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.

IgA vasculitis (IgAV) is an immune complex-mediated small-vessel vasculitis that typically affects the skin, gastrointestinal tract, kidneys and joints. Childhood-onset IgAV is a common disease and usually follows a self-limiting course, whereas adult-onset IgAV is considerably less frequent and is associated with a poorer prognosis. The diagnosis, assessment and management of adult-onset IgAV remain challenging owing to the absence of validated diagnostic criteria for adults and lack of...

CONCLUSIONS: Pediatric kidney transplant recipients encounter substantial future challenges in social and professional integration as they progress through adulthood. Our cohort demonstrated moderate educational achievement and employment rates, and most were unmarried. Our findings emphasize the importance of long-term multidisciplinary support to promote successful transitions into adulthood, addressing not only medical needs but also social and occupational needs.

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...

Diabetic kidney disease is a complication of inadequately controlled diabetes of any type. It is the main reason for end-stage renal disease and the need to start dialysis, leading to a great burden for health care systems. Moreover, it strongly diminishes the quality of life and shortens life expectancy. The pathophysiology, diagnostics and treatment methods of diabetic kidney disease are not yet fully understood. This complication is underestimated and most often diagnosed in an advanced stage...

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: Using the HOUSES Index and COI, we identified a 3-fold to fivefold higher risk of pediatric graft loss among recipients with lower SDOH. These tools provide robust non-biological predictors for transplant outcomes; however, larger studies are required to compare their relative impact.

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.

Background: Hemodialysis patients are particularly vulnerable to hepatitis B virus (HBV) due to immunosuppression and repeated vascular access. While universal childhood vaccination has reduced population-level HBV prevalence, dialysis units require tailored prevention and monitoring strategies. This study aimed to characterize HBV serologic profiles, evaluate immune responses, and assess the kinetics of antibody waning in a diverse hemodialysis population. Methods: We retrospectively analyzed...

CONCLUSIONS: Although DAAs lead to metabolic and hepatic improvements, long-term prognosis in T2D patients remains largely determined by baseline liver disease severity, insulin resistance, and genetic background. These findings emphasize the importance of early antiviral treatment and optimized metabolic management in this high-risk population.

CONCLUSION: The burden of XLH disease in adulthood is determined by skeletal manifestations and dental disease and may be more severe in males. Additionally, cardiometabolic impairment may not be common. The disease burden impacts most of the individuals, beyond those presenting the criteria for burosumab reimbursement.

CONCLUSION: Despite identical mutations, phenotypic differences highlight complex genotype-phenotype relations, stressing the need for research, genetic counseling, and family member screening.

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). Evidence-based 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...

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...

CONCLUSION: While children and adults demonstrate comparable long-term kidney survival, elderly patients face significantly worse outcomes due to advanced chronicity and systemic damage. These findings highlight the need for tailored interventions in late-onset LN. Older-onset LN, in fact, was an independent predictor of CKD or death together with AKD, arterial hypertension, SLICC >0, and no remission at 1 year.

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.

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...

CONCLUSION: This is the first report of the TRPC6 p.Pro44Leu variant, expanding the variant spectrum of TRPC6-associated FSGS. The clinical decision to withhold immunosuppression was guided primarily by the patient's phenotype (young age, sub-nephrotic proteinuria, FSGS-NOS, and no secondary causes); the TRPC6 variant, although classified as a VUS, provided supportive evidence for a genetic etiology and reinforced this management approach. This case demonstrates that genetic testing can guide...

Proteinuria promotes renal Na^(+) retention through aldosterone-independent activation of the epithelial Na^(+) channel (ENaC). While this mechanism is well documented in rodent models, its relevance and mechanistic basis in human disease remain poorly defined. Using a cross-sectional design, we isolated urinary extracellular vesicles (uEVs) from healthy subjects (control, n=20), patients with proteinuria (2.5±1.7g albumin/g creatinine, n=20) and nephrotic syndrome (5.5±3.4g albumin/g...

A case of analytical interference in urine immunofixation electrophoresis (IFE) caused by daratumumab is presented. Daratumumab, an IgG1-kappa monoclonal antibody, is well-known to produce a false IgG-kappa band in serum IFE, but urinary interference has not been reported because intact IgG is generally not filtered through the glomerulus. A 67-year-old man presented with nephrotic syndrome and was found to have markedly elevated serum creatinine (3.10 mg/dL), free kappa light chains (453.42...

Autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome is rare and incompletely defined. Its association with renal disease is less frequently reported. Systemic disorders such as systemic lupus erythematosus, immunoglobulin G4-related disease, light chain disorders, amyloidosis, and pediatric autoimmune polyendocrine syndromes may cause concurrent hepatic and renal involvement; however, renal disease with liver pathology in the absence of systemic illness is exceedingly uncommon....

Refractory peri-procedural bleeding may be the first manifestation of an occult systemic bleeding disorder. A 46-year-old woman with heavy menstrual bleeding developed severe uterine hemorrhage after endometrial sampling and intrauterine device removal, refractory to conservative treatment and requiring balloon tamponade and uterine artery embolization. Her history revealed spontaneous ecchymoses, hematemesis, and a previous nontraumatic subcapsular liver hematoma. Further evaluation showed...

Zebra bodies-electron-dense lamellar lysosomal inclusions-are the renal hallmark of Fabry disease, caused by deficient α-galactosidase A (GLA) activity. However, several pharmacological agents with cationic amphiphilic properties produce morphologically indistinguishable deposits, resulting in drug-induced phospholipidosis. We describe a woman in her 50s referred to nephrology with non-nephrotic proteinuria and non-visible haematuria following an ischaemic stroke treated with prednisolone and...

CONCLUSIONS: Clinical phenotype strongly predicts anti-PLA2R diagnostic yield in nephrotic-range proteinuria. These findings support KDIGO guidelines emphasizing nephrotic syndrome presence for non-invasive anti-PLA2R-positive membranous nephropathy diagnosis and suggest prioritizing anti-PLA2R testing in nephrotic syndrome patients for optimal resource utilization in limited settings.

Onconephrology is an emerging subspecialty that encompasses comprehensive kidney management in patients with cancer. Trastuzumab emtansine (T-DM1) is an antibody-drug conjugate composed of trastuzumab linked to the microtubule inhibitor. T-DM1 is widely used in the treatment of human epidermal growth factor receptor 2-positive breast cancer, and glomerular disease arising during T-DM1 therapy remains rarely reported. The present report describes the case of a 50-year-old Japanese woman who...

Objective: To evaluate the efficacy and safety of obinutuzumab (OBZ) in children with refractory nephrotic syndrome (RNS) who have a poor response to rituximab (RTX) therapy. Methods: This retrospective cohort study enrolled children with primary RNS who received OBZ because of poor response to RTX at the Children's Medical Center of Peking University First Hospital from July 2024 to December 2025. For each patient, outcomes during the OBZ treatment phase were compared with those during their...

A 62-year-old man with biopsy-proven focal segmental glomerulosclerosis presented with nephrotic syndrome and enlarging bilateral perinephric masses. Contrast-enhanced CT showed large low-density lesions surrounding and compressing both kidneys, raising concern for retroperitoneal malignancy. Needle biopsy revealed bland spindle cells with low proliferative activity in myxoid stroma. Because he declined repeat biopsy, dual-tracer PET/CT was performed, showing diffuse mild-to-moderate 18F-FDG...

Renal disease in people living with HIV extends beyond classical HIV-associated nephropathy to include immune-mediated and vasculitic lesions, of which pauci-immune crescentic glomerulonephritis is among the rarest and most easily missed. We describe two biopsy-proven cases with anti-myeloperoxidase anti-neutrophil cytoplasmic antibody positivity. The first, established on antiretroviral therapy, presented with a pulmonary-renal syndrome after empirical anti-tubercular treatment had failed;...

RATIONALE: Secondary (amyloid A [AA]) amyloidosis is a rare but serious systemic complication of chronic inflammatory disorders. Hidradenitis suppurativa (HS) represents an uncommon cause, with approximately 20 cases reported, usually associated with severe, extensive, or long-standing disease.

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CONCLUSIONS: Reduced respiratory infection exposure during the pandemic may be associated with lower new-onset NS incidence. Preventive measures against respiratory infections may help reduce NS occurrence.

Background: Idiopathic nephrotic syndrome (NS) in children is characterized by urinary protein loss and potential disruptions in trace element homeostasis. The dynamic changes in zinc, copper, and magnesium levels in relation to disease activity remain incompletely defined. Objective: This study aimed to evaluate serum zinc, copper, and magnesium and urinary copper and magnesium alterations in homeostasis in pediatric nephrotic syndrome and to examine their associations with disease stage,...

CONCLUSIONS: In conclusion, our results revealed that the anti-ANGPTL3/IL-22 bifunctional fusion protein ameliorates NS by protecting mitochondria, inhibiting apoptosis, and suppressing autophagy, highlighting a novel therapeutic approach for NS.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody-associated vasculitis that typically presents with asthma, eosinophilia and small-to-medium vessel inflammation. Central nervous system infarction is an uncommon complication and typically occurs during active disease. Recurrent stroke despite apparent disease remission is rare and presents a diagnostic challenge.We report a man in his 60s with EGPA who developed recurrent multifocal cerebral...

CONCLUSION: This case suggests a recognition and expansion of the knowledge of an extremely rare tubular involvement in relation to anti-phospholipase A2 receptor antibody-positive membranous nephropathy.

Lupus nephritis (LN) is a frequent and severe manifestation of systemic lupus erythematosus (SLE) associated with progression to kidney failure in up to 30% of cases, higher morbidity, and increased mortality. Recent advances in the management of LN have highlighted a shift toward a combination of immunosuppressive therapies, with agents such as belimumab and voclosporin expanding the treatment options. However, the optimal use and timing of these agents in relapsing disease remains to be...

