ResultsAmong 341 patients (71.3% male), 36.2% had severe and 63.8% had mild-to-moderate urinary tract dilation. Mean AP diameter was 14.4 ± 7.0 mm. Urinary tract dilation resolved in 96.2% of patients, typically within 3–44 months (mean 11 ± 6.3). Surgery was required in 22.6% of patients, mostly for ureteropelvic junction obstruction and vesicoureteral reflux, and was significantly more common in severe cases (56.1% vs. 3.7%, p < 0.001). Severe urinary tract dilation was significantly associated with family history of congenital anomalies of the kidney and urinary tract, history of urinary tract infection (UTI), reduced kidney function, and abnormal dimercaptosuccinic acid renal scintigraphy findings (p < 0.001). ROC analysis demonstrated high predictive performance (area under the receiver operating characteristic curve (AUC) = 0.936, 95% confidence interval: 0.908–0.963; p < 0.001). An AP diameter cutoff value of 17 mm predicted surgical intervention, with 88.3% sensitivity and 84.1% specificity.ConclusionAn AP diameter ≥17 mm, decreased differential kidney function, UTI history, and family history of congenital anomalies of the kidney and urinary tract are strong indicators for surgical intervention.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1774632</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1774632</link>
        <title><![CDATA[A case report of neonatal renal pseudohypoaldosteronism type I caused by a de novo variant in the NR3C2 gene]]></title>
        <pubdate>2026-04-30T00:00:00Z</pubdate>
        <category>Case Report</category>
        <author>Hongcai Ma</author><author>Huijiao Han</author><author>Yanyan Liu</author><author>Yong An</author>
        <description><![CDATA[Pseudohypoaldosteronism type I (PHA I) is a rare hereditary disorder of mineralocorticoid resistance, with the renal form predominantly caused by NR3C2 variants. We report a male neonate presenting with severe hyperbilirubinaemia who was subsequently diagnosed with renal PHA I. Born at 38⁺⁶ weeks, he was admitted on day 7 for jaundice. Laboratory evaluation revealed refractory hyponatraemia, markedly elevated plasma aldosterone and renin levels, and ineffective conventional electrolyte therapy. Whole-exome sequencing identified a de novo heterozygous nonsense variant, c.1954C>T (p.Arg652*), in NR3C2, confirming the diagnosis. Aggressive sodium supplementation corrected the electrolyte imbalance, and follow-up showed normal growth and stable serum sodium. This case underscores the need to consider inherited renal tubulopathies in neonates with refractory hyponatraemia and highlights the pivotal role of genetic testing in precise diagnosis.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1779651</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1779651</link>
        <title><![CDATA[Challenges and outcomes in pediatric and adult kidney transplantation]]></title>
        <pubdate>2026-04-28T00:00:00Z</pubdate>
        <category>Review</category>
        <author>Viola Weeda</author><author>Nikolaos Koliakos</author><author>Thomas Caës</author><author>Adrian Khelif</author><author>Dimitri Mikhalski</author><author>Pierre Lingier</author>
        <description><![CDATA[The preoperative evaluation and postoperative management of kidney transplantation are fundamentally tailored to the distinct needs of adult and pediatric populations. The pediatric assessment prioritizes growth, congenital anomalies (like CAKUT), neurodevelopmental progress, and achieving pre-emptive transplantation. In contrast, adult evaluation focuses on comprehensive cardiovascular screening, malignancy risk, and managing accumulated comorbidities, with a growing emphasis on frailty assessment. Surgical technique in children is adapted for size, requiring meticulous microvascular anastomosis and specific urinary reconstruction strategies, especially in the context of complex urological histories. Postoperatively, complication profiles differ adults face higher rates of delayed graft function and cardio metabolic sequelae, while children's unique vascular technical risks and the critical challenge of adolescent non-adherence prevail in children. For both groups of patients, urinary tract infections are a major concern, necessitating structured prophylaxis and management. Looking ahead, pediatric transplantation is exploring minimally invasive robotic techniques. Ultimately, successful long-term outcomes depend on a multidisciplinary, life-stage-specific approach that balances immunological risks with the distinct developmental or chronic disease trajectories of each patient to optimize graft survival and quality of life.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1753010</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1753010</link>
        <title><![CDATA[Management of hypogammaglobulinemia in pediatric patients with refractory lupus nephritis: a focus on belimumab]]></title>
        <pubdate>2026-04-22T00:00:00Z</pubdate>
        <category>Original Research</category>
        <author>Yanan Han</author><author>Yanjun Yang</author><author>Peitong Han</author><author>Xiaoying Yuan</author><author>Chunzhen Li</author><author>Jieyuan Cui</author>
