Pediatric Nephrology最新文献

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New prognostic markers for IgA nephropathy in children. 儿童IgA肾病的新预后指标。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-09-25 DOI: 10.1007/s00467-025-06956-2
Chiara Casuscelli, Elisa Longhitano, Giovanni Conti, Veronica Maressa, Silvia Di Carlo, Claudia Spinella, Luigi Peritore, Vincenzo Calabrese, Domenico Santoro
{"title":"New prognostic markers for IgA nephropathy in children.","authors":"Chiara Casuscelli, Elisa Longhitano, Giovanni Conti, Veronica Maressa, Silvia Di Carlo, Claudia Spinella, Luigi Peritore, Vincenzo Calabrese, Domenico Santoro","doi":"10.1007/s00467-025-06956-2","DOIUrl":"https://doi.org/10.1007/s00467-025-06956-2","url":null,"abstract":"<p><p>IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide, with significant implications for adults and children. The disease progresses variably, from asymptomatic hematuria to severe glomerulonephritis, and around 10-20% of children diagnosed in childhood develop stage 5 chronic kidney disease (CKD 5) within 20 years. Identifying reliable prognostic markers is crucial for early intervention and long-term management. The International IgAN Prediction Tool combines clinical, laboratory, and histological data and has been adapted for pediatric use. It is highly accurate in predicting CKD 5. Markers such as the Oxford MEST-C score, proteinuria, eGFR, and blood pressure assist in risk stratification. Emerging biomarkers, including the IgA/C3 ratio, galactose-deficient IgA1, soluble CD89, and urinary cytokines like IL-6 and TGF-β1, show potential for predicting disease progression. Although promising, further research is needed to validate these biomarkers in pediatric populations and refine predictive tools for IgAN progression. These advances will guide targeted therapies and improve long-term renal outcomes in children.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145138291","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
Correlation between polymorphisms of the aquaporin-1 gene and peritoneal function in children on chronic peritoneal dialysis. 慢性腹膜透析患儿水通道蛋白-1基因多态性与腹膜功能的相关性
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-09-25 DOI: 10.1007/s00467-025-06959-z
Jiani Yao, Chunyan Wang, Xiaoyan Fang, Jing Chen, Zhiqing Zhang, Jiaojiao Liu, Jialu Liu, Rufeng Dai, Xiaotian Chen, Yihui Zhai, Hong Xu, Qian Shen
{"title":"Correlation between polymorphisms of the aquaporin-1 gene and peritoneal function in children on chronic peritoneal dialysis.","authors":"Jiani Yao, Chunyan Wang, Xiaoyan Fang, Jing Chen, Zhiqing Zhang, Jiaojiao Liu, Jialu Liu, Rufeng Dai, Xiaotian Chen, Yihui Zhai, Hong Xu, Qian Shen","doi":"10.1007/s00467-025-06959-z","DOIUrl":"https://doi.org/10.1007/s00467-025-06959-z","url":null,"abstract":"<p><strong>Background: </strong>Aquaporins (AQPs) are a class of proteins that transport water molecules across membranes, which can promote water transport in cells. We aimed to explore the correlation between different polymorphisms of AQP1 and peritoneal function in children on peritoneal dialysis (PD).</p><p><strong>Methods: </strong>Children who underwent PD at the Children's Hospital of Fudan University from January 1, 2014, to December 31, 2023, were included. The AQP1 genotypes of the four polymorphisms were rs2075574 (TT, CT, CC), rs1049305 (GG, CG, CC), rs10253374 (TT, CT, CC) and rs17159702 (TT, CT, CC).</p><p><strong>Results: </strong>A total of 187 children on chronic PD were included in the study. We found that the TT group with rs2075574 exhibited a lower baseline peritoneal equilibration test (PET) ultrafiltration level than the CC group (302 ± 129 vs. 408 ± 168 ml/m<sup>2</sup>, P = 0.015). For rs1049305, the CC group had a higher pKT/V than both the GG (2.71 ± 1.25 vs. 2.27 ± 0.79, P = 0.04) and CG groups (2.71 ± 1.25 vs. 2.24 ± 0.88, P = 0.03). Additionally, at 12-month follow-up, the CC (410 ± 160 ml/m<sup>2</sup>, P = 0.04) and CG (393 ± 174 ml/m<sup>2</sup>, P = 0.04) groups of rs1049305 showed higher PET ultrafiltration than the GG group (239 ± 288 ml/m<sup>2</sup>). No significant correlation was observed between the four genotypes and adverse events.</p><p><strong>Conclusions: </strong>AQP1 rs2075574 and rs1049305 polymorphisms might be associated with ultrafiltration and urea transport in children with PD.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145150533","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
Early fluid status and severe intraventricular hemorrhage or death in extremely preterm infants. 极早产儿早期液体状态与严重脑室内出血或死亡
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-09-24 DOI: 10.1007/s00467-025-06962-4