CONCLUSION: Serum proteomics analysis identified a group of patients with NS who were potentially at higher risk of developing VTE. Extracellular matrix and endothelial glycocalyx damage might play an important role in VTE development among patients with NS.

This case study details an 86-year-old male with a history of hypertension, dyslipidemia, and bilateral blindness, who presented with recurrent vomiting and a progressive proteinuric acute kidney injury. Initial evaluations revealed nephrotic syndrome and acute kidney injury, alongside imaging findings consistent with a renal mass, later confirmed as grade 3 clear renal cell carcinoma (RCC) upon biopsy. Concurrently, systemic amyloid A (AA) amyloidosis was diagnosed based on the result of the...

Malignant pleural effusions (MPEs) are almost always exudative, with only an estimated 3-4% meeting biochemical criteria for a transudate. Light's criteria remain the standard method for classifying pleural effusions, and transudative effusions typically do not undergo cytologic evaluation. This may delay the diagnosis of malignancy in rare cases where malignant cells are present in a biochemically transudative effusion. We present a 41-year-old homeless man with no established primary care who...

CONCLUSION: In this multicenter real-world cohort, combined plasma cell and B-cell depletion was associated with meaningful remission rates in refractory rFSGS and was accompanied by dynamic changes in antinephrin antibodies in selected cases. Prospective trials are warranted to define optimal patient selection and dosing, and to clarify its place in therapy.

CONCLUSION: ARA are associated with impaired B-cell depletion and higher relapse rates. Monitoring ARA may help identify patients at risk of treatment failure and guide individualized therapeutic strategies.

CONCLUSION: Subclinical cochlear dysfunction is present in a subset of children with INS and is undetectable by routine clinical examination. The findings are consistent with shared renal-cochlear vulnerability and cumulative corticosteroid-associated cochlear toxicity. Objective hearing screening should be integrated into standard follow-up protocols for pediatric nephrotic syndrome.

CONCLUSION: CF-associated AA amyloidosis is a devastating complication characterized by predominant renal involvement and poor survival. Regular urinary protein assessment may facilitate earlier diagnosis, while further studies are needed to establish effective disease-modifying therapies.

CONCLUSION: PCD represents a core mechanism mediating podocyte injury and MN progression. TCM effectively modulates PCD imbalance through multi-target regulation, demonstrating prominent clinical efficacy and safety. These findings provide a theoretical basis and translational direction for developing high-efficacy, low-toxicity therapeutic strategies for MN.

Hypothyroidism is a rare cause of rhabdomyolysis and an even less common cause of acute kidney injury (AKI). Because the symptoms may be mild or non-specific, the diagnosis can easily be overlooked. We report the case of a 44-year-old man with no significant past medical history who was admitted after renal impairment was discovered on routine blood testing. On clinical examination, he had sinus bradycardia, puffy facies with bilateral periorbital edema, Hertoghe sign, muffled heart sounds,...

A 74-year-old man presented with leg edema and was found to have nephrotic-range proteinuria. Renal biopsy revealed AL amyloidosis. Subsequent bone marrow examination demonstrated an increase in monoclonal plasma cells accounting for 13% of nucleated cells. Right-sided pleural effusion developed and was confirmed to be chylothorax. Although treatment with daratumumab, lenalidomide, and dexamethasone (DLd regimen) was initiated, the pleural effusion continued to increase. Lymphangiography using...

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Infantile nephrotic syndrome is rare and frequently associated with an underlying genetic cause. Oligogenic inheritance has been proposed in selected cases, although supporting evidence remains limited. We report the case of an 8-month-old infant presenting with nephrotic syndrome characterized by massive proteinuria (25 g/g creatinine), hypoalbuminemia (17 g/L), and preserved renal function. Genetic testing using a targeted nephrotic syndrome panel identified two heterozygous variants of...

CONCLUSION: The developed model provides a foundation for model-informed precision dosing of MMF in children with nephrotic syndrome.

CONCLUSIONS: In conclusion, PSI predicts proteinuria remission and treatment response in PMN. Prospective multicenter studies are needed to validate its clinical utility.

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Background: Diabetic kidney disease (DKD) is one of the leading causes of chronic kidney disease and kidney failure worldwide. Although most patients with diabetes are diagnosed clinically on the basis of albuminuria, estimated glomerular filtration rate decline, and diabetic retinopathy, clinical parameters alone may not reliably distinguish biopsy-proven diabetic nephropathy (DN) from non-diabetic kidney disease (NDKD) or mixed lesions. The role of kidney biopsy in diabetic patients therefore...

CONCLUSION: In RNS, adding a statin to RTX-ACEI improved 12-month remission, proteinuria, lipids, and podocyte markers; prospective validation is needed.

CONCLUSION: In this exploratory, hypothesis-generating study, obinutuzumab was associated with a favorable relapse profile, sustained peripheral B-cell depletion, and reduction of background immunosuppression in a highly selected pediatric population. Larger prospective studies are required to confirm these preliminary observations and define long-term safety.

CONCLUSION: This case highlights the potential role of staged integrative Ayurvedic intervention in achieving sustained remission and reducing inflammatory severity in chronic plaque psoriasis associated with psychological stress and persistent urinary abnormalities. Parallel improvement in dermatological severity, stress assessment, digestive-metabolic status, and urinary parameters suggests possible systemic effects involving interconnected inflammatory and psychoneuroimmunological pathways....

Membranous nephropathy (MN) and minimal change disease (MCD) are the most common causes of nephrotic syndrome following hematopoietic stem cell transplantation (HSCT), a complication conventionally attributed to chronic graft-versus-host disease (GVHD). Paraneoplastic MCD is well described in lymphoid malignancies but is rarely reported in myeloid neoplasms. We report two cases of biopsy-confirmed MCD presenting as the initial manifestation of acute myeloid leukemia (AML) relapse following...

CONCLUSIONS: Up to 5% of youth with nephrotic syndrome use tobacco, alcohol, or recreational drugs, and 16% are exposed to a household smoker. While this study was underpowered to evaluate the independent impact of personal tobacco and recreational drug use, no clear association between substance exposure and longitudinal outcomes was identified.

CONCLUSION: This study conducted a systematic bibliometric evaluation of pediatric NS, and clarified its current research status and identified future research hotspots and development trends. The exploration of novel immunosuppressants and the elucidation of complex pathogenic mechanisms remain enduring hotspots in the evolving landscape of pediatric NS.

Daratumumab, a human IgG1 monoclonal antibody targeting CD38, is widely used in multiple myeloma and AL amyloidosis. Despite its clinical success, many patients fail to achieve durable responses or relapse, underscoring the importance of understanding resistance mechanisms. Drawing on experience from other better-studied monoclonal antibodies, resistance to daratumumab can be categorized into four main mechanisms: (1) reduced CD38 expression on plasma cells; (2) increased expression of...

Primary membranous nephropathy (PMN) in pregnancy is rare and poses significant risks including pre-eclampsia, preterm delivery, low birth weight and fetal death. Anti-phospholipase A2 receptor (PLA2R) antibodies are used to confirm diagnosis.We report a case of biopsy-confirmed PLA2R-positive but seronegative PMN in a woman in her late 20s with a dichorionic-diamniotic twin pregnancy. Initial hypoalbuminaemia was attributed to intercurrent infection but was later recognised as nephrotic...

We present two cases (females aged 23 and 36 years) with newly diagnosed systemic lupus erythematosus who developed lupus myocarditis concurrent with lupus nephritis. Both exhibited acute decompensated heart failure with dyspnea, edema, and cardiomegaly. Common key findings included strongly positive ANA blot, nephrotic-range proteinuria, acute kidney injury, and markedly elevated NT-proBNP (>35,000 pg/mL). Transthoracic echocardiography confirmed biventricular dysfunction (left ventricular...

CONCLUSIONS: These findings suggest that anti-podocin and anti-KIRREL1 antibodies associate with steroid resistance, FSGS and disease recurrence after transplantation in autoimmune podocytopathies.

CONCLUSION: This case underscores the diagnostic uncertainty inherent in distinguishing coincident MGUS from MGRS when polyclonal immunofluorescence coexists with monoclonal gammopathy but gold-standard pathologic workup is incomplete. It highlights the need for standardized pathologic evaluation, including pronase-digested paraffin immunofluorescence, and illustrates that routine urinalysis in older adults with non-renal symptoms can reveal clinically significant kidney disease.

Membranous nephropathy (MN), a leading cause of nephrotic syndrome, is associated with hypercoagulability and an increased risk of thromboembolic events; however, the relationship between coagulation-related alterations and the immune microenvironment remains incompletely understood. In this study, microarray datasets (GSE73953 and GSE140713) and single-cell RNA sequencing data (GSE233275) were obtained from the Gene Expression Omnibus (GEO), and a broad set of coagulation-related genes was...

Membranous nephropathy (MN) is a major cause of adult nephrotic syndrome characterized by immune complex deposition and podocyte injury. Oxidative stress and chronic inflammation contribute significantly to disease progression. The present study was conducted to explore the therapeutic effects of podocyte-abatacept loaded exosomes (Abt@Exos) on MN through regulating aryl hydrocarbon receptor (AhR) regulated nuclear factor-kappa B (NF-κB)/ nuclear factor erythroid 2-related factor 2 (Nrf2)...

Perinuclear antineutrophil cytoplasmic antibodies (P-ANCAs) and myeloperoxidase (MPO) antibodies are detected in 15%-25% of lupus nephritis patients, but systemic lupus erythematosus (SLE)/ANCA-associated vasculitis (AAV) overlap syndrome is rare, occurring in approximately 2% of cases. We present a 57-year-old woman with SLE and antiphospholipid syndrome (APS) on belimumab, hydroxychloroquine, and prednisone, who presented with acute ischemic stroke requiring thrombectomy and rapidly...

