Mafalda Félix Cabral, Inês Martins, Miguel Pereira, Gonçalo Vale, Madalena Almeida Borges, Maria Soto-Maior Costa, Fernando Caeiro, Mário Góis, Helena Sousa, Telma Francisco, Gisela Neto, Margarida Abranches, Rute Baeta Baptista
{"title":"在IgA肾病儿童队列中,真实肾脏预后与诊断时和活检后预测预后的比较","authors":"Mafalda Félix Cabral, Inês Martins, Miguel Pereira, Gonçalo Vale, Madalena Almeida Borges, Maria Soto-Maior Costa, Fernando Caeiro, Mário Góis, Helena Sousa, Telma Francisco, Gisela Neto, Margarida Abranches, Rute Baeta Baptista","doi":"10.1590/2175-8239-JBN-2024-0260en","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis, with a variable clinical course. This study aimed to evaluate real-world kidney outcomes in children with IgAN and compare these with predictions from the International IgAN Prediction Tool for children (IIgAN-PT).</p><p><strong>Methods: </strong>A single-center, longitudinal retrospective study was conducted on pediatric patients diagnosed with IgAN from 2010 to 2022. Data on clinical, laboratory, and histological parameters were analyzed. The IIgAN-PT score was calculated for each patient at biopsy and one year after biopsy. The primary outcome was a composite endpoint of ≥30% eGFR decrease or progression to end stage kidney disease (ESKD).</p><p><strong>Results: </strong>Among 23 patients (57% male, median age at biopsy 13.8 years), MEST-C scores showed M1 in 87%, E1 in 22%, S1 in 39%, T1/2 in 13%, and C1 in 26%. During a median 3.1-year follow-up, 26% reached the primary outcome, while the median predicted risk based on IIgAN-PT was 6.5%. Additionally, 57% experienced eGFR decline (annual median decline of 5.6 mL/min/1.73 m2). Application of the updated IIgAN-PT one-year post-biopsy (n = 13) resulted in a median predicted risk of 1.79%, while 23% met the primary outcome.</p><p><strong>Conclusion: </strong>The observed eGFR decline or progression to ESKD was higher than predicted, highlighting the need for early diagnosis, monitoring, and treatment. Small-scale studies like ours underscore the importance of early intervention and may inform the design of larger studies to improve the predictive ability of the IIgAN-PT tool in diverse clinical settings.</p>","PeriodicalId":14724,"journal":{"name":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","volume":"47 3","pages":"e20240260"},"PeriodicalIF":1.3000,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12448116/pdf/","citationCount":"0","resultStr":"{\"title\":\"Comparison of real-world kidney outcomes with predicted outcomes at the time of diagnosis and post-biopsy in a cohort of children with IgA nephropathy.\",\"authors\":\"Mafalda Félix Cabral, Inês Martins, Miguel Pereira, Gonçalo Vale, Madalena Almeida Borges, Maria Soto-Maior Costa, Fernando Caeiro, Mário Góis, Helena Sousa, Telma Francisco, Gisela Neto, Margarida Abranches, Rute Baeta Baptista\",\"doi\":\"10.1590/2175-8239-JBN-2024-0260en\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis, with a variable clinical course. This study aimed to evaluate real-world kidney outcomes in children with IgAN and compare these with predictions from the International IgAN Prediction Tool for children (IIgAN-PT).</p><p><strong>Methods: </strong>A single-center, longitudinal retrospective study was conducted on pediatric patients diagnosed with IgAN from 2010 to 2022. Data on clinical, laboratory, and histological parameters were analyzed. The IIgAN-PT score was calculated for each patient at biopsy and one year after biopsy. The primary outcome was a composite endpoint of ≥30% eGFR decrease or progression to end stage kidney disease (ESKD).</p><p><strong>Results: </strong>Among 23 patients (57% male, median age at biopsy 13.8 years), MEST-C scores showed M1 in 87%, E1 in 22%, S1 in 39%, T1/2 in 13%, and C1 in 26%. During a median 3.1-year follow-up, 26% reached the primary outcome, while the median predicted risk based on IIgAN-PT was 6.5%. Additionally, 57% experienced eGFR decline (annual median decline of 5.6 mL/min/1.73 m2). Application of the updated IIgAN-PT one-year post-biopsy (n = 13) resulted in a median predicted risk of 1.79%, while 23% met the primary outcome.</p><p><strong>Conclusion: </strong>The observed eGFR decline or progression to ESKD was higher than predicted, highlighting the need for early diagnosis, monitoring, and treatment. Small-scale studies like ours underscore the importance of early intervention and may inform the design of larger studies to improve the predictive ability of the IIgAN-PT tool in diverse clinical settings.</p>\",\"PeriodicalId\":14724,\"journal\":{\"name\":\"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia\",\"volume\":\"47 3\",\"pages\":\"e20240260\"},\"PeriodicalIF\":1.3000,\"publicationDate\":\"2025-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12448116/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1590/2175-8239-JBN-2024-0260en\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"UROLOGY & NEPHROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1590/2175-8239-JBN-2024-0260en","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
Comparison of real-world kidney outcomes with predicted outcomes at the time of diagnosis and post-biopsy in a cohort of children with IgA nephropathy.
Introduction: IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis, with a variable clinical course. This study aimed to evaluate real-world kidney outcomes in children with IgAN and compare these with predictions from the International IgAN Prediction Tool for children (IIgAN-PT).
Methods: A single-center, longitudinal retrospective study was conducted on pediatric patients diagnosed with IgAN from 2010 to 2022. Data on clinical, laboratory, and histological parameters were analyzed. The IIgAN-PT score was calculated for each patient at biopsy and one year after biopsy. The primary outcome was a composite endpoint of ≥30% eGFR decrease or progression to end stage kidney disease (ESKD).
Results: Among 23 patients (57% male, median age at biopsy 13.8 years), MEST-C scores showed M1 in 87%, E1 in 22%, S1 in 39%, T1/2 in 13%, and C1 in 26%. During a median 3.1-year follow-up, 26% reached the primary outcome, while the median predicted risk based on IIgAN-PT was 6.5%. Additionally, 57% experienced eGFR decline (annual median decline of 5.6 mL/min/1.73 m2). Application of the updated IIgAN-PT one-year post-biopsy (n = 13) resulted in a median predicted risk of 1.79%, while 23% met the primary outcome.
Conclusion: The observed eGFR decline or progression to ESKD was higher than predicted, highlighting the need for early diagnosis, monitoring, and treatment. Small-scale studies like ours underscore the importance of early intervention and may inform the design of larger studies to improve the predictive ability of the IIgAN-PT tool in diverse clinical settings.