Lucinda J Weaver, Samuel J Gentle, Arie Nakhmani, Fazlur Rahman, Namasivayam Ambalavanan, Vivek V Shukla, Christine Stoops, David Askenazi, Colm P Travers
{"title":"Early fluid status and severe intraventricular hemorrhage or death in extremely preterm infants.","authors":"Lucinda J Weaver, Samuel J Gentle, Arie Nakhmani, Fazlur Rahman, Namasivayam Ambalavanan, Vivek V Shukla, Christine Stoops, David Askenazi, Colm P Travers","doi":"10.1007/s00467-025-06962-4","DOIUrl":"https://doi.org/10.1007/s00467-025-06962-4","url":null,"abstract":"<p><strong>Background: </strong>Measures of early postnatal fluid balance may be associated with severe intraventricular hemorrhage (sIVH) and/or death in extremely preterm infants in the first postnatal week.</p><p><strong>Methods: </strong>A single-center, retrospective cohort study including actively treated inborn infants weighing ≥ 400 g and 22-27 weeks' gestation from 2014-2021. Longitudinal mixed effect models compared daily fluid balance covariates including serum sodium, percent weight change, total fluid intake, urine output, and fluid balance (daily weight - birth weight /birth weight × 100) among infants with and without sIVH or death, during the first seven postnatal days. Multiple regression and machine learning models were developed to predict sIVH and/or death. Variables that were incorporated into the models included measures of fluid balance, gestational age, birth weight, antenatal corticosteroids, multiples, and sex.</p><p><strong>Results: </strong>We included 932 infants with mean ± SD gestational age of 25w2d ± 11d and birth weight of 746 ± 212 g of whom 195 (20.9%) had sIVH and/or death. Lower percentage weight change (p < 0.001), higher total fluid intake (p = 0.007), higher sodium (p = 0.007), and positive early fluid balance (p < 0.001) were associated with sIVH and/or death even after adjustment for baseline characteristics. The area under the receiver-operating curve (AUC) for regression models predicting sIVH and/or death incorporating baseline characteristics improved after adding fluid balance measures from 0.75 to 0.80, while the AUC for machine learning models improved from 0.72 to 0.84.</p><p><strong>Conclusions: </strong>In extremely preterm infants, early fluid status measures were associated with risk of sIVH and/or death. The addition of fluid status measures improves the performance of models predicting sIVH and/or death.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145131615","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
A 1-year-old boy with MIRAGE syndrome and nephrotic syndrome, whose kidney histopathology revealed membranous nephropathy-like findings: a case report. 1岁男童伴有MIRAGE综合征和肾病综合征,其肾脏组织病理学显示膜性肾病样表现:1例报告。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-09-23 DOI: 10.1007/s00467-025-06971-3
Shingo Ishimori, Aya Imaide, Tomoyuki Yokota, Satoshi Narumi, Norishige Yoshikawa
{"title":"A 1-year-old boy with MIRAGE syndrome and nephrotic syndrome, whose kidney histopathology revealed membranous nephropathy-like findings: a case report.","authors":"Shingo Ishimori, Aya Imaide, Tomoyuki Yokota, Satoshi Narumi, Norishige Yoshikawa","doi":"10.1007/s00467-025-06971-3","DOIUrl":"https://doi.org/10.1007/s00467-025-06971-3","url":null,"abstract":"<p><p>MIRAGE syndrome is a rare multisystem disorder caused by gain-on-function SAMD9 variants. Kidney biopsies in some MIRAGE syndrome patients have shown glomerular sclerosis or interstitial nephritis. A boy with genetically confirmed MIRAGE syndrome, who showed microhematuria and nephrotic range proteinuria, underwent kidney biopsy at 18 months, revealing diffuse mesangial proliferation and partial segmental lobular accentuation associated with mesangial cell proliferation with neither crescentic lesions nor sclerotic glomeruli. Immunofluorescence staining showed diffuse granular deposition of IgG along the glomerular basement membrane and dominant deposition of IgG in the mesangial matrix. Electron microscopy revealed diffuse subepithelial electron-dense deposits. Treatment with prednisolone for membranous nephropathy-like findings was initiated but the proteinuria persisted. At 21 months of age, the patient died of multi-organ failure as a result of severe gastrointestinal infection and necrotizing enterocolitis. This is the first case with histologically membranous nephropathy-like findings and marked mesangial proliferation in a child with MIRAGE syndrome with nephrotic syndrome.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145125672","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
How can we make novel treatments of glomerular diseases available for children as early and safely as possible? 我们怎样才能使儿童肾小球疾病的新治疗方法尽可能早和安全?