Diabetes is often assumed to cause chronic kidney disease (CKD) in patients with diabetes, though non-diabetic pathology may coexist. We reviewed 84 patients with diabetes who underwent native kidney biopsy (2017-2023). Indications included active urinary sediment, nephrotic-range proteinuria and rapid renal decline. Non-diabetic pathology was found in 49% and mixed lesions in 21%, mainly IgA nephropathy, acute tubular necrosis and interstitial nephritis. Biopsy altered management in 23% of...

Human leukocyte antigen (HLA) donor-specific antibodies (DSA) are among the most important determinants of late allograft loss after kidney transplantation. However, no uniform monitoring strategy has been validated in pediatric recipients. This educational review recommends that post-transplant HLA antibody monitoring in children should be tailored to the individual patient's immunological risk profile rather than applied as a fixed schedule. Pediatric recipients face a heterogeneous risk...

Sustainable nephrology should be recognized not only as an environmental priority but also as an economic and clinical imperative. Kidney care, particularly dialysis, is among the most resource-intensive areas of healthcare, generating substantial costs and environmental impacts through high consumption of water, energy, plastics, and pharmaceuticals. Increasing evidence suggests that strategies such as chronic kidney disease prevention, risk-based follow-up, conservative kidney management,...

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CONCLUSION: Pediatric KT recipients were more likely to be male, White, and aged 12-18 years. Racial disparities persist, with ethnic minority groups-particularly Black patients-facing reduced access to KT. These findings underscore the necessity of implementing targeted interventions to promote equitable access to pediatric KT.

Biallelic pathogenic variants in the CTC1 gene are associated with cerebroretinal microangiopathy with calcifications and cysts (CRMCCs), also known as Coats plus syndrome (CPS; OMIM #612199). This rare multisystem condition is characterized by early childhood onset of intracranial calcifications, leukodystrophy and cysts, along with retinal telangiectasia and exudates (Coats disease). We report a woman referred to genetics with complex multisystem clinical manifestations beginning in her 30s...

Lupus nephritis (LN), a severe manifestation of systemic lupus erythematosus (SLE), is a heterogeneous disease driven by diverse immune and tissue cell types. We obtained 538,194 single-cell and 142,881 single-nuclear profiles from kidney biopsies of 155 patients with LN and 30 preimplantation transplant biopsy controls, along with 327,326 single-cell blood profiles. We characterized key stromal and immune cell types and cell states; moreover, we distinguished cell states that were tissue...

CONCLUSIONS: The HD carbon footprint varies nearly threefold across European settings. Centre-specific emission hotspots identified through absolute life cycle stage comparisons define the most actionable intervention priorities. Where public transport is structurally feasible, modal shift is the single largest modifiable lever.

CONCLUSION: The reported incidence of postoperative AKI in children undergoing noncardiac surgery remains uncertain due to substantial heterogeneity and overrepresentation of high-risk populations. AKI incidence was high in neonates and liver transplant recipients and was associated with younger age and higher ASA class, although data were limited. Further prospective studies in broader pediatric surgical populations are needed to better assess the risk of postoperative AKI in children.

CONCLUSIONS: CNS infections in SOT recipients are rare and presentation can be non-specific, though associated with a high mortality. These findings highlight the importance of maintaining clinical awareness of CNS infection in SOT recipients.

CONCLUSIONS: ICIs alone or in combination with oncolytic immunotherapy may serve as novel downstaging strategies in advanced, ruptured HCC. Successful LT with long-term disease-free survival challenges traditional concerns about peritoneal seeding. Larger studies are required to define optimal patient selection, timing, and safety of pre-transplant ICIs. A 54-year-old man with advanced, ruptured liver cancer had an unexpectedly stable course over four years while receiving immunotherapy and an...

CONCLUSIONS: Nephrologists in Australia face considerable barriers in delivering reproductive care. Despite expressing support for patient autonomy, many clinicians defer or avoid reproductive discussions, especially in women with advanced CKD, limiting informed decision making. Addressing these barriers through structured training, clear guidelines, and systematic integration of reproductive care into routine nephrology practice is essential to ensuring that women receive timely, equitable, and...

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CONCLUSION: Addressing the burden of pediatric ESKD will require coordinated action to improve early detection, reinforce health systems, and expand equitable access to KRT globally, particularly in low-resource settings.

Background: Overweight, obesity, and metabolic dysfunction-associated fatty liver disease (MASLD) are increasingly prevalent in adolescents and are linked to alterations in the gut-liver axis. Gut microbiota may contribute to early metabolic disturbances preceding overt disease. Objective: To compare gut microbiota composition and fecal metabolite profiles, including short-chain fatty acids (SCFAs) and amino acids (AAs), between adolescents with overweight/obesity and normal-weight peers, and to...

CONCLUSIONS: CKD affects a substantial and growing proportion of pediatric HSCT survivors in this cohort, particularly those transplanted at older ages. These findings are associative rather than causal, given the retrospective, single-center design and limited number of CKD events. They nonetheless support systematic long-term renal surveillance and nephrology referral as part of post-HSCT survivorship care, with prospective, adequately powered studies needed to confirm risk factors and clarify...

Mutations in the transcription factor gene Wilms Tumor 1 (WT1) are one of the leading causes of congenital glomerular disease, characterized by severe urinary protein loss and glomerular scarring. No disease-modifying therapies exist for WT1 glomerulopathies, and affected children rely on dialysis or kidney transplantation. We evaluated a previously uncharacterized treatment in a mouse model with an orthologous human mutation in Wt1 (Wt1^(+/R394W)) that replicates the pathology of WT1...

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CONCLUSIONS: Online searches generated limited high-quality resources tailored to pediatric needs. Healthcare providers should guide families to vetted resources and consider co-creating materials to improve relevance and impact.

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CONCLUSION: This study shows that secondary TMA is the predominant form of pediatric TMA. Secondary TMA, heart failure/shock, and the requirement for mechanical ventilation were identified as independent risk factors for death or kidney failure in children.

INTRODUCTION: WT1-associated kidney disease is an important cause of early-onset end-stage kidney disease (ESKD) in children and may be accompanied by Wilms tumor, gonadal tumors, and disorders of sex development (DSD), creating distinctive challenges for kidney transplantation and post-transplant management.

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CONCLUSIONS: In a US VLBW population, the unmodified STARZ score demonstrates limited clinical utility for AKI risk stratification, thus limiting its routine clinical use and highlighting the need for population-specific validation and redevelopment of AKI risk models.

Emerging studies have identified autoantibodies targeting the podocyte protein nephrin in patients with post-transplant recurrent focal segmental glomerulosclerosis (FSGS). These antibodies bind nephrin, directly disrupting its downstream signaling pathways. This disruption profoundly impacts podocyte structure and function, thereby enriching our understanding of antibody-mediated podocytopathies and their classification. The presence of these autoantibodies correlates with disease activity and...

CONCLUSION: Daratumumab was associated with histologic improvement, reduction of microvascular inflammation, and stabilization of graft function in two children with refractory ABMR after KTx. Controlled studies to determine the safety, efficacy, and optimal dosage of daratumumab in children with ABMR are needed.

CONCLUSIONS: This pilot study suggests that factors such as HLA mismatches, donor eGFR, and donor age, consistently guided decisions, independently of participant's allocation organization. This underscores the potential value of developing evidence-informed donor-offer acceptance guidelines and investigating the impact of these factors on kidney transplantation outcome.

Abernethy malformation is a rare congenital vascular anomaly defined by the absence or severe hypoplasia of the portal vein, resulting in portosystemic shunting. The metabolic and immunologic consequences such as hepatic encephalopathy and pulmonary complications are well recognized. However, renal involvement of Abernethy malformation has been reported rarely . We report a 13-year-old boy who presented with severe anemia, pneumonia and nephrotic-range proteinuria (14.18 g/24 h). Imaging...

CONCLUSION: The Phoenix Sepsis criteria achieves superior mortality risk identification over IPSCC criteria and score outperforms conventional scoring systems in predicting PICU mortality, supporting its risk stratification in pediatric liver transplant recipients.

CONCLUSIONS: Among pediatric kidney transplant patients with diarrhea, the rate of intestinal pathogens was slow but reduced normal flora in stool cultures was common, indicating microbiota -related etiologies. The high rate of cytomegalovirus infection among patients suggested that cytomegalovirus infection should be considered as a potential etiological factor in pediatric transplant patients presenting with diarrhea. Noninfectious causes should also be considered in differential diagnosis of...

CONCLUSIONS: Pediatric kidney transplant recipients exhibit distinct inflammatory and biochemical profiles during upper respiratory tract infection compared with healthy peers but show comparable clinical outcomes. Early molecular diagnosis, vigilant monitoring, and preventive strategies such as vaccination remain essential to optimize infection management in this vulnerable population.

CONCLUSIONS: Kidney transplant is feasible and generally effective in patients with Joubert syndrome, offering durable renal replacement in most cases. However, the universal occurrence of delayed graft function and the effect of infectious complications, which led to 1 graft loss and 1 death, emphasize the need for careful perioperative management, urological assessment, and close long -term follow -up within a multidisciplinary framework.

CONCLUSIONS: The transition to adult care in pediatric solid -organ transplant recipients presents emotional, informational, and systemic challenges. Structured, culturally sensitive transition programs are urgently needed in Türkiye to optimize continuity of care and health outcomes.

CONCLUSIONS: Pediatric renal transplant recipients with cystinosis demonstrated comparable graft survival and favorable posttransplant outcomes versus recipients without cystinosis, with potential advantages, including lower incidence of new -onset diabetes after transplant and better early renal function. These findings support renal transplant as a viable treatment option for pediatric patients with cystinosis and end -stage renal disease.

