CONCLUSION: Rituximab offers reasonable efficacy in young children with FRSDNS, with a trend toward a shorter relapse-free period and more potential complications. Rituximab should be reserved until established treatments are exhausted.

INTRODUCTION: There is an unmet clinical need for the development of novel treatment strategies to improve the outcome of children with frequent relapsing or steroid-dependent nephrotic syndrome. Obinutuzumab (OBI) is a second-generation anti-CD20 monoclonal antibody that has demonstrated its superiority to rituximab (RTX) in vitro and in vivo. Our assumption is that a single infusion of low-dose OBI will induce longer B-cell depletion, longer sustained remission and reduce the frequency of...

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CONCLUSIONS: Systematic association of IgIA + ECZ is not supported for all neurological STEC-HUS pediatric patients; potential rescue therapy for severe cases warrants consideration.

CONCLUSIONS: Eculizumab is effective and safe in inducing and maintaining remission in aHUS secondary to anti-FH antibodies and renders reduction of anti-FH titers less urgent. Anti-FH antibody titers decreased in most patients irrespective of the immunosuppressive treatment chosen, so that a strategy consisting of combining eculizumab with MMF monotherapy seems sufficient at least in non-Indian or less severe forms of anti-FH antibody-associated HUS.

CONCLUSION: cIgAN with minimal proteinuria at time of biopsy might be linked with acute and chronic glomerular lesions.

CONCLUSION: A obinutuzumab and daratumumab combination seems to be a promising strategy in post-transplantation SRNS recurrence without response to standard treatment options.

Pediatric steroid-sensitive nephrotic syndrome (pSSNS) is the most common childhood glomerular disease. Previous genome-wide association studies (GWAS) identified a risk locus in the HLA Class II region and three additional independent risk loci. But the genetic architecture of pSSNS, and its genetically driven pathobiology, is largely unknown. Here, we conduct a multi-population GWAS meta-analysis in 38,463 participants (2440 cases). We then conduct conditional analyses and population specific...

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CONCLUSIONS: This study shows that ARA are frequent in children with FR/SDNS and that close immuno- and pharmacological monitoring may help personalizing rituximab treatment in patients needing repeated injections.

CONCLUSION: Global antiB cell strategy combining obinutuzumab and daratumumab induces prolonged peripheral B cell depletion and remission in children with difficult-to-treat SDNS.

Rituximab is an effective treatment for steroid-dependent/ frequently-relapsing nephrotic syndrome (SDFRNS) in children. However, the optimal rituximab regimen remains unknown. To help determine this we conducted an international, multicenter retrospective study at 11 tertiary pediatric nephrology centers in Asia, Europe and North America of children 1-18 years of age with complicated SDFRNS receiving rituximab between 2005-2016 for 18 or more months follow-up. The effect of rituximab prescribed...

CONCLUSIONS: This study confirms that NSAIDs reduce urine wasting of sodium and calcium in patients with BS. Monitoring serum renin levels may be useful to identify the lowest effective dose of NSAIDs that optimizes reduction of urine electrolyte losses.

CONCLUSIONS: The HR genotype is frequent in FSGS patients with African ancestry in our cohort, especially in those originating from the West Indies, and confer a poor renal prognosis. It is usually not associated with other causative mutations in monogenic SRNS genes.

CONCLUSIONS: High PWV and increased cIMT indicating arterial stiffness and hypertrophic vasculopathy may be present in children with ADPKD regardless BP status, and prior to GFR decline, suggesting that vascular disease precedes chronic kidney disease in ADPKD.

CONCLUSION: This case illustrates the interpretative challenges posed by VUS in complex glomerular disease and underscores the need for disciplined variant classification, careful gene-disease validation, and rigorous clinical-genetic correlation to avoid misclassification and inappropriate management decisions in SRNS.

Chronic kidney disease (CKD) in childhood, although uncommon, has profound lifelong consequences. Because disease onset occurs early, even modest slowing of CKD progression may translate into decades free from dialysis, transplantation, and premature death. Progressive proteinuria is a central driver of nephron loss in pediatric CKD, making early and sustained antiproteinuric strategies particularly impactful. Despite heterogeneous etiologies, including congenital and immune-mediated kidney...

Hereditary thrombotic thrombocytopenic purpura (TTP) is a rare autosomal recessive disorder caused by ADAMTS13 deficiency, typically presenting in childhood or early adulthood. We describe an atypical presentation of hereditary TTP in a 55-year-old man presenting with acute kidney injury on a background of diabetic kidney disease, without prior suggestive history or identifiable triggers. Initial evaluation revealed features of thrombotic microangiopathy, including haemolysis, thrombocytopenia,...

Persistent microscopic hematuria in children is often considered benign, yet recent evidence shows that a substantial proportion of affected individuals have underlying glomerular disease, particularly collagen IV-related nephropathies. We report a case of autosomal dominant Alport syndrome (ADAS) diagnosed just before discontinuation of long-term follow-up in a young woman initially presumed to have benign familial hematuria. The proband had persistent microscopic hematuria from early...

Cystinosis is a rare autosomal recessive lysosomal storage disorder caused by pathogenic variants in CTNS, which encodes cystinosin, a H^(+)/cystine symporter that mediates cystine efflux from lysosomes. Defective cystinosin leads to accumulation of cystine in lysosomes and the formation of cystine crystals in most tissues. In its more severe and frequent form, infantile nephropathic cystinosis, patients present with renal Fanconi syndrome in the first 2 years of life, which progresses to...

CONCLUSION: One-third of families of inpatient pediatric oncology decedents with cancer agreed to autopsy. Demographic and diagnostic factors were not universally strong predictors, underscoring the personal nature of autopsy decisions. Further research should include multisite prospective designs and direct engagement with bereaved families.

CONCLUSIONS: This single-institution disease-specific analysis revealed distinct comorbidity patterns among childhood cancer survivors treated with PBT. Although severe late effects were rare, musculoskeletal and endocrine disorders were frequent, underscoring the need for diagnosis-tailored, long-term follow-up strategies.

CONCLUSIONS: This case suggests a possible link between ACTL6B-related neurodevelopmental disorders and gastrointestinal dysmotility; if confirmed, it could expand the known clinical spectrum of the disease. Pyridostigmine could be considered as adjunctive therapy in PIPO, especially when a neuropathic etiology is suspected.

CONCLUSIONS: Early markers of kidney damage remain very common in children with homozygous sickle cell disease in the DRC. This persistence highlights the lack of effective kidney prevention strategies and the urgent need for systematic screening using simple and accessible tools in resource-limited settings.

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IgA nephropathy (IgAN) is the most frequently reported glomerular disease associated with inflammatory bowel disease (IBD), particularly Crohn's disease (CD), although pediatric cases remain rare. We report IgAN in a 16-year-old male with CD following intestinal surgery and during long-term infliximab therapy, with renal impairment occurring independently of bowel disease activity. The patient presented with recurrent macroscopic hematuria, proteinuria, and acute kidney injury despite sustained...

Steroid-resistant nephrotic syndrome (SRNS) in childhood frequently reflects monogenic podocytopathies in which immunosuppression is ineffective. Biallelic variants in MYO1E, encoding the class I myosin Myo1E, cause a distinctive form of focal segmental glomerulosclerosis (FSGS) often accompanied by "Alport-like" multilamination of the glomerular basement membrane (GBM). Early recognition has therapeutic and prognostic implications. A previously healthy 4-year-old boy presented with generalized...

The gut microbiota, a vast community of symbiotic microorganisms inhabiting our gut, has been recognized as a key-lever for human health, shaping immune system resilience and being essential for immunological homeostasis throughout the life course. Gut microbiota composition may influence both initiation and/or perpetuation of intestinal inflammation, but recent research has highlighted its contribution to both rising and progression of protean non-intestinal inflammatory diseases: indeed, a...

CONCLUSION: Our results in this primarily paediatric cohort highlight the importance of metabolic control and support increasing the blood bicarbonate level for therapy to 24 mmol/L to improve growth. Compared to the overall population, patients with dRTA are at higher risk of CKD from childhood, particularly if they have underlying SLC4A1 variants.

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Hyperphosphatemic familial tumoral calcinosis (HTC) is a rare disease caused by autosomal recessive loss of function variants in the genes encoding fibroblast growth factor 23 (FGF-23), Klotho, or GalNAc-T3. This results in reduced phosphate excretion in the renal proximal tubule, leading to hyperphosphatemia. The clinical manifestations of HTC are mainly periarticular calcifications accompanied by pain and disability, inflammation, and dental problems. Inactive forms or reduced levels of FGF-23...

High-dose methotrexate (HDMTX) is a cornerstone of contemporary treatment protocols for both pediatric and adult acute lymphoblastic leukemia (ALL); however, up to 4% of children and 15% of adults develop renal toxicity with severely delayed MTX elimination (DME). Evidencebased guidance on re-exposure after DME is lacking, and omission of further HDMTX may compromise anti-leukemic efficacy and potentially increase the risk of relapse. This study, conducted within the Ponte di Legno international...

CONCLUSION: Women commencing KRT within 12 months postchildbirth represents a high-risk group with complex medical needs. Maternal death during early childhood years is an underrecognized phenomenon and warrants further research.

CONCLUSIONS: Patients with childhood-onset AAV show good overall and graft survival after kidney transplantation and a low rate of post-transplant relapse. Further studies are warranted to confirm whether positive ANCA at the time of transplantation is associated with poorer graft outcomes.

The increasing prevalence of pediatric obesity has raised numerous questions about its health implications, particularly regarding renal transplant outcomes. These complications often hinder medical interventions in these children. While kidney transplants are often viewed from an organocentric perspective, the overall health of the patient is critical to the success of the procedure. Current discussions make it clear that childhood obesity poses significant problems not only for graft survival,...

CONCLUSIONS: The review and cases emphasise the importance of early genetic testing in paediatric renal anomalies, the necessity of multidisciplinary surveillance even in asymptomatic individuals, and the relevance of 17q12 deletion as a model of variable expressivity in genomic medicine.

Background: Survivors of childhood brain cancer survivors (CBCS) have a higher risk of endothelial dysfunction and cardiovascular mortality. Recombinant human growth hormone (rhGH) replacement therapy may help reduce endothelial damage and the development of cardiovascular diseases (CVD). This study aimed to assess biochemical and biophysical endothelial function in CBCS with GH deficiency (GHD). Methods: CBCS who were at least two years post-treatment underwent clinical evaluation, including...

CONCLUSION: Patients diagnosed with chronic kidney disease during the pediatric period demonstrate an elevated risk of cardiovascular complications from the time of diagnosis onwards. A possible correlation between reduced bone mineral density in these patients and cardiovascular events represents another factor that increases mortality and morbidity.

CONCLUSIONS: These findings provide new insights into GS, highlight disease burden, and suggest areas for future research.

CONCLUSION: Most patients were steroid sensitive, with minimal change being the most common. Focal segmental glomerulosclerosis was the predominant histopathology in the steroid-resistant cases. SRNS patients had worse outcomes, with more infections, CKD, and ESKD.

Background: Free thyroxine (FT4) reference intervals (RIs) provided by many laboratories do not adequately represent the differences in FT4 levels observed across age groups, limiting their usefulness in the diagnosis and management of disease, most particularly at the extremes of age. Interpretive criteria specific to neonates, young children, and older adults are rarely provided. This work was undertaken to develop comprehensive age-based RIs from birth to age 100 to provide clinicians with...

CONCLUSIONS: Involvement of the MSK system is a common morbidity in children with hemodialysis. Calcium × phosphate product (p = 0.026) and vitamin D level (p = 0.003) were the most significant factors associated with MSK pain in multivariate regression analysis.

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Diagnosing nutcracker syndrome can be challenging, particularly when symptoms are suggestive of more common conditions. In such cases, the syndrome is often not considered as an initial differential diagnosis. We report the case of a 30-year-old woman with a history of microhematuria since childhood as well as previous episodes of macrohematuria, abdominal pain and urinary tract infections. As her mother, sister and other relatives are affected by Alport syndrome and chronic kidney disease, this...

CONCLUSION: Transient isolated RTA is observed in infants and young children with mild metabolic acidosis, isolated bicarbonaturia, and moderate failure to thrive and/or growth faltering. It resolves spontaneously within a few years, usually requiring only low-dose alkalizing therapy.

Congenital anomalies of the kidneys and urinary tract (CAKUT) are developmental disorders that commonly cause pediatric chronic kidney disease and mortality. We examine here rare coding variants in 248 CAKUT trios and 1742 singleton CAKUT cases and compare them to 22,258 controls. Diagnostic and candidate diagnostic variants are detected in 14.1% of cases. We find a significant enrichment of rare damaging variants in constrained genes expressed during kidney development and in genes associated...

Epidermolysis bullosa (EB) is a rare, heterogeneous, hereditary, chronic skin disorder with severe cutaneous and extracutaneous involvement. With the significant increase in survival of EB patients, kidney complications have become more common. Among the EB subtypes, recessive dystrophic epidermolysis bullosa (RDEB) is associated with the development of amyloidosis. Secondary amyloidosis affecting the kidneys in RDEB is fatal due to its rapid progression and difficulty in dialysis. Herein, we...

BACKGROUND: Hypertension affects 6% of all children and adolescents, is increasing in prevalence, and is associated with adverse cardiovascular outcomes. In childhood chronic kidney disease, hypertension is associated with progression to kidney failure. However, direct evidence linking childhood hypertension with long-term adverse kidney outcomes is scarce. We aimed to determine the long-term risk of major adverse kidney events (MAKEs) among children and adolescents diagnosed with hypertension.

Karyomegalic interstitial nephritis (KIN) is a rare hereditary form of chronic interstitial nephritis that was first described over 50 years ago. It is characterized by karyomegalic tubular epithelial cells and progressive chronic kidney disease, often leading to end-stage renal disease by the fifth decade of life. Recent studies have identified FAN1 mutations as a key genetic contributor, with additional associations to environmental factors and toxic exposures, such as ochratoxin A, alkylating...

CONCLUSION: SM still carries a significant risk of increased mortality, the need for dialysis, and mechanical ventilation support. The first 24 h after admission, as well as the shock, are determinants of increased mortality.

Congenital anomalies of the kidney and urinary tract (CAKUT) are a common cause of chronic kidney disease in children. Most patients will reach end-stage renal function and dialysis or transplantation in childhood or early adulthood. Patients with CAKUT deserve a careful evaluation before a kidney transplant; detailed imaging and functional studies are necessary, particularly in the presence of lower urinary tract abnormalities, and surgical procedures are advisable in selected cases. A higher...

Autosomal recessive polycystic kidney disease (ARPKD) is a congenital hepatorenal fibrocystic pathology and is one of the most significant childhood nephropathies leading to chronic kidney disease (CKD). While kidney damage has been well studied in this pathology, only a few studies have investigated specific cardiac damage during ARPKD. This study aimed to conduct a large analysis of heart dysfunction during the progression of CKD. ARPKD rats with the Pkhd1 gene mutation (IVS35-2A>T) were...

Childhood-onset systemic lupus erythematosus (cSLE) is a multi-systemic, inflammatory autoimmune disease that affects many organs including the heart. Pericardial effusion as a primary manifestation of SLE in early infancy is very rare. It has been reported as the first symptom of SLE in adult and adolescent case reports only and the youngest reported case was a three-year-old. We report a case of a 22-month-old infant who had previously been healthy but presented with pericardial effusion and a...

