Pediatric Nephrology最新文献

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Impact of acute kidney injury and dysnatremia on length of stay in infants after cardiac surgery. 急性肾损伤和钠血症对婴儿心脏手术后住院时间的影响。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-06-14 DOI: 10.1007/s00467-025-06846-7
Jonas Rønne Kronborg, Rasmus Bo Lindhardt, Niels Vejlstrup, Line Marie Holst, Klaus Juul, Morten Holdgaard Smerup, Jakob Gjedsted, Hanne Berg Ravn
{"title":"Impact of acute kidney injury and dysnatremia on length of stay in infants after cardiac surgery.","authors":"Jonas Rønne Kronborg, Rasmus Bo Lindhardt, Niels Vejlstrup, Line Marie Holst, Klaus Juul, Morten Holdgaard Smerup, Jakob Gjedsted, Hanne Berg Ravn","doi":"10.1007/s00467-025-06846-7","DOIUrl":"10.1007/s00467-025-06846-7","url":null,"abstract":"<p><strong>Background: </strong>Acute kidney injury (AKI) and dysnatremia following pediatric cardiac surgery are common conditions associated with worse outcomes. While the multifactorial etiology of AKI is well-known, the role of concomitant dysnatremia is limited. This study aims to describe the occurrence of AKI, its association with the length of stay in the intensive care unit (ICU-LOS), and the impact of dysnatremia in the context of AKI.</p><p><strong>Methods: </strong>Retrospective study comprising 228 congenital heart procedures in 213 infants at Rigshospitalet, Copenhagen, Denmark, from 2017 to 2019. AKI development was evaluated separately in neonates and infants > 1 month and its impact on ICU-LOS. Risk factors for AKI were analyzed across age groups using the univariate and multivariate logistic regression analysis.</p><p><strong>Results: </strong>AKI occurred in 61% of neonates and 62% of infants. Severity was comparable across age groups, except for KDIGO-stage 3, where seven out of eight children treated with peritoneal dialysis were neonates. Urine output was well-preserved despite AKI development, but children with AKI required more than double the furosemide dose. In multivariate analysis, prolonged cardiopulmonary bypass (CPB) duration, higher furosemide doses, and hypernatremia were independently associated with AKI. AKI was only associated with prolonged ICU-LOS in infants, while hyponatremia was associated with prolonged ICU-LOS in all individuals with AKI.</p><p><strong>Conclusions: </strong>AKI occurs frequently in neonates and infants after congenital heart surgery but is only associated with prolonged ICU-LOS in infants. The co-occurrence of AKI and hyponatremia leads to longer ICU-LOS in both neonates and infants. Independent predictors of AKI were prolonged CPB duration, hypernatremia, and reduced furosemide sensitivity.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3281-3291"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12402015/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144294761","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}
引用次数: 0
Impact of online hemodiafiltration on bone turnover in children with CKD-5d: A prospective cohort study. 在线血液滤过对CKD-5d患儿骨转换的影响:一项前瞻性队列研究
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-05-19 DOI: 10.1007/s00467-025-06805-2
Mohammed F Kasem, Ragia M Said, Aliaa Mourad, Noha R Mohamed, Noha U Hashem
{"title":"Impact of online hemodiafiltration on bone turnover in children with CKD-5d: A prospective cohort study.","authors":"Mohammed F Kasem, Ragia M Said, Aliaa Mourad, Noha R Mohamed, Noha U Hashem","doi":"10.1007/s00467-025-06805-2","DOIUrl":"10.1007/s00467-025-06805-2","url":null,"abstract":"<p><strong>Background: </strong>Mineral bone disorder (MBD) is a systemic disorder associated with chronic kidney disease (CKD). Online hemodiafiltration (OL-HDF) combines hemodialysis (HD) and hemofiltration and has shown promising results in children with CKD-5d considering co-morbidities.