Childhood kidney diseases最新文献_第3页

Genome-based medicine in Korea: the Korea National Institute of Health infrastructure for precision medicine. 韩国基因组医学：韩国国立卫生研究院精准医学基础设施。

Childhood kidney diseases Pub Date : 2026-02-01 Epub Date: 2026-02-27 DOI: 10.3339/ckd.26.002

Hyun-Young Park

{"title":"Genome-based medicine in Korea: the Korea National Institute of Health infrastructure for precision medicine.","authors":"Hyun-Young Park","doi":"10.3339/ckd.26.002","DOIUrl":"10.3339/ckd.26.002","url":null,"abstract":"Since the completion of the Human Genome Project, genome-based medicine has progressed from a predominantly research-driven endeavor to a field of increasing clinical relevance. In Korea, the Korea National Institute of Health (KNIH) has played a central role in the establishment of the necessary research infrastructure that supports the secure and responsible use of genomic and clinical data. These efforts have enabled the generation of comprehensive genomic datasets representative of the Korean population and, together with the Korea Biobank Array optimized for population-specific variants, have strengthened discovery-driven research and accelerated advances in disease gene identification and risk prediction. More recently, KNIH has expanded analyses based on whole-genome sequencing data to support clinical translation, enabling more comprehensive variant detection and facilitating the application of genomic information to disease diagnosis and precision medicine research. These national genomic resources provide an important foundation for improving the diagnosis and management of genetically mediated conditions, including pediatric kidney diseases, where early etiologic diagnosis can substantially influence clinical decision-making and long-term outcomes. Further strengthening of institutional and regulatory frameworks will be essential to support routine clinical implementation and maximize the public health impact of genomics in Korea.","PeriodicalId":524218,"journal":{"name":"Childhood kidney diseases","volume":"30 1","pages":"15-23"},"PeriodicalIF":0.0,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13587174/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149033047","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Anti-glomerular basement membrane disease with concomitant myeloperoxidase antibodies in a pediatric patient: a case report of presentation, treatment and outcome a rare disease. 抗肾小球基底膜病伴髓过氧化物酶抗体的儿科患者：一个罕见疾病的表现、治疗和结果的病例报告

Childhood kidney diseases Pub Date : 2026-02-01 Epub Date: 2025-11-08 DOI: 10.3339/ckd.25.015

Yiressy Pina, Emily Frierson, John Hicks, Partha Chakraborty, Alvaro Orjuela, Amit Thakral

引用次数: 0

Follow-up of congenital abnormalities of the kidney and urinary tract from the antenatal period to the first year of life: a retrospective study from Turkey. 先天性肾脏和尿路异常的随访从产前期到生命的第一年：来自土耳其的回顾性研究。

Childhood kidney diseases Pub Date : 2025-10-01 Epub Date: 2025-10-31 DOI: 10.3339/ckd.25.027

Nazlı Idil Fil, Ozge Surmeli Onay, Melih Velipasaoglu, Aslı Kavaz Tufan, Nuran Cetin, Tugba Barsan Kaya, Ozge Aydemir

{"title":"Follow-up of congenital abnormalities of the kidney and urinary tract from the antenatal period to the first year of life: a retrospective study from Turkey.","authors":"Nazlı Idil Fil, Ozge Surmeli Onay, Melih Velipasaoglu, Aslı Kavaz Tufan, Nuran Cetin, Tugba Barsan Kaya, Ozge Aydemir","doi":"10.3339/ckd.25.027","DOIUrl":"10.3339/ckd.25.027","url":null,"abstract":"Purpose: This study aimed to evaluate the progression of renal function and clinical outcomes during the first year of life in infants with congenital abnormalities of the kidney and urinary tract (CAKUTs) detected via antenatal ultrasonography.Methods: This retrospective, observational cohort study included 55 infants, categorized into two groups: those with collecting system anomalies (CSAs) and those with renal parenchymal malformations (RPMs). The primary outcomes were the incidences of acute kidney injury (AKI) and chronic kidney disease (CKD) as well as the prognostic indicators linking the antenatal and postnatal periods.Results: CSAs were more common in both the antenatal and postnatal periods, with incidence rates of 54.5% and 56.4%, respectively. Hydronephrosis was the most frequently detected CAKUT type during the antenatal period (47.2%). Multicystic dysplastic kidney was the most predominant lesion in both antenatal and postnatal diagnoses within the RPM group, accounting for 38.2%. Although the incidence of AKI did not differ significantly between the two groups, CKD was more prevalent in the RPM group (P<0.05). Gestational age, presence of oligohydramnios, and several ultrasonographic findings (including loss of corticomedullary differentiation, bladder trabeculation, and cysts) were significantly associated with the development of AKI. In multivariate logistic regression analysis, loss of corticomedullary differentiation remained an independent predictor of AKI (odds ratio, 13.5; 95% confidence interval, 1.8-100.0; P=0.011).Conclusions: Loss of corticomedullary differentiation on postnatal ultrasound is an important predictor of AKI, highlighting the need to investigate its relevance during the prenatal period.","PeriodicalId":524218,"journal":{"name":"Childhood kidney diseases","volume":"29 3","pages":"125-135"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13587195/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032977","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Steroid-sensitive nephrotic syndrome in children: Korean Society of Pediatric Nephrology clinical practice recommendations. 儿童类固醇敏感性肾病综合征：韩国儿科肾病学会临床实践建议。