        <description><![CDATA[BackgroundAlthough the use of belimumab in children with lupus nephritis (LN) has increased over the past few years, there are limited data on the safety of belimumab in such patients with hypogammaglobulinemia. There are scarce reports of an association between hypogammaglobulinemia and infection in patients with LN who are receiving belimumab treatment.MethodsWe reviewed the cases of 27 patients with lupus nephritis and nephrotic-range proteinuria admitted to Hebei Children's Hospital from January 2019 to October 2022. Among the 27 patients, 12 received intravenous (IV) belimumab (at a dose of 10 mg per kilogram of body weight) plus the standard systemic lupus erythematosus therapy (SoC) (belimumab group) and the other 15 received the SoC (glucocorticoids plus cyclophosphamide or mycophenolate mofetil) (control group). Estimated Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score; total amount of protein in urine in 24 h; the serum levels of IgG, IgM, IgA, and C3; total B lymphocyte count (BLC); total white lymphocyte count (WBC); erythrocyte sedimentation rate (ESR); and C-reactive protein level were measured five times (at weeks 0, 4, 12, 24, and 52, respectively) in the two groups.ResultsHypogammaglobulinemia was observed in 22/27 (81.5%) participants with LN prior to initiating treatment. Participants developed hypogammaglobulinemia by week 4. There were no significant differences between the two groups in the total amount of protein in urine in 24 h, serum IgG level, and total B cell count at 0 weeks. Furthermore, no IgG replacement therapy was used in either group until week 15 of observation. However, five patients in the belimumab group and one patient in the control group, whose serum IgG level was below 4 g/L, received 1–2 intravenous immunoglobulin (IVIG) treatments in weeks 16–26 due to severe or recurrent infections. The incidence of infection in the belimumab group was significantly higher than that in the control group, and the IgG serum level in the belimumab group was significantly lower than that in the control group. We also found that the ESR in the belimumab group was significantly lower than that in the control group at weeks 12 and 24 (P < 0.05). At weeks 24 and 52, the C3 level in the belimumab group was significantly higher than that in the control group, and the SLEDAI score in the belimumab group was significantly lower than that in the control group (P < 0.05). At week 52, the WBC in the belimumab group was significantly higher than that in the control group (P < 0.05). However, there was no significant difference in the total amount of protein in urine in 24 h between the two groups at the five time points.ConclusionsHypogammaglobulinemia is common in refractory LN. Belimumab treatment may increase the possibility of IgG reduction and the risk of infection. Pediatric patients with LN whose serum IgG levels are below 4 g/L always receive IVIG replacement therapy because of infection. In patients treated with belimumab, monitoring IgG levels is necessary, and IgG replacement therapy should be more aggressive.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1838282</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1838282</link>
        <title><![CDATA[Correction: Acute cyclosporine overdose in a child with nephrotic syndrome: a case report and literature review]]></title>
        <pubdate>2026-04-15T00:00:00Z</pubdate>
        <category>Correction</category>
        <author>Eun Song Song</author><author>Eun Mi Yang</author>
        <description></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1767283</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1767283</link>
        <title><![CDATA[Repeat kidney biopsy in pediatric lupus nephritis: an analysis of associated clinical-pathological features and outcomes]]></title>
        <pubdate>2026-04-15T00:00:00Z</pubdate>
        <category>Original Research</category>
        <author>Wenyan Wang</author><author>Fei Zhao</author><author>Guixia Ding</author><author>Sanlong Zhao</author><author>Guoliang Ma</author><author>Xueqin Cheng</author>
        <description><![CDATA[BackgroundThe utility of repeat kidney biopsy in pediatric lupus nephritis (LN) remains debated. This study aims to evaluate pathological evolution, therapeutic implications, and prognostic impact of repeat biopsies in children with LN.MethodsWe conducted a retrospective analysis of 19 pediatric LN patients (aged 5–18 years) who underwent ≥2 kidney biopsies at Nanjing Children's Hospital (2009–2022). Pathological classification (ISN/RPS criteria), activity/chronicity indices, and treatment responses were compared between biopsies. Statistical analyses were performed using SPSS 21.0, with P < 0.05 considered significant.ResultsThe primary indication for repeat biopsy was proteinuria recurrence (73.7%, 14/19). Pathological class transformation occurred in 47.4% (9/19) of cases (e.g., class III to IV), with a higher proportion of non-nephrotic type presentation in the transformation group (77.8% vs. 20.0%, P < 0.05). Treatment regimens were adjusted in 63.2% (12/19) of patients post-biopsy (e.g., cyclophosphamide to mycophenolate mofetil). Although chronicity scores increased significantly (e.g., glomerulosclerosis: 57.9% to 89.5%, P < 0.05), short-term kidney remission rates did not differ between groups with and without pathological transformation (77.8% vs. 80.0%, P > 0.05).ConclusionRepeat kidney biopsy detects pathological progression (e.g., increased chronicity) and guides treatment modifications in over 60% of pediatric LN cases. However, pathological class change alone does not dictate short-term outcomes, highlighting initial treatment response as a stronger prognostic indicator. Individualized use of repeat biopsies is recommended for children with proteinuria relapse to optimize management while mitigating procedural invasiveness.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1790656</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1790656</link>