</p><p><strong>Methods: </strong>Children with CKD-5d who were stable for at least 3 months on thrice weekly 3-h HD sessions via an arteriovenous fistula (AVF) using polysulphone membrane were shifted to post-dilution OL-HDF and followed up for 12 months. Baseline Ca, PO4, serum albumin, alkaline phosphatase, iPTH, CRP, soluble Klotho, FGF-23, BALP and TRAP-5b were assessed and repeated at the end of the 12-month follow-up period.</p><p><strong>Results: </strong>We included 31 children (17 males) with median age of 12.5 (IQR = 9.7-13.3) years and median HD vintage of 60.1 (IQR = 9.1-37.5) months. OL-HDF resulted in a statistically significant decrease in FGF-23 and FGF-23/Klotho ratios and insignificant increase in the levels of Klotho compared to their baseline values. It also led to statistically significant increase in BALP, decrease in TRAP-5b and elevation of the BALP/TRAP-5b ratio compared to their baseline values. A 12-month period of OL-HDF treatment had no significant effect on height Z-score before and after exclusion of patients having deformities of lower limbs.</p><p><strong>Conclusion: </strong>OL-HDF resulted in a significant decrease in FGF-23, TRAP-5b and FGF-23/Klotho ratio with a significant increase in BALP and BALP/TRAP ratio. This might signify a promising positive impact on bone turnover in children with CKD-5d.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3253-3262"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12401763/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144094268","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}
引用次数: 0
Primary renal diffuse large B-cell lymphoma presenting as new-onset kidney failure. 原发性肾弥漫性大b细胞淋巴瘤表现为新发肾衰竭。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-06-10 DOI: 10.1007/s00467-025-06833-y
Massimiliano Bertacchi, Lea Vasey, Alexandra Wilhelm-Bals, Anne-Laure Rougemont, Frederic Baleydier, Paloma Parvex
{"title":"Primary renal diffuse large B-cell lymphoma presenting as new-onset kidney failure.","authors":"Massimiliano Bertacchi, Lea Vasey, Alexandra Wilhelm-Bals, Anne-Laure Rougemont, Frederic Baleydier, Paloma Parvex","doi":"10.1007/s00467-025-06833-y","DOIUrl":"10.1007/s00467-025-06833-y","url":null,"abstract":"<p><strong>Background: </strong>Primary renal lymphoma is a rare form of kidney tumor, accounting for less than 1% of all kidney masses, and with only a few pediatric cases reported in the literature. Bilateral forms can present with kidney failure, constituting a therapeutic challenge for both hematologists and nephrologists, with the need to combine complex chemotherapy regimens and kidney replacement therapy.</p><p><strong>Methods: </strong>We present a rare case of primary bilateral renal diffuse large B-cell lymphoma in a teenage girl presenting with kidney failure (eGFR of 7 ml/min/1.73 m<sup>2</sup>), proposing a practical approach to optimize chemotherapy on kidney replacement therapy. We discuss diagnostic methods, the management and adjustment of chemotherapy and hyperhydration protocols, and the tailoring of highly efficient hemodiafiltration sessions to grant sufficient drug exposure while avoiding drug accumulation and toxicity in the context of kidney failure.</p><p><strong>Results: </strong>This approach permitted a substantial reduction of chemotherapy adverse effects while inducing remission and partial recovery of kidney function in our patient, with hemodialysis discontinuation after 6 months, and an eGFR that had improved to 28 ml/min/1.73 m<sup>2</sup> at 12 months.</p><p><strong>Conclusions: </strong>Managing complex chemotherapy protocols in kidney failure can be challenging, and the collaboration of a multidisciplinary team is essential. The adjustment of drug dosing and the tailoring of hemodialysis can increase patients' tolerance while maintaining chemotherapy efficiency.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3181-3186"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12401750/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144258667","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}
引用次数: 0