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Pediatric acute liver failure (PALF) is a rare but life-threatening condition characterized by rapid clinical deterioration and high mortality. Viral infections represent a major etiology of PALF, although the causative agent remains unidentified in a substantial proportion of cases. Human Enteroviruses (EVs) are typically associated with self-limiting illnesses; however, they may rarely cause severe systemic disease, including fulminant hepatitis, particularly in neonates and young children. We...

The cardiovascular-kidney-metabolic framework recognizes the interconnected biological, clinical, and societal drivers of cardiovascular disease, chronic kidney disease, diabetes, and obesity. To advance an integrated perspective, aligned with the kidney community focus, the International Society of Nephrology convened an International Expert Forum, bringing together a global and multidisciplinary team of leaders in this field. This meeting report synthesizes key discussions spanning...

CONCLUSIONS: These findings suggest that anti-podocin and anti-KIRREL1 antibodies associate with steroid resistance, FSGS and disease recurrence after transplantation in autoimmune podocytopathies.

CONCLUSIONS: adolescents with PH show significant alterations in the expression of adrenergic and cholinergic receptors in PBL. These changes correlate with hypertension mediated organ damage, hemodynamic and metabolic parameters typical of childhood hypertension.

INTRODUCTION: Successful pregnancies with healthy newborns have been reported after all types of solid organ transplantation (SOT). Limited data in young children suggest similar development and health compared with the general population. However, fetal development may be influenced by factors such as immunosuppressive medication and the increased incidence of pregnancy complications, and forthcoming health problems may only become apparent later in the life of the offspring. To allow for...

CONCLUSIONS: Anemia was frequently encountered after pediatric kidney transplantation. Both early and late PTA were strongly linked to iron deficiency. Pre-transplant iron status emerged as a key modifiable risk factor. Anemia was associated with graft dysfunction, proteinuria, hyperparathyroidism, and rejection. In particular, patients with persistent anemia should undergo a comprehensive evaluation for potential etiological factors and be closely monitored with respect to graft functions.

As a commonly used immunosuppressant, mycophenolate mofetil (MMF) is widely prescribed after organ transplantation because it is not toxic to the liver and kidney. However, its use is associated with certain gastrointestinal toxicity and increased risk of opportunistic infections. Meanwhile, whether it affects vascular development is unclear. Herein, a zebrafish model was utilized to evaluate whether MMF affects vascular development, and the results showed that MMF exhibited teratogenicity,...

CONCLUSION: pDBD donors have fallen over the last 25 years, whereas pDCD donors have increased over 10-fold over the same period. Given the ongoing need for pediatric organ transplantation and the ethical importance of preserving opportunities for donation, there is an urgent need to develop a parallel communication and ethical framework to support families, clinicians, and transplant teams in navigating these donation opportunities.

Acute kidney injury is a frequent complication after pediatric heart transplant and is associated with long-term renal dysfunction and mortality. Factors associated with acute kidney injury have been studied; however, the role of intraoperative physiologic monitoring, particularly renal near infrared spectroscopy, remains incompletely understood. This study aimed to evaluate the association between intraoperative hemodynamic markers with the development of postoperative acute kidney injury in...

CONCLUSIONS: Excess adiposity is linked to marked kidney enlargement and BMI-related differences in filtration-marker-derived eGFR estimates. Increased KV is frequent despite low prevalence of albuminuria, supporting KV as a sensitive marker of early obesity-related kidney remodeling. The functional significance of lower estimated eGFR values requires confirmation using measured GFR methods.

In recent years, extracorporeal therapies have expanded beyond their traditional role in acute kidney injury (AKI), providing new possibilities for extracorporeal blood purification, immunomodulation, and organ support in critically ill children. Novel systems such as CytoSorb® and oXiris® enable extracorporeal removal of inflammatory mediators and cytokines, whereas Toraymyxin® and Seraph® target endotoxins and pathogen-associated molecules. The Selective Cytopheretic Device for Pediatrics...

CONCLUSIONS: Delayed diagnosis remains a major challenge in pediatric PH. Early recognition, genotype-guided therapy, and timely implementation of appropriate transplantation strategies are essential to improve outcomes.

CONCLUSION: Considering wider benefits of genomic medicine can materially change its estimated cost-effectiveness. Lower genomic test cost and better quantification of genotype-guided tacrolimus dosing benefits could improve its cost-effectiveness in Australia.

After kidney transplantation, there is the lifelong need to take immunosuppressive drugs, most of which have a narrow therapeutic window and a high inter- and intra-patient variability, entailing the risk of over- and under-immunosuppression. This balance is specifically challenging in pediatric recipients due to age-specific challenges, such as infections, a developing immune system, their continuously changing body composition, and their longer life expectancy. Currently, the balance leans...

CONCLUSIONS: This retrospective analysis confirms that age at KPE and postoperative corticosteroid use are key favorable factors for successful CoJ, with steroid use showing the strongest association. The excellent overall patient survival highlights the critical role of management in expert centers, even within a decentralized model.

Monoclonal gammopathy, paraprotein secreted by a clonal B-lymphoproliferative or plasma cell disorder, is quite rare in children and adolescents and may be transient. Kidney injury related to monoclonal proteins, or monoclonal gammopathy of renal significance (MGRS), is correspondingly rare and may be relatively unfamiliar to pediatric nephrologists when encountering such diagnoses on kidney biopsy (e.g., amyloid, light chain tubulopathy, light chain cast nephropathy). Several recently described...

CONCLUSION: The profile of glomerulopathies reflects not only local biopsy indications but also the heterogeneity of the population of Northeast Brazil and its particular ethnic and socioeconomic characteristics. Glomerulopathies, such as LN, accounted for the majority of cases, indicating the influence of ancestral factors in this region.

Primary membranous nephropathy (PMN) in pregnancy is rare and poses significant risks including pre-eclampsia, preterm delivery, low birth weight and fetal death. Anti-phospholipase A2 receptor (PLA2R) antibodies are used to confirm diagnosis.We report a case of biopsy-confirmed PLA2R-positive but seronegative PMN in a woman in her late 20s with a dichorionic-diamniotic twin pregnancy. Initial hypoalbuminaemia was attributed to intercurrent infection but was later recognised as nephrotic...

CONCLUSIONS: Rituximab was associated with better kidney function preservation than calcineurin inhibitors over long follow-up. Proteinuria remission and relapse results favored rituximab but did not reach statistical significance. Long-term treatment comparative effectiveness in rare diseases can be evaluated with real-world data.

IgA nephropathy (IgAN), IgA vasculitis (IgAV), focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), and minimal change disease (MCD) account for the majority of idiopathic glomerulo-nephropathies (GN). These disorders involve immune system dysregulation and have a complex genetic architecture. Currently, there are no adequately powered blood transcriptomic datasets coupled to genetic data from patients with GN that can delineate disease-context specific genetic effects on the...

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.

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.

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Chronic interferon (IFN) activation is a hallmark of autoimmune diseases such as systemic lupus erythematosus and Sjögren's disease (SjD), where epithelial cells are key contributors. Although viral and retroelement triggers have been proposed as triggers, direct evidence in patient tissues is limited, and endogenous mechanisms of epithelial IFN dysregulation remain unclear. Mitochondrial double-stranded RNA (mt-dsRNA) is a potent type I IFN (IFN-I) inducer, but its regulation in epithelial...

Rheumatological disorders encompass a broad and complex spectrum of conditions, often driven by dysregulated immune responses and autoantibody formation. Increasing evidence highlights the significant overlap between rheumatological diseases and inborn errors of immunity (IEIs). The 2024 update of the International Union of Immunological Societies phenotypic classification describes 559 IEI, including 67 novel monogenic defects and 2 new phenocopies. This review examines the clinical spectrum of...

CONCLUSION: A multivariate logistic regression model incorporating serum TC, Ca, and IgG levels demonstrates strong discriminatory utility for prevalent LN in children with SLE.

CONCLUSIONS: This first genetically confirmed Moroccan case series expands the clinical and molecular spectrum of Wiskott-Aldrich syndrome in North Africa. Our findings highlight the marked clinical and genetic heterogeneity of WAS and underscore the importance of early molecular diagnosis to guide appropriate management, genetic counseling, and timely referral for curative therapy in resource-limited settings.

CONCLUSION: This case suggests that acute co-infection with EBV and CMV may play a role in the pathogenesis of SLE, possibly via molecular mimicry and B-cell activation. Therefore, routine screening for EBV and CMV in adolescent SLE patients may be warranted to advance etiological research and inform personalized treatment strategies.

CONCLUSION: In this nationwide, population-based study from an Asian population, maternal SARDs were associated with higher risks of childhood MDs. Our findings suggest early monitoring of MDs among offspring of mothers with SARDs as part of clinical practice.

CONCLUSION: This data-driven and expert/patient consensus-based process has proposed 39 candidate items, some with subitems, and definitions for a revised SDI. Weighting of items and subitems is underway to develop a clinical scoring system.

CONCLUSION: CD8+ HLA-DRB1+ T cells represent a dysfunctional effector memory and proliferative population expanded in SLE. Type I IFN drives this paradoxical state by promoting exhaustion and impaired degranulation.

CONCLUSION: SC belimumab demonstrated expected steady-state exposure and tolerability in Chinese paediatric patients with SLE, supporting its use in this patient population.

CONCLUSION: Reduced communication frequency between families with LPOE with care teams may drive language-related disparities, particularly during periods of system stress. Enhanced outreach to families with barriers to health care engagement may facilitate more equitable care delivery.