        <title><![CDATA[Case Report: A rare coexistence with severe aortic root dilatation and nutcracker phenomenon in pediatric Marfan syndrome]]></title>
        <pubdate>2026-04-14T00:00:00Z</pubdate>
        <category>Case Report</category>
        <author>Xiaoyu Qiao</author><author>Yanyu Chen</author><author>Danyan Su</author><author>Lifeng Shang</author><author>Yusheng Pang</author>
        <description><![CDATA[Marfan syndrome (MFS) is a multisystem connective tissue disorder affecting the cardiovascular, ocular, and skeletal systems. We report a case of a 13.5-year-old boy who presented with excessive linear growth. Diagnostic evaluations revealed severe aortic root dilatation, repeatedly positive occult blood in urine, and ultrasonographic findings suggestive of left renal vein entrapment. Genetic testing identified a pathogenic variant in the FBN1 gene. The patient was ultimately diagnosed with Marfan syndrome complicated by left renal vein entrapment syndrome (nutcracker phenomenon). The co-occurrence of severe aortic root dilatation and left renal vein entrapment syndrome in childhood Marfan syndrome is relatively uncommon. This case may provide valuable insights for clinical diagnosis and management.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1671377</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1671377</link>
        <title><![CDATA[The burden of protein-energy wasting in children with CKD3-5D: an observational study]]></title>
        <pubdate>2026-04-08T00:00:00Z</pubdate>
        <category>Original Research</category>
        <author>Ying Liang</author><author>Ying Shen</author><author>Yeping Jiang</author>
        <description><![CDATA[BackgroundProtein-energy wasting (PEW) is a critical complication in children with advanced chronic kidney disease (CKD), yet its burden remains poorly characterized in pediatric populations. This observational study aimed to determine the prevalence and clinical correlates of PEW in children with CKD stages 3-5D.MethodsA retrospective analysis was conducted on 170 children (aged 0–18 years) treated at Beijing Children's Hospital from January 2015 to December 2024. PEW was diagnosed using modified pediatric-specific criteria, which is fully aligned with the core nutritional assessment and management principles of the 2020 KDOQI Clinical Practice Guidelines for Nutrition in CKD, requiring growth retardation (height SDS < −1.88 or growth velocity <10th percentile) plus two additional parameters (hypoalbuminemia, reduced intake, weight loss, or muscle wasting). Data on anthropometry, biochemical markers, three consecutive 24-h dietary recalls (standardized), and nutritional interventions (oral nutritional supplements, dietary counseling, specialist referral) were analyzed. A multivariate linear regression analysis was performed to assess PEW as an independent predictor of height velocity after adjusting for metabolic acidosis, GH/IGF-1 axis resistance and CKD-MBD; Spearman correlation and logistic regression analyses were conducted to explore the association between inflammatory markers (hs-CRP) and PEW.ResultsThe prevalence of PEW was 30.6%, increasing with CKD severity: 0% (stage 3), 25.9% (stage 4), 39.7% (stage 5 non-dialysis), and 20.0% (stage 5D). Hypoalbuminemia (73.1%) and reduced intake (73.1%) were predominant features. Short stature (height SDS < −1.88) affected 45.3% of the cohort. Dialysis patients exhibited lower PEW rates than non-dialysis stage 5 counterparts (P = 0.002), a disparity associated with standardized nutritional interventions (oral nutritional supplements, regular dietary counseling) in 5D patients (86.7% coverage) vs. low intervention rates in stage 5 non-dialysis patients (12.8% coverage). Over 46% of stage 5 patients were not on dialysis, primarily due to delayed referral or parental preference, which further contributed to inadequate nutritional management in this subgroup. PEW was an independent predictor of impaired height velocity after adjusting for confounders (β = −0.32, P < 0.001); hs-CRP levels were positively correlated with PEW scores (r = 0.41, P < 0.001), and elevated hs-CRP (>3 mg/L) was an independent risk factor for PEW (OR = 2.86, 95% CI: 1.52–5.38, P = 0.001).ConclusionPEW is highly prevalent in advanced pediatric CKD, driven by inflammation and nutritional deficits, and acts as an independent predictor of growth retardation. Early screening and stage-specific, evidence-based dietary and pharmacological interventions in accordance with the 2020 KDOQI guidelines are imperative to mitigate morbidity, particularly for stage 5 non-dialysis patients with low access to standardized nutritional care.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1712781</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1712781</link>