Acute leukemias represent the first cause of cancer in children. Their prognosis has improved significantly due to remarkable advances in therapeutic management, despite the risk of long-term consequences, especially for patients who underwent allogenic hematopoietic stem cell transplantation (aHSCT). Through the Leukemia in Children and Adolescents (LEA) long-term follow-up cohort (clinicaltrials gov. Identifier: NCT01756599), we conducted a French national multicenter prospective study on the...

Time to remission (TTR) has been largely considered one of the predictive factors for the risk of relapse and steroid dependency in childhood steroid-sensitive nephrotic syndrome, yet conflicting opinions exist. However, the factors influencing TTR have never been studied. We performed a post-hoc analysis of the prospective pediatric cohort enrolled in a previous multicenter study (ClinicalTrials.gov Id: NCT01386957) to evaluate the possible influence of some clinical and laboratory parameters...

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CONCLUSION: Our study highlights KT access disparities particularly for females, the youngest recipients, high-risk age (15-19 years), and diseases with recurrence risk. Notably, pre-emptive transplants and enduring previous grafts offer advantages regarding re-transplantation.

CONCLUSION: Type B lactic acidosis in aggressive malignancies indicates a poor prognosis. In such cases, as in our case, lactic acidosis improves only with appropriate and sufficient chemotherapy, and its improvement is an important indicator that the case is responsive to treatment.

Various aggressive lymphomas entities have been associated with immunodeficiency. To provide further evidence that also MYC-negative high-grade B-cell (formerly Burkitt-like) lymphoma with 11q aberrations comprises an immunodeficiency-related subtype, we here conducted a comprehensive pathological and genetic workup of a 25-year-old patient with this type of lymphoma and simultaneous papillary renal cell carcinoma. The patient developed both malignancies following extensive childhood...

CONCLUSION: The majority of patients with pediatric AAV achieve inactive renal disease by 12 months; however, almost half have evidence of damage. Renal function at diagnosis is a strong predictor of renal function at 12 months.

Idiopathic Nephrotic Syndrome (INS) is a common childhood glomerular disease requiring intense immunosuppressive drug treatments. Prediction of treatment response and the occurrence of relapses remains challenging. Biofluid-derived extracellular vesicles (EVs) may serve as novel liquid biopsies for INS classification and monitoring. Our cohort was composed of 105 INS children at different clinical time points (onset, relapse, and persistent proteinuria, remission, respectively), and 19 healthy...

The molecular mechanisms responsible for the heightened reactivity of quiescent T cells in human early life remain largely elusive. Our previous research identified that quiescent adult naïve CD4^(+) T cells express LINE1 (long interspersed nuclear elements 1) spliced in previously unknown isoforms, and their down-regulation marks the transition to activation. Here, we unveil that neonatal naïve T cell quiescence is characterized by enhanced energy production and protein synthesis. This...

Maintenance peritoneal dialysis (PD) is the most used kidney replacement therapy for children with kidney failure throughout the world. Underlying causes of kidney failure, indications for dialysis, body size, and nutritional requirements differ between children and adults on PD. These differences, along with the ongoing growth and development that occurs throughout childhood, impact PD access, prescription, and monitoring in children. This review highlights the unique challenges and management...

CLINICAL CHARACTERISTICS: WT1 disorder is characterized by congenital/infantile or childhood onset of steroid-resistant nephrotic syndrome (SRNS), a progressive glomerulopathy that does not respond to standard steroid therapy. Additional common findings can include disorders of testicular development (with or without abnormalities of the external genitalia and/or müllerian structures) and Wilms tumor. Less common findings are congenital anomalies of the kidney and urinary tract (CAKUT),...

CLINICAL CHARACTERISTICS: Primary hyperoxaluria type 1 (PH1) is caused by deficiency of the liver peroxisomal enzyme alanine-glyoxylate aminotransferase (AGT), which catalyzes the conversion of glyoxylate to glycine. When AGT activity is reduced or absent, glyoxylate is converted to oxalate, which cannot be metabolized and must be excreted by the kidneys. Insoluble calcium oxalate crystals form due to high urinary oxalate concentration. Urinary crystals aggregate, leading to nephrolithiasis...

Hidradenitis suppurativa (HS) (Verneuil's disease) is a chronic inflammatory cutaneous disorder with systemic inflammatory potential. A rare and severe sequela of this sustained inflammation is reactive (AA) amyloidosis, typically presenting with nephrotic-range proteinuria and progressive renal failure. Here, we report a 50-year-old male with severe (Hurley Stage III) HS who developed nephrotic syndrome secondary to biopsy-proven renal AA amyloidosis. Initiation of the TNF-α inhibitor...

Steroid-sensitive nephrotic syndrome (SSNS) encompasses a heterogeneous spectrum of podocytopathies, ranging from classical T-cell-mediated forms to those with an autoimmune component. Anti-nephrin autoantibodies have recently emerged as serological markers in a subset of immune-mediated podocytopathies, particularly in minimal change disease (MCD), primary FSGS, and immunoresponsive SSNS/SDNS. Although they correlate with disease activity and remission in selected cases, their sensitivity...

CONCLUSIONS: Current evidence does not support a definitive prophylactic strategy to prevent recurrent FSGS after kidney transplantation. These findings remain inconclusive, and confirmation of potential benefit-or lack thereof-requires adequately powered randomized trials. Future multicenter studies should standardize recurrence definitions, timing of prophylaxis, and safety reporting.

This case highlights the use of intravenous immunoglobulin (IVIG) in a patient with refractory pure class V lupus nephritis (LN), a challenging subtype of LN. A woman in her 30s with systemic lupus erythematosus developed severe proteinuria and could not tolerate standard immunosuppressants due to life-threatening infections. IVIG, though not a guideline-recommended therapy for class V LN, was initiated as an alternative. Over a 6-month period, the urinary protein-to-creatinine ratio decreased...

CONCLUSIONS: This study reveals the genetic and phenotypic heterogeneity of primary SRNS in Indonesian children. The identification of diverse pathogenic variants underscores the utility of WES, even in cases responsive to cyclosporin. Genetic testing should be integrated into routine SRNS evaluation, especially in atypical presentations or early-onset of the disease.

CONCLUSION: Obinutuzumab may be an effective and safe follow-up option for children with RNS who fail to maintain sustained remission after RTX.

CONCLUSIONS: Children with nephrotic syndrome (NS) have poorer oral hygiene and gingival health despite lower sugar intake and reported oral practices, underscoring the influence of dentition stage and the need for integrated dental care and targeted oral health education in pediatric NS management.

CONCLUSIONS: These preliminary results suggest that obinutuzumab is a promising first-line anti-CD20 therapy for FRNS and SDNS in children, offering sustained remission with a good safety profile.

CONCLUSION: Circulating antinephrin antibodies mark a podocytopathy-like phenotype in IgAN with MCD (MCD-IgAN) and define a distinct NS phenotype in patients with IgAN without overt podocytopathy. Targeted serologic testing could facilitate subgroup identification and guide tailored management.

CONCLUSION: This study indicates a potential link between disulfidptosis-related genes and MCD, and presents an exploratory diagnostic and molecular classification system that requires further validation in larger cohorts.

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ABSTRACT: The emergence of non-factor therapies has fundamentally changed inhibitor management in haemophilia. Historically, immune tolerance induction (ITI) was considered indispensable for eradicating factor VIII (FVIII) or IX inhibitors and restoring responsiveness to replacement therapy. However, the introduction of rebalancing therapies (TFPI inhibitors, tissue factor pathway inhibitors) and emicizumab, a bispecific antibody mimicking factor VIIIa activity, provides highly effective bleed...

BACKGROUND Respiratory tract infections (RTIs) are a major complication and prognostic determinant in children with nephrotic syndrome (NS), yet reliable predictors for infection risk remain lacking. Although fibrinogen (FIB) and urinary a1-microglobulin (alpha1-MG) have been individually linked to inflammation and renal injury, their combined prognostic value in pediatric NS has not been established. This study investigated the correlation between FIB, urinary alpha1-MG, and RTI occurrence in...

Posterior reversible encephalopathy syndrome (PRES) is an uncommon but potentially life-threatening neurological complication of systemic lupus erythematosus (SLE). Its pathogenesis is multifactorial, especially in patients with active lupus nephritis receiving intensive immunosuppression. We report a 24-year-old woman with active class III/V lupus nephritis, persistent antiphospholipid antibody positivity, and profound hypoalbuminemia who developed severe atypical PRES with extensive multifocal...

Podocytopathies are kidney diseases caused by podocyte injury or dysfunction that drives proteinuria or nephrotic syndrome. A significant expansion in understanding of the complex causes and mechanisms of podocyte injury, as well as potential therapeutic approaches, has been achieved over the past two decades with rapid advances in genomics in both research and clinical practice. Podocytopathies associated with monogenic mechanisms account for approximately 30% of cases with steroid-resistant...

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INTRODUCTION: Renal vein thrombosis (RVT) is a rare but potentially serious condition, particularly when extending into the inferior vena cava (IVC). It can arise from nephrotic syndrome, hypercoagulable states, or infection, and may compromise renal function or lead to embolic complications. While anticoagulation remains the cornerstone of therapy, endovascular mechanical thrombectomy is emerging as a viable option in selected cases. This case report describes a right RVT with IVC involvement...

CONCLUSION: Renal involvement in FD is often underdiagnosed due to the heterogeneity of clinical presentation. Early diagnosis and timely initiation of enzyme replacement therapy can delay progression to chronic kidney disease and improve long-term outcomes.

Early recognition of nephrotic syndrome and neurological complications in pediatric Hodgkin's lymphoma is essential. This case highlights a rare association between Hodgkin's lymphoma, nephrotic syndrome, and posterior reversible encephalopathy syndrome, emphasizing the importance of multidisciplinary management and prompt blood pressure control to optimize outcomes.

CONCLUSIONS: Ipi+Nivo combination therapy is associated with prominent glomerular-predominant renal injury and pancreatic toxicity signals, potentially reflecting distinct immunological mechanisms whereby CTLA-4-dependent disruption of B-cell tolerance may predispose to glomerular injury while PD-1-mediated release of T-cell cytotoxicity may preferentially target pancreatic islets. These findings suggest the need for enhanced clinical vigilance and tailored monitoring strategies beyond...

CONCLUSIONS: Long-term linear growth was largely preserved in children with difficult-to-treat NS, and short stature was uncommon. Favorable growth outcomes appear achievable with contemporary management strategies incorporating effective disease control and steroid-sparing therapy.

CONCLUSIONS: Low-dose SC OFA is a feasible preemptive therapy for remission maintenance in pediatric FR/SDNS, particularly for those with RTX resistance or intolerance. This outpatient-compatible route may reduce hypersensitivity risks and healthcare costs. Larger prospective trials are warranted to confirm long-term efficacy.

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Exostosin-2 (EXT2)-associated membranous nephropathy (MN) represents a subgroup of immune complex-mediated renal diseases, where transplantation may facilitate clearance of immune deposits. We present a successful kidney transplantation from a 19-year-old deceased donor with previously undiagnosed EXT2-associated MN into a 75-year-old male recipient. Despite significant pretransplant donor proteinuria (> 300 mg/dL) and hypoalbuminemia, renal biopsies at procurement demonstrated well-preserved...

Minimal change disease (MCD) is a common cause of nephrotic syndrome in adults, with limited evidence available on its treatment and prognosis. In this study, we retrospectively included the clinical characteristics and treatment results of adult MCD patients in our center and explored and analyzed potential risk factors for relapse in MCD patients. We included 51 adult MCD patients with a median age of 29 years, and 30 were men. Among them, 16 patients (31.37%) had acute kidney injury (AKI) at...

We report a rare case of synchronous stage IV mantle cell lymphoma (MCL) and paraneoplastic membranous nephropathy (MN) in a 48-year-old man. The patient presented with a nasopharyngeal mass; biopsy confirmed cyclin D1-positive MCL harboring the CCND1::IGH rearrangement, along with nephrotic syndrome. Kidney biopsy revealed MN with IgG1/IgG3-dominant glomerular deposits but was negative for PLA2R, THSD7A, and NELL-1 antigens in both renal and tumor tissues, with corresponding serum antibodies...

CONCLUSION: In patients with a long-standing history of diabetes, the presence of a preceding infection, hypocomplementemia, nephrotic-range proteinuria, and rapidly progressive renal dysfunction should prompt suspicion for non-diabetic kidney disease. In such cases, renal biopsy, together with serum and urine immunofixation electrophoresis, free light chain assays, complement testing, and rheumatologic autoantibody screening, is essential for the diagnosis and differential diagnosis of PGNMID...

Idiopathic membranous nephropathy (IMN) is one of the main causes of adult nephrotic syndrome. A subset of untreated or inadequately treated patients eventually progress to end-stage renal disease (ESRD), posing a significant clinical challenge. Although the discovery of novel podocyte target antigens has deepened our understanding of IMN pathogenesis, the precise molecular mechanisms remain incompletely elucidated, and effective targeted therapies are still lacking. Animal models play an...

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Purtscher-like retinopathy is a rare occlusive microvasculopathy that causes acute, painless visual loss and characteristic retinal lesions. Although typically trauma-related, it can occur in systemic conditions such as nephrotic syndrome (NS), driven by hypercoagulability, complement activation, and microembolization. We report a 3-year-old girl with steroid-resistant NS presenting with sudden vision loss, including inability to fixate and intermittent exotropia, without trauma or hypertension....

CONCLUSION: Measurement of anti-PLA2R demonstrates high diagnostic accuracy for PMN in our setting and may allow kidney biopsy to be avoided in a selected subgroup of patients with a typical clinical presentation, thereby optimizing resources and reducing invasive procedures. Larger local studies are needed to confirm these findings.

CONCLUSION: Infection-related glomerulonephritis in adult patients was found to present with diverse clinicopathological features, often post-infection. Most patients recovered, but progression to chronic kidney disease and end-stage renal disease correlated with hypertension and chronic histological changes.

CONCLUSION: This case highlights the therapeutic complexity of EBV-HLH in a solid-organ transplant recipient and underscores both the rationale for and risks of cellular immunotherapy amid severe hyperinflammation and organ dysfunction.

Renal venous thrombosis (RVT) is a location of unusual-site venous thromboembolism. RVT occurs more commonly in males, and shows a bimodal age distribution, with a neonatal and adult peak. Abdominal malignancies and nephrotic syndrome are prominent risk factors in adults, whereas hypotension, birth asphyxia, sepsis, umbilical venous catheters and prematurity are the predominant causes in children. The most common symptoms of RVT include abdominal pain and macroscopic haematuria. A palpable...

CONCLUSIONS: SRNS/FSGS accounts for approximately 29% of paediatric CKD 5 in this cohort and is characterised by a high prevalence of monogenic variants reflecting regional consanguinity. Genetic testing reliably stratifies recurrence risk: non-genetic SRNS carries a substantially higher recurrence rate, yet combined plasmapheresis and rituximab achieves remission in most affected patients. Graft outcomes are comparable to those seen in other CKD 5 aetiologies.

Background: Septic Charcot neuroarthropathy is a limb- and life-threatening condition characterized by the coexistence of neuropathic joint destruction and infection. In patients with severe systemic compromise, major amputation is often considered inevitable. Case Presentation: A 47-year-old man with untreated diabetes mellitus presented with progressive painless swelling of the left foot. He had morbid obesity (120 kg, 165 cm; body mass index 44.1 kg/m²), severe hypoalbuminemia, and chronic...