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-09-22 DOI: 10.1007/s00467-025-06963-3
Kjell Tullus
{"title":"How can we make novel treatments of glomerular diseases available for children as early and safely as possible?","authors":"Kjell Tullus","doi":"10.1007/s00467-025-06963-3","DOIUrl":"https://doi.org/10.1007/s00467-025-06963-3","url":null,"abstract":"","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145125555","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
Comparison of different equations for estimating the glomerular filtration rate in pediatric kidney transplant recipients. 估算儿童肾移植受者肾小球滤过率的不同公式的比较。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-09-22 DOI: 10.1007/s00467-025-06942-8
Paphawadee Sukboonthong, Julaporn Pooliam, Maturin Jantongsree, Achra Sumboonnanonda, Anirut Pattaragarn, Suroj Supavekin, Nuntawan Piyaphanee, Kraisoon Lomjansook, Yarnarin Thunsiribuddhichai, Intraparch Tinnabut, Nuttiporn Khueankong, Thanaporn Chaiyapak
{"title":"Comparison of different equations for estimating the glomerular filtration rate in pediatric kidney transplant recipients.","authors":"Paphawadee Sukboonthong, Julaporn Pooliam, Maturin Jantongsree, Achra Sumboonnanonda, Anirut Pattaragarn, Suroj Supavekin, Nuntawan Piyaphanee, Kraisoon Lomjansook, Yarnarin Thunsiribuddhichai, Intraparch Tinnabut, Nuttiporn Khueankong, Thanaporn Chaiyapak","doi":"10.1007/s00467-025-06942-8","DOIUrl":"https://doi.org/10.1007/s00467-025-06942-8","url":null,"abstract":"<p><strong>Background: </strong>Accurate glomerular filtration rate estimation (eGFR) is essential for managing pediatric kidney transplant recipients. Given the physiology of pediatric patients receiving adult-donor kidneys, identifying the most appropriate plasma creatinine (PCr)-based formula-pediatric or adult-specific-is crucial.</p><p><strong>Methods: </strong>This cross-sectional study included pediatric kidney transplant recipients (age 1-18 years) who received adult-donor kidneys. We compared agreement thresholds of various pediatric and adult PCr-based GFR equations with CKiD 2012 combined PCr‒cystatin C (PCr-CystC) equation via intraclass correlation coefficients (ICCs), concordance correlation coefficients (CCCs), total deviation index (TDI), P30 performance metric (P30), Bland-Altman plots, and receiver-operating characteristic (ROC) analysis. Correlation between CKiD under 25 (U25) PCr-CystC and reference CKiD 2012 equation was also evaluated.</p><p><strong>Results: </strong>One hundred twenty samples were collected from 23 recipients (mean age = 14.2 ± 3.4 years) and donors (mean age = 31.7 ± 10.0 years). Schwartz-Lyon equation demonstrated the highest performance with the reference (ICC = 0.913, CCC = 0.911, TDI = 14.0 mL/min/1.73 m<sup>2</sup>, P30 = 99.2%). U25 (ICC = 0.922, CCC = 0.882, P30 = 93.3%), full age spectrum (FAS)-height (ICC = 0.897, CCC = 0.877, P30 = 96.7%), and Bedside Schwartz equations (ICC = 0.850, CCC = 0.819, P30 = 89.2%) showed comparable performance. Bland-Altman plots revealed proportional bias (p < 0.05), leading to ROC analysis, which identified eGFR < 70 mL/min/1.73 m<sup>2</sup> for Schwartz-Lyon, U25, and FAS-height, and < 60 mL/min/1.73 m<sup>2</sup> for Bedside Schwartz as optimal agreement thresholds, beyond which each equation showed increased bias. Subgroup analyses also showed better performance in patients aged 10-18 years. Additionally, U25 PCr-CystC equation showed excellent agreement with the reference (ICC = 0.993, CCC = 0.990, P30 = 100%).</p><p><strong>Conclusions: </strong>Schwartz-Lyon equation demonstrated the highest performance among PCr-based equations with the reference in pediatric kidney transplant recipients, particularly when eGFR was < 70 mL/min/1.73 m<sup>2</sup> and in patients aged 10-18 years. U25 PCr-CystC equation showed best overall agreement with the reference and should be preferred where CystC measurement is feasible.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145125474","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