        <title><![CDATA[Single-center analysis of etiology and complications in hospitalized children with chronic kidney disease stages 3–5]]></title>
        <pubdate>2026-03-27T00:00:00Z</pubdate>
        <category>Original Research</category>
        <author>Ying Liang</author><author>Ying Shen</author><author>Yeping Jiang</author>
        <description><![CDATA[ObjectiveTo investigate the etiology spectrum and complication profiles of hospitalized children with chronic kidney disease (CKD) stages 3–5, identify independent risk factors for CKD progression, and provide evidence for optimizing early clinical management. We hypothesized that congenital anomalies of the kidney and urinary tract (CAKUT) are the predominant etiology in this cohort, and baseline eGFR decline plus uncontrolled hypertension would independently predict progression to stage 5 CKD.MethodsA retrospective cohort study was conducted on 170 children with CKD stages 3–5 admitted to the Nephrology Department of Beijing Children's Hospital from January 2016 to December 2023. Etiology, complication rates, and their associations with CKD stages were analyzed. Multivariate logistic regression models were used to identify independent risk factors for progression to stage 5 CKD, adjusting for age, sex, and underlying etiology.ResultsCAKUT accounted for 51.8% of cases, followed by glomerular diseases (22.4%). The prevalence of anemia (80.5%), hypertension (64.1%), and CKD-MBD (90.6%) increased significantly with CKD progression (P < 0.001 for all). Anemia occurred in 100% of stage 5 non-dialysis patients, while hypertension control rates were only 45.3%. Multivariate analysis identified baseline eGFR <30 mL/min/1.73 m² (OR = 4.2, 95% CI: 2.1–8.3, P < 0.001) and uncontrolled hypertension (OR = 2.5, 95% CI: 1.5–4.2, P = 0.001) as independent predictors of progression to stage 5 CKD. The overall infection rate was 28.2%.ConclusionCAKUT is the leading etiology of CKD stages 3–5 in hospitalized children, with a high burden of complications. Early intervention for anemia, hypertension, and CKD-MBD, combined with enhanced infection prevention, may improve clinical outcomes. Baseline eGFR <30 mL/min/1.73 m² and uncontrolled hypertension are key targets for delaying CKD progression. Notably, the distribution of etiology and complication burden in this cohort parallels patterns reported in North America and Europe, supporting the global applicability of KDIGO-based management strategies.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1706611</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1706611</link>
        <title><![CDATA[Clinical value of luciferase-based bioluminescence assay in diagnosis of Alport syndrome]]></title>
        <pubdate>2026-03-12T00:00:00Z</pubdate>
        <category>Original Research</category>
        <author>Yue Cai</author><author>Ying-qi Lin</author><author>Xin-yu Kuang</author><author>Lei Sun</author><author>Meng-ying Li</author><author>Yu-jie Hu</author><author>Wen-yan Huang</author>
        <description><![CDATA[ObjectivesAlport syndrome (AS) is an inherited kidney disorder caused by pathogenic variants in COL4A3, COL4A4, or COL4A5. In this study, we aim to apply a split-luciferase bioluminescence assay to functionally assess COL4A3, COL4A4, or COL4A5 variants of uncertain significance (VUS) identified in pediatric patients with Alport syndrome, and to explore its value in supporting variant interpretation and diagnostic evaluation.MethodsA retrospective analysis included 31 children who met established clinical and pathological diagnostic criteria for Alport syndrome, but in whom genetic testing identified VUS in COL4A3, COL4A4, or COL4A5. Genomic DNA was analyzed using next-generation sequencing (NGS) to identify variant loci. Recombinant plasmids corresponding to the identified variants were constructed and transfected into HEK293T cells. The split-luciferase bioluminescence assay (NanoBiT® system) was employed to measure luminescence signals in living cells. Luminescence intensity was compared between the VUS-associated plasmids and the wild-type (WT) plasmid, and the sensitivity and specificity of this technique were evaluated.ResultsThe luminescence intensity of all 31 plasmids carrying VUS identified in children with Alport syndrome was reduced by more than 50% compared with the WT plasmid group (P < 0.01, n = 3). When the luminescence intensity was below 397.8, the sensitivity for distinguishing VUS from WT was 96.77%, and the specificity was 100%.ConclusionsThe split-luciferase bioluminescence assay was successfully applied as an in vitro functional approach to assess COL4A3, COL4A4, or COL4A5 VUS, and may reliably assist in the diagnostic evaluation of pediatric patients with suspected Alport syndrome whose genetic testing reveals VUS, particularly in cases where pathological assessment is unavailable or not feasible.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1779744</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1779744</link>