IgA nephropathy with minimal change disease (IgAN-MCD) is a rare glomerulopathy combining features of both entities, typically presenting as steroid-sensitive nephrotic syndrome (NS) with frequent relapses. While rituximab (RTX) is commonly used in pediatric glomerular diseases, its efficacy in IgAN-MCD remains unclear. Our retrospective study evaluated the efficacy and safety of RTX in eight children with IgAN-MCD who remained steroid-dependent or frequently relapsed despite treatment with...

CONCLUSIONS: This case demonstrates that rituximab monotherapy can achieve clinical remission in iMCD-NOS with concurrent autoimmune manifestations when anti-IL-6 therapy is unavailable. The delayed response pattern-with biomarker improvement preceding clinical recovery-highlights the importance of serial VEGF monitoring and persistence with B-cell-directed therapy before concluding treatment failure.

Idiopathic nephrotic syndrome is one of the most common glomerular diseases in children and adolescents. Monogenic nephrotic syndrome is associated with lower rates of remission following treatment with standard immunosuppressive therapies and a higher risk of progression to end-stage kidney disease. Advances in genetic testing technologies have identified more than 100 single-gene causes of nephrotic syndrome and made genetic testing more accessible in clinical practice. The vast majority of...

Objective The main objective of this study is to assess bone mineral density (BMD) and ocular complications in children with steroid-sensitive nephrotic syndrome (SSNS) receiving prolonged corticosteroid therapy and to identify factors associated with these complications. Methods This cross-sectional observational study was conducted at a tertiary care center over 18 months. Children aged 1-18 years with SSNS who had received corticosteroids for more than six months were enrolled. Clinical...

CONCLUSION: IgM sialylation status tracks disease activity and modulates podocyte structural, metabolic, and signaling responses, supporting immune glycan remodeling as a disease-associated modifier of podocyte vulnerability in iNS.

CONCLUSIONS: Our NELL1 LIPS assay provides a noninvasive tool for diagnosing NELL1-associated MN, characterizing clinical subsets, and monitoring therapeutic response.

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CONCLUSIONS: This MR study supports the possibility of a genetic causal association between peripheral blood B cell subtypes and MN. The results improve our understanding of the immunological basis of MN and may inform the development of personalized medicine.

We report a case of normolipidemic lipoprotein glomerulopathy (LPG) associated with the APOE Kyoto variant. A 54-year-old Chinese man was referred for evaluation of persistent low-level proteinuria detected on routine screening, with preserved renal function and no clinical features of nephrotic syndrome. Laboratory evaluation showed proteinuria without overt dyslipidemia. Extensive evaluation excluded secondary causes. Renal biopsy revealed markedly dilated capillary loops filled with...

CONCLUSIONS: RTX demonstrates good efficacy and safety in children with FRNS or SDNS after treatment with three immunosuppressive agents.

Objective: To analyze the risk factors for poor prognosis in children with steroid-resistant nephrotic syndrome (SRNS) and to construct and validate a prognostic model. Methods: A retrospective cohort study was conducted. Clinical data of 456 children with SRNS who were initially diagnosed and hospitalized at the Children's Hospital of Chongqing Medical University from January 2009 to December 2024 was collected, including general information, laboratory and pathological indicators, gene types,...

A growing number of target antigens have been identified in membranous nephropathy (MN) in recent years. Clinical correlations exist for some MN antigens, whereas others remain poorly characterized because of their rarity. High-temperature requirement A serine peptidase 1 (HTRA1) is the target antigen in approximately 1%-2% of MN cases without any established disease associations. Recent studies suggest HTRA1-MN may associate with malignancies in approximately 12% of cases, mostly solid tumors....

CONCLUSION: This report highlights the potential for life-threatening hypersensitivity upon rituximab re-challenge, even with a remote history of uneventful use. It emphasizes the need for prolonged post-infusion observation, patient education regarding delayed symptoms, and preparedness for immediate management of severe allergic phenomena. Further studies are warranted to elucidate risk factors and mechanisms underlying these rare but serious events.

We herein report the case of a 2-year-old girl with novel compound heterozygous NPHS1 variants, p.R460Q (a known loss-of-function mutation) and p.V822M (a rare variant with reported pathogenicity and relatively mild functional effects). She initially presented with typical features of idiopathic nephrotic syndrome and achieved complete remission on Day 10 of corticosteroid therapy. After tapering, the patient developed recurrent episodes of infection-associated heavy proteinuria, which often...

Recognizing its clinical utility, the 2024 Kidney Disease Improving Global Outcomes (KDIGO) guideline recommends using equations that integrate serum creatinine and cystatin C for chronic kidney disease (CKD) diagnosis and staging. Cystatin C-inclusive equations generally outperform creatinine-based equations in bias and accuracy across diverse populations, including children and patients with comorbidities. Moreover, cystatin C-inclusive equations consistently demonstrate greater prognostic...

CONCLUSION: In our matched cohort, the vast majority of patients, received no more than 8 units of blood. Intraoperative allogeneic blood transfusion does not influence postoperative acute kidney injury and in-hospital mortality in liver transplantation patients. Multicenter, prospective studies are needed in the future to validate our findings.

Allograft nephrectomy (AN) after graft failure remains controversial, balancing symptom control and facilitation of immunosuppression withdrawal against operative risk and uncertain immunological consequences. A systematic review of literature published from January 2000 to March 2024 in accordance with PRISMA guidelines was performed. We included studies addressing indications, timing, complications and outcomes related to subsequent transplantation. Thirty-nine studies were included. Reported...

CONCLUSION: Kidney transplantation offers children and adolescents a renewed chance at life, boosting their self-esteem and overall well-being. However, they often face considerable psychological stress, including anxiety about the future and limited support. To address these challenges, healthcare providers and social organizations must focus on their unique needs through comprehensive psychological assessments, identification of unmet needs, and strengthened collaboration among medical teams,...

CONCLUSION: Abnormal high delta power may be indicative of clinically apparent encephalopathy in young patients with kidney failure. The role of increased functional connectivity as potential compensatory mechanism should be further explored.

CONCLUSIONS: Kidney transplantation in carefully selected older recipients, including those aged ≥ 70 years, was associated with sustained patient and graft survival. Infectious complications occurred frequently and constituted the leading attributed cause of DWFG; this underscores the importance of understanding why some recipients recover from infectious stress, while others experience subsequent clinical deterioration.

CONCLUSIONS: The delay in PH diagnosis in France has been halved over the past five years. This improvement may be associated with recent therapeutic developments and increased awareness of the disease. Earlier diagnosis may enable earlier management, which might ultimately contribute to improved overall prognosis.

No abstract

Chronic kidney disease (CKD) in childhood, although uncommon, has profound lifelong consequences. Because disease onset occurs early, even modest slowing of CKD progression may translate into decades free from dialysis, transplantation, and premature death. Progressive proteinuria is a central driver of nephron loss in pediatric CKD, making early and sustained antiproteinuric strategies particularly impactful. Despite heterogeneous etiologies, including congenital and immune-mediated kidney...

CONCLUSIONS: Circulating NSE in childhood reflects developmental stage rather than CKD status. Group comparisons in pediatric biomarker studies require age adjustment. Transplantation alters the NSE-age relationship beyond what kidney function explains. BDNF tracks kidney function in pediatric CKD. Age-stratified reference intervals are required before either marker can guide clinical decisions.

CONCLUSION: This multistakeholder prioritization distilled broad kidney care domains into actionable subtopics, centered around slowing CKD progression and improving symptoms during kidney replacement therapy, providing targeted systematic reviews and guideline updates supporting shared decision-making in CKD.

Cancer is a critically important outcome for kidney transplant recipients that inconsistently and infrequently reported in trials. We convened a consensus workshop to establish a core outcome measure for cancer for trials in kidney transplant recipients. Workshop attendees included 21 transplant recipients, 2 caregivers and 46 health professionals from 12 countries. Transcripts were analyzed thematically. Three themes were identified. "Fear of cancer occurrence due to immunosuppression"...

CONCLUSION: Although causality cannot be established, the concurrence of bilateral dysplastic kidneys and kidney failure in this rare genetic disorder raises important clinical questions. The case also highlights the importance of mutlidisciplinary care in dialysis and transplant planning. Further studies are required to clarify the potential role of TBL1XR1 gene in kidney development and long-term outcomes in Pierpont syndrome.

CONCLUSIONS: Pediatric KTx recipients with structural myocardial alterations on CMR imaging exhibit detectable abnormalities in routine echocardiographic diastolic parameters. Especially a markedly elevated septal E/e' could identify patients at increased risk for underlying myocardial involvement and justify the use of CMR imaging in post-transplant follow-up.

Background/Objectives: Advanced technologies in type 1 diabetes mellitus (T1DM) management have reshaped the strategies used to achieve optimal glucose control. Continuous subcutaneous insulin infusion (CSII) and automated insulin delivery (AID) systems are effective alternatives to multiple daily injections (MDI). This study aims to evaluate glycemic regulation in children and adolescents transitioning from MDI to insulin pumps and to raise awareness among patients and their families regarding...

Background/Objectives: Opportunistic infections remain clinically important after kidney transplantation and may contribute to morbidity and graft dysfunction in pediatric recipients. Data regarding their timing, spectrum and clinical course in children remain limited. Methods: We retrospectively reviewed pediatric kidney transplant recipients followed at a single tertiary center between 2014 and 2024. Demographic and clinical characteristics, infection type, timing after transplantation,...

CONCLUSIONS: The EKFC equation was overall the best suited creatinine-based eGFR equation for this Norwegian population from 2 years of age. Clinicians should be informed about clinical implications of shifting from other equations to the EKFC equation.

CONCLUSIONS: Timing and initial regimen selection were strong. The main baseline process measure requiring prospective review was continuation of protocol-linked antibiotics beyond cutoffs in abdominal transplantation. This audit shows how routine electronic data can be used to create reproducible measures and organ-specific targets for future stewardship and quality-improvement studies.

CONCLUSION: We describe treatment allocation and short-term outcomes in pediatric MN using a modified risk stratification-based approach. Higher risk categories appeared to have less favorable responses, while anti-PLA2R positivity was associated with better outcomes.

CONCLUSION: Tac-SD decreased after conversion from IR-Tac to LCPT among those with high baseline variability but increased among those with low baseline variability; there were no significant changes in subjective adherence measures. The effectiveness of conversion from IR-Tac to LCPT for adherence is unclear.

CONCLUSION: This case illustrates the broad spectrum of autoimmunity in WAS/XLT, highlighting the potential for renal involvement. It emphasizes the importance of early recognition, genetic confirmation, and multidisciplinary management. Registry data remain essential for guiding prognosis, monitoring complications, and informing therapeutic strategies, including hematopoietic stem cell transplantation.

CONCLUSION: Using large-scale, real-world multi-institutional data, we investigated the natural history of a large contemporary cohort of children with MPGN. As patients classified as having C3G compared to IC-MPGN had similar baseline clinical characteristics and rates of progressive disease despite untargeted treatment approaches, the availability of newer complement-targeted agents hold the promise of benefit to this population.

CONCLUSION: Accordingly, these guidelines strongly recommend early RAS blockade as the cornerstone of AS, while no recommendations are made for routine use of cyclosporine, MRAs, or SGLT2i. Well-designed clinical trials are needed to expand therapeutic options and improve long-term outcomes in AS.

CONCLUSION: The clinical phenotype of C3 p.Arg161Trp-associated CaHUS has significant heterogeneity, with differences in presentation and outcomes between children and adults. Children present with more profound hemolysis but milder acute kidney injury (AKI). Furthermore, C3 p.Arg161Trp is linked to a high risk of relapse.

Cotransplantation of donor bone marrow (BM) has yielded a therapeutic benefit in clinical trials of kidney transplantation by inducing chimerism-based tolerance, but this was dependent on unfavorable recipient irradiation. Additional regulatory T (T(reg)) cell administration can overcome the need for myelosuppression, but this has been demonstrated only in mice so far. Here, we conducted a controlled, phase 1/2a trial of combined BM and T(reg) cell therapy in HLA-mismatched living-donor kidney...

BACKGROUND: Candidates awaiting repeat kidney transplantation face substantial immunological barriers due to prior human leukocyte antigen (HLA) allo-sensitisation. Re-exposure to previously mismatched HLA antigens may increase the risk of acute rejection and premature graft loss. Although avoidance of repeat HLA mismatches (RMM) has been standard practice, emerging evidence suggests this risk varies according to the specific HLA allelic RMM and anti-HLA antibody profile to the RMM. Here we...

CONCLUSIONS: AKI is common, mostly mild, and predominantly occurs early after transplantation, highlighting the need for risk-stratified renal monitoring, tailored immunosuppressive management, and long-term nephrological follow-up in FA patients surviving HSCT.

Whether metabolites enriched at early developmental stages affect cellular and organismal aging remains unclear. In this study, we comprehensively profiled the metabolic landscape of mouse oocytes in comparison to cleavage-stage embryos. Our analysis revealed that oocytes display accumulation of reductive metabolites that diminish following fertilization. Notably, we identified serotonin (5-hydroxytryptamine, 5-HT) as an oocyte-enriched metabolite with protective roles in aging. The underlying...

The ongoing shortage of kidney donations increases pressure to optimize utility and equity in kidney allocation; simulation models allow evaluation of policy changes before implementation. Using data from the German national transplant registry (19,517 kidney transplants), human leukocyte antigen (HLA) information from the German bone marrow donor registry, and the Eurotransplant database, we developed a discrete time simulation model that reconstructs the current German kidney allocation...

The global epidemic of chronic kidney disease (CKD) is becoming well recognised. Current CKD treatment strategies, however, focus on using drugs to protect kidneys in clinically overt nephropathies together with the optimisation of dialysis and transplantation techniques. Here, a complementary perspective is made, starting with a focus on early adverse events, or 'hits', that perturb the prenatal development or early postnatal maturation of the kidney. A more widespread awareness of these...

CONCLUSIONS: Kidney function laboratory values, although seemingly straightforward in their intended purpose and use, are more complex from the patient and family perspective, as they also closely align with youth kidney transplant recipients' and their families' emotions. This study demonstrates the value of designing visualizations that capture these complexities, which can create opportunities for increased understanding of health information and self-reflection to support self-management.

CONCLUSIONS: Although urinary allantoin, adenosine, and the allantoin/uric acid ratio were comparable between pediatric patients with ADPKD and controls, inverse associations between allantoin and TKV were observed between the groups. However, the lack of correlation with LIC score and the negative correlation with age limit its potential as a marker in pediatric ADPKD.

CONCLUSIONS: We identified a three-protein panel (TSPAN1, KLK3, EDA2R) which can predict incident BPH. The findings highlight potential targets for non-invasive risk assessment and therapy.

CONCLUSION: HLA mismatch is a stronger predictor of graft outcomes than KDPI in pediatric kidney transplantation. The limited number of high-KDPI donors in pediatric recipients may explain the lack of significant association with KDPI. These findings support efforts to minimize HLA mismatches in pediatric allocation strategies.

CONCLUSIONS: Adequate family social support was significantly associated with access to PKT. Emphasis should be placed on strengthening family support networks and bolstering caregivers' resilience to increase the likelihood of PKT among pediatric candidates.

CONCLUSION: Fungal infections after kidney transplantation show distinct epidemiological patterns with pathogen-specific risk profiles, supporting risk-adapted monitoring and diagnostics.

CONCLUSION: Glomerulonephritis in allograft biopsies can impact patient and graft outcome. Diagnosis requires high index of clinical suspicion, correlation with clinical features and treatment history, knowledge of native kidney disease for categorization into recurrent or de novo disease, assessment of renal function parameters such as urine PCR, serum urea and creatinine, and tissue biopsy for histopathological, immunofluorescence, and electron microscopic examination.