Growth dynamics of transversal body dimensions and proportions, with related clinical determinants in children with X-linked hypophosphatemia treated with phosphate supplements and active vitamin D. 在接受磷酸盐补充剂和活性维生素D治疗的x连锁低磷血症儿童中,横向身体尺寸和比例的生长动态及其相关临床决定因素
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-06-10 DOI: 10.1007/s00467-025-06841-y
Laura Celine Brieger, Stephan Przygodda, Alina Verena Bohlen, Mirko Rehberg, Martin Konrad, Karl Peter Schlingmann, Olaf Hiort, Dorothee Schmidt, Ulrike John-Kroegel, Elke Wuehl, Markus Josef Kemper, Ute Derichs, Ludwig Patzer, Norbert Albers, Desiree Dunstheimer, Sabine Heger, Karina Grohmann-Held, Carmen Schroeder, Norbert Jorch, Elmar Schmid, Hagen Staude, Marcus Weitz, Clemens Freiberg, Angela Huebner, Anke Heitmeyer-Pyper, Giuseppina Sparta, Carl-Joachim Partsch, Michaela Marx, Christof Land, Inka Baus, Frauke Wilkening, Kristina Moeller, Gunter Simic-Schleicher, Susann Empting, Oliver Metzing, Verena Wagner, Martin Holder, Mislav Stjepan Žebec, Dirk Schnabel, Dieter Haffner, Miroslav Zivicnjak
{"title":"Growth dynamics of transversal body dimensions and proportions, with related clinical determinants in children with X-linked hypophosphatemia treated with phosphate supplements and active vitamin D.","authors":"Laura Celine Brieger, Stephan Przygodda, Alina Verena Bohlen, Mirko Rehberg, Martin Konrad, Karl Peter Schlingmann, Olaf Hiort, Dorothee Schmidt, Ulrike John-Kroegel, Elke Wuehl, Markus Josef Kemper, Ute Derichs, Ludwig Patzer, Norbert Albers, Desiree Dunstheimer, Sabine Heger, Karina Grohmann-Held, Carmen Schroeder, Norbert Jorch, Elmar Schmid, Hagen Staude, Marcus Weitz, Clemens Freiberg, Angela Huebner, Anke Heitmeyer-Pyper, Giuseppina Sparta, Carl-Joachim Partsch, Michaela Marx, Christof Land, Inka Baus, Frauke Wilkening, Kristina Moeller, Gunter Simic-Schleicher, Susann Empting, Oliver Metzing, Verena Wagner, Martin Holder, Mislav Stjepan Žebec, Dirk Schnabel, Dieter Haffner, Miroslav Zivicnjak","doi":"10.1007/s00467-025-06841-y","DOIUrl":"10.1007/s00467-025-06841-y","url":null,"abstract":"<p><strong>Background: </strong>Children with X-linked hypophosphatemia (XLH) present with rickets, leg deformities, and growth failure. Bone stability depends on balanced bone growth in both length and width. Data on body proportions, including transverse body dimensions, in children with XLH treated with phosphate supplements and active vitamin D are lacking.</p><p><strong>Methods: </strong>Six major transverse body dimensions of the trunk and extremities, and the frame index (FI), i.e., ratio between bicondylar humerus diameter and height, were measured annually along with clinical characteristics in 109 pediatric patients with XLH, all on supplementation therapy, participating in a prospective multicenter observational study conducted since 1998. Associations between anthropometric and clinical parameters were investigated using linear mixed-effects models.</p><p><strong>Results: </strong>Children with XLH exhibited persistent hypophosphatemia and elevated alkaline phosphatase z scores despite supplementation treatment. This was associated with disproportionate transversal skeletal growth, which was most pronounced during adolescence (13-17 years). Bicondylar diameter z scores (tubular bone width) and FI progressively increased with age (each p < 0.05). In addition, FI was identified as a superior indicator of stunting when compared to other measures of transversal dimensionality across all age groups. In young children (2-6 years), transversal growth was most synchronized and associated most strongly with clinical characteristics.</p><p><strong>Conclusions: </strong>Our data show disproportionate growth in transversal body dimensions despite supplementation treatment in children with XLH, suggesting compensatory widening of tubular bones as adaptation for mineral loss caused by persisting rickets. The FI can be used as a general indicator of bone health in children with XLH in clinical practice and trials.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3187-3200"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12401769/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144258666","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}