        <title><![CDATA[Etiology and longitudinal kidney outcomes in children with nephrocalcinosis: a retrospective cohort study]]></title>
        <pubdate>2026-03-06T00:00:00Z</pubdate>
        <category>Original Research</category>
        <author>Mihriban İnözü</author><author>Sibel Çetince Şenses</author><author>Özlem Yüksel Aksoy</author><author>Burcu Yazıcıoğlu</author><author>Sare Gülfem Özlü</author><author>Fatma Şemsa Çaycı</author><author>Umut Selda Bayrakçı</author>
        <description><![CDATA[BackgroundNephrocalcinosis refers to the deposition of calcium salts within the kidney parenchyma and is a condition encountered in various metabolic, genetic, and systemic disorders in childhood. Nephrocalcinosis is increasingly recognized in children; however, data on long-term kidney outcomes and prognostic factors remain limited.MethodsThis study was designed as a retrospective cohort study. We evaluated the clinical characteristics, etiological spectrum, and longitudinal kidney outcomes of children with nephrocalcinosis. Changes in estimated glomerular filtration rate (eGFR) over follow-up were assessed, and factors associated with kidney function decline were analyzed using multivariable regression.ResultsAmong 73 children with nephrocalcinosis, 43 were included in longitudinal analyses with a median follow-up of 52 months. Median eGFR declined significantly over time (p = 0.006). Kidney outcomes varied according to underlying etiology, with lower final eGFR observed in children with hereditary or genetic causes. In multivariable analysis, systemic/syndromic etiology, history of urinary tract infection, and older age at diagnosis were independently associated with greater eGFR decline, whereas traditional metabolic risk factors were not independently associated with longitudinal changes in kidney function.ConclusionOur findings suggest that pediatric nephrocalcinosis may be associated with a decline in kidney function over long-term follow-up and that kidney outcomes are influenced by underlying etiological and clinical factors. Given the paucity of studies addressing the long-term prognosis of nephrocalcinosis, these results highlight the need for early diagnosis, careful etiological evaluation, individualized risk stratification, and close follow-up, particularly in high-risk subgroups. Furthermore, our study underscores the need for prospective studies to better characterize prognostic factors and optimize long-term care in children with nephrocalcinosis.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1740485</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1740485</link>
        <title><![CDATA[Case Report: Ataxia telangiectasia with severe hemorrhagic cystitis]]></title>
        <pubdate>2026-02-26T00:00:00Z</pubdate>
        <category>Case Report</category>
        <author>Hua Song</author><author>Yi Lin</author><author>Yuwei Xian</author>
        <description><![CDATA[BackgroundAtaxia telangiectasia (AT) is a rare autosomal recessive genetic disorder caused by variants in the ataxia-telangiectasia mutated (ATM) gene. AT is characterized by progressive cerebellar degeneration, telangiectasia, immunodeficiency, cancer susceptibility, and radiosensitivity. This report presents a case of classic AT complicated by severe hemorrhagic cystitis, a rare clinical manifestation. Genetic analysis revealed novel variants in the ATM gene.Case presentationA 12-year-old Han Chinese boy presented with recurrent gross hematuria that progressed in frequency and severity after completion of chemotherapy for T-cell acute lymphoblastic leukemia (ALL). He had developed gait instability at age 2, and brain MRI showed cerebellar atrophy. Genetic testing revealed compound heterozygous ATM variants: c.8357G>T (p.Gly2786Val) (maternal) and IVS54+3A>C (paternal) (NM_000051). Cystoscopy revealed multiple telangiectatic lesions of the bladder mucosa with associated yellow-brown sedimentation. Emergency cystoscopic electrocoagulation controlled the bleeding.ConclusionWe report two novel ATM variants (c.8357G>T, IVS54+3A>C) in a patient with classic AT who developed severe hemorrhagic cystitis associated with bladder wall telangiectasia. AT patients may be at risk for delayed, potentially life-threatening hemorrhagic cystitis, particularly following cyclophosphamide exposure. Cystoscopy is essential for diagnosis and enables timely endoscopic management.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1714324</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1714324</link>
        <title><![CDATA[Integrating machine learning for advanced analysis of bioelectrical impedance parameters in children with nephrotic syndrome]]></title>
        <pubdate>2026-02-16T00:00:00Z</pubdate>
        <category>Original Research</category>
        <author>Josephine Reinert Quist</author><author>Leigh C. Ward</author><author>Lars Jødal</author><author>René Frydensbjerg Andersen</author><author>Christian Lodberg Hvas</author><author>Steven Brantlov</author>