Dysregulated activation of the stimulator of interferon genes (STING) pathway underlies various inflammatory and autoimmune pathologies. Since STING oligomerization is fundamental to its biological function, targeted modulation of this polymerization process presents a promising therapeutic approach. However, achieving precise control over STING polymerization has remained a significant challenge. In this study, we report that benzofuran derivatives serve as molecular glues to potently inhibit...

Treatment strategies for pediatric rheumatic diseases have changed substantially over the past two decades, driven by the development of biologics and cytokine-targeted molecular therapy. Therapeutic approaches that modulate both innate and adaptive immune responses have improved prognosis in these immune-mediated disorders, and early diagnosis with timely intervention is associated with better outcomes. However, affected children often present with non-specific symptoms, and reliable biomarkers...

Single-cell transcriptomics technology offers unprecedented insights into molecular heterogeneity. However, capturing sample-level representations that reflect both systemic and cellular states remains challenging, especially when disease annotations are mostly available as coarse sample-level labels. Here, we introduce Phenoverse, an interpretable deep learning framework that learns sample-level disease state representations through cell type-aware residual encoding, prototype learning, and...

ObjectivesAlthough pregnancy and childbirth are critical for patients with systemic lupus erythematosus (SLE), patients who continue to parent their children during treatment have received little attention. In this study, we aimed to investigate the impact of parenting on the quality of life (QoL) of patients with SLE.MethodsThis cross-sectional study used data from the Lupus Registry of Nationwide Institutions. The participants were females with SLE. The exposure was parenting, categorized...

CONCLUSIONS: In our descriptive meta-analyses of crude IRRs among databases from various countries and settings, we did not observe increased rates of incident POTS, ME/CFS, RA, IBD, SLE and T1DM in COVID-19 versus test-negative or reinfection versus COVID-19 during the first 9 months of the post-acute phase of COVID-19 or reinfection (>90 days postinfection until month 12). Since causal interpretation cannot be made from this study, further causal research is warranted.

Complement component 2 deficiency (C2D) is the most common inherited complement deficiency and is associated with severe bacterial infections and autoimmunity. We describe a 14-year-old Ashkenazi Jewish boy presenting with bullous cutaneous lupus erythematosus and lupus nephritis, whose diagnosis of C2D was prompted by a family history of fatal pneumococcal meningitis in a sibling. Genetic analysis identified homozygosity for the recurrent C2 c.841_849 + 19del variant, and complement studies...

ABSTRACT: This study aimed to assess whether co-treatment with gonadotropin-releasing hormone agonists during cyclophosphamide therapy protects ovarian function and preserves fertility in women with systemic lupus erythematosus. We performed a systematic review and meta-analysis of comparative cohort studies including premenopausal women with systemic lupus erythematosus treated with intravenous cyclophosphamide with or without gonadotropin-releasing hormone agonists. The primary outcome was...

Background and Clinical Significance: Varicella, caused by the varicella-zoster virus (VZV), is typically a childhood disease; however, adult cases carry substantially higher morbidity and mortality. Immunocompromised individuals are particularly vulnerable to severe outcomes. Many adults in Japan lack documented varicella vaccination, representing a missed opportunity for preventing primary VZV infection. None of the patients had documented prior varicella vaccination. Case Presentation: We...

CONCLUSION: In a prospective cohort of well-controlled SLE patients with low disease activity delivering mostly at term, SGA and small placentas remain common. The increased prevalence of placental malperfusion lesions together with an altered balance of pro- and anti-angiogenic proteins suggests that the role of vascular and angiogenesis-related factors should be further explored in relation to SGA in SLE pregnancy.

Systemic lupus erythematosus (SLE) is a complex autoimmune disease in which neutrophils, especially the pro-inflammatory low-density neutrophil (LDN) subset, play a central pathogenic role. Yet, the molecular mechanisms that link neutrophil ferroptosis, degranulation, and interferon amplification remain incompletely understood. Through integrative bioinformatics and validation in clinical samples, we identified acyl-CoA synthetase long-chain family member 1 (ACSL1) as a pivotal regulator in SLE....

This study aimed to investigate mitochondrial gene mutations and expression in peripheral blood mononuclear cells (PBMCs) of systemic lupus erythematosus (SLE) patients, focusing on MT-ND5, and assess expression changes under lipopolysaccharide (LPS), tumor necrosis factor-α (TNF-α), and dexamethasone stimulation. Peripheral blood was collected from female SLE patients. Mitochondrial DNA (mtDNA) from PBMCs was sequenced using the HiSeq PE150 platform. Quantitative reverse transcription PCR and...

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CONCLUSION: The SLICC 2012 criteria showed superior sensitivity compared to ACR 1997, while EULAR/ACR 2019 also performed well. However, a small subset of clinically diagnosed cSLE cases remained unclassified by all criteria, highlighting the need for pediatric-specific classification frameworks.

CONCLUSION: The reduced LP activity and the higher anti-PS/PT IgG/IgM titers in PTD aPL-positive women during the first trimester suggest their use as early prognostic tools for PTD in aPL-positive pregnant women.

CONCLUSION: The LBFSS is a new, valid and feasible PROM to assess brain fog and cognitive symptoms in SLE.

CONCLUSION: Patients with JSLE, particularly those with lupus nephritis, demonstrated lower retinal vascular perfusion in the superficial and deep retinal capillary plexus compared to normative control subjects. Elevated disease activity correlated with reduced vascular density in the superficial and deep retinal capillary plexus.

Calciphylaxis is a rare and life-threatening vascular calcification disorder characterized by occlusion of subcutaneous microvessels and subsequent ischemic skin necrosis. While it is well described in adults, pediatric data remain extremely limited. A systematic literature search was conducted in PubMed, Web of Science, and Embase from inception to January 2026. Studies involving patients ≤18 years old were included. Two additional pediatric cases from our institution were also analyzed....

CONCLUSIONS: In 2016-2022 PCOS/PMOS research funding by the NIH increased by ∼50% compared to 2006-2015, albeit still less than the increase and total funding for RA and SLE. There appeared to be greater interest in PCOS/PMOS by a higher number of ICs. Recent advocacy efforts appear to be associated with increasing NIH funding. However, much remains to be done to address persistent disparities in research funding for this highly prevalent and morbid women's health disorder.

Severe pediatric pneumonia associated with thromboembolism is notable, yet its pathogenic mechanism remains elusive, suggesting a potential nonnegligible role of genetic susceptibility. Five previously healthy children were admitted with severe pneumonia, primarily caused by Mycoplasma pneumoniae and other pathogens. All patients exhibited markedly elevated inflammatory markers and D-dimer levels. Crucially, whole-exome sequencing identified the methylenetetrahydrofolate reductase (MTHFR)...

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...

Lupus myocarditis (LM) is a rare but potentially life threatening manifestation of systemic lupus erythematous (SLE). LM may be rarely the initial presentation of SLE or ma y be presented during the course of the disease. Herein, we report a case of an adult female patient who presented with a 3-day history of abdominal pain, fever and recent onset dyspnea on exertion. Twenty-four hours later the patient developed acute heart failure went into cardiac arrest during her transfer from the internal...

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.

CONCLUSION: This case supports the pathogenicity of the DNASE1L3 p.Ile60Ser variant broadening the genetic spectrum. Plasma DNA fragment analysis provides a sensitive biomarker of impaired nuclease function, and JAK inhibition may offer partial therapeutic benefit in DNASE1L3-related systemic inflammation.

CONCLUSIONS: Attaining LLDAS and remission was lower in our cohort than in other contemporary paediatric lupus cohorts. Remission but not LLDAS was associated with lower risk of new damage over 4.3 years of follow-up. None of these DAS were associated with mortality.

Nailfold videocapillaroscopy (NVC) provides direct, non-invasive access to the peripheral microcirculation and has become a central tool in adult rheumatology. Its primary clinical value lies in the evaluation of patients presenting with signs of Raynaud phenomenon (RP) and scleroderma-spectrum disorders. In particular, NVC facilitates the differentiation between primary and secondary (scleroderma-related) RP. The characteristic scleroderma pattern observed on NVC, including giant capillaries,...

CONCLUSION: Our study demonstrated transition readiness increases at each clinic visit, with female sex and older age at enrollment positively predicting higher Transition-Q scores over time; however, longitudinal trajectories did not differ by sex. This suggests that male individuals and younger adolescents may require additional supports to optimize transition readiness.

CONCLUSIONS: Persistent ANA positivity in patients with AIC strongly predicts subsequent SLE, particularly in females older than 10 years with ITP. This finding warrants further investigation in larger, prospective studies, including follow-up of patients transitioning to adult care, as SLE may manifest later in adulthood.

Lupus nephritis (LN) is a kidney injury caused by systemic lupus erythematosus (SLE) and can lead to serious impairment of renal function. Glucocorticoid (GC) combined with cyclophosphamide (CTX) is currently a commonly used treatment for LN; however, it is associated with several limitations, including a high proportion of refractory cases, a high recurrence rate after remission, and a long treatment cycle. The purpose of this study was to evaluate the safety and efficacy of Tacrolimus (Tac)...

OBJECTIVE: This scoping review aims to identify, map, and synthesize evidence on the sexual and reproductive health (SRH) information needs of youth 15-24 years living with epilepsy, congenital heart disease (CHD), or systemic lupus erythematosus (SLE) in the USA and Canada, and identify barriers and facilitators to access to SRH information and services.

Systemic lupus erythematosus (SLE) is a lifelong autoimmune condition with multi-system involvement that is associated with morbidity, mortality, and poor quality of life. The key aim of management should be to empower individuals with SLE to manage their condition, suppressing systemic disease activity, and preventing organ damage. This guideline builds on and expands the recommendations developed for the first guideline published in 2017 for adults living with SLE. This comprehensive...