引用次数: 0
Serum sclerostin as a marker of microvascular and macrovascular complications among children and adolescents with type 1 diabetes mellitus. 血清硬化蛋白作为1型糖尿病儿童和青少年微血管和大血管并发症的标志。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-05-12 DOI: 10.1007/s00467-025-06793-3
Dina E Sallam, Yasmine Ibrahim Mahmoud Elhenawy, Aya Mohamed Abdullah Ahmed, Sara Ibrahim Abdelfattah Taha, Eman Mohamed Elsayed
{"title":"Serum sclerostin as a marker of microvascular and macrovascular complications among children and adolescents with type 1 diabetes mellitus.","authors":"Dina E Sallam, Yasmine Ibrahim Mahmoud Elhenawy, Aya Mohamed Abdullah Ahmed, Sara Ibrahim Abdelfattah Taha, Eman Mohamed Elsayed","doi":"10.1007/s00467-025-06793-3","DOIUrl":"10.1007/s00467-025-06793-3","url":null,"abstract":"<p><strong>Background: </strong>Uncontrolled diabetes mellitus (DM) accelerates atherosclerosis and vascular diseases, leading to micro- and macrovascular complications. Early cardiac and kidney involvement necessitates an early biomarker. Sclerostin is a Wnt-signaling inhibitor, having a pathophysiological role in vasculopathy, and could be used as a vasculopathy marker. Nevertheless, few data are available in pediatric patients with type 1 diabetes mellitus (T1DM). We aimed at assessing its serum level, and relation to diabetic microvascular and macrovascular complications.</p><p><strong>Methods: </strong>This is a case control study on patients with T1DM, and healthy controls. Patients were divided into non-diabetic nephropathy (DN), and DN groups according to proteinuria. Patients' clinicodemographic and anthropometrics were obtained, with withdrawal of fasting serum lipid profile, kidney function test, and serum sclerostin. Carotid intimal media thickness (CIMT), a marker of subclinical atherosclerosis, was measured.</p><p><strong>Results: </strong>We had 75 comparable subjects, where median (IQR) serum sclerostin levels were significantly higher in DN, compared to non-DN and controls [90.83 (82.32 - 115.1), vs. 33.29 (28.37 - 38.53), vs. 13.5 (10.32 - 15.72) ng/mL,respectively, p, < 0.001]. Similarly, median (IQR) CIMT was significantly higher in DN, than in non-DN and controls [1.1 (0.8 - 1.3), vs. 0.11 (0.1 - 0.2), vs. 0.11 (0.1 - 0.2) mm, respectively, p < 0.001]. Serum sclerostin level correlated positively with disease duration, higher HgbA1c%, albuminuria level, and CIMT in all patients. The cut-off values of serum sclerostin > 60.0 ng/mL and CIMT > 0.3 mm were able to detect DN.</p><p><strong>Conclusions: </strong>Serum sclerostin levels may serve as a potential biomarker for microvascular and macrovascular complications in pediatric patients with T1DM.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3155-3162"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12402022/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144047434","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}
引用次数: 0
Current etiology of hypertension in European children - factors associated with primary hypertension. 欧洲儿童高血压的当前病因学——与原发性高血压相关的因素。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-05-20 DOI: 10.1007/s00467-025-06761-x
Łukasz Obrycki, Krzysztof Skoczyński, Maksymilian Sikorski, Jan Koziej, Kacper Mitoraj, Jakub Pilip, Michał Pac, Janusz Feber, Mieczysław Litwin