        <description><![CDATA[BackgroundNephrotic syndrome (NS) in children, characterised kidney-related protein leakage and peripheral oedema, remains challenging to assess. Bioelectrical impedance analysis (BIA) provides indices of body water (oedema), and analysis with machine learning (ML) may improve clinical care. We tested an ML model to identify NS in children compared with healthy children.MethodsThis cross-sectional study included children with active NS in the acute phase (aNS group) recruited from the Department of Paediatrics and Adolescent Medicine, Aarhus University Hospital, Denmark. Anonymised MF-BIA data from frequencies between 5 and 1000 kHz were analysed using the web-based ML platform JustAddDataBio (JADBio)® to identify potential biomarkers for improved diagnosis.ResultsEight children with aNS and 38 healthy children of similar ages were included. The ML software employed ridge logistic regression with the penalty hyperparameter lambda = 0.001 and a selected threshold of 0.81 by JADBio. The best model achieved an area under the curve (AUC) of 0.84 [95% confidence interval (CI): 0.72;0.94]. The software selected the following features: height, age, resistance at 50 kHz, impedance at 50 kHz, the characteristic frequency, phase angle at 50 kHz, and sex. The model demonstrated a statistically significant true positive classification rate of 0.92 (92%) [CI: 0.88;0.96] and a specificity of 0.22 (22%) [CI: 0.08;0.36].ConclusionApplying ML-supported evaluation of BIA affirmed diagnostics. However, low specificity limits clinical applications. A larger population of patients and inclusion of additional biomarkers may be needed to develop a more acceptable model.]]></description>
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        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1690215</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1690215</link>
        <title><![CDATA[Tacrolimus-induced reversible posterior leukoencephalopathy syndrome in a child with nephrotic syndrome: a case report]]></title>
        <pubdate>2026-01-29T00:00:00Z</pubdate>
        <category>Case Report</category>
        <author>Dengyan Wu</author><author>Lina Ma</author><author>Huimin Wu</author><author>Jie Li</author><author>Jing Yang</author><author>Li Huang</author>
        <description><![CDATA[BackgroundTacrolimus is widely used as an immunosuppressant in the management of refractory nephrotic syndrome. Although effective, it may occasionally lead to rare but serious adverse effects such as posterior reversible leukoencephalopathy syndrome (PRES). PRES has traditionally been associated with hypertension and elevated drug concentrations.Case presentationIn the present study, we describe the case of a 10-year-old Chinese girl who was diagnosed with steroid-resistant nephrotic syndrome (SRNS) and pathologically confirmed to have minimal change nephropathy. Following 40 days of full-dose glucocorticoid therapy with inadequate improvement in proteinuria, tacrolimus was initiated at 1.5 mg twice daily (0.09 mg/kg/day). Neurological symptoms, including headache and nausea, developed 18 h after the first dose—before steady-state drug levels were reached. Within 24 h, hypertension emerged, and magnetic resonance imaging (MRI) revealed abnormal signals in the bilateral parietal cortical and subcortical regions, consistent with PRES. Tacrolimus was immediately discontinued, and the patient was treated with nifedipine, low-dose furosemide, and vitamin B6. Symptoms resolved within 48 h, and blood pressure normalized. Immunosuppressive therapy was subsequently switched to mycophenolate mofetil (MMF). Follow-up brain MRI at three months demonstrated complete resolution of the detected abnormalities.ConclusionTacrolimus-associated PRES may occur very early in treatment, even before stable drug concentrations are achieved. Vigilant clinical monitoring, prompt recognition of neurological symptoms, and timely intervention are critical to avoid long-term sequelae.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2025.1710286</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2025.1710286</link>
        <title><![CDATA[C3 molecular structural and histopathological analyses in a pediatric case of atypical hemolytic uremic syndrome with life-threatening gastrointestinal bleeding—a case report]]></title>
        <pubdate>2026-01-27T00:00:00Z</pubdate>
        <category>Case Report</category>
        <author>Takuji Enya</author><author>Kohei Miyazaki</author><author>Sakina Kuge</author><author>Yuichi Morimoto</author><author>Hiroki Kondou</author><author>Naoki Sakata</author><author>Kensuke Joh</author><author>China Nagano</author><author>Kandai Nozu</author><author>Nobutoshi Ito</author><author>Yoshiyuki Hakata</author><author>Keisuke Sugimoto</author><author>Masaaki Miyazawa</author>