Adequacy and safety of pediatric native kidney biopsy using 16- and 18-gauge needles. 使用16号和18号针头进行小儿原生肾活检的充分性和安全性。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-09-22 DOI: 10.1007/s00467-025-06973-1
Petr Ananin, Anastasiia Milovanova, Kirill Kulikov, Ekaterina Stolyarevich, Alexey Tsygin
{"title":"Adequacy and safety of pediatric native kidney biopsy using 16- and 18-gauge needles.","authors":"Petr Ananin, Anastasiia Milovanova, Kirill Kulikov, Ekaterina Stolyarevich, Alexey Tsygin","doi":"10.1007/s00467-025-06973-1","DOIUrl":"https://doi.org/10.1007/s00467-025-06973-1","url":null,"abstract":"<p><strong>Background: </strong>The native kidney biopsy is an important diagnostic procedure in pediatric nephrology. Recent meta-analyses did not find the size of the needle as a risk factor for bleeding complications, but they were predominantly based on adult studies. There are few papers comparing the safety and core adequacy in pediatric native kidney biopsy.</p><p><strong>Methods: </strong>We present a large single-center retrospective study performed in a tertiary pediatric nephrology center. Data of children who received a real-time ultrasound-guided native kidney biopsy with a 16- or an 18-gauge needle from 2018 to 2024 were analyzed.</p><p><strong>Results: </strong>Overall, 1040 children (644 boys) were included, with a median age of 10.25 (6.6; 14.23) years. One hundred three (9.9%) patients experienced bleeding complications. Perinephric hematoma was reported in 86 (8.3%) cases, gross hematuria in 18 (1.7%), and 3 (0.3%) children required transfusion. Multivariate regression analysis revealed the needle size (OR for 16-gauge 2.06, 95% CI 1.22-3.47, p = 0.007) as a risk factor for complications in the overall cohort and in children under 12 years old. The needle size did not affect complication rates in children aged 12-18 years. Inadequate kidney cores were reported in 37 (4.5%) cases; OR for 18-gauge needles (OR 5.08, 95% CI 1.07-24.21, p = 0.041) was found.</p><p><strong>Conclusions: </strong>Use of a 16-gauge needle reduces the risk of obtaining an inadequate core in comparison with an 18-gauge. An 18G needle has a safety advantage over a 16G needle in children younger than 12 years. A 16G needle is as safe as an 18G needle and should be used for native kidney biopsy in children older than 12 years.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145125714","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
Health-related quality of life, mental health and caregiver burden in children with autosomal recessive polycystic kidney disease. 常染色体隐性多囊肾病患儿的健康相关生活质量、心理健康和照顾者负担
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-09-18 DOI: 10.1007/s00467-025-06795-1
Charlotte Gimpel, Susanne Schaefer, Franz Schaefer
{"title":"Health-related quality of life, mental health and caregiver burden in children with autosomal recessive polycystic kidney disease.","authors":"Charlotte Gimpel, Susanne Schaefer, Franz Schaefer","doi":"10.1007/s00467-025-06795-1","DOIUrl":"https://doi.org/10.1007/s00467-025-06795-1","url":null,"abstract":"<p><strong>Background: </strong>Pediatric chronic kidney disease (CKD) causes significantly impaired health-related quality of life (hrQOL) and caregiver burden, but no studies focus specifically on autosomal recessive polycystic kidney disease (ARPKD).