To investigate the role of non-human leukocyte antigen (HLA) antibodies in kidney allograft rejection, we developed a non-HLA antibody Luminex assay to identify kidney transplant recipients at risk of allograft rejection. Protein microarray analysis (>1.5 fold; P < .05) of 16 rejection-positive, endothelial cell crossmatch-positive patients identified 135 non-HLA antigens. From these, 12 novel antigens were prioritized for their membrane localization, kidney expression, and identification in...

CONCLUSION: Pediatric patients present a more active kidney histology than adults, a more frequent nephrotic-range proteinuria and more severe anemia at presentation. Despite this, outcomes are comparable to adults. Berden, ARRS, and AKRiS can be used for predicting kidney prognosis in the pediatric population.

CONCLUSION: These findings highlight a persistently elevated risk in adolescents and young adults in the contemporary era.

Two pediatric patients with dilated cardiomyopathy and end-stage heart failure (ESHF) were bridged with extracorporeal devices to heart transplant (HTx).A 6-year-old boy received veno-arterial extracorporeal membrane oxygenation (V-A ECMO) support with left ventricular unloading via subclavian drainage and later conversion to a hybrid configuration involving left and right ventricular assist devices (LVAD and RVAD) for 8 days. After HTx, acute kidney injury and hemorrhagic stroke occurred.A...

Infections after kidney transplantation are a principal cause of morbidity, allograft failure and mortality. Paediatric recipients are less likely to have immunity through prior exposure to pathogens linked to graft failure such as CMV and EBV. This immunological naivety results in a need for meticulous donor screening, the deployment of strategies to minimise the risk of severe disease, and prompt treatment once infection is diagnosed. This review provides an up-to-date overview of major...

CONCLUSION: The frequency of severe hemorrhage in children treated with kidney transplantation in our center was higher than reported in the literature. The type of donor and the combination of small recipients and large donors were the main risk factors. The need for a larger abdominal dissection area in small children receiving large grafts, contributing to the risk of bleeding, is a possible explanation for this finding.

CONCLUSIONS: This study characterizes aspects of the systemic and mucosal humoral immune response to BKPyV infection, offering preliminary insights into immunity in the general population.

CONCLUSION: This simple survey revealed that definitive diagnosis of PH occurs at older age accompanied with higher rate of kidney transplantation.

CONCLUSIONS: PD displays substantial environmental impact variability across centres, driven by differences in material consumption, waste management, energy sourcing, and clinical protocols. Cleaner energy profiles, effective waste management, and reduced reliance on single-use consumables may lower environmental burdens. These findings underscore the importance of integrating environmental performance into clinical decision-making and health system planning.

CONCLUSIONS: Despite the increased hemodynamic demands after pediatric KT with large donor-recipient size mismatch, improved LV function and reduced left ventricular mass were found within 1 year after transplantation. These results suggest reversed cardiac remodeling.

CONCLUSIONS: Despite similar KT characteristics and rejection rates, children transplanted for PUV experience more infections and earlier deterioration of graft function and progression to CKD compared with children transplanted for non-urologic causes. These findings suggest the need for long-term, close monitoring and the proactive use of strategies to optimize bladder function in PUV KT recipients.

No abstract

Microbiome systems encompass diverse ecological niches and host associations, with their scale and complexity challenging traditional analytical frameworks. Advances in artificial intelligence are transforming microbiome research by enabling improved integrative analyses of microbial genomes, community structure, and functional potential. In this Review, we outline how these developments create opportunities for microbiome research to move beyond descriptive analyses towards predictive modelling...

CONCLUSION: Acetate ameliorates experimental MN through coordinated immunomodulatory effects and amelioration of podocyte foot process effacement. These findings support targeting acetate pathways as a mechanistically plausible adjunct strategy with low potential toxicity for MN.

CONCLUSION: IgM sialylation status tracks disease activity and modulates podocyte structural, metabolic, and signaling responses, supporting immune glycan remodeling as a disease-associated modifier of podocyte vulnerability in iNS.

Membranous nephropathy (MN) is uncommon in children overall, but secondary MN is relatively common in younger children. Inflammatory bowel disease (IBD) can be complicated by kidney disease, but IBD complicated by MN is rarely reported. A 3-year-old boy diagnosed with very early onset IBD (VEO-IBD) a year earlier was incidentally found to have proteinuria via urine screening system. Laboratory tests revealed nephrotic syndrome with microscopic hematuria and signs of ongoing inflammation...

CONCLUSIONS AND RELEVANCE: In this exploratory analysis, treatment with finerenone slowed kidney function decline, reduced albuminuria, and lowered the risk of kidney failure or substantial loss of kidney function in patients with glomerular diseases. These findings suggest an important role for finerenone in preserving kidney function in this population.

A 10-year-old boy was admitted with facial edema and proteinuria for two months, occurring nine months after hematopoietic stem cell transplantation. He was clinically diagnosed with nephrotic syndrome and showed no remission after four weeks of standard glucocorticoid therapy, suggesting steroid-resistant disease. Renal biopsy was consistent with membranous nephropathy. Mass spectrometry identified granular co-deposition of IgG and semaphorin 3B (Sema3B) along the glomerular basement membrane,...

Bullous systemic lupus erythematosus (BSLE) is a rare blistering manifestation of systemic lupus erythematosus (SLE), characterized by widespread tense bullae resulting from autoantibodies against type VII collagen. It is predominantly seen in women and is exceptionally rare in pediatric males. We report a case of a 14-year-old South Asian male with recurrent tense blisters over the trunk, face, extremities, palms, and mucosa, alongside systemic features including photosensitivity, arthralgia,...

Treatments that deplete or modulate B cells are in use or being investigated for several immune-mediated glomerular diseases. Kidney Disease: Improving Global Outcomes (KDIGO) convened a Controversies Conference in Panama City, Panama, in June 2025 to review current evidence and identify key gaps in knowledge and research needs to effectively apply such therapies. Availability, effectiveness, and safety of B cell-targeted therapies vary substantially across glomerular diseases. In IgA...

Membranous nephropathy (MN) is an uncommon cause of nephrotic syndrome in children, accounting for fewer than 5% of cases. Primary MN mediated by antibodies against the phospholipase A2 receptor (PLA2R) is particularly rare in the paediatric population. We report the case of a 14-year-old girl with type 1 diabetes mellitus and autoimmune hypothyroidism who presented with nephrotic syndrome and was subsequently diagnosed with PLA2R-positive primary MN. She presented with periorbital oedema,...

We aimed to systematically evaluate the strength and credibility of evidence linking exposure to five major heavy metals, including arsenic, cadmium, lead, mercury, and chromium, with health outcomes (PROSPERO, CRD420251169899). Literature searches of PubMed/Embase, CINAHL, and Google Scholar up to April 20, 2025, identified meta-analyses of observational studies assessing these associations. Effect sizes were recalculated using random-effects models and expressed as equivalent odds ratios (eOR)...

CONCLUSION: Anti-nephrin antibodies have a relatively high positive rate in podocytopathies and have a differentiating effect on SSNS and non-SSNS in children. Anti-nephrin antibodies are associated with the clinical severity and recurrence of podocytopathies.

Juvenile idiopathic arthritis is common rheumatic disease in children and adolescents, but renal involvement is uncommon. Renal involvement is mostly in the form of secondary renal amyloidosis presenting as proteinuria. Membranous nephropathy is an uncommon renal manifestation of juvenile idiopathic arthritis. Here, we report a case of HLA-B27-positive oligoarticular juvenile idiopathic arthritis presenting as subnephrotic proteinuria. The patient also had positive anti-phospholipase A2 receptor...

Nephrotic syndrome (NS) occurring in children with cancer represents a rare yet clinically important paraneoplastic complication. Within pediatric oncology, both primary (idiopathic) and secondary forms of glomerular disease have been identified, most frequently presenting as minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), or membranous nephropathy (MN). Emerging evidence highlights the involvement of anti-nephrin autoantibodies in a significant subset of idiopathic...

CONCLUSION: FSGS exhibits a notably high prevalence in SRNS and remains the most frequently observed histopathological lesion associated with this condition.

Chronic kidney disease affects 1 in 10 people worldwide, with damage to specialized blood filter cells of the kidney, called podocytes, playing a critical role. In membranous nephropathy (MN), a major cause of nephrotic syndrome, circulating autoantibodies attack proteins on podocyte foot processes (FPs), damaging the kidney's filtration barrier. Our study shows that these autoantibodies trigger the formation of antigen-autoantibody aggregates on the podocyte FP plasma membrane. These aggregates...

CONCLUSIONS: JAK/STAT pathway overactivity is present in pediatric patients with primary FSGS and predicts the severity of disease. JAK/STAT hyperactivity is likely driven by cytokine signaling and may be targeted by JAK inhibition.

INTRODUCTION: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a rare and chronic fibroinflammatory condition hallmarked by tumefactive lesions that can affect nearly any organ of the body and lead to fibrotic organ destruction. Parenchymal and non-parenchymal affection of the kidney and urogenital tract are subsumed under the umbrella term IgG4-related kidney disease (IgG4-RKD), which is a severe and quite common organ manifestation in IgG4-RD. The immunopathogenesis in IgG4-RD is depicted...

Early data have shown the potential of chimeric antigen receptor (CAR) T-cell therapies to expand the therapeutic landscape in systemic lupus erythematosus (SLE). While many CAR T-cell therapy learnings can be drawn from the experience of this modality in oncology, key questions remain regarding clinical development considerations unique to lupus. To assess and discuss these issues, the Lupus Accelerating Breakthroughs Consortium, a public-private partnership, convened a multi-partner working...

CONCLUSIONS: This study provides robust genetic evidence for repurposing GLP-1RAs in CKD and IgAN through anti-inflammatory (FGF23) and metabolic pathways, extending their utility beyond glucose control. While European ancestry data limit generalisability, our framework prioritises FGF23 and metabolic modulation as key targets for clinical trials in renal protection.

CONCLUSION: Our commentary underscores the need for increased participation in clinical trials to validate regional applicability and improve long-term outcomes for people with GD in Australia and New Zealand. Clinical trials of new medications have led to more treatment options that are awaiting approval.

CONCLUSIONS: This system of reporting urine sediment is a sensitive and efficient method for predicting the severity of underlying kidney disease and need for performing renal biopsy.

CONCLUSION: In this study, we found several PLA2R1 and HLA-DQA1 single-nucleotide polymorphism loci associated with primary membranous nephropathy morbidity and that some PLA2R1 single-nucleotide polymorphism loci were related to the treatment response of patients with primary membranous nephropathy.

CONCLUSION: This study unveils self-reported Black race, young age (aged < 18 years) and Latinx ethnicity as potential risk factors associated with worse kidney outcomes.

There is rapidly increasing evidence of the role of complement in different forms of kidney disease and this has broadened the field to involve not only atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G), but also a number of other glomerular diseases, mainly ANCA-associated renal vasculitis, immune-complex glomerulonephritis, membranous nephropathy, and IgA nephropathy (IgAN). In parallel, the field of therapeutic agents able to target the three complement pathways at...

CONCLUSIONS: In the CureGN cohort, elevated risk of incident CV and TE events is associated with severity of kidney disease rather than GD subtype.

Allogeneic hematopoietic progenitor cell transplantation is a widely used procedure, and graft-versus-host disease (GVHD) is a common complication. Glomerular involvement due to GVHD is exceptional.

CONCLUSIONS: Our study demonstrated that IMRCs inhibited TGF-β1-induced fibrosis in HESCs, suppressed the EMT process ex vivo, reduced the inflammatory response, and reversed endometrial damage and fibrosis in IUA rats. IMRCs exerted their effects through the paracrine pathway, with specific miRNAs in Exos downregulating the TGF-β/Smad signaling pathway to inhibit uterine endometrial fibrosis. IMRCs provide a new direction for the treatment of IUA.

CONCLUSIONS: The spectrum of kidney disease has changed within the last 14 years. The relative frequency of MN and DN increased significantly, while that of HBVN decreased significantly. These findings highlight the need for ongoing public health efforts tailored to the changing spectrum of kidney diseases.

CONCLUSIONS: This genetic-level investigation uncovers causal associations between immunophenotypes and PGDs, providing valuable insights into the immunological underpinnings of PGDs. Our findings suggest potential targets for treatment strategies, thereby facilitating more personalized and effective therapeutic approaches in PGDs management.

CONCLUSIONS: In the Nephrotic Syndrome Study Network cohort, combined PLA2R-Ab testing with ELISA and IIF provided optimal test characteristics in making a noninvasive diagnosis of MN before or soon after kidney biopsy, including in patients with subnephrotic proteinuria. Further studies in multiethnic populations are needed to assess whether genetic data can augment this approach.

CONCLUSIONS: CTLA4-Ig ameliorated induction of experimental membranous nephropathy, potentially through suppression of Th17 cells in the kidney, and may represent an effective adjunct treatment in membranous nephropathy.

Our review summarizes and evaluates the current state of knowledge on lipid metabolism in relation to the pathomechanisms of kidney disease with a focus on common pediatric kidney diseases. In addition, we discuss how nutrition in early childhood can alter kidney development and permanently shape kidney lipid and protein metabolism, which in turn affects kidney health and disease throughout life. Comprehensive integrated lipidomics and proteomics network analyses are becoming increasingly...

CONCLUSION: Genetically influenced plasma levels of PLA2R1 and NFKB1 impact MN risk, while FCGR3B and BTN3A1 levels are causally linked to IgAN risk, suggesting potential drug targets for further clinical exploration, notably BTN3A1 for IgAN.

Background: A renal biopsy is essential for the identification and management of renal disorders. Although considered an invasive operation, it is necessary for a definitive diagnosis and treatment of many renal diseases. The primary goal of this study was to assess the clinicopathological aspect of renal diseases undergoing biopsy in children receiving tertiary care.Patients and Methods: Children (≤18 years) hospitalized with nephrotic syndrome were the subjects of this cross-sectional study,...

Patients with nephrotic syndrome (NS) present with edema, proteinuria, hypoalbuminemia, and hyperlipidemia. In children, the most common causes are idiopathic minimal change disease and focal segmental glomerulosclerosis (FSGS). In adults, FSGS and membranous nephropathy (MN) are the most common primary causes. There are numerous secondary causes, including diabetes, amyloidosis, systemic lupus erythematosus, hematologic malignancies, and infections. In addition to confirming the diagnosis of NS...

DNASE1L3 is an extracellular nuclease that digests chromatin released from apoptotic cells. DNASE1L3 variants impair the enzyme function, enhance autoantibody production and type I interferon (IFN-I) responses, and cause different autosomal recessive phenotypes ranging from hypocomplementemic urticarial vasculitis syndrome to full-blown systemic lupus erythematosus (SLE). Kidney involvement in patients with DNASE1L3 variants is poorly characterized. Herein, we describe the clinical course of 3...

CONCLUSION: The pathological type of NS may be associated with specific malignancies in patients with PNS. Prompt identification of PNS coupled with suitable therapeutic intervention has a significant impact on the outcome for patients.

Treatment of glomerulonephritis presents several challenges, including limited therapeutic options, high costs, and potential adverse reactions. As a recognized Chinese patent medicine, Tripterygium wilfordii poly-glycosides (TWP) have shown promising benefits in managing autoimmune diseases. To evaluate clinical effectiveness and safety of TWP in treating glomerulonephritis, we systematically searched PubMed, Cochrane Library, Web of Science, and Embase databases for controlled studies...