        <description><![CDATA[Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening disease characterized by uncontrolled complement activation and is associated with various genetic factors, including multiple variants at the gene locus encoding the complement component 3 (C3). However, only a few functional amino acid substitutions have been identified in C3. We report a pediatric case of aHUS presenting with refractory hypertension and massive gastrointestinal bleeding. Comprehensive histopathological, immunohistochemical, genetic, and molecular structural analyses were performed. Biopsy specimens were stained for renin and the membrane-attack complex of the complement system (C5b-9). Plasma levels of the Ba fragment of complement factor B were measured to evaluate the alternative pathway activation. Genomic DNA was obtained with consent and analyzed by targeted next-generation sequencing using a custom gene panel, followed by Sanger sequencing for variant confirmation. The possible effects of the identified amino acid substitution on the molecular structure of C3 were analyzed using computer-aided simulation with MODELLER and DoGSiteScorer. As a result, the juxtaglomerular apparatus was hyperplastic and intensely stained for renin. Endothelial cells of renal and intestinal blood vessels were positive for C5b-9. The plasma Ba level was elevated compared to the control level. The ileal and colonic mucosae were denuded and highly edematous, with epithelial cells undergoing regenerative and metaplastic changes in the active phase. Mucosal blood vessels contained intraluminal red cell fragments and neutrophils attached to swollen endothelial cells. However, the colonic mucosa showed near-normal histology after disease convalescence. Genetic analyses identified a single nucleotide C3 variant NM_000064.4(C3):c.4811T>C (p.Met1604Thr), resulting in an M1604T substitution in the functional C345C domain. Molecular structural analyses indicated that this amino acid substitution can cause the formation of a large cavity within the hydrophobic core of the C-terminal domain, possibly destabilizing the spherical structure of C3. Our study highlights that the M1604T substitution in the C3 C345C domain may drive the observed excessive complement activation, C5b-9 deposition on endothelial cells, and severe circulatory disturbances in the intestinal mucosa.]]></description>
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        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2026.1737399</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2026.1737399</link>
        <title><![CDATA[Acute cyclosporine overdose in a child with nephrotic syndrome: a case report and literature review]]></title>
        <pubdate>2026-01-22T00:00:00Z</pubdate>
        <category>Case Report</category>
        <author>Eun Song Song</author><author>Eun Mi Yang</author>
        <description><![CDATA[BackgroundCalcineurin inhibitors are widely used in organ transplantation and nephrotic syndrome, with chronic toxicities well documented, but acute toxicity is rarely reported.Case presentationWe report a 3.9-year-old boy with steroid-dependent nephrotic syndrome who accidentally ingested a single dose of cyclosporine A (CsA) at 75 mg/kg (1,500 mg)— over 20 times the intended dose of 3.5 mg/kg (70 mg)—due to medication error. He developed transient abdominal pain, diarrhea, and vomiting, which resolved without treatment. Error was discovered 12 h post-ingestion, and he was hospitalized with a CsA trough level of 1003 ng/mL (measured 13 h after ingestion), yet remained clinically stable. Management included CsA discontinuation, intravenous hydration, rifampin- and phenobarbital-induced cytochrome P450 activation, resulting in normalization of CsA levels within 48 h. A literature review identified 28 pediatric cases of acute CsA overdose, with presentations ranging from asymptomatic to severe neurotoxicity and acute kidney injury. The varied, including supportive care, gastrointestinal decontamination, and enzyme induction, with generally favorable outcomes.ConclusionsAlthough rare, acute CsA overdose in children can pose serious risks. This case and review underscore the symptoms of overdose and prompt intervention to prevent complications.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2025.1728887</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2025.1728887</link>
        <title><![CDATA[Case Report: Spinal muscular atrophy with IgA nephropathy: a coincidence or association?]]></title>
        <pubdate>2026-01-22T00:00:00Z</pubdate>
        <category>Case Report</category>
        <author>Yuxuan Gu</author><author>Le Wang</author><author>Xiaoying Yuan</author><author>Yanan Han</author><author>Peitong Han</author><author>Jieyuan Cui</author><author>Xinlei Wang</author><author>Yuchan Huang</author><author>Lili Zhang</author><author>Chunzhen Li</author>
        <description><![CDATA[BackgroundSpinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder caused by biallelic loss-of-function variants of the survival motor neuron 1 (SMN1) gene on chromosome 5q13. It has been reported that SMA may affect the function of the kidneys. Here, we report a patient with co-occurrence of SMA and IgA nephropathy (IgAN).Case presentationA 14-year-old girl presented with six months of limb weakness, progressive exacerbation of symptoms of left lower limb muscle weakness; her left lower limb muscle strength decreased, and bilateral knee tendon reflexes and Achilles tendon reflexes were not elicited. The patient was diagnosed with SMA type 3 in conjunction with the results of genetic testing. The patient had proteinuria and hematuria, and a renal biopsy was performed. Considering the patient's clinical and pathological characteristics, the final diagnosis was spinal muscular atrophy combined with IgA nephropathy. To the best of our knowledge, this is the first reported case that demonstrates the coexistence of SMA and IgAN.Discussion and conclusionsThe exact mechanism of renal impairment due to SMA is not fully understood, and the combination of SMA with IgAN is extremely rare. Our report suggests that there may be a potential association between them.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2025.1709402</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2025.1709402</link>