{"title":"Current etiology of hypertension in European children - factors associated with primary hypertension.","authors":"Łukasz Obrycki, Krzysztof Skoczyński, Maksymilian Sikorski, Jan Koziej, Kacper Mitoraj, Jakub Pilip, Michał Pac, Janusz Feber, Mieczysław Litwin","doi":"10.1007/s00467-025-06761-x","DOIUrl":"10.1007/s00467-025-06761-x","url":null,"abstract":"<p><strong>Background: </strong>While hypertension (HT) in pediatric patients is often secondary (SH), recent trends show a rise in primary hypertension (PH), which is associated with an increasing global prevalence of obesity. A relationship between serum uric acid and PH has also been suggested. Our study aimed to assess the etiology of HT and factors associated with PH in a large European cohort of children referred for HT based on office blood pressure (BP) measurements.</p><p><strong>Methods: </strong>We performed a retrospective analysis of 2008 children aged 0-18 years (12.3 ± 4.9 years) diagnosed with HT. Patients were classified into white coat hypertension (WCH), PH, or SH groups based on office BP, 24-h ambulatory BP monitoring (ABPM) and clinical evaluation. Anthropometric, hemodynamic, and biochemical data were collected.</p><p><strong>Results: </strong>Out of 2008 patients included in the analysis, 200 (10%) were excluded due to multifactorial HT diagnosis after kidney transplantation (KTx). Among the remaining patients HT was confirmed in 1260 (548 were classified as WCH). Of 1260 patients with HT: 49.3% had PH, while 50.7% SH, mainly secondary to renal parenchymal disease (43.5% of SH patients), aortic coarctation (20.7%), and renovascular HT (18%). Age > 12.5 years, obesity (BMI SDS (standard deviation score) ≥ 1.65), and serum uric acid > 4.8 mg/dL were identified as significant factors associated with PH.</p><p><strong>Conclusions: </strong>Our study provides valuable insights into the current etiology of pediatric HT and highlights the role of age, obesity, and uric acid level in the diagnosis of PH in children.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3233-3244"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12402006/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144111537","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}
引用次数: 0
Kidney pathological findings of MYH9-related disease: a cross-sectional nationwide survey in Japan. myh9相关疾病的肾脏病理表现:日本横断面全国调查
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-05-15 DOI: 10.1007/s00467-025-06802-5
Ryo Nakatani, Kenichiro Miura, Yoko Shirai, Yasufumi Ohtsuka, Yoko Ohwada, Takao Konomoto, Tamaki Morohashi, Koji Tsugawa, Sekiko Taneda, Kazuho Honda, Shinji Kunishima, Kenji Ishikura, Motoshi Hattori
{"title":"Kidney pathological findings of MYH9-related disease: a cross-sectional nationwide survey in Japan.","authors":"Ryo Nakatani, Kenichiro Miura, Yoko Shirai, Yasufumi Ohtsuka, Yoko Ohwada, Takao Konomoto, Tamaki Morohashi, Koji Tsugawa, Sekiko Taneda, Kazuho Honda, Shinji Kunishima, Kenji Ishikura, Motoshi Hattori","doi":"10.1007/s00467-025-06802-5","DOIUrl":"10.1007/s00467-025-06802-5","url":null,"abstract":"<p><strong>Background: </strong>MYH9-related disease (MYH9-RD) is characterized by macrothrombocytopenia, hearing loss, and progressive kidney dysfunction. Due to thrombocytopenia, kidney biopsy is seldom performed, and pathological findings remain unclear. Although case reports have described focal segmental glomerulosclerosis (FSGS) and glomerular basement membrane (GBM) abnormalities like Alport syndrome, no cross-sectional studies of MYH9-RD have been performed. This study aimed to clarify kidney pathological findings through a nationwide survey.</p><p><strong>Methods: </strong>We conducted a nationwide survey of MYH9-RD patients and collected tissues from kidney biopsies, along with immunofluorescence and electron microscopy images. Multiple pathologists examined all samples.</p><p><strong>Results: </strong>Nine kidney biopsy samples were included. Mesangial matrix expansion was observed in all samples (100%), while FSGS was observed in two (22%). Segmental foot process effacement was observed in all samples (100%), with irregularly aggregated podocyte dense material in seven (78%). Immunofluorescence analysis revealed that three samples (33%) had immunoglobulin and/or complement deposition: the types of depositions varied among patients. Electron dense deposits (EDD) were found in five samples (56%). GBM abnormalities - thinning, irregular thickening, and splitting of the lamina densa - were observed in five samples (56%), but no basket-weave appearance was noted. Endothelial cell swelling was found in seven samples (78%).