</p><p><strong>Methods: </strong>This prospective case-control study assessed hrQOL (using PedsQL®ESRD) and screened for psychosocial problems (strength and difficulties questionnaire (SDQ)) in 43 children with ARPKD. Fifty-eight caregivers reported on the disease's impact on family (FaBel) and their own QOL (Ulm inventory of parental caregiver QOL (ULQIE)). As controls, we questioned 36 matched healthy children and 57 parents under similar pandemic restrictions and used published historical controls (healthy and with advanced CKD).</p><p><strong>Results: </strong>Patients were aged 9.0 ± 4.8 years with CKD stage G1-4 (45%), on dialysis (14%) or after kidney transplantation (26%). Nine patients had developmental delay secondary to medical complications. PedsQL®ESRD total scores correlated significantly to kidney function, but could not capture liver-specific symptoms. All 4 measures showed significant differences between treatment modalities with best scores in patients during CKD stages G1-4 and worst on dialysis, except SDQ, which was worst after transplantation. The most significant extra-renal risk factor for all 4 scores was developmental delay of the child. SDQ scores were elevated in contemporary vs. historical controls, but even further in ARPKD especially for peer relationship problems.</p><p><strong>Conclusion: </strong>In summary, ARPKD causes significantly impaired hrQOL, psychosocial problems and caregiver burden, which were equal to, if not greater than, that of controls with more advanced kidney failure. Treatment modality and developmental delay were the most important risk factors.</p><p><strong>Trial registration: </strong>Trial registered 06/2020 DRKS S00021059.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145081216","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Macrophage migration inhibitory factor and angiopoietin-like protein 4 as markers for steroid response in children with idiopathic nephrotic syndrome. 巨噬细胞迁移抑制因子和血管生成素样蛋白4作为特发性肾病综合征儿童类固醇反应的标志物
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-09-18 DOI: 10.1007/s00467-025-06966-0
Hanaa Al Dash, Heba Mostafa Ahmed, Shireen Ragab Shihatah, Noha Khalifa Abdelghaffar, Mona Gamal Mostafa, Sherin Khamis Hussein
{"title":"Macrophage migration inhibitory factor and angiopoietin-like protein 4 as markers for steroid response in children with idiopathic nephrotic syndrome.","authors":"Hanaa Al Dash, Heba Mostafa Ahmed, Shireen Ragab Shihatah, Noha Khalifa Abdelghaffar, Mona Gamal Mostafa, Sherin Khamis Hussein","doi":"10.1007/s00467-025-06966-0","DOIUrl":"https://doi.org/10.1007/s00467-025-06966-0","url":null,"abstract":"<p><strong>Background: </strong>Idiopathic nephrotic syndrome (INS) is a significant kidney disorder in pediatrics. Early diagnosis of minimal change disease (MCD) is difficult in children with nephrotic syndrome (NS). Angiopoietin-like protein 4 (ANGPTL4), found on the surface of podocytes, has been linked to nephrotic syndrome (NS) and plays a role in triggering proteinuria. Macrophage migration inhibitory factor (MIF) functions as a crucial modulator of the innate immune system and partly counteracts glucocorticoid-induced immune system inhibition. This study aimed to assess the role of ANGPTL4 and MIF as biomarkers in steroid responsiveness of INS.</p><p><strong>Methods: </strong>This cross-sectional comparative study involved 70 children with NS and 40 healthy children as a control group.</p><p><strong>Results: </strong>Urinary MIF/creatinine levels were significantly elevated in steroid-resistant nephrotic syndrome (SRNS) relative to in steroid-sensitive nephrotic syndrome (SSNS) and controls (p < 0.001). However, ANGPTL4 levels were significantly elevated in the SSNS group relative to the SRNS and control groups (p < 0.001). Regarding plasma MIF and urinary MIF/creatinine levels, there were no significant differences between MCD and FSGS, whereas ANGPTL4 levels were significantly elevated in MCD relative to FSGS (p < 0.001).</p><p><strong>Conclusions: </strong>Elevated levels of serum and urinary MIF levels were consistent with SRNS. Furthermore, ANGPTL4 was found to be highly upregulated in SSNS, unlike SRNS, which serves as a potential marker to distinguish between these two diseases.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145086852","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 the estimated glomerular filtration rate in asphyxiated neonates undergoing therapeutic hypothermia during the first 10 days of life. 在生命最初10天内接受治疗性低温治疗的窒息新生儿估计肾小球滤过率的进展。