        <title><![CDATA[Efficacy and safety of corticosteroid and immunosuppressive agent combination therapy for pediatric IgA nephropathy: a meta-analysis]]></title>
        <pubdate>2026-01-15T00:00:00Z</pubdate>
        <category>Systematic Review</category>
        <author>Xilin Xiong</author>
        <description><![CDATA[BackgroundIgA nephropathy (IgAN) is a common primary glomerular disease in children. The efficacy and safety of glucocorticoid (GC) and immunosuppressive therapies remain debated. This study aimed to evaluate their clinical effectiveness and safety in pediatric IgAN.MethodsA systematic search was conducted in six databases (PubMed, Web of Science, Cochrane, Embase, CNKI, Wanfang, and VIP), yielding 404 studies, of which eight met inclusion criteria. Eligible studies included RCTs or retrospective studies involving pediatric patients (≤18 years) treated with immunosuppressants plus GC. Outcomes assessed included proteinuria, hematuria, serum creatinine, and adverse events. Two researchers independently extracted and analyzed data. Network meta-analysis (NMA) was performed using R software, incorporating network plots, forest plots, and SUCRA rankings.ResultsNetwork topology showed strong links between GC monotherapy and regimens such as “tacrolimus + GC” and “mycophenolate mofetil (MMF) + GC.” SUCRA rankings indicated superior efficacy of combination therapies in reducing proteinuria and hematuria. Forest plots revealed that all combination regimens significantly reduced proteinuria compared to GC alone (P < 0.05), with only MMF + GC significantly improving hematuria (P < 0.05). No significant differences in adverse event rates were found among treatment groups (P > 0.05). Funnel plots suggested minimal publication bias.ConclusionCombination therapies, especially tacrolimus + GC and MMF + GC, offer greater efficacy than GC monotherapy in pediatric IgAN, without increasing adverse events. These findings support their clinical application, though larger studies are needed to validate results and optimize treatment strategies.]]></description>
      </item><item>
        <guid isPermaLink="true">https://www.frontiersin.org/articles/10.3389/fped.2025.1755954</guid>
        <link>https://www.frontiersin.org/articles/10.3389/fped.2025.1755954</link>
        <title><![CDATA[Urodynamic characterization in children with lower urinary tract symptoms and comorbid ADHD: a retrospective matched case-control study]]></title>
        <pubdate>2026-01-15T00:00:00Z</pubdate>
        <category>Original Research</category>
        <author>Ping Bai</author><author>Lili Liu</author><author>Diyi Luo</author>
        <description><![CDATA[BackgroundLower urinary tract symptoms (LUTS) are common functional urinary disorders in children and can markedly impair quality of life. Attention-deficit/hyperactivity disorder (ADHD) is a prevalent neurodevelopmental condition, and emerging evidence suggests that affected children are at increased risk of LUTS. Nevertheless, systematic investigations into the relationship between ADHD and urodynamic characteristics in pediatric LUTS remain limited. This study aimed to evaluate the association between ADHD and urodynamic features in children with LUTS using a case–control design.MethodsWe conducted a retrospective case–control study including 144 children with LUTS, of whom 36 were diagnosed with ADHD. All participants underwent standardized urodynamic testing, with assessments of bladder capacity, detrusor pressure at maximum filling, and detrusor overactivity (DO). Children were categorized into ADHD and non-ADHD groups, and intergroup comparisons of urodynamic parameters were performed. Multivariable regression analysis was used to assess the independent association between ADHD and urodynamic abnormalities.ResultsCompared with the non-ADHD group (n = 108), children with ADHD (n = 36) exhibited significantly reduced volumes at first urge, strong urge, and maximum cystometric capacity (all p < 0.05). Conversely, detrusor pressure at maximum filling and the prevalence of DO were significantly higher in the ADHD group (both p < 0.05). Multivariable regression analysis identified DO as an independent predictor of urodynamic abnormalities in children with ADHD (OR = 3.43, 95% CI: 1.32–8.91, p = 0.012).ConclusionsChildren with ADHD display significant functional bladder abnormalities on urodynamic testing, particularly reduced bladder capacity, increased detrusor pressure during filling, and heightened detrusor activity. ADHD may influence bladder function, at least in part, through neurobehavioral mechanisms. These findings provide valuable clinical insights for the management of LUTS in children with ADHD and underscore the importance of early screening and intervention. Further research is needed to elucidate the underlying mechanisms and to develop effective therapeutic strategies.]]></description>
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