</p><p><strong>Conclusions: </strong>Mesangial expansion and segmental foot process effacement were commonly observed in MYH9-RD patients. Additional findings included immunoglobulin and complement deposition with EDD, and GBM abnormalities. Although patients may have had relatively severe disease, which limits generalizability, these results provide valuable insights into the disease mechanisms and potential therapeutic targets of MYH9-RD.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3201-3209"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144078910","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}
引用次数: 0
Acute kidney injury with cast nephropathy following creatine loading in a 17-year-old. 17岁患者肌酸负荷后急性肾损伤伴铸型肾病。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-04-30 DOI: 10.1007/s00467-025-06784-4
Guido Filler, Eugene Maung, Maria Esther Díaz González de Ferris, Nancy Gain Chan, Ajay Parkash Sharma
{"title":"Acute kidney injury with cast nephropathy following creatine loading in a 17-year-old.","authors":"Guido Filler, Eugene Maung, Maria Esther Díaz González de Ferris, Nancy Gain Chan, Ajay Parkash Sharma","doi":"10.1007/s00467-025-06784-4","DOIUrl":"10.1007/s00467-025-06784-4","url":null,"abstract":"<p><p>This case highlights a 17-year-old male who developed acute kidney injury (AKI) with cast nephropathy following a 6-day high-dose creatine loading regimen. The patient presented with bilateral flank pain, a parallel rise in cystatin C and creatinine, and kidney enlargement on ultrasound despite adequate hydration and the absence of rhabdomyolysis markers. Renal biopsy confirmed cast nephropathy without evidence of light chain disease. This case underscores the potential risks of high-dose creatine use in adolescents and highlights the importance of kidney function monitoring in athletes using sports supplements.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3089-3092"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144026641","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}
引用次数: 0
Progression of acute kidney injury to chronic kidney disease: a prospective cohort study. 急性肾损伤进展为慢性肾脏疾病:一项前瞻性队列研究
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-05-21 DOI: 10.1007/s00467-025-06810-5
Shreyashi Karmakar, Deblina Dasgupta, Shakil Akhtar, Sanjukta Poddar, Prabhas Prasun Giri, Yincent Tse, Rajiv Sinha
{"title":"Progression of acute kidney injury to chronic kidney disease: a prospective cohort study.","authors":"Shreyashi Karmakar, Deblina Dasgupta, Shakil Akhtar, Sanjukta Poddar, Prabhas Prasun Giri, Yincent Tse, Rajiv Sinha","doi":"10.1007/s00467-025-06810-5","DOIUrl":"10.1007/s00467-025-06810-5","url":null,"abstract":"<p><strong>Background: </strong>Prospective studies on kidney outcomes in critically ill children with acute kidney injury (AKI) are scarce from low- and middle-income countries (LMIC). We conducted a pilot study to evaluate the continuum of transient AKI-persistent AKI-acute kidney disease (AKD) and chronic kidney disease (CKD).</p><p><strong>Methods: </strong>Children (1-18 years) admitted to our tertiary Pediatric Intensive Care Unit (PICU) and developing AKI with no known pre-existing kidney co-morbidities from January 2021 to June 2022 were included with follow up visits at 1 and 3 months after AKI onset. AKI and CKD were defined as per KDIGO 2012. At risk of CKD was defined by albuminuria, hypertension, estimated glomerular filtration rate (eGFR) 60-90 ml/kg/1.73 m<sup>2</sup> or hyperfiltration (eGFR ≥ 150 ml/kg/1.73 m<sup>2</sup>).