CONCLUSIONS: In this study, circulating antinephrin autoantibodies were common in patients with minimal change disease or idiopathic nephrotic syndrome and appeared to be markers of disease activity. Their binding at the slit diaphragm induced podocyte dysfunction and nephrotic syndrome, which highlights their pathophysiological significance. (Funded by Deutsche Forschungsgemeinschaft and others.).

CONCLUSIONS: Cyclophosphamide can induce immunological remission earlier than rituximab at the span of 6 months. The PLA2R-CTLD1-IgG4 has a better predict value than total PLA2R-IgG for remission of proteinuria at the 6th month.

Recent progress in glomerular immune complex and complement-mediated diseases have refined diagnostic categories and informed mechanistic understanding of disease development in pediatric patients. Herein, we discuss selected advances in 3 categories. First, membranous nephropathy antigens are increasingly utilized to characterize disease in pediatric patients and include phospholipase A2 receptor (PLA2R), Semaphorin 3B (Sema3B), neural epidermal growth factor-like 1 (NELL1), and protocadherin...

Here, we used digital spatial profiling (DSP) to describe the glomerular transcriptomic signatures that may characterize the complex molecular mechanisms underlying progressive kidney disease in Alport syndrome, focal segmental glomerulosclerosis, and membranous nephropathy. Our results revealed significant transcriptional heterogeneity among diseased glomeruli, and this analysis showed that histologically similar glomeruli manifested different transcriptional profiles. Using glomerular...

CONCLUSION: The results of this study suggest a potential link between PCa and a higher risk of ED.

The deposition of antipodocyte autoantibodies in the glomerular subepithelial space induces primary membranous nephropathy (MN), the leading cause of nephrotic syndrome worldwide. Taking advantage of the glomerulus-on-a-chip system, we modeled human primary MN induced by anti-PLA2R antibodies. Here we show that exposure of primary human podocytes expressing PLA2R to MN serum results in IgG deposition and complement activation on their surface, leading to loss of the chip permselectivity to...

CONCLUSIONS: IHC PLA(2)R staining of glomerular tissue is a useful diagnostic marker of IMN. Though PLA(2)R prevalence is lower in children, its role in guiding treatment needs further exploration.

CONCLUSIONS: We confirm that changes better known in adults with either type 1 or type 2 diabetes mellitus can occur in children with type 1 diabetes mellitus: overt diabetic nephropathy either on its own or combined with other conditions and kidney disorders other than diabetic nephropathy.

No abstract

CONCLUSIONS: Most scored pathology features showed excellent reproducibility, demonstrating consistency for these features across multiple pathologists. Correlations between certain pathologic features and expected clinical characteristics show the value of this approach for future studies on clinicopathologic correlations and biomarker discovery.

CONCLUSION: Pegcetacoplan may provide therapeutic benefit for C3G and has a favorable safety profile across the 4 glomerular diseases studied.

Kidney function is strongly influenced by genetic factors with both monogenic and polygenic factors contributing to kidney function. Monogenic disorders with primarily autosomal dominant inheritance patterns account for 10% of adult and 50% of paediatric kidney diseases. However, kidney function is also a complex trait with polygenic architecture, where genetic factors interact with environment and lifestyle factors. Family studies suggest that kidney function has significant heritability at...

CONCLUSION: The Edema ClinRO (V1) measure is developed specifically to measure edema in nephrotic syndrome. The tool assesses edema across multiple body parts, and it includes a training module to ensure standardized administration across raters. Future examination of this measure is ongoing to establish its reliability and validity.

No abstract

CONCLUSIONS: Anti-ENO1 and anti-Histones 2A serum levels identify LN patients at the onset of renal symptoms and decrease following response to therapies. Both antibodies are associated with proteinuria and renal function loss. Our data support their use as predictive biomarkers for LN follow-up.

Autoimmune diseases are chronic and heterogeneous disorders resulting from the breakdown of immune tolerance and subsequent tissue damage. Beyond genetic predisposition, viral infections are increasingly recognized as pivotal environmental contributors to disease onset. In this study, we performed comprehensive viral metagenomic profiling of blood samples from 205 patients with systemic lupus erythematosus (SLE), Sjögren's syndrome (SS), ankylosing spondylitis (AS), and undifferentiated...

CONCLUSION: Mental health likely influences how adolescents with rheumatic disease view the impact of their disease on their quality of life and functioning. This is important to consider when applying PGA scores in research studies and clinical care. The rheumatology community must develop efficient ways to better evaluate and treat potential factors mediating their patients' overall health and functioning.

Hemophagocytic lymphohistiocytosis (HLH), a severe, life-threatening hyperinflammatory syndrome driven by dysregulated immune activation, is characterized by rapid clinical deterioration and poor outcomes that pose critical challenges for clinical management. Here, we report on a female patient aged 10 years and 3 months who was diagnosed with systemic lupus erythematosus (SLE)-associated hemophagocytic lymphohistiocytosis with central nervous system (CNS) involvement. Treatment with the...

CONCLUSION: Critically ill children co-managed by rheumatology and intensive care teams most often present with hyperinflammatory syndromes. Early rheumatology involvement and multidisciplinary care appear essential for optimal patient outcomes.

CONCLUSION: In a field often lacking high-quality data, these consensus statements from APLAR provide expert opinion-based guidance to support clinical decision-making. It is envisaged that it will assist in educational and training purposes and help shape future research priorities.

CONCLUSIONS: Neonatal TPN exposure was not independently associated with childhood autoimmune diseases. These findings strongly reassure clinicians that perceived long-term immunological risks reflect baseline clinical severity rather than the TPN intervention itself.

CONCLUSIONS: We have developed updated interdisciplinary clinical practice guidelines to facilitate a patient-centred T2T approach to the management of patients with jSLE.

Neuropsychiatric systemic lupus erythematosus (NPSLE) is a severe and potentially life-threatening complication of systemic lupus erythematosus (SLE), particularly in pediatric populations, in whom central nervous system involvement is often more aggressive and associated with long-term neurocognitive sequelae. Diagnosis remains challenging due to heterogeneous clinical manifestations and the lack of specific biomarkers. Traditionally, management has relied on high-dose corticosteroids,...

CONCLUSIONS: The superior sensitivity and likelihood ratio of the 2023 ACR-EULAR criteria suggest they may be a valuable tool for the classification of paediatric APS; however, further prospective validation is essential to optimise their clinical application.

No abstract

CONCLUSION: These results have the potential to enhance our understanding of cSLE. Further studies must be conducted to expand our findings.

Coccidioidomycosis (CM) is an emerging public health challenge due to an increasing number of cases in the U.S. Diagnosis may be challenging because of its nonspecific presentation, which may lead to treatment delays. In addition, the optimal management of severe pulmonary CM remains unclear. We present the case of an 18-year-old immunocompromised renal transplant recipient with a history of systemic lupus erythematosus who developed acute respiratory distress syndrome (ARDS) secondary to severe...

CONCLUSION: This report describes a novel STAT1 variant consistent with a gain-of-function phenotype associated with disseminated tuberculosis and early-onset SLE, expanding the clinical and molecular spectrum of this disorder. In children presenting with overlapping infectious and autoimmune features, underlying inborn errors of immunity should be considered.

Stress-induced upregulation of interleukin-6 (IL-6) signaling and its downstream pathophysiological consequences have garnered considerable attention in recent years. However, no comprehensive review has specifically examined the association between stress-induced IL-6 and its implications for bone health in children. During childhood, linear growth and peak bone mass acquisition are tightly regulated processes. Hence, this review aims to investigate current evidence on stress-induced IL-6...

CONCLUSION: First-trimester screening using PlGF, PAPP-A, and maternal factors shows preliminary associations with common obstetric complications, suggesting the potential utility of these biomarkers in early risk stratification, providing a proof-of-concept tool for early risk stratification and improved management of high-risk pregnancies to reduce maternal and fetal morbidity. However, these models require validation in larger, more diverse populations before clinical consideration.

Crohn disease (CD), a subtype of inflammatory bowel disease, may be complicated by extraintestinal manifestations. Although cutaneous findings are among the most frequent, metastatic Crohn disease (MCD), defined as mucocutaneous CD at sites noncontiguous with the gastrointestinal tract, is rare. Children with MCD are more likely than adults to develop genital inflammation. Case reports describe the vulva, scrotum, and penis as common locations of involvement in children with genital MCD. We...

The fourth LBMR-Tim (Toulouse Referral Medical Laboratory of Immunology) symposium was convened on December 15th, 2025, in Toulouse, France, to discuss recent advances in the understanding and management of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). Pathophysiological mechanisms underlying SSc and SLE were discussed from a genetic perspective, with particular emphasis on the X-chromosomal TLR7/TLR8 locus and the interferon signaling pathway. Cellular aspects were explored,...

No abstract

CONCLUSIONS: Monogenic causes were found in at least 6.1% of the overall cohort of pSLE and in 22.7% of genetically screened cases, with the highest yield in EOSLE (28.4%). C1QA was the most common single-gene defect (7.2%). These findings underscore the value of genetic testing in pSLE, especially those with EOSLE or suggestive clinical features.

CONCLUSION: Thrombocytopenia frequently occurs in pediatric patients with SLE and demonstrates a significant correlation with leukopenia, the presence of antiphospholipid antibodies, and involvement of major organs. Additionally, further multicenter prospective investigations are necessary to clarify the contribution of platelets to the pathogenesis of SLE. In clinical practice, when thrombocytopenia is identified in pediatric SLE patients, thorough evaluation for antiphospholipid antibodies and...

Paediatric-onset systemic lupus erythematosus (SLE) is a rare but clinically significant condition, often presenting with central nervous system (CNS) involvement. The most common CNS manifestations include headaches, seizures and psychosis. We present two cases of neuropsychiatric SLE with unusual clinical and imaging features.The first case involves an adolescent girl who presented with unilateral vision loss and elevated intracranial pressure. MRI revealed large well-defined ring-enhancing...

CONCLUSION: Our findings do not support the use of the BI multiplex immunoassay as a primary ANA screening tool in the primary-care setting. On the basis of these findings, the Clalit Health Services laboratory network discontinued simultaneous IIF and BI testing.

CONCLUSIONS: Caregivers of children with cSLE face substantial psychological distress, with threat perception and negative coping as key modifiable correlates. Interventions to reshape cognitive appraisal and promote adaptive coping, alongside expanded health insurance coverage and optimised caregiving role distribution, are needed to alleviate caregiver burden.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organ systems. In SLE, T-cell subsets are closely associated with autoimmunity and organ inflammation. Detecting T-cell exhaustion (TEX)-related biomarkers is crucial for early SLE diagnosis and offers new insights into disease mechanisms. Transcriptomic data for SLE were collected from public databases, and TEX-related genes were obtained from the literature. Biomarkers were identified using analyses such...

Recent advances in immunologic and molecular research on rheumatic diseases have significantly improved understanding of the diverse group of immune-mediated disorders. Rheumatic diseases were traditionally considered autoimmune diseases, caused by impaired adaptive immunity and often associated with pathogenic autoantibodies. However, in the late 20th century, certain genetic rheumatic diseases-such as tumor necrosis factor receptor-associated periodic syndrome (TRAPS) and familial...

Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease that disproportionately affects women of reproductive age and is associated with substantial morbidity, premature mortality, and racial and ethnic disparities. Advances in diagnosis and immunosuppressive therapy have improved survival, yet patients remain at increased risk for kidney failure, cardiovascular disease, infection, pregnancy complications, and treatment related damage. Primary care and hospital-based...

Neonatal lupus erythematosus (NLE) is a rare, passively acquired autoimmune disease. The manifestations of NLE are varied, and the outcomes of the majority of cases result in spontaneous resolution. However, severe cases are limited. Emerging evidence highlights the genetic heterogeneity of NLE. The missense variant p.Arg90His in NCF1 has been demonstrated to be associated with autoimmune diseases, including systemic lupus erythematosus. Chromosomal abnormalities manifest as developmental and...

Type I interferon (IFN-I) is a proinflammatory cytokine that activates signalling pathways crucial for antiviral defence in innate immunity. The IFN-I system is a self-preserving mechanism that normally responds to exogenous nucleic acids derived from pathogens, but not to endogenous nucleic acids from host cells. However, genetic defects that constitutively activate this pathway can lead to aberrant IFN-I production (the "IFN signature") and sterile inflammation (autoinflammation), giving rise...

CONCLUSION: Children with AD are more likely to have OSA than children without AD, emphasizing the need for careful management, especially when surgical options are considered.

Systemic lupus erythematosus (SLE) is an autoimmune disease marked by diverse clinical manifestations and profound immune dysregulation. Among the immune cells implicated in SLE pathogenesis, double-negative T (DNT) cells, a unique subset of CD3^(+) T lymphocytes that lack both CD4 and CD8 co-receptors, have emerged as important and context-dependent contributors to disease progression. Pathogenic DNT cells are markedly expanded in SLE patients and disrupt immune tolerance by promoting the...

CONCLUSIONS: This case emphasizes the importance of early recognition and systematic evaluation of complement deficiencies in pediatric patients with lupus manifestations and recurrent infections. Identifying hereditary C1q deficiency has crucial implications for diagnosis, prognosis, and timely selection of advanced therapies, including hematopoietic stem cell transplantation, in severe forms of cSLE.

CONCLUSIONS: Pregnant women with SLE exhibited persistently elevated BAFF levels, which were associated with lower B cell numbers, SLE-related autoantibody positivity and shorter pregnancy duration. Together with a disease-specific placental enrichment of IFNα, these findings support the presence of an inflammatory and potentially pathogenic IFN-BAFF signature in SLE pregnancy. Further studies are needed to determine the functional consequences of these immunological alterations on...

CONCLUSION: Poor sleep quality is common among adolescents with cSLE and is significantly associated with psychosocial distress, particularly reflected in daytime dysfunction. These findings support integrating routine screening of sleep quality and psychosocial distress into comprehensive cSLE care and highlight the need for larger longitudinal studies to clarify causal pathways and identify modifiable drivers of sleep disturbance.

CONCLUSION: IgG AnxA5-Abs and A5R are enriched in Sapporo-defined obstetric APS, with weaker and less consistent associations in thrombosis-only APS. These findings support the biological plausibility of AnxA5 disruption in obstetric APS and identify AnxA5-related biomarkers as promising candidates for future validation. However, prospective external validation using standardized assays, pre-specified thresholds, and demonstration of incremental prognostic value are essential prerequisites...

CONCLUSIONS: In cSLE patients with nephritis, the VD of the SCP decreases further. Although this decline does not parallel the severity of nephritis, it may help identify cSLE patients with nephritis. Key Points • Retinal blood flow of cSLE patients is affected even before visual acuity is impaired or lupus retinopathy develops. • When cSLE patients are complicated with LN, the VD of the SCP is further reduced.

CONCLUSIONS: MMF and CYC had similar efficacy as initial therapies for pLN. RTX may augment long-term response. However, response rates were suboptimal. Large variations in initial therapy were observed. Given the paucity of prospective data in pLN, our study further illustrates the need for data-driven, pediatric-specific protocols to standardize care and improve outcomes.

Chronic granulomatous disease (CGD) is an inborn error of immunity (IEI) characterized by defective NADPH oxidase activity, leading to severe infections, hyperinflammation, and immune dysregulation. Autoimmune manifestations are increasingly recognized, whereas systemic lupus erythematosus (SLE) with renal involvement is exceedingly rare. We report an 11-year-old boy with X-linked CGD who developed SLE complicated by class IV/V lupus nephritis (LN), presenting with progressive cutaneous lesions,...

