Tubulointerstitial damage is a common diabetic kidney disease (DKD) complication caused by diabetes mellitus. However, many patients may not present with albuminuria or impaired glomerular filtration rate (GFR), making it difficult to detect kidney dysfunction. Kidney injury molecule-1 (KIM-1) is an emerging biomarker of early DKD, reflecting tubular injury before glomerular dysfunction. We explored the association between KIM-1 and DKD in children and adolescents with type 1 diabetes (T1D). We included 10 studies with 842 participants published between 2016 and 2025. A literature search of MEDLINE/PubMed, Cochrane Library, and Scopus was conducted from inception through January 5, 2026. The mean difference with a 95% confidence interval (CI) was calculated, and a P-value of <0.05 was considered statistically significant. A quality assessment of the included studies was conducted using the Newcastle-Ottawa Scale. In the meta-analysis of four studies, the urinary KIM-1 (uKIM-1)/creatinine ratio was elevated before changes in estimated GFR (95% CI, 0.02–0.48; P=0.03). This review suggests that uKIM-1/creatinine ratio and uKIM-1 may act as promising tubular biomarkers associated with early kidney injury in pediatric patients with T1D. However, further data are needed to clarify their role in pediatric DKD.

Pediatric kidney disease has a relatively lower prevalence than do other pediatric conditions and has a notably different etiology from kidney diseases observed in adults. Furthermore, the pediatric population is unique in that they experience ongoing growth and development, distinguishing them from adult patients. Consequently, pediatric patients with kidney disease require more specialized and meticulous nutritional management than do adults. To address this need and promote optimal dietary practices for pediatric patients with kidney disease, pediatric nephrologists from the Korean Society of Pediatric Nephrology and nutritionists from the Korean Society of Clinical Nutrition have collaborated to establish nutritional guidelines specifically tailored to Korean dietary patterns. These guidelines offer detailed, nutrient-specific recommendations covering energy, protein, calcium, phosphorus, and potassium consumption while providing practical, culturally relevant guidance intended to support both pediatric patients and their caregivers.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder and primarily arises from mutations in either the polycystic kidney disease 1 (PKD1) or 2 (PKD2) gene. Although traditionally categorized as an adult-onset disease, cystogenesis often begins in utero, and structural changes may become evident during childhood. Although most pediatric patients with ADPKD remain asymptomatic, hypertension occurs in 20%–40% of cases, and albuminuria may also be present. Both findings represent early, treatable manifestations that can significantly influence long-term prognosis. Ultrasonography is the preferred diagnostic modality; however, genetic testing is indicated in very-early-onset or atypical cases. The cornerstone of disease management is rigorous blood pressure control using renin–angiotensin system inhibitors, along with lifestyle modifications such as sodium restriction and adequate hydration. Early diagnosis and intervention are essential for optimizing long-term clinical outcomes in pediatric patients with ADPKD. This study aimed to provide a comprehensive review of the genetics, pathophysiology, clinical manifestations, diagnosis, treatment, and future management of pediatric ADPKD.

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.

Purpose: Idiopathic nephrotic syndrome (NS) often necessitates prolonged or repeated corticosteroid treatment, which increases the risk for steroid-induced posterior subcapsular cataract (SI-PSC). Previous research on its burden remains limited. Here, we aimed to determine the prevalence and predictive risk factors of SI-PSC in children with NS. Methods: This cross-sectional study was conducted in the Pediatric Nephrology Department of The Kidney Centre, Karachi, Pakistan, from October 2023 to October 2025. The study included 150 children with NS, aged 2–16 years, who had received oral prednisolone for more than 6 months. Demographic and clinical information were collected through caregiver interviews and medical record review. All participants underwent a detailed slit-lamp examination by the same ophthalmologist. Associations between cataract formation and demographic or treatment-related variables were analyzed statistically. A P-value of ≤0.05 was considered significant. Results: Cataracts were detected in 23 children (15.3%). Most patients had early lenticular changes, but only two required surgical intervention. Other ocular abnormalities were uncommon (4.7%). Cataracts occurred more frequently in males and in children younger than 10 years. Cumulative corticosteroid doses were nearly identical in patients with and without cataracts. Children with frequently relapsing NS accounted for the highest proportion of those with SI-PSC. Systemic hypertension was the adverse effect most strongly associated with cataract development. Conclusions SI-PSC is a notable complication in children receiving long-term corticosteroid therapy. Our findings underscore the importance of routine ophthalmologic screening. Individual susceptibility, including potential genetic factors, might play a role in cataract development and warrant further investigation.

Cystinosis is a rare autosomal recessive lysosomal storage disorder with an incidence of approximately 1 in 100,000 to 200,000 live births. It is the most common cause of inherited pediatric Fanconi syndrome (FS). Here, we describe the cases of two infants from unrelated families who presented with polyuria and features of proximal renal tubular dysfunction. Although no corneal cystine deposition was observed at presentation, clinical suspicion and genetic analysis confirmed the diagnosis of nephropathic cystinosis. Both patients carried the same pathogenic variant in the CTNS gene, suggesting that it is a hotspot in this region. These patients were managed with oral cysteamine therapy, cysteamine eye drops, and supportive therapy for FS and are currently doing well. Genetic diagnosis plays a crucial role in the early detection of cystinosis, facilitating timely initiation of cysteamine therapy, and should be considered in infants with FS.

Urinary tract infections (UTIs) are among the most common bacterial infections in infants and a significant cause of antibiotic exposure. Recent advances in culture-independent sequencing and improved urine culture techniques have revealed that urine contains resident microbial communities, collectively known as the urinary microbiome (urobiome). Additionally, emerging evidence supports the existence of a gut-bladder axis, where intestinal dysbiosis may lead to uropathogen colonization, immune modulation, and UTI recurrence. This review summarizes current pediatric research connecting alterations in the urinary and gut microbiomes to UTI susceptibility, recurrence, and clinical outcomes, specifically in infants. We also address the unique methodological challenges in pediatric urobiome research and outline future directions for applying microbiome and metabolomics insights to clinical practice.

Anti-glomerular basement membrane disease (anti-GBMD) is a rare small-vessel vasculitis presenting as rapidly progressive glomerulonephritis and/or diffuse alveolar hemorrhage. A third of patients with anti-GBMD have antineutrophil cytoplasmic antibodies and are known as double-positive patients. There are a limited number of pediatric double-positive cases published in the literature. In this case report, we summarize the presentation, treatment, and outcome of a patient with anti-GBMD with both anti-myeloperoxidase and anti-GBM antibodies, highlighting the safe use of rituximab. We also describe atypical complications in our case, including significant weight loss of unclear etiology and development of recurrent episodes of acute pericalcific arthritis.