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

CONCLUSIONS: Belimumab plus standard therapy showed a potential glucocorticoid-sparing effect in pediatric SLE, but larger prospective studies are needed to confirm its efficacy and safety.

CONCLUSION: Together, these findings suggest that interferon driven transcriptional rewiring, impaired IL-10 signaling, and aberrant lipid metabolic programming converge to compromise DCs tolerogenic capacity in SLE. This highlights key mechanistic pathways that could be targeted to restore immune tolerance and reduce chronic inflammation.

CONCLUSIONS: Mental health monitoring over 6-12 months apart revealed changes in depressive symptoms, anxiety and sleep quality during routine paediatric SLE care. The absence of associations with cLLDAS or remission underscores the importance of integrating mental health outcomes into the treat-to-target strategies.

儿童系统性红斑狼疮（SLE）是一种以免疫炎症为特征，可累及多系统的自身免疫性疾病，心血管受累是影响预后的重要因素之一。除心包炎、心肌损伤及瓣膜病变等临床表现外，儿童SLE在疾病早期即可出现以血管炎症和内皮功能障碍为特征的亚临床损伤，其发生发展与持续炎症反应、自身抗体及抗磷脂抗体相关机制密切相关。临床常规筛查手段虽可识别显性心脏病变，但对早期及隐匿性损伤的识别仍存在不足。现系统梳理儿童SLE心血管受累机制与临床表现，总结早期识别要点，对相关影像技术的合理应用进行分析。.

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.

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,...

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Nanoplastics (NPs) are ubiquitous environmental pollutants capable of penetrating biological barriers and disrupting immune homeostasis. However, their immunotoxic potential to trigger or exacerbate autoimmune diseases (AIDs), such as systemic lupus erythematosus (SLE), remains largely unexplored. Herein, using polystyrene NPs (PS-NPs) as a model, we systematically investigated their immunotoxicity and underlying mechanisms exacerbating SLE progression via neutrophil extracellular traps (NETs)....

Macrophage activation syndrome (MAS) is driven by a hyperinflammatory response characterized by aberrant activation of lymphocytes and phagocytes. While monocytes and macrophages are thought to be important in MAS pathogenesis, their role remains poorly understood. We used bulk and single-cell RNA sequencing (RNA-Seq) on sorted monocytes from children with MAS and healthy controls to identify transcriptional changes during MAS. We defined a MAS signature in classical monocytes that correlated...

Since October 7th, 2023, the healthcare system in Gaza has suffered severe damage. Patients with chronic kidney diseases, particularly those needing dialysis, are among the most affected. This study aimed to summarize the impact of the war on patients with kidney failure. Data were collected by either visiting the dialysis centers or contacting them through phone calls and text messages. We found that the total number of patients had decreased from 1022 patients in late 2022 to 750 patients in...

CONCLUSIONS: This review presents a structured set of provisional, expert consensus-informed clinical practice points for the nutritional management of patients undergoing tandem ECMO/CRRT therapy. While numerous studies address nutrition in the context of either ECMO or CRRT, none were found to specifically examine nutritional strategies in patients receiving both therapies simultaneously. This gap significantly limits the direct applicability of existing evidence to patients on ECMO and CRRT....

Sustainable nephrology should be recognized not only as an environmental priority but also as an economic and clinical imperative. Kidney care, particularly dialysis, is among the most resource-intensive areas of healthcare, generating substantial costs and environmental impacts through high consumption of water, energy, plastics, and pharmaceuticals. Increasing evidence suggests that strategies such as chronic kidney disease prevention, risk-based follow-up, conservative kidney management,...

CONCLUSION: Early CMV-specific CD4^(+)/CD8^(+) imbalances measured by AIM are associated with CS-CMVi and reflect distinct trajectories of immune reconstitution after KT. These findings support prospective evaluation of CMV-AIM assays as precision immune-monitoring tools in larger studies.

Individuals with bipolar disorder (BD) face elevated rates of chronic kidney disease (CKD) and premature mortality yet remain underrepresented in cardiorenal trials. Although sodium-glucose cotransporter-2 inhibitors (SGLT2is) and glucagon-like peptide-1 receptor agonists (GLP-1 RAs) have established renoprotective benefits in the general population, their comparative effectiveness in BD with comorbid CKD is unknown. This observational cohort study examined risks of kidney replacement therapy...

Congenital abnormalities of the kidney and urinary tract (CAKUT) encompass diverse diagnoses that commonly present as urinary tract dilatation and are the leading cause of kidney failure in children. Antenatal hydronephrosis occurs in ~1% of pregnancies; most resolve spontaneously, but conditions such as posterior urethral valves (PUV), pelviureteric junction obstruction (PUJO), vesicoureteric junction obstruction (VUJO), duplex systems, ureteroceles, and vesicoureteric reflux (VUR) require...

Cardiovascular laterality defects occur with an estimated birth prevalence of 1.1/10,000 live births, associated with congenital heart defects (CHD) and situs abnormalities. Known disease genes explain about 20% of all cases often correlated with primary ciliary dyskinesia (PCD). We aimed to identify disease genes beyond PCD-related aetiologies using exome sequencing in 16 case-parent trios followed by exome survey in 2,109 individuals with situs inversus totalis, heterotaxy, or isolated CHD. We...

CONCLUSIONS: Dietary patterns featuring processed meats, seasonings, and refined oils may worsen CKD risk profiles in West Africans. We need prospective studies and randomized trials investigating African diets and their impact on BP and proteinuria.

CONCLUSION: Children on maintenance HD exhibit elevated sICAM-1 levels. Our findings suggest that higher-efficiency dialysis modalities, such as OL-HDF, may attenuate dialysis-related inflammation and endothelial activation in these patients.

CONCLUSIONS: Combined OCs were observed to modify steroid metabolism and lower androgen levels in women with classic 21OHD. OC therapy may serve as a useful adjunct in the management of androgen excess in this population, particularly when conventional glucocorticoid therapy alone does not achieve satisfactory biochemical control.

BACKGROUND: Kidney transplant rejection remains a leading cause of graft loss, and diagnosis still depends largely on invasive biopsies. Urinary extracellular vesicles (uEVs) offer a potential noninvasive alternative for detecting allograft rejection through molecular profiling of vesicle cargo.

BACKGROUND: Congenital heart disease (CHD) is the most common structural birth defect affecting 1% of live births. With improvements in medical and surgical management, there are now more than 2 million children and adults in the United States with CHD, a number that continues to grow. Children who undergo cardiac surgery for CHD face elevated risks of hypertension, chronic kidney disease (CKD), kidney failure, and premature mortality-complications that are more prevalent in those with single...

Monoclonal gammopathy, paraprotein secreted by a clonal B-lymphoproliferative or plasma cell disorder, is quite rare in children and adolescents and may be transient. Kidney injury related to monoclonal proteins, or monoclonal gammopathy of renal significance (MGRS), is correspondingly rare and may be relatively unfamiliar to pediatric nephrologists when encountering such diagnoses on kidney biopsy (e.g., amyloid, light chain tubulopathy, light chain cast nephropathy). Several recently described...

CONCLUSION: Renal mortality in Chiapas shows a dual burden in which CKDu exhibits autonomy, consistent with chronic interstitial nephritis in agricultural communities, and supports surveillance and prevention addressing occupational and environmental determinants.

INTRODUCTION: Ureteral wall thickening or a periureteral "sleeve" on imaging often raise concern for malignancy, but rare non-carcinomatous disorder can mimic this presentation. We report radiology and pathology findings of four prototypical cases of ureteral obstruction due to rare diseases.

CONCLUSION: Kidney transcriptome sequencing improves molecular diagnosis across the Alport spectrum, enables interpretation of splice-altering variants, and captures signatures linked to progression. Integrating RNA-based analysis may refine classification, enhance prognostic assessment, and support precision medicine in hereditary nephropathies.

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CONCLUSION: Intraoperative hemodynamic parameters were not independently associated with DGF or early graft function. At day 7, early graft function was primarily associated with cold ischemia time, whereas at day 30 it was mainly associated with preemptive transplantation status and DGF.

Lupus nephritis (LN), a severe manifestation of systemic lupus erythematosus (SLE), is a heterogeneous disease driven by diverse immune and tissue cell types. We obtained 538,194 single-cell and 142,881 single-nuclear profiles from kidney biopsies of 155 patients with LN and 30 preimplantation transplant biopsy controls, along with 327,326 single-cell blood profiles. We characterized key stromal and immune cell types and cell states; moreover, we distinguished cell states that were tissue...

SLC29A3-related disorders are autosomal recessive conditions characterized by histiocytic infiltration, autoinflammatory and autoimmune manifestations, and variable degrees of immune deficiency. SLC29A3-related disorders are rare and complex, with a broad spectrum of manifestations, even among individuals carrying the same pathological genetic variants. Initially reported as H syndrome, pigmented hypertrichosis with insulin-dependent diabetes mellitus syndrome (PHID), Faisalabad histiocytosis or...

CONCLUSIONS: Adolescents with and without PMS showed broadly similar BP values. However, statistically significant phase-related changes in systolic and diastolic BP were observed within the PMS group, suggesting that menstrual cycle-related physiological variation may influence BP regulation in these adolescents. Larger longitudinal studies are needed to determine the clinical significance and underlying mechanisms of these findings.

CONCLUSIONS: We confirmed the high prevalence of AH at WT diagnosis and identified tumor-related factors associated with an increased risk of developing AH. The key elements for the adequate management of these patients include the use of appropriate antihypertensive therapy and proper routine blood pressure screening for all patients with a new WT diagnosis. In the absence of standardized treatment guidelines, we propose recommendations developed through collaboration among pediatric...