IF 2.6 3区 医学
Pediatric Nephrology Pub Date : 2025-09-18 DOI: 10.1007/s00467-025-06957-1
Karel Allegaert, Julia Macente, Djalila Mekahli, John van den Anker, Pieter Annaert, Anne Smits
{"title":"Progression of the estimated glomerular filtration rate in asphyxiated neonates undergoing therapeutic hypothermia during the first 10 days of life.","authors":"Karel Allegaert, Julia Macente, Djalila Mekahli, John van den Anker, Pieter Annaert, Anne Smits","doi":"10.1007/s00467-025-06957-1","DOIUrl":"https://doi.org/10.1007/s00467-025-06957-1","url":null,"abstract":"<p><strong>Background: </strong>Serum creatinine (Scr) centile values were recently described in a cohort of 1136 (near)-term neonates that underwent therapeutic hypothermia (TH) because of moderate to severe hypoxic-ischemic encephalopathy. Recent methodological progress enables conversion of these Scr centiles to estimated glomerular filtration rate (eGFR) values.</p><p><strong>Methods: </strong>Scr centiles in the TH dataset during the first 10 days of life were converted to eGFR values, using the Schwartz formula, with the Smeets k-value (0.31) and fixed body length (50 cm) to generate postnatal reference eGFR values, centiles, and an equation for median eGFRs. These findings were compared to published eGFR data in term controls.</p><p><strong>Results: </strong>A polynomial function was estimated: <math><mrow><mi>eGFR</mi> <mfenced><mfrac><mi>mL</mi> <mi>min</mi></mfrac> <mo>∙</mo> <mn>1.73</mn> <msup><mrow><mi>m</mi></mrow> <mn>2</mn></msup> </mfenced> <mo>=</mo> <mn>9.1667</mn> <mo>+</mo> <mn>7.1173</mn> <mo>-</mo> <mn>0.3439</mn> <msup><mrow><mi>x</mi></mrow> <mn>2</mn></msup> <mo>,</mo> <mrow><mo>(</mo> <mi>x</mi> <mo>=</mo> <mi>days</mi> <mo>)</mo></mrow> </mrow> </math> for eGFR in TH neonates. The median eGFR increases 2- to threefold over the first week (day 1: 16.1; day 2: 19.4; day 7: 41.2 mL/min∙1.73 m<sup>2</sup>), while the polynomial function does not fully reflect the interindividual variability in eGFR values (intra-day variability is also 2- to threefold). Patterns in acute kidney injury (AKI) TH cases differ significantly from non-AKI TH cases. Based on pooling of published eGFR data, this was compared to a function in healthy term neonates: <math><mrow><mi>eGFR</mi> <mfenced><mfrac><mi>mL</mi> <mi>min</mi></mfrac> <mo>∙</mo> <mn>1.73</mn> <msup><mrow><mi>m</mi></mrow> <mn>2</mn></msup> </mfenced> <mo>=</mo> <mn>14.2167</mn> <mo>+</mo> <mn>6.7644</mn> <mo>-</mo> <mn>0.3901</mn> <msup><mrow><mi>x</mi></mrow> <mn>2</mn></msup> <mrow><mo>(</mo> <mi>x</mi> <mo>=</mo> <mi>days</mi> <mo>)</mo></mrow> </mrow> </math> (day 1: 20; day 2: 26; day 7: 42 mL/min/1.73 m<sup>2</sup>).</p><p><strong>Conclusions: </strong>Based on a pooled dataset in TH cases, we converted Scr centiles to eGFR centiles. Based on median values, this resulted in a polynomial function in TH cases, compared to healthy term neonates. This eGFR function enables precision pharmacotherapy for GFR-cleared drugs in this vulnerable population.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":""},"PeriodicalIF":2.6,"publicationDate":"2025-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145086795","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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