Childhood kidney diseases Pub Date : 2025-10-01 Epub Date: 2025-07-24 DOI: 10.3339/ckd.25.019

Hyun Kyung Lee, Myung Hyun Cho, Min Hyun Cho, Yeon Hee Lee, Jeong Yeon Kim, Joo Hoon Lee, Hee Gyung Kang, Eujin Park, Jin-Soon Suh, Dong Ah Park, Heeyeon Cho

{"title":"Steroid-sensitive nephrotic syndrome in children: Korean Society of Pediatric Nephrology clinical practice recommendations.","authors":"Hyun Kyung Lee, Myung Hyun Cho, Min Hyun Cho, Yeon Hee Lee, Jeong Yeon Kim, Joo Hoon Lee, Hee Gyung Kang, Eujin Park, Jin-Soon Suh, Dong Ah Park, Heeyeon Cho","doi":"10.3339/ckd.25.019","DOIUrl":"10.3339/ckd.25.019","url":null,"abstract":"Pediatric nephrotic syndrome is a clinical condition characterized by severe proteinuria, hypoalbuminemia, hyperlipidemia, and generalized edema, most commonly affecting children aged 2 and 6 years. Although standard oral steroid therapy is effective in managing most cases, relapses are common during childhood. Nephrotic syndrome presents significant challenges due to frequent relapses and the potential side effects of long-term drug therapy. In Korea, the absence of a unified national clinical guideline has led physicians to manage pediatric nephrotic syndrome based on diverse clinical guidelines and individual clinical judgment. To address this, the Korean Society of Pediatric Nephrology has developed an evidence-based guideline specifically tailored to the Korean healthcare system. This guideline provides recommendations for the diagnosis and management of steroid-sensitive nephrotic syndrome in children under the age of 18 years.","PeriodicalId":524218,"journal":{"name":"Childhood kidney diseases","volume":"29 3","pages":"92-103"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13587206/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Comparative diagnostic value of NGAL and multibiomarker models in pediatric sepsis-associated acute kidney injury in Indonesia: a preliminary study. NGAL和多生物标志物模型在印度尼西亚儿童败血症相关急性肾损伤中的比较诊断价值：一项初步研究

Childhood kidney diseases Pub Date : 2025-10-01 Epub Date: 2025-10-31 DOI: 10.3339/ckd.25.031

Fina Meilyana Andriyani, Jenifer Kiem Aviani, Ahmed Widiasta, Dzulfikar Djalil Luk Hakim, Dedi Rachmadi Sjambas

{"title":"Comparative diagnostic value of NGAL and multibiomarker models in pediatric sepsis-associated acute kidney injury in Indonesia: a preliminary study.","authors":"Fina Meilyana Andriyani, Jenifer Kiem Aviani, Ahmed Widiasta, Dzulfikar Djalil Luk Hakim, Dedi Rachmadi Sjambas","doi":"10.3339/ckd.25.031","DOIUrl":"10.3339/ckd.25.031","url":null,"abstract":"Purpose: Sepsis is a leading cause of pediatric critical illness and a major contributor to acute kidney injury (AKI). Sepsis-associated AKI (SA-AKI) worsens outcomes, yet current diagnostic criteria based on serum creatinine and urine output lack sensitivity for early detection. This study evaluated the diagnostic performance of neutrophil gelatinase-associated lipocalin (NGAL), angiopoietin-2, and syndecan-1 for early identification of SA-AKI in children with sepsis.Methods: A prospective nested case-control study was conducted in children aged 1-18 years with sepsis. Participants were classified into SA-AKI and non-SA-AKI groups using pediatric Risk, Injury, Failure, Loss, End-stage Renal Disease (pRIFLE) criteria based on estimated creatinine clearance index (eCCI). Blood and urine samples were collected at baseline, 12, 24, and 48 hours to measure NGAL, angiopoietin-2, and syndecan-1 levels by enzyme-linked immunosorbent assay. Diagnostic accuracy was assessed using receiver operating characteristic (ROC) analysis.Results: Of 23 children enrolled, eight (34.8%) developed SA-AKI. Baseline creatinine, eCCI, urinary NGAL, and mean corpuscular volume (MCV) differed significantly between groups, whereas angiopoietin-2 and syndecan-1 did not. ROC analysis showed that 24-hour eCCI (area under the curve [AUC]=1.000; sensitivity 100%; specificity 100%) and 12- to 24-hour urinary NGAL (AUC=0.900; sensitivity 87.5%; specificity 86.7%) were the strongest predictors. MCV had moderate accuracy (AUC=0.767). Urinary NGAL correlated negatively with eCCI (r=-0.67).Conclusions: Urinary NGAL measured within 12-24 hours is a reliable early biomarker for SA-AKI in pediatric sepsis, complementing eCCI and pRIFLE criteria to enhance early diagnosis and management.","PeriodicalId":524218,"journal":{"name":"Childhood kidney diseases","volume":"29 3","pages":"112-124"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13587207/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149032994","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Exploring complement activation as a therapeutic target in lupus nephritis. 探索补体活化作为狼疮性肾炎的治疗靶点。