CONCLUSIONS: Serial electronic stethoscope monitoring was feasible. In one illustrative patient with Doppler-confirmed stenosis, persistent acoustic abnormalities were recognized before routine nursing assessment but not before imaging. The candidate acoustic threshold and exploratory correlates of high-risk AVF status require confirmation in larger, externally validated prospective cohorts before clinical implementation.

Gene therapy with onasemnogene abeparvovec (OA) has dramatically improved the prognosis of spinal muscular atrophy (SMA) since its approval. Thrombotic microangiopathies (TMA), including atypical complement-mediated hemolytic uremic syndrome (aHUS), represent rare but potentially life-threatening complications associated with OA. Complement activation and immune response against the viral vector capsid are considered the main pathogenic mechanisms. We report a 10-month-old girl with SMA type 1...

BACKGROUND: Despite a general improvement in the HIV burden over the past two decades, gendered social determinants including intimate partner violence (IPV) continue to affect women's vulnerability to HIV, which could be further exacerbated as resources dwindle over the coming years. Our study aimed to quantify recent trends in the HIV burden, the magnitude of the HIV burden associated with IPV, and the potential impact of declining financial support globally.

CONCLUSIONS: This nationwide study demonstrated age- and disease subtype-specific heterogeneity in temperature-related kidney disease risks. Our findings suggest that, in addition to older adults, younger and middle-aged populations should also be primarily considered in climate change adaptation strategies targeting kidney health.

Objective: To analyze the clinical characteristics of lower urinary tract dysfunction (LUTD) in school-age children, examine their correlation with emotional-behavioral problems, and explore the potential influencing factors for such problems in this population. Methods: This case-control study enrolled 130 LUTD patients aged 6-12 years who were admitted to Shanxi Children's Hospital from June 2024 to October 2025, comprising the LUTD group. A control group of 125 age- and gender-matched...

近年来，随着单细胞组学、空间转录组学、多组学整合、人工智能及细胞治疗等技术的迅猛发展，儿童肾脏病学正经历从传统表型与病理分类向机制驱动的精准医学模式的深刻转型。国际上，在足细胞自身免疫机制、肾脏免疫微环境重塑、新型生物制剂、补体靶向治疗、嵌合抗原受体T细胞治疗及慢性肾脏病多通路保护等方面已取得突破性进展。本文围绕疾病机制解析、精准诊断、靶向治疗、细胞治疗及疾病管理等领域进行系统论述，展望儿童肾脏病精准医学体系的建设方向，为构建符合中国儿童特点的精准诊疗模式提供参考。.

Objective: To evaluate the efficacy and safety of lonafarnib, in children with Hutchinson-Gilford progeria syndrome(HGPS). Methods: This was a prospective cohort study. A total of 12 children with HGPS who were treated with lonafarnib at the Children's Hospital of Zhejiang University School of Medicine between April 2022 and November 2025 were enrolled. Clinical data collected within 1 week before initial medication were taken as the baseline. The first follow-up was performed 4 to 12 months...

医学人文精神是医学的灵魂，在技术飞速发展的今天，其价值愈发凸显。本文以现代医学人文精神的核心内涵为起点，结合中国儿科肾脏病学专业的临床实践与学科发展，探讨医学人文传统如何在当代医疗环境中被传承与创新发展。提出在儿童肾脏病的诊疗中，医者应将“仁爱、共情、责任、尊重”融入医患沟通、伦理实践、人文服务创新中，承济世仁心，融医技仁术，实现技术与人性的有机融合，助力守护儿童肾脏健康。.

CONCLUSION: Both admission lactate level and early dynamic lactate trajectory provide valuable prognostic information in pediatric AKI patients. Identifying lactate patterns facilitates early risk stratification and guides individualized management.

OBJECTIVES: ANCA-associated vasculitis (AAV) presents with rapidly progressive glomerulonephritis, yet the tissue-level molecular landscape in pediatric AAV remains poorly understood. We aimed to characterize spatially resolved renal disease programs across different histopathological stages of pediatric AAV and compared with adult AAV.

Tumor immune escape is not determined solely by immune checkpoints, suppressive cytokines, or changes in immune-cell composition; it is also organized by the metabolic architecture of the tumor microenvironment. Reprogrammed amino acid metabolism contributes to this process by redistributing nutrients, generating immunoregulatory metabolites, and reshaping immune-cell states. Rather than viewing individual amino acid pathways as independent mechanisms, this review proposes a network framework in...

Renal artery variations are recognized as potential contributors to hypertension through altered renal perfusion and activation of renin-angiotensin system. However, their clinical and hemodynamic significance in children remains unclear. This retrospective study included 14 pediatric patients (0-18 years) with hypertension and renal artery variations. Demographic, laboratory, echocardiographic and ambulatory blood pressure monitoring (ABPM) data were analyzed at baseline and follow-up. Patients...

Bardet-Biedl syndrome (BBS) is a rare autosomal recessive disorder characterized by clinical and genetic heterogeneity. Data on the prevalence of clinical manifestations and comorbidities in BBS, particularly in pediatric patients, are limited. This study aimed to assess the prevalence and various manifestations of endocrine and other clinical comorbidities among pediatric and adult patients with BBS. This retrospective chart review included pediatric and adult patients with BBS evaluated at an...

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Background/Objectives: Compression therapy serves as first-line conservative management for low-flow vascular malformations and Klippel-Trénaunay syndrome (KTS). However, ready-made garments are frequently ill-fitting for patients with limb overgrowth, asymmetry, deformity, or heterogeneous body habitus. Custom-made compression elastic garments offer an individualized solution, yet systematic data across diverse clinical entities remain scarce. This study evaluated objective and subjective...

CONCLUSION: Despite the higher dose of alemtuzumab and rates of leukopenia in the SQ group, there were no other differences in tolerability and outcomes compared to IV alemtuzumab. Overall, alemtuzumab is well tolerated during the first year post-transplant as an induction immunosuppression agent in the pediatric population.

CONCLUSIONS: Our nomogram model provides a novel tool for the early identification of children at high risk of ANE, assisting clinicians in formulating timely interventions to improve patient prognosis.

CONCLUSIONS: Our findings emphasize multifaceted barriers to enrolling children with OSA in clinical trials. Furthermore, we provided several underutilized solutions to facilitate children's participation in clinical trials. However, formal targeted strategies are still needed in the future, because these recommendations alone might not solve the evidence gap in children.

CONCLUSION: TIPS implantation induces progressive biventricular dilatation, increased stroke volume, and eccentric remodelling. Despite these marked structural adaptations, myocardial tissue characteristics remained largely unchanged during short- and mid-term follow-up, suggesting predominantly preload-driven cardiac remodelling.

CONCLUSIONS: Long-term kidney follow-up for children born preterm or with low birth weight remains insufficiently established in Japan. A shared care pathway across neonatology and pediatric nephrology from NICU discharge is needed.

INTRODUCTION: Cardiovascular risk assessment and management in the paediatric population is a relatively uncharted territory. However, atherogenesis starts during childhood, making childhood and adolescence an important window of opportunity to prevent atherosclerotic cardiovascular disease (ASCVD) later in life. An emerging group at risk for early ASCVD are children with chronic conditions. This paper describes the rationale, design and methods for the Secondary Manifestations of ARTerial...

CONCLUSIONS: HDR syndrome identified in kidney disease cohorts often presents with incomplete clinical features. In patients with CKD and sensorineural deafness, HDR syndrome should be considered, and comprehensive genetic testing, including copy number analysis, is essential for accurate diagnosis.

CONCLUSION: Acute and chronic kidney injury are common in patients with CNS tumors, but detection is limited by heterogeneity of tumor type and adequate SCr availability.

CONCLUSION: The NLR demonstrated strong predictive value for a complicated disease course in children with STEC-HUS. External validation in independent cohorts is required before routine clinical implementation.

Hereditary adrenocortical unresponsiveness to adrenocorticotropin (HAUA) is a rare congenital disorder characterized by isolated glucocorticoid deficiency with preserved mineralocorticoid production. HAUA encompasses familial glucocorticoid deficiency (FGD) and triple A syndrome (AAAS) and is caused by autosomal recessive defects in ACTH-signaling-related genes, including MC2R, MRAP, AAAS, NNT, TXNRD2, and MCM4. To clarify the current clinical characteristics of HAUA (including FGD and AAAS) in...

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To investigate the incidence of acute kidney injury (AKI) and factors associated with AKI in children with functional single ventricle (FSV) undergoing the bidirectional Glenn (BDG) procedure. This retrospective cohort study included patients aged < 18 years with FSV who underwent BDG at Guangdong Provincial People's Hospital between January 2005 and December 2024. Postoperative AKI was defined using the serum creatinine component of the Kidney Disease: Improving Global Outcomes criteria....

CONCLUSIONS: MIS-C with cardiovascular involvement has a substantial incidence of acute myocardial dysfunction, particularly in patients with higher levels of TroponinT and BNP and in those who developed MAS. Cardiovascular abnormalities are usually transient. However, in 10% of cases, mild abnormalities are still detected by cardiovascular imaging in the long-term, without any correlation with the severity of the acute phase.

CONCLUSIONS: Our nomogram model provides a novel tool for the early identification of children at high risk of ANE, assisting clinicians in formulating timely interventions to improve patient prognosis.

CONCLUSION: Kidney dysfunction is common in pediatric COVID-19 cases, particularly in severe disease. AKI was strongly associated with worse outcomes, including mortality. Early detection and management of kidney involvement are essential to improving prognosis in pediatric COVID-19 patients.

CONCLUSION: This review sheds light on the importance of testing PROMs in patients with PCC using these questionnaires and the need for further testing their validity in this condition.

CONCLUSIONS: AlloHCT recipients achieve humoral immunity comparable to healthy individuals after three vaccine doses, supporting efficacy and safety of repeated SARS-CoV-2 vaccination in this vulnerable population.