</p><p><strong>Results: </strong>Of 390 children, 15% (n = 57) developed AKI. 75% (n = 43) with AKI had underlying primarily non-kidney systemic etiology. Fourteen (25%) died at median 5 days (IQR 4-7) after admission, and three were lost to follow up after discharge. For the 40 AKI survivors with three months data, incidence of transient AKI was 40% (n = 16), persistent AKI 20% (n = 8), AKD 32% (n = 13), and CKD 8% (n = 3). In addition, 18% (n = 7) were at risk of CKD. 38% with AKI for > 48 h vs. 6% with AKI < 48 h developed CKD or were at risk of CKD (p = 0.025). All three AKI survivors who progressed to CKD had an underlying primarily kidney etiology and progressed from AKD to CKD.</p><p><strong>Conclusions: </strong>In this LMIC study, kidney sequelae were high at 3 months among PICU AKI survivors. This pilot supports the need and feasibility of larger prospective trials in LMIC settings to understand outcomes for all children with AKI.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3293-3300"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144111550","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}
引用次数: 0
Efficacy of desmopressin and enuresis alarm in the treatment of monosymptomatic nocturnal enuresis: a multicenter prospective randomized controlled study. 去氨加压素和遗尿报警治疗单症状性夜间遗尿的疗效:一项多中心前瞻性随机对照研究。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-10-01 Epub Date: 2025-06-10 DOI: 10.1007/s00467-025-06840-z
Rongqun Zhai, Sida Shao, Lei Lv, Shuai Li, Yihe Wang, Yanping Zhang, Qingli Li, Yibo Wen, Jing Yang, Huiqing Zhang, Wei Lu, Chaoming Zhou, Guoxing Wu, Qingwei Wang, Jian Guo Wen
{"title":"Efficacy of desmopressin and enuresis alarm in the treatment of monosymptomatic nocturnal enuresis: a multicenter prospective randomized controlled study.","authors":"Rongqun Zhai, Sida Shao, Lei Lv, Shuai Li, Yihe Wang, Yanping Zhang, Qingli Li, Yibo Wen, Jing Yang, Huiqing Zhang, Wei Lu, Chaoming Zhou, Guoxing Wu, Qingwei Wang, Jian Guo Wen","doi":"10.1007/s00467-025-06840-z","DOIUrl":"10.1007/s00467-025-06840-z","url":null,"abstract":"<p><strong>Background: </strong>To compare the therapeutic effect of desmopressin (DDAVP) and enuresis alarm (EA) in treating primary monosymptomatic nocturnal enuresis (MNE) and identified prognostic factors.</p><p><strong>Methods: </strong>A total of 213 children (6-16 years) with MNE were randomized to the DDAVP or EA group at five hospitals in mainland China from January 2019 to December 2023. Comprehensive medical histories were collected, and voiding diaries were maintained for two consecutive weeks. All participants underwent 12-week follow-up evaluations, with therapeutic outcomes assessed at the endpoint. Children achieving complete response were monitored for relapse for an additional 3 months post-treatment.</p><p><strong>Results: </strong>After excluding 28 patients (16 lost to follow-up, 12 incomplete diaries), 185 completed the study (63.24% male, mean age 10.25 ± 2.36 years). The loss to follow-up rate in the EA group was higher than in the DDAVP group (11.71% vs. 2.94%, P < 0.05). Ninety-four children were treated with EA and 91 children with DDAVP. After 12 weeks, there was no significant difference in the therapeutic effect between the DDAVP and EA group (P > 0.05). Relapses occurred in 1/30 children in the EA group and 6/30 children in the DDAVP group (P < 0.05). Family history (OR = 2.37, 95%CI: 1.16-4.84), enuresis frequency > 4 times/week (OR = 2.30, 95%CI: 1.08-4.89), and reduced bladder capacity (OR = 2.29, 95%CI: 1.12-4.66) were negative prognostic factors.</p><p><strong>Conclusion: </strong>Both therapies showed comparable short-term efficacy, but EA exhibited superior durability with lower relapse. Family history, severity of enuresis, and reduced bladder capacity are negative prognostic factors for therapeutic effect.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3137-3144"},"PeriodicalIF":2.6,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144266898","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}
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