Childhood kidney diseases Pub Date : 2025-10-01 Epub Date: 2025-10-27 DOI: 10.3339/ckd.25.029

Beom Jin Lim

引用次数: 0

Milky white serum in an infant with anasarca: a case report. 乳白色血清在婴儿无肌炎：1例报告。

Childhood kidney diseases Pub Date : 2025-10-01 Epub Date: 2025-10-29 DOI: 10.3339/ckd.25.026

Suchismita Saha, Menka Yadav, Anjali Tomar, Sanya Chopra, Abhijeet Saha

{"title":"Milky white serum in an infant with anasarca: a case report.","authors":"Suchismita Saha, Menka Yadav, Anjali Tomar, Sanya Chopra, Abhijeet Saha","doi":"10.3339/ckd.25.026","DOIUrl":"10.3339/ckd.25.026","url":null,"abstract":"Milky white serum in infancy is rare and usually reflects a metabolic disorder; renal causes are distinctly uncommon. We report a 7-month-old male infant who presented with generalized edema and strikingly lipemic serum. Examination revealed lipemia retinalis, while investigations showed extreme hypertriglyceridemia and nephrotic range proteinuria. Initial tests also suggested hyperproteinemia and hyperphosphatemia, but repeat measurements confirmed hypoalbuminemia. Whole exome sequencing identified compound heterozygous variants in the NPHP1 gene, comprising one pathogenic variant and one variant of uncertain significance. Although parental testing was not performed, the combined clinical and genetic findings supported a diagnosis of congenital nephrotic syndrome. Parental testing, though not performed, combined clinical and genetic findings supported a diagnosis of congenital nephrotic syndrome. This case highlights that, although rare, congenital nephrotic syndrome can present with severe dyslipidemia and milky serum in infancy, and underscores the need to recognize potential laboratory estimation errors in lipemic samples.","PeriodicalId":524218,"journal":{"name":"Childhood kidney diseases","volume":"29 3","pages":"136-141"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13587179/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149033018","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Clinical impacts and management of urinary tract infections caused by extended-spectrum beta-lactamase-producing Enterobacteriaceae in children: a narrative review. 儿童广谱β -内酰胺酶产肠杆菌科引起的尿路感染的临床影响和管理：叙述性回顾。

Childhood kidney diseases Pub Date : 2025-10-01 Epub Date: 2025-10-27 DOI: 10.3339/ckd.25.028

Jisun Park, Eun Young Joo, Ji-Eun Lee, Su Jin Kim

{"title":"Clinical impacts and management of urinary tract infections caused by extended-spectrum beta-lactamase-producing Enterobacteriaceae in children: a narrative review.","authors":"Jisun Park, Eun Young Joo, Ji-Eun Lee, Su Jin Kim","doi":"10.3339/ckd.25.028","DOIUrl":"10.3339/ckd.25.028","url":null,"abstract":"The incidence of pediatric urinary tract infections (UTIs) caused by extended-spectrum beta-lactamase-producing Enterobacteriaceae (ESBL-PE) has increased worldwide; however, optimal empirical treatment strategies remain controversial. This review addresses the prevalence, virulence factors, clinical impacts, and antibiotic management of UTIs caused by ESBL-PE in children. The prevalence of ESBL-PE among pediatric UTI isolates exceeds 20% in many regions, including Korea. No significant differences exist in the clinical manifestations between ESBL(+) UTIs and other UTIs. Despite in vitro resistance, third-generation cephalosporins frequently achieve therapeutic success because of their high urinary drug concentrations. Based on the patient's condition and risk factors, an initial empirical antibiotic should be selected, and early de-escalation and carbapenem-sparing strategies are essential to balance treatment efficacy and resistance prevention. Therefore, Korean guidelines for the treatment of pediatric UTIs are required.","PeriodicalId":524218,"journal":{"name":"Childhood kidney diseases","volume":"29 3","pages":"104-111"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13587220/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149033020","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Hypomagnesemia as a reversible complication of cyclosporine in pediatric nephrotic syndrome: a case report. 低镁血症是环孢素治疗儿童肾病综合征的可逆并发症：1例报告。