CONCLUSION: IFI27 accelerates cNPSLE progression by stimulating the IFN-I pathway in CD8^(+) T cells, driving their differentiation into cytotoxic phenotypes, and enhancing microglial activation and neuroinflammation.

CONCLUSION: Baricitinib was well-tolerated and associated with clinical improvement in patients with treatment-refractory monogenic lupus. These findings support its potential therapeutic role and highlight the need for larger, long-term studies.

CONCLUSIONS: Most patients with SLE intended to breastfeed, but this intention decreased during active disease. Hydroxychloroquine PILs appeared to discourage breastfeeding despite the drug's safety.

ObjectiveTo investigate the clinical characteristics of pediatric systemic lupus erythematosus complicated by myositis.MethodsA retrospective analysis was conducted on the clinical data of six patients with SLE-associated myositis, examining manifestations of multisystem involvement, muscle injury markers, and autoantibody profiles.ResultsAmong the six patients, five were female and one male, with a median age of 12 years. All patients presented with myalgia and/or muscle weakness. Physical...

CONCLUSION: Skin manifestations often present as the initial complaint in pediatric rheumatic diseases. A thorough cutaneous examination is crucial for identifying the underlying systemic conditions and guiding appropriate management.

Lupus nephritis (LN) stands out as one of the most critical complications of systemic lupus erythematosus (SLE), affecting almost 60% of the patient population. Even though more therapies have been made available for LN in the past decade, clinical outcomes remain less than ideal: nearly 10% to 30% of LN cases still advance to end-stage kidney disease (ESKD), still making the management of LN a clinical challenge. Therefore, the primary aim of treatment of LN is simple: to halt the progression...

This article presents a case of recurrent autoimmune hemolytic anemia in a child with a gain-of-function (GOF) mutation of the TLR7. This patient's condition contrasts with the six previously documented cases of GOF mutations in the TLR7, thereby expanding the phenotypic spectrum of such mutations and enhancing clinical comprehension of childhood systemic lupus erythematosus (cSLE). The article discusses the mechanisms by which TLR7 GOF mutations can result in autoimmune hemolytic anemia,...

CONCLUSIONS AND RELEVANCE: This study identified novel autoantibody signatures for atherosclerosis progression and statin response in JSLE, with potential utility for precision medicine approaches for CVD-risk management.

CONCLUSION: Associations of SLEDAI-2K domains with damage accrual were variable and were discordant with domain weightings. Alternative weighting strategies for disease activity instruments could improve their prognostic utility.

No abstract

Systemic lupus erythematosus (SLE) is an autoimmune disease that predominantly affects women of childbearing age, and active disease during pregnancy is associated with increased maternal and fetal morbidity. Belimumab is an effective biologic therapy for active SLE; however, its use during pregnancy has long been limited by the scarcity of safety data. Recent evidence and updated international recommendations suggest that belimumab may be considered in selected cases when required to maintain...

CONCLUSION: These findings indicate that Plk1 contributes to the maladaptive downregulation of renal water and sodium transporters during obstruction. Pharmacological inhibition or genetic reduction of Plk1 preserves transporter expression, highlighting the Plk1-cPLA(2)-PGE(2)-NEDD4/NEDD4L axis as a potential therapeutic target for correcting water and sodium imbalance in obstructive nephropathy.

Recognizing its clinical utility, the 2024 Kidney Disease Improving Global Outcomes (KDIGO) guideline recommends using equations that integrate serum creatinine and cystatin C for chronic kidney disease (CKD) diagnosis and staging. Cystatin C-inclusive equations generally outperform creatinine-based equations in bias and accuracy across diverse populations, including children and patients with comorbidities. Moreover, cystatin C-inclusive equations consistently demonstrate greater prognostic...

CONCLUSION: Children and adolescents with primary hypertension demonstrated lower health-related quality of life scores, particularly in physical health-related domains, compared with healthy controls. These findings suggest that primary hypertension may be associated with impaired quality of life in pediatric patients. Larger, multicenter, longitudinal studies are needed to validate these results.

CONCLUSION: This systematic review presented a comprehensive overview of rituximab's pharmacokinetics across various clinical populations. It highlighted the complexity and variability of rituximab's pharmacokinetics. Future research should place emphasis on the model informed precision dosing of rituximab based on pharmacokinetics and various influencing factors in clinical practice.

Adrenal overt Cushing syndrome (CS) is a rare disease but presents with a high risk of serious, life-threatening complications. Although florid CS is less frequently reported now, the interval between symptom onset and diagnosis still remains as long as four years. In this study, we investigated the current status of patients presenting with adrenal overt CS in the 2000s using data from the "Study of Advancing Care and Pathogenesis of Intractable Adrenal Diseases in Japan (ACPA-J study)." Based...

CONCLUSION: Obinutuzumab may be an effective and safe follow-up option for children with RNS who fail to maintain sustained remission after RTX.

Primary enlargement of the cerebrospinal fluid (CSF)-filled brain ventricles, known as congenital cerebral ventriculomegaly (CCV), is a hallmark of congenital hydrocephalus. CCV is also enigmatically but frequently associated with autism and other neurodevelopmental disorders. To gain insight into the developmental genetic regulation of the human CSF-ventricular system, we conducted an integrated, multiomic study of about 2700 trio-based exomes from patients with primary CCV. We found that about...

CONCLUSIONS: Graft survival for kidneys from donors aged 8-18 y is not inferior to that of adult donors with a KDPI of ≤20%. These data support the use of pediatric donors aged 8 y and older for those recipients who traditionally received adult donor kidneys with a KDPI of ≤20%.

CONCLUSIONS: This study reveals the genetic and phenotypic heterogeneity of primary SRNS in Indonesian children. The identification of diverse pathogenic variants underscores the utility of WES, even in cases responsive to cyclosporin. Genetic testing should be integrated into routine SRNS evaluation, especially in atypical presentations or early-onset of the disease.

CONCLUSIONS: Our study identifies oxalate as a systemic immunometabolic stressor and IL-17A as a central mediator of oxalate-induced cardiorenal injury. These findings establish the oxalate-IL-17A axis as a mechanistic link between CKD and CVD and suggest IL-17A inhibition as a potential therapeutic strategy to reduce cardiovascular damage in CKD.

CONCLUSION: Pediatric GS shows significant clinical heterogeneity. Severe hypokalemia is accompanied by early dyslipidemiaindicates. SLC12A3 variants are diverse; functional domain alone cannot predict phenotype. Early screening and long-term follow-up are essential for optimal management.

Lipoprotein glomerulopathy is a rare, inherited kidney disorder caused by pathogenic APOE variants that leads to obstruction of glomerular capillaries by lipoprotein thrombi, steroid-resistant nephrotic syndrome, and progressive chronic kidney disease. Immunosuppressive therapy is ineffective; instead, lipid-lowering measures reduce intraglomerular lipoprotein deposition and preserve kidney function. We describe a 23-month-old boy presenting with nephrotic syndrome and severe anaemia. Family...

CONCLUSIONS: PCOQ10D exhibits marked phenotypic heterogeneity, characterized by variations in age of onset, organ involvement, and clinical severity, as well as significant interindividual differences in treatment responses to CoQ10 supplementation. This case expands the phenotypic spectrum of the disease. Moreover, the therapeutic outcomes suggest that all diagnosed patients require long-term supplementation with adequate doses of CoQ10, which is of great significance for delaying disease...

CONCLUSION: Nurses are pivotal in the successful implementation and sustainability of remote patient care (RPC) platforms, yet they require adequate time and organisational support. As primary users alongside patients, nurses should actively be involved in remote patient care (RPC) practices and take leadership in defining their responsibilities. This study enhances understanding of nurses' experiences with remote patient care (RPC), emphasizing the importance of their role in maintaining the...

CONCLUSIONS AND IMPLICATIONS OF KEY FINDINGS: Age at onset and cumulative initial dose of prednisolone, and not duration of extended therapy, determine disease course in pediatric steroid-sensitive nephrotic syndrome. The benefit of extending initial therapy on risk of first and frequent relapses in young patients requires examination in controlled trials using BSA-based dosing.

CONCLUSIONS: The IR-FI are significantly associated with the incidence of cardiovascular events, an association that appears to be mediated through systemic inflammatory pathways. These novel integrated indices represent promising biomarkers for early screening and targeted clinical intervention among individuals with CKM syndrome stages 0-3.

CONCLUSIONS: These preliminary results suggest that obinutuzumab is a promising first-line anti-CD20 therapy for FRNS and SDNS in children, offering sustained remission with a good safety profile.

The parental imprinted GNAS complex locus encodes the α-subunit of the stimulatory G protein. Pseudohypoparathyroidism type Ib (PHP1B) is characterized by renal resistance to parathormone and is caused by epigenetic anomalies, ie, at least the loss of methylation (LOM) at the A/B differentially methylated region (DMR). LOM or gain of methylation (GOM) may also affect other DMRs and different transcripts expressions within the locus (NESP, exon H, AS1, XLαs, A/B). Most PHP1B are sporadic and...

CONCLUSION: Abnormal high delta power may be indicative of clinically apparent encephalopathy in young patients with kidney failure. The role of increased functional connectivity as potential compensatory mechanism should be further explored.

CONCLUSIONS: Children undergoing peritoneal dialysis experience significant emotional, physical and psychosocial burdens. Family-centred nursing care, routine psychological screening, proactive school liaison and structured peer support are essential to improve outcomes for this population.

CONCLUSIONS: Our study demonstrated the feasibility of identifying a real-world pediatric cohort with CKD and severely increased proteinuria using adapted criteria from the FIONA trial. Findings from this study highlight factors that should be considered in the design of future hybrid studies evaluating treatment effectiveness using data from both trials and the real world.

CONCLUSIONS: Kidney transplantation in carefully selected older recipients, including those aged ≥ 70 years, was associated with sustained patient and graft survival. Infectious complications occurred frequently and constituted the leading attributed cause of DWFG; this underscores the importance of understanding why some recipients recover from infectious stress, while others experience subsequent clinical deterioration.

Adenosine deaminase 2 deficiency (DADA2) is an inborn error of immunity leading to systemic vasculitis, haematological manifestations, immune deficiency and/or autoimmunity. We report the case of a 26-year-old female with an initial diagnosis of systemic lupus erythematosus (SLE). However, atypical evolution patterns for SLE (hypogammaglobulinaemia, moderate B-cell lymphopenia, disappearance of anti-dsDNA) led to the identification of a homozygous class 5 missense variant of CECR1/ADA2, thus to...

No abstract

BACKGROUND Respiratory tract infections (RTIs) are a major complication and prognostic determinant in children with nephrotic syndrome (NS), yet reliable predictors for infection risk remain lacking. Although fibrinogen (FIB) and urinary a1-microglobulin (alpha1-MG) have been individually linked to inflammation and renal injury, their combined prognostic value in pediatric NS has not been established. This study investigated the correlation between FIB, urinary alpha1-MG, and RTI occurrence in...

CONCLUSIONS: Pediatric cancer diagnosis communication in Japan is shaped by five approaches that prioritize the child's best interests. Locally grounded strategies highlight the importance of trust, relationship building, and being present, but may limit children's voices when family preferences dominate. It is essential to employ nurses who specialize in pediatric cancer care. Future research should incorporate the children's and families' perspectives and evaluate culturally sensitive...

Alport syndrome (AS) is a genetically heterogeneous disorder caused by mutations in type IV collagen genes, clinically characterized by progressive renal function deterioration. Despite advances in genetic screening technologies, cases resulting from non-canonical splice site variants remain diagnostically challenging, frequently leading to missed diagnoses or delayed therapeutic intervention. Here, we report a pediatric patient with AS and his mother, both harboring a novel splicing variant in...

CONCLUSION: The findings suggest that pressure-related factors may contribute more strongly to renal injury than the isolated pathogen itself. Long-term renal protection should prioritize storage pressure control and upper tract drainage. Zusammenfassung Hintergrund: Bei Kindern mit neurogener Blase steuern Urinkulturen und Uropathogenmuster häufig klinische Entscheidungen. Ob diese Muster über anatomische und urodynamische Faktoren hinaus zur Nierenschädigung beitragen, ist unklar.

To investigate the frequency and characteristics of HCQ-induced pigmentation in Japanese paediatric patients. This retrospective cohort study enrolled Japanese children who received HCQ for ≥ 3 months. Data were collected from medical records, caregiver or patient questionnaires on awareness of pigmentation, and physical examination findings. The primary outcome was the frequency of HCQ-induced pigmentation. Twenty-six patients were included. Of these, 25 (96.2%) were female (median age: 15...

CONCLUSION: VEO-ADPKD represents a distinct, high-risk pediatric phenotype characterized by earlier and more severe kidney involvement. These findings emphasize the importance of early diagnosis, comprehensive genetic evaluation, and close monitoring. Genetic testing is essential to identify children with biallelic mutations, representing a clinical entity more akin to mild autosomal recessive polycystic kidney disease. Standardized phenotyping and prospective pediatric studies are needed to...

Current guidelines for the management of meticillin-susceptible Staphyloccocus aureus bloodstream infection/bacteraemia (SAB) recommend intravenous (flu)cloxacillin as the first-line treatment. This is based on decades of clinical practice. The high acute kidney injury rates seen with (flu)cloxacillin in the recently published SNAP trial, which is the largest randomized clinical trial ever in S. aureus bacteraemia (SAB), shows us the need to regularly test and question our usual clinical...

Endocrine hypertension refers to elevated blood pressure resulting from an identifiable hormonal cause that is potentially treatable. Endocrine hypertension occurs in ∼30% of young adults with hypertension (18-40 years), in 30-50% of patients with resistant hypertension, most commonly due to primary aldosteronism, and can occur in a small yet clinically significant subset of children with hypertension. Other causes of endocrine hypertension include excess mineralocorticoid production, cortisol...

Introduction. The need to perform voiding cystourethrography (VCUG) in patients with a first episode of urinary tract infection (UTI) caused by atypical pathogens is controversial. The primary objective of this study was to evaluate the diagnostic accuracy of renal ultrasound for predicting vesicoureteral reflux (VUR) in these patients. Population and methods. Retrospective study of diagnostic accuracy. We analyzed medical records of patients under 2 years of age with a first episode of UTI...

CONCLUSION: This review maps the evidence on educational support programs for HD patients, revealing variability in intervention types, delivery methods, study designs, and outcome measures. These inconsistencies highlight the need for more rigorous, standardized research particularly in LMICs to determine the most effective educational strategies. The findings provide a foundation for developing evidence-based, patient-centered educational programs adaptable to diverse global populations.

CONCLUSION: The advent of targeted treatment opportunities for PH1 comes with an increased need to provide guidance to the field. Filling the existing gaps will ensure that a growing number of patients get access to optimal care and novel life-changing therapies.

CONCLUSION: This case illustrates how extended terminal deletions of 10q can disrupt key structural and metabolic gene networks. To our knowledge, this is the first transcriptomic characterization of a 10.77 Mb deletion in the 10q26.13-q26.3 region together with a large terminal 4q duplication. Integrating functional transcriptomics with clinical and cytogenetic data may enhance our understanding of rare chromosomal disorders and inform individualized management, including reproductive...

CONCLUSIONS: Higher time-adjusted cHDAS was associated with an increased risk of damage accrual, indicating that greater cumulative exposure to high disease activity is associated with damage. Neuropsychiatric involvement and time-adjusted cHDAS ≥5% were significantly associated with damage accrual.

CONCLUSIONS: Children younger than 6 years and individuals not using glucose-responsive insulin therapy were less likely to meet the recommended glycemic targets. It is imperative to minimize these disadvantages.

CONCLUSIONS: Low-dose SC OFA is a feasible preemptive therapy for remission maintenance in pediatric FR/SDNS, particularly for those with RTX resistance or intolerance. This outpatient-compatible route may reduce hypersensitivity risks and healthcare costs. Larger prospective trials are warranted to confirm long-term efficacy.

Autoinflammatory diseases are increasingly defined at the genetic and pathway levels, yet therapeutic predictability has not advanced in parallel. This disconnect is especially evident in monogenic disorders, in which molecular diagnosis can identify the initiating lesion but does not reliably predict the dominant effector process, organ-specific risk, or the treatment most likely to achieve durable control. Similar clinical phenotypes may arise from distinct molecular defects, whereas...

[This corrects the article DOI: 10.1016/j.isci.2023.107019.].

CONCLUSIONS: Urinary trehalase, particularly when adjusted for urinary creatinine may capture early tubular injury in dehydrated children before serum creatinine rises. Larger studies are needed to establish clinically useful cut-offs and to confirm diagnostic performance.

CONCLUSION: This study highlights the particularly early kinetics of CMV, BK virus, and EBV in our pediatric patients after HSCT, with CMV appearing around week 3, transient EBV reactivations in initially seronegative patients, and an early BK virus peak linked to hemorrhagic cystitis.

CONCLUSIONS: Long-term linear growth was largely preserved in children with difficult-to-treat NS, and short stature was uncommon. Favorable growth outcomes appear achievable with contemporary management strategies incorporating effective disease control and steroid-sparing therapy.

No abstract

CONCLUSION: Kidney genetics care is expanding but remains unevenly implemented. Nephrologist-led models can be effective with support. Patient selection may influence diagnostic yield more than testing modality. Standardized outcome reporting and theory-driven implementation evaluation are essential for equitable and sustainable genomic services.

CONCLUSION: The SONG-LP instrument is an internally consistent and reliable measure of LP for people receiving dialysis. This study provides preliminary evidence supporting its psychometric validity, offering a foundation for further evaluation of its use in clinical trials.

Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura, is a small‑vessel vasculitis characterized by immunoglobulin A-predominant immune complex deposition in vessel walls and the glomerular mesangium. Although most commonly observed in children, adult‑onset disease is less frequent and is associated with a higher risk of significant renal involvement and poorer clinical outcomes. We report the case of a 56‑year‑old man with alcoholic cirrhosis and chronic...

CONCLUSIONS: These findings establish an AI-based framework for therapeutic ASO design and provide proof-of-concept evidence that targeting the 3'UTR of LMNA transcripts can effectively suppress pathogenic lamin A isoforms and ameliorate systemic progeroid phenotypes.

No abstract

CONCLUSION: ESKD involvement in U.S. mortality rose from 1999 to 2023 with marked subgroup inequities and shifts in underlying-cause pathways. Monitoring ESKD as a contributing cause, together with cause-structure and scale-penetration analyses, provides information beyond underlying-cause surveillance to support integrated prevention addressing CKD progression and its cardiometabolic and infectious complications.

No abstract

[This corrects the article DOI: 10.1016/j.isci.2023.107019.].

Background: Post-COVID-19 condition (PCC) is a complication following acute COVID-19 infection, which may lead to long-term cardiac abnormalities. This review aimed to assess the prevalence of structural/functional deviations in echocardiography in individuals with PCC compared to patients without PCC. Methods: We searched three databases. Two reviewers independently screened articles using LASER Al and extracted relevant data using a piloted Excel sheet. We performed meta-analysis using...

CONCLUSIONS: The COVID-19 pandemic has had a significant impact on the incidence of HUS in the pediatric population. SARS-CoV-2 infection most markedly increases the risk of neurological complications but does not affect overall mortality.

BACKGROUND: Enteric infectious diseases claim more than 1 million lives annually and are among the top ten causes of death in children younger than 5 years. Remarkable global investment has been dedicated to enteric infectious disease prevention and control; however, the shifting global health landscape is testing the continuance of progress. To evaluate the current status and guide future interventions, we present the latest epidemiological estimates of enteric infectious diseases from the...

The activation of pattern recognition receptors (PRRs) orchestrates inflammation and regulates adaptive immunity. To test whether tuning inflammation through PRR stimulation enhanced the efficacy of mRNA vaccines, we combined an mRNA-based vaccine generated against the ancestral spike protein of SARS-CoV-2 with mannadjuvant, a formulation of fungal mannan and aluminum hydroxide targeting the PRR dectin-2. In mice and non-human primates, mannadjuvant increased the magnitude and durability of the...

CONCLUSIONS: S. pneumoniae was detected in 20.4% Iranian children's NP samples, with the serotype 23 F dominancy. Emerging of 15B and lower PCVs prediction coverage compared to other unvaccinated countries, might stem from limited administration of PCV13, environmental/epidemiological factors, and COVID-19-related shifts in colonization. Ongoing surveillance of pneumococcal carriage, serotype distribution, and antimicrobial resistance are required due to the existence of antibiotic resistance...

Multisystem inflammatory syndrome in children (MIS-C) is a severe complication of SARS-CoV-2 infection. The long-term impact on vascular and cardiac health in post-MIS-C patients remains unclear. We aimed to evaluate subclinical cardiovascular changes in children two years after MIS-C. This cross-sectional study included 42 children diagnosed with MIS-C (29 boys, 13 girls, median age 10.7 years) and 38 age- and sex-matched healthy controls. Participants underwent comprehensive cardiovascular...

PURPOSE: Canadian researchers have made significant contributions to the advancement of organ transplantation globally. The COVID-19 pandemic made transparent the importance of reflecting on our accomplishments and the current and future challenges that limit the lives of our patients and to celebrate individual and collective achievement.

Multisystem inflammatory syndrome in children (MIS-C) is a rare hyperinflammatory disorder that occurs in previously healthy pediatric patients after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) exposure or mild infection. MIS-C typically has mild kidney symptoms that resolve quickly. The kidney biopsy experience in pediatric coronavirus disease 2019 (COVID-19) and MIS-C is limited in the literature. Here, we describe a 17-year-old SARS-CoV-2 positive boy with features of MIS-C...

BACKGROUND: Although individual rare and complex diseases (RDs) affect small patient populations, together they impact an estimated 27–36 million people across the European Union. Addressing this major public health challenge has been a long-term priority for the European Union, leading to the establishment of the European Reference Networks (ERNs) in 2017. MAIN BODY: ERNs are cross-border networks connecting clinical expert centres to share knowledge, improve and harmonise diagnosis and care...

CONCLUSION: SOT recipients in Canada, especially lung transplant recipients, experience high rates of hospitalization, SCU admission, and in-hospital mortality. Notable differences observed between organ subtypes for admissions with and without a COVID-19 diagnosis may reflect differences in immunosuppressive medication regimens, informing areas for future research.

CONCLUSIONS AND RELEVANCE: This multicenter cohort study found that among severely ill adults hospitalized with babesiosis, the adjusted risk of in-hospital death or 30-day readmission was nearly 5-fold lower in those treated with ET vs those not treated with ET. These data support ET for severely ill patients with babesiosis, although the findings may be susceptible to unmeasured confounding. Further research is needed to identify which patients are most likely to benefit.

CONCLUSIONS: In this Registry, CytoSorb® therapy was associated with significant early clinical benefits in patients with septic shock, including hemodynamic stabilization and improved fluid balance. Further systematic research is needed to optimize its use and identify patient populations that benefit most.

CONCLUSIONS: hMPV causes clinically significant disease in patients with hematological malignancy, often necessitating hospital and ICU care, and leading to mortality. In the absence of specific treatments or vaccines, this virus remains an underrecognized pathogen in patients with hematological malignancy. Enhanced clinical awareness and investment in diagnostics, prevention, and therapeutics are needed.

Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C), associated with SARS-CoV-2 infection share overlapping clinical and laboratory features, making differential diagnosis particularly challenging during the COVID-19 pandemic. Accurate distinction is essential due to differences in pathophysiology, management strategies, and cardiovascular outcomes. We report the case of a 7-year-old boy presenting with prolonged fever, mucocutaneous manifestations, arthritis, and...

Pediatric patients with SARS-CoV-2 infection are at an increased risk of severe disease and adverse outcomes. Nevertheless, comprehensive data on COVID-19 vaccine effectiveness (VE) in children with diabetes during the post-pandemic period remain limited. This study assessed the VE against severe COVID-19 outcomes during both the pandemic and post-pandemic phases in children with and without diabetes mellitus (DM). A cohort study based on population data was carried out, including all patients...

CONCLUSIONS: The rate of paediatric KT in Europe has remained stable, with differences between GDP groups. Low-GDP countries had the lowest KT rates, but with an increasing trend over time. Opportunities to further increase access to paediatric KT should be explored.

CONCLUSION: Mean ISE performance declined from 2016 to 2023 across all PGY levels, with the greatest decreases observed in general urology subtopics. Scores after 2020 were lower across most PGY levels and content domains, while performance on repeated questions remained stable. These trends may reflect increased examination difficulty, expanded content, changes in question composition, evolving study strategies, and variability in clinical exposure rather than diminished knowledge.

CONCLUSIONS: Our results suggest that vaccination provided similar protection against COVID-19-related mortality in individuals with and without schizophrenia. However, the magnitude of the intervention effect was double for individuals with schizophrenia due to their higher baseline risk.

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CONCLUSIONS: Our findings in this exploratory observational study suggest that higher frequencies of atypical B cells in the peripheral blood of pediatric SOTRs may identify intact cellular but absent humoral responsiveness to vaccination. Intact T cell responsiveness to antigens may be sufficient to monitor protective immunity after vaccination in SOTRs.

BACKGROUND: Lower respiratory infections (LRIs) remain the world's leading infectious cause of death. This analysis from the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2023 provides global, regional, and national estimates of LRI incidence, mortality, and disability-adjusted life-years (DALYs), with attribution to 26 pathogens, including 11 newly modelled pathogens, across 204 countries and territories from 1990 to 2023. With new data and revised modelling techniques,...

CONCLUSION: The intensity and the nature of the anti-viral immune alterations depend on the type and the degree of the immune impairment. Evaluating the specific host immune actors responsible for maintaining a protective response appears essential to adapt vaccine strategy in these patients, opening the door to new, more personalized vaccination approaches.

Avian influenza A virus (IAV) H5N1 is an emerging threat of human pandemic. We describe a 71-year-old man who died of H5N1 pneumonia in Louisiana and whose blood contained autoantibodies neutralizing type I IFNs (AAN-I-IFNs), including the 12 IFN-α subtypes (1-10 ng/ml) and IFN-ω (100 pg/ml). Causality between these AAN-I-IFN and lethal outcome of avian influenza in this patient is based on (1) our previous report that AA-I-IFN underlie about 5% of cases of critical pneumonia triggered by...

Viral infections are well-known causes of systemic illness in children, but their kidney involvement, particularly acute tubulointerstitial nephritis (TIN), remain underdiagnosed and clinically underestimated. A wide range of viruses has been implicated in pediatric TIN, including Epstein-Barr virus, cytomegalovirus, BK virus, parvovirus B19, respiratory syncytial virus, and SARS-CoV-2. Among these, adenovirus stands out for its potential to cause severe kidney injury. Delayed diagnosis remains...

Coronavirus disease 2019 (COVID-19) vaccine has been extended to children 6 months and older and boosters to those 12 years and older, and vaccine safety continues to be monitored. A 12-year-old female presented with non-oliguric acute kidney injury 6 days after receiving the second dose of Pfizer COVID-19 vaccine. Renal biopsy revealed idiopathic severe acute tubulointerstitial nephritis (TIN), which had a temporal relationship with the second dose of the COVID-19 vaccine. Patient received...

CONCLUSION: Critical pulmonary impairment after mild COVID-19 is rarely detected by spirometry and DLCO but may affect the LCI. Within 3 months, impaired pulmonary function improved in most patients. Children were less affected by severe pulmonary sequelae and respiratory complaints than adults. Complaints like dyspnoea or chest pain may be an early indicator of lung function impairment, suggesting that further diagnostic tests for treatable post-COVID-19 complications may be needed....

COVID-19 signs and symptoms varied among patients, with the most common being fever, fatigue, sore throat, cough, anorexia, and shortness of breath. (1) Background: This study aimed to assess effort, dyspnea, and cooperation scores in patients with mild and moderate post-COVID-19 forms, both at baseline and after completing a structured physical recovery program. (2) Methods: Our study included 160 post-COVID-19 patients who had experienced mild or moderate disease. (3) Results: Effort and...

CONCLUSION: This study comprehensively analyzes the current research landscape and identifies key hotspots in influenza co-infection. The findings offer crucial guidance for future studies in this field.

CONCLUSION: 15-19% of youth and young adults with CKD endorsed elevated rates of C19-associated emotional distress and worry. Findings suggest that children with poorer kidney function and lower income were more likely to endorse distress and worry related to C19.

BACKGROUND: For more than three decades, the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) has provided a framework to quantify health loss due to diseases, injuries, and associated risk factors. This paper presents GBD 2023 findings on disease and injury burden and risk-attributable health loss, offering a global audit of the state of world health to inform public health priorities. This work captures the evolving landscape of health metrics across age groups, sexes, and...

CONCLUSION: Asthma is associated with lower odds of death, but the strength of this protective association diminishes in early adulthood and again in later life. These age-related differences warrant further investigation and, if confirmed, could inform age-tailored care strategies. Maintaining broad vaccine coverage and timely antiviral use remains advisable for all patients. Future studies that incorporate detailed information on asthma control, medication adherence and lifestyle factors are...

CONCLUSION: 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.

CONCLUSIONS: Between 2020 and 2021, COVID-19 intervention measures significantly lowered the transmission of Mycoplasma pneumoniae. However, data from 2022 suggest a risk of rebound. We need to be alert the possible resurgence of Mycoplasma pneumoniae in children. This calls for clinical action: increasing polymerase chain reaction (PCR) testing during the seasonal peak and focusing on monitoring school-aged children and girls.

INTRODUCTION: Our aim is to develop a Framework of Measurement for people living with Long COVID and their caregivers for use in Long COVID research and clinical practice. Specifically, we will characterise evidence pertaining to outcome measurement and identify implementation considerations for use of outcome measures among adults and children living with Long COVID and their caregivers.

Prognostic scores that help allocate resources and time to the most critical patients could have potentially improved the response to the SARS-CoV-2 pandemic. We assessed the performance of five risk scores in predicting death or transfer to the intensive care unit (ICU) or sub-intensive care unit (SICU) in hospitalised patients with SARS-CoV-2 infection, with the three aims of retrospectively analysing the effectiveness of these tools, identifying frail patients at risk of death or...

Advanced age is the most important risk factor for severe disease or death from COVID-19, but a thorough mechanistic understanding of the molecular and cellular underpinnings is lacking. Multi-omics analysis of 164 samples from SARS-CoV-2-infected persons aged 1 to 84 years reveals a rewiring of type I interferon (IFN) signaling with a gradual shift from signal transducer and activator of transcription 1 (STAT1) to STAT3 activation in monocytes, CD4^(+) T cells, and B cells with increasing age....

Post-Acute Sequelae of SARS-CoV-2 infection (PASC or "Long COVID"), includes numerous chronic conditions associated with widespread morbidity and rising healthcare costs. PASC has highly variable clinical presentations, and likely includes multiple molecular subtypes, but it remains poorly understood from a molecular and mechanistic standpoint. This hampers the development of rationally targeted therapeutic strategies. The NIH-sponsored "Researching COVID to Enhance Recovery" (RECOVER)...