Childhood kidney diseases Pub Date : 2025-10-01 Epub Date: 2025-09-17 DOI: 10.3339/ckd.25.018

Quang Chi Ngo, Hung Do Tran, Ngoc Bao Thi Nguyen, Tham Hong Thi Nguyen, Trang Thuy Mai

{"title":"Hypomagnesemia as a reversible complication of cyclosporine in pediatric nephrotic syndrome: a case report.","authors":"Quang Chi Ngo, Hung Do Tran, Ngoc Bao Thi Nguyen, Tham Hong Thi Nguyen, Trang Thuy Mai","doi":"10.3339/ckd.25.018","DOIUrl":"10.3339/ckd.25.018","url":null,"abstract":"Calcineurin inhibitors remain a cornerstone in the treatment of steroid-resistant nephrotic syndrome (SRNS) in pediatric patients. Hypomagnesemia, a clinically relevant yet frequently neglected complication of calcineurin inhibitors like cyclosporine, may exacerbate hypokalemia and induce neuromuscular symptoms such as tetany, challenging patient management. However, serum magnesium is not routinely monitored in many clinical settings. A 46-month-old girl with SRNS had been receiving high-dose cyclosporine for over 1 year. She was admitted with vomiting, diarrhea, generalized edema, and hypovolemic shock. After stabilization, she developed persistent hypokalemia refractory to supplementation, despite normal renal function and basic electrolyte panel. On hospital day 10, carpal-type tetany prompted the first measurement of serum magnesium, revealing severe hypomagnesemia (0.14 mmol/L). Cyclosporine was temporarily withheld, and intravenous then oral magnesium was administered. Tetany resolved within 48 hours, and both serum magnesium and potassium levels normalized. This case highlights the risk of delayed recognition of symptomatic hypomagnesemia during prolonged cyclosporine therapy in children. Tetany was the first neurological manifestation of a preventable complication. Current guidelines lack clear recommendations regarding magnesium surveillance in pediatric SRNS receiving cyclosporin. This case underscores a critical gap in practice and calls for the integration of magnesium monitoring into standard care for this vulnerable population.","PeriodicalId":524218,"journal":{"name":"Childhood kidney diseases","volume":"29 3","pages":"142-148"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13587219/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149033024","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies. 非典型溶血性尿毒症综合征：病理生理、临床表现和治疗策略。

Childhood kidney diseases Pub Date : 2025-06-01 Epub Date: 2025-06-27 DOI: 10.3339/ckd.25.017

Ji Yeon Song

{"title":"Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies.","authors":"Ji Yeon Song","doi":"10.3339/ckd.25.017","DOIUrl":"10.3339/ckd.25.017","url":null,"abstract":"Atypical hemolytic uremic syndrome (aHUS) is a rare and potentially life-threatening thrombotic microangiopathy, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Without timely intervention, it may progress to end-stage kidney disease. The condition is primarily attributed to dysregulation of the alternative complement pathway, with up to 60% of patients exhibiting genetic mutations in complement regulatory proteins such as complement factor H, complement factor I, membrane cofactor protein, and thrombomodulin. The introduction of complement inhibitors, such as eculizumab and ravulizumab, has significantly improved clinical outcomes by reducing recurrence and preserving renal function. These advances have redefined treatment approaches, particularly in pediatric patients and those undergoing kidney transplantation. The prophylactic use of complement inhibitors is now recommended for transplant recipients with moderate to high genetic risk. This review aimed to examine the pathophysiology, clinical manifestations, and treatment strategies of aHUS. Early and targeted complement inhibition is essential for preventing irreversible kidney damage and optimizing outcomes, particularly in children and transplant recipients.","PeriodicalId":524218,"journal":{"name":"Childhood kidney diseases","volume":"29 2","pages":"52-59"},"PeriodicalIF":0.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13587204/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149033036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0