CONCLUSION: This study underscores the need for personalized follow-up care for pediatric patients recovering from COVID-19. Comprehensive monitoring and support programs are crucial for addressing the specific complications observed in this population, thereby ensuring improved long-term outcomes.

Nicotinamide adenine dinucleotide (NAD^(+)) plays an important role in the innate immune response and is depleted during SARS-CoV-2 infection due to increased turnover. It is unknown whether treatment with NAD^(+) precursors can safely raise NAD^(+) levels in patients with COVID-19. To determine whether MIB-626 (β-nicotinamide mononucleotide), an NAD^(+) precursor, can safely increase blood NAD^(+) levels and attenuate acute kidney injury (AKI) and inflammation in hospitalized patients with...

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CONCLUSION: Initiating a CRRT program in LMICs is feasible despite challenges. Creating a team with members willing to shoulder additional responsibility and training them gave impetus to our program. Tapping governmental and non-governmental support helped us circumvent financial challenges. However, in a resource limited setting, sustainability requires in-house technical and financial support. Survival to discharge was 25%, with hyperlactatemia at CRRT initiation predicting mortality.

Recent literature gives different results on morbidity and mortality after COVID-19 hospitalization as compared to Influenza. In this registry-based study in Austria, we compared the short- and long-term outcomes after COVID-19 or Influenza hospitalization and associations with their baseline medication load. Data were provided on children and adolescents hospitalized with COVID-19 (sample size: 1061) in the years 2020 and 2021 or with Influenza in 2016-2021 (sample size: 2781) as well as on...

The COVID-19 pandemic has catalyzed the application of advanced digital technologies such as artificial intelligence (AI) to predict mortality in adult patients. However, the development of machine learning (ML) models for predicting outcomes in children and adolescents with COVID-19 remains limited. This study aimed to evaluate the performance of multiple machine learning models in forecasting mortality among hospitalized pediatric COVID-19 patients. In this cohort study, we used the...

The 5th International Symposium on Regulatory Autoantibodies Targeting GPCR (RAB-GPCRs) advanced the understanding of the significant role played by autoantibodies targeting G-protein-coupled receptors (GPCRs) in various human diseases. Once considered passive markers, RAB-GPCRs are now recognized as active modulators of cellular signaling, immune regulation, and inflammation. The symposium highlighted their involvement in multiple prominent pathologies, including autoimmune diseases, cardio-...

During the COVID-19 pandemic, adenoviral vaccines drew attention owing to a potential life-threatening coagulation disorder, the vaccine-induced immune thrombotic thrombocytopenia (VITT). Patients deceased of VITT have been accepted as organ donors despite safety concerns regarding the transmission of VITT to recipients. The outcome of adult kidney graft recipients was reported favorable in most cases; however, (thrombotic) complications were observed more frequently. We present 2 pediatric...

Cystinuria, characterized by defective renal absorption of cystine causing recurrent nephrolithiasis, demands ongoing management. This study examines the effects of COVID-19-related disruptions in tiopronin availability on the clinical outcomes of pediatric cystinuria patients. This retrospective cohort study analyzed medical records of 11 pediatric patients with cystinuria, followed from 2001 to 2023. Patients were diagnosed using urine microscopy/biochemistry and stone composition analysis....

To investigate the real-world effectiveness of COVID-19 vaccines in a large cohort of patients with diabetes mellitus (DM), we analyzed all >18-year-old patients with COVID-19 registered in a Brazilian nationwide surveillance database between February 2020 and February 2023. The primary outcome of interest was vaccine effectiveness against death, evaluated using multivariate logistic regression models. Among the 2,131,089 patients registered in the SIVEP-Gripe, 482,677 (22.6%) had DM. After...

CONCLUSION: In this study, the amount of albuminuria was independently associated with the yearly loss of kidney function in patients with AS. Combined measurement of albuminuria and urinary IgG may identify patients with the highest risk of rapid decline in kidney function. Following external validation in a larger, prospective cohort, this approach could be used to identify patients who could potentially benefit from closer monitoring and earlier intervention.

CONCLUSIONS: Despite moderate awareness of EULAR CV recommendations, substantial knowledge gaps and practical barriers persist, indicating the need for focused education and improved clinical pathways to enhance CV risk management in RMD care.

CONCLUSIONS: Pegcetacoplan demonstrated rapid and sustained efficacy with good safety despite two potential drug-related concerns in C3G and IC-MPGN, highlighting its potential for broader application and the need for further research to optimize patient selection and treatment strategies.

[This corrects the article DOI: 10.3389/fimmu.2026.1809192.].

Acute kidney injury (AKI) is common in hospitalized patients, and its incidence is rising sharply in intensive care units. It is associated with significant morbidity and mortality due to a profound change in its epidemiological profile - multifactorial in origin, often septic, and associated with other organ failures. The mortality rate reaches 30-50% in the most severe forms, particularly when AKI requires renal replacement therapy (RRT). Temporary RRT, when indicated, must be part of an...

CONCLUSIONS: In frail, ASCT-ineligible patients with biopsy-proven renal AL amyloidosis, daratumumab monotherapy yielded higher hematologic and renal response rates compared with bortezomib-based regimens. These findings support early anti-CD38 therapy as a potential strategy to improve renal preservation and survival, warranting confirmation in multicenter trials.

CONCLUSIONS: The non-neoplastic renal parenchyma in renal cell carcinoma patients frequently exhibits occult pathological changes, predominantly tubulointerstitial damage likely driven by the tumor microenvironment. The study highlights a higher-than-expected prevalence of undiagnosed nephropathies (24%), including paraneoplastic cases. Routine histological evaluation during radical nephrectomy is essential for optimizing patient management, avoiding unnecessary subsequent biopsies, and guiding...

CONCLUSIONS AND CLINICAL IMPLICATIONS: This summary of the 2025 EAU/ESPU/ERN eUrogen/ERN ITHACA/ERN ErkNet/IFSBH guideline provides updated guidance for evidence-based management of children and adolescents with spinal dysraphism.

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CONCLUSION: This real-world study suggests a potential nephroprotective role of SGLT2i in adult patients with AS, including heterozygous COL4A3/COL4A4 carriers. Benefits appeared independent of baseline BMI and renal function, supporting consideration of earlier initiation. Prospective studies are required to validate these findings and refine treatment timing.

BACKGROUND: Although individual rare and complex diseases (RDs) affect small patient populations, together they impact an estimated 27–36 million people across the European Union. Addressing this major public health challenge has been a long-term priority for the European Union, leading to the establishment of the European Reference Networks (ERNs) in 2017. MAIN BODY: ERNs are cross-border networks connecting clinical expert centres to share knowledge, improve and harmonise diagnosis and care...

CONCLUSIONS: Patients carrying monoallelic COL4A3 p.Gly407Arg pathogenic variant exhibit variable phenotypic expression, with proteinuria representing the strongest predictor of renal function decline.

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CONCLUSION: Our case suggests that rituximab, without cyclophosphamide, may represent a promising therapeutic approach in children with double-seropositive anti-GBM disease, even in severe presentations.

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Background: Antiphospholipid syndrome (APS) is diagnosed by characteristic clinical manifestations supported by positivity for lupus anticoagulant, anticardiolipin, and anti-β2-glycoprotein I antibodies. However, a proportion of patients, especially those with systemic lupus erythematosus, remain seronegative despite high clinical suspicion. Anti-phosphatidylserine/prothrombin antibodies (aPS/PT) have emerged as potential biomarkers in this setting. We conducted an expert perception-based Health...

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CONCLUSION: In clinically euvolemic children on dialysis, the combined use of LUS, BIS, and IVC-CI (multiparametric approach) effectively quantified subclinical hypervolemia, which was correlated with the risk of LVH.

Primary hyperoxalurias (PHs) are a group of rare autosomal recessive disorders of glyoxylate metabolism leading to excessive oxalate production, recurrent nephrolithiasis, nephrocalcinosis, and progression to kidney failure with systemic oxalosis in the most severe forms. Until recently, treatment options were limited to conservative measures and double liver/kidney transplantation. The advent of small interfering RNA therapies has revolutionized the field by enabling targeted hepatic enzyme...

CONCLUSION: Although guidelines recommend vaccination alone, our findings indicate that combined protection offers substantially greater protection against IMD in patients receiving long-term C5i. Continued prospective monitoring will be essential to define the optimal preventive strategies in this high-risk population.

Refractory lupus nephritis (LN) poses a significant clinical challenge in the management of systemic lupus erythematosus (SLE) due to its resistance to conventional immunosuppressive therapies. This study evaluates the immunological, anti-inflammatory and anti-fibrotic effects of daratumumab, a CD38-targeting monoclonal antibody, in patients with refractory LN who failed standard treatments. Previous findings demonstrated daratumumab safety and efficacy, improving renal function and reducing...

Cystinosis is a rare autosomal recessive lysosomal storage disorder characterized by systemic cystine accumulation. Cysteamine is the only currently approved cystine-depleting therapy, available in immediate- and delayed-release (DR cysteamine) formulations. DR cysteamine contains methacrylic acid copolymer, an excipient associated with fibrosing colonopathy in patients with cystic fibrosis. Here, we report on a case of a 10-year-old girl with cystinosis who developed severe gastrointestinal...

CONCLUSION: There is significant global variability in the spectrum of diseases leading to pediatric KF, partially attributable to genetic, environmental, and macroeconomic factors.

Polycystic liver disease (PLD) is a rare genetic disorder characterised by progressive liver enlargement due to multiple cysts. The main symptoms are liver volume-related. Although randomised controlled trials have shown that somatostatin analogues (SSAs) reduce liver volume as well as symptoms, specific guidance on when and how to use SSAs in clinical practice is still lacking. A panel of 15 hepatologists and nephrologists developed practical guidance on SSA use, based on a systematic...

CONCLUSION: Thrombocytopenia in APS patients, particularly in severe cases, correlates with heightened thrombotic risk and systemic manifestations. These findings highlight the importance of customized strategies that balance thrombosis prevention with bleeding risk, especially in complex cases.

CONCLUSIONS: Using data-driven and consensus methodology, EAPSDAS was developed and initial validation was performed. Further validation in prospective studies is warranted.

Congenital lower urinary tract obstruction (cLUTO) describes a heterogeneous spectrum of congenital lower urinary tract defects with variable postnatal outcomes, ranging from high morbidity and mortality to spontaneous resolution. In the past, fetal intervention studies aimed at mitigating the disease sequelae of cLUTO have yielded inconclusive results, which contributed to the current heterogeneous antenatal management of fetuses with cLUTO across fetal surgery centers. The recent development...

CONCLUSIONS: Advanced PH1 is associated with high morbidity and mortality rates.

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CONCLUSIONS: This study strengthens the real-world evidence on aHUS and adds to previously published Global aHUS Registry data. In addition, it provides insights into the differential epidemiology of the disease in Belgium and demonstrates the increased susceptibility of women to aHUS across the whole spectrum of recognized complement gene variants.

CONCLUSIONS: TAK is a rare, potentially life-threatening large-vessel vasculitis. Early recognition is crucial for timely diagnosis and aggressive treatment initiation. Children with TAK often experience a complex disease course requiring multiple treatment adjustments and surgical or endovascular interventions. Large, multinational collaborations are essential for advancing our knowledge and improving patient outcomes.

Cystinosis is a rare monogenic autosomal recessive disorder caused by pathogenic variants in the CTNS gene, encoding cystinosin. Loss-of-function of cystinosin leads to intralysosomal cystine accumulation, resulting in cellular dysfunction and multisystem involvement. In addition to symptomatic treatment, early initiation of cysteamine therapy and its strict adherence are essential to delay kidney failure and minimize extrarenal complications. We report the case of a 28-year-old woman diagnosed...

CONCLUSION: These data represent the longest published follow-up of lumasiran-treated patients with PH1 (ages 6-43 years) to date. Long-term lumasiran treatment for PH1 had acceptable safety and led to sustained and substantial reduction of UOx with preservation of kidney function.

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CONCLUSIONS: The study emphasized the significant association between CKD and CVD persisting across socioeconomic strata. The findings highlight socioeconomic disparities, emphasizing the importance of a multidisciplinary care approach and further research to address inequalities in the CKD-CVD relationship.

CONCLUSION: Long-term data support the good safety and efficacy profile of Sibnayal^(®) in the treatment of dRTA with adequate control of metabolic acidosis, stable kidney function and significant positive long-term clinical outcomes.

CONCLUSIONS: GF increased with subsequent KTx. GF and death with a functioning graft after second transplantation improved with calendar year of transplantation, reflecting improvements in transplant care over time. Older donor age, DD KTx, short primary graft survival, high PRA, and increasing HLA-DR mismatch were associated with a higher predicted composite outcome.

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CONCLUSION: Nedosiran was well-tolerated, reduced average Uox levels, reduced kidney stone occurrence, and maintained stable renal function for over 3 years.

CONCLUSIONS: The present study confirms that FGN is primarily a B-cell-driven disease and provides evidence that FGN can be effectively managed by achieving a profound depletion of CD20+ B lymphocytes; the disease is highly progressive and probably requires prolonged maintenance treatment; and, last, early diagnosis is critical for long-term outcome because a significant glomerular sclerosis at the time of the first biopsy precludes the possibility of reversing or stabilizing the course of the...

Genetic investigations in nephrology have long been viewed as the prerogative of paediatricians or restricted to archetypal genetic nephropathies with highly penetrant variants affecting young adults. However, genetic testing has emerged as a pivotal tool in the field of adult nephrology, with the ability to revolutionize the understanding and management of adult kidney diseases. Here, we explore the multifaceted role of genomic testing (such as exome or genome sequencing) in chronic kidney...

CONCLUSION: Heatmap analyses reveal a significant but incomplete overlap of RD clinical trial sites between ERNs and c4c in parts of Europe, suggesting strong potential for cross-network collaboration to enhance paediatric RD trial recruitment and outcomes.

JAK inhibitors (JAKi) are small molecules that interact with JAK proteins, modulating the JAK-STAT signaling pathway, which plays a significant, though not yet fully understood, role in immune regulation. Due to the breadth of their mechanism of action, JAKi have shown promising results in the treatment of various immune-mediated diseases across different fields such as rheumatology or dermatology, and may represent a valuable therapeutic option for patients with multiple coexisting...

CONCLUSION: DAILY-LUMA is the largest cohort of patients receiving lumasiran in real life, confirming its safety and efficacy at 2 years.

CONCLUSIONS: The updated recommendations provide consensus guidance and will help to improve the quality of care of patients during the phases of reproduction, pregnancy, and lactation.

CONCLUSIONS: Current amoxicillin-clavulanate dosing regimens for critically ill children after cardiac surgery need to be updated to avoid subtherapeutic concentrations and clinical failure due to augmented clearance (ClinicalTrials.gov NCT02456974).

CONCLUSION: Pretreating formulas with resins is a reproducible and straightforward method when specific diets for CKD are unavailable. However, it is important to keep in mind that resins may impact the overall composition (osmolality) and the concentration of other nutrients (folates).

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Background/PurposeAPS ACTION Registry was created to study the natural course of antiphospholipid syndrome (APS) over 10 years in persistently antiphospholipid antibody (aPL) positive patients with or without systemic autoimmune rheumatic diseases (SARDs). Our primary objective was to compare the characteristics of aPL-positive patients with or without thrombocytopenia (TP) and/or autoimmune hemolytic anemia (AIHA).MethodsThe registry inclusion criteria are positive aPL based on the Revised...