CONCLUSIONS: DKA frequency increased across the study period, with a peak during the pandemic. This findings should be interpreted in the context of global epidemiological data, where the prevalence of DKA at diagnosis of type 1 diabetes remains high and varies widely between countries, reaching around 50% in some populations. Therefore, coordinated country-level actions aimed at improving awareness of early diabetes symptoms are needed to reduce the persistently high rate of DKA.

CONCLUSIONS: In this US cohort, severe PrAKI appears to be associated with acute systemic illness occurring in the setting of underlying chronic cardiometabolic vulnerability. The dissociation between rising mortality and stable dialysis utilization underscores the need for earlier risk stratification and multidisciplinary care to reduce maternal mortality.

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

Background: More than 750 million cases of COVID-19 have been reported worldwide. The respiratory system, particularly the lungs, is one of the main targets of SARS-CoV-2 infection. Although persistent pulmonary function abnormalities have been described in adults, evidence in pediatric populations remains limited and inconsistent. Children usually experience a milder course of COVID-19; however, the long-term impact of SARS-CoV-2 infection on respiratory function in this group is still unclear....

CONCLUSIONS: The use of COVID + donors for pediatric kidney transplantation has increased over time. The posttransplant outcomes are similar between COVID + and COVID - pediatric recipients, supporting the use of COVID + donors in this population.

Persistent symptoms following SARS-CoV-2 infection in children remain poorly understood, and objective biological correlates are scarce. The vascular endothelium is considered a central target of post-viral dysregulation, yet paediatric evidence for microvascular involvement is limited. Retinal imaging enables non-invasive assessment of microvascular structure and function and may help to clarify whether endothelial dysregulation is present in children with post-COVID-19 syndrome (PCS). Retinal...

CONCLUSION: Sex differences exist in post-vaccination symptoms after BNT162b2 administration in young children and adolescents. These are of importance for the conception of approval studies, for post-vaccination monitoring and for future vaccination strategies.

CONCLUSION: MIS-C occurred predominantly after first SARS-CoV-2 infections; while evidence for a lower risk following reinfection was suggestive but not conclusive.

This retrospective study presents a 10-year-old male with multi-systemic venous thromboembolism (VTE) secondary to COVID-19, including right ventricular thrombus(40 mm × 18 mm), bilateral iliac vein thrombosis, pulmonary embolism, and renal vein thrombosis. The child presented with fever, abdominal pain, and elevated inflammatory markers (CRP 222.72 mg/L, WBC 22.41 × 10^(⁹)/L). Imaging confirmed extensive thrombi in the right ventricle, pulmonary arteries, and lower extremities. Anticoagulation...

In the SARS-CoV-2 endemic phase, assessing the effectiveness of COVID-19 booster doses in children is essential for public health policy. This study evaluated the vaccine effectiveness (VE) of three doses (primary series plus booster) against severe outcomes, comparing the pandemic and endemic periods and children with and without comorbidities. We carried out a cohort study based on the population, utilizing comprehensive Brazilian data from individuals under 18 years of age with confirmed...

BACKGROUND: Better evaluation of the contribution of the main diseases, injuries, and risk factors for mortality and life expectancy is crucial for more efficient policy making at the national and subnational levels in Iran. The aim of this study is to assess the effect of emerging causes of mortality on health, specifically COVID-19, which can help policy makers implement preventive measures in similar situations.

BACKGROUND: Atypical hemolytic uremic Syndrome (aHUS), a form of thrombotic microangiopathy (TMA), had a poor prognosis until the development of complement C5-inhibiting monoclonal antibodies, eculizumab and ravulizumab. While ravulizumab has shown effectiveness in treating postpartum TMA, data about its use during pregnancy remains lacking. CASE PRESENTATION: A 32-year-old woman with a history of aHUS was initially diagnosed in 2018 at the age of 27 after presenting with microangiopathic...

CONCLUSION: The humoral response to COVID-19 was similar in children with idiopathic nephrotic syndrome compared to control children, suggesting that routine vaccination schedules remain appropriate in this group. These findings suggest preserved antibody responses in this population; however, due to the exploratory nature of this study, larger studies are needed before clinical recommendations can be modified.

The European Confederation of Medical Mycology Candida III was a pan-European, multicenter observational study of adult patients with blood culture-proven candidemia. Among a total of 632 patients with candidemia across 64 institutions in 20 European countries, a subanalysis of 396 (63%) cases occurring outside the intensive care unit (ICU) was conducted. Compared with ICU patients, non-ICU patients had a higher comorbidity burden (median Charlson comorbidity index [CCI] 6 vs 5 in ICU patients,...

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.

Health care is shifting towards a digital-guided system, integrating digital diagnostics, biomarkers and therapeutics in many care pathways. However, despite rapid technological advancement and preliminary adoption accelerated by the COVID-19 pandemic, a significant implementation gap persists. This narrative review explores the causes of this gap, highlighting several examples from early development to final implementation. These show that technical validation alone is insufficient. Success...

CONCLUSION: Post-COVID condition remains a burden despite vaccination. Distinct symptomatology patterns across VoC and timelines highlight the need for tailored management strategies to mitigate long-term global impacts.

BACKGROUND: Breast cancer is a leading cause of mortality and morbidity among females worldwide. As part of the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2023, we provided an updated comprehensive assessment of the epidemiological trends, disease burden, and risk factors associated with breast cancer globally, regionally, and nationally from 1990 to 2023.

CONCLUSIONS: Real-world CytoSorb® use as part of standard care in critically ill patients was associated with improvements in several clinical and laboratory parameters; however, these findings should be interpreted cautiously given the observational design and absence of a control group. Observed mortality was lower than mortality estimates historically associated with established severity scores.

Background/Objectives: Post-COVID-19 muscle weakness is common even after mild or moderate infection, driven by systemic inflammation, prolonged inactivity, and reduced functional reserve. This study aimed to describe changes in global muscle strength assessed using the Medical Research Council (MRC) scale in adults recovering from mild or moderate COVID-19 who participated in a structured two-week rehabilitation program, and to compare these changes with those observed under standard medical...

Background: Acute kidney injury (AKI) is increasingly recognised in children with acute COVID-19 and multisystem inflammatory syndrome in children (MIS-C), yet the long-term renal consequences in younger paediatric populations remain unclear. Most studies focus on acute illness or mixed-age cohorts, with limited data specific to children aged 0-12 years. Objectives: This study aimed to systematically identify, evaluate, and synthesise evidence on post-acute (≥30 days) and long-term (≥90 days)...

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Respiratory pathogen dynamics in western China following COVID-19 restrictions remain poorly characterized. We analyzed 50,247 specimens across 14 pathogens from January 2020-December 2024 using multiplex PCR at Sichuan Provincial People's Hospital. Pathogen positivity is increased by 314% post-pandemic, with H1N1 showing 1,826% and Mycoplasma pneumoniae showing 519% increases. Human rhinovirus exhibited highest overall detection at 9.05%. Correlation analysis revealed 89% of pathogen pairs...

CONCLUSION: Combined daratumumab and anti-CD20 therapy appears to be an effective rescue strategy for refractory INS, in native kidneys and post-transplant. It induces rapid and sustained remission, enabling discontinuation of apheresis. Prospective studies are warranted to optimize treatment regimens and identify predictive biomarkers of response.

CONCLUSION: Long-term respiratory sequelae and fatigue occurred after both MIS-C and severe COVID-19, but respiratory symptoms and impaired HRQoL were more frequent after COVID-19. Lung function and CPET abnormalities in children with COVID-19 often corresponded with symptoms. Children with MIS-C often showed CPET abnormalities without respiratory complaints or lung function changes.

CONCLUSION: The findings highlight a complex interplay between pandemic conditions and observed positivity rates. The increase likely stemmed from multiple factors, including shifted testing focus, altered healthcare-seeking behavior, and potential viral reactivation. The COVID-19 response offers insights for optimizing future viral hepatitis control strategies during public health emergencies. Future research should expand demographic and geographic scope and investigate behavioral/social...

Background and Objectives: COVID-19 has been associated with prolonged inactivity and reduced physical performance, even in mild and moderate cases. This study aimed to evaluate changes in functional mobility and gait speed, assessed with the Timed Up and Go (TUG) and 10-Meter Walk Test (10MWT), in patients with mild to moderate post-COVID-19 conditions undergoing a structured rehabilitation program. Materials and Methods: A controlled observational study was conducted on 193 patients (115...

CONCLUSIONS: The HAYATI app effectively filled a critical surveillance gap during the early pandemic phase in Lebanon. By integrating GIS technology, automated risk stratification, and community-level engagement, it provided a scalable model for public health surveillance in resource-limited settings. This approach has potential for broader applications in managing future outbreaks and endemic diseases through decentralized, real-time digital health strategies.

CONCLUSION: China's pandemic control measures created significant barriers to dialysis access and contributed to heightened psychological distress among patients. In response, many individuals employed self-management strategies to reduce the impact of these disruptions. The findings highlight the need for patient-centered interventions, particularly those aimed at enhancing transportation accessibility, incorporating mental health support, and addressing disparities in rural healthcare. Future...

CONCLUSIONS: The COSMOS registry highlights CS-associated improvements in lactate, creatinine, norepinephrine needs, fluid balance, and oxygenation. Mortality was favorable compared with risk-based predictions.Trial registration Clinicaltrials.gov Identifier: NCT05146336.

CONCLUSION: We found a significantly higher incidence of APSGN and its associated complications during the post-COVID period.

BACKGROUND: The global burden of sepsis, a life-threatening dysregulated host response to infection leading to organ dysfunction, remains challenging to quantify. We aimed to comprehensively estimate the global, regional, and national burden of sepsis, including the impact of the COVID-19 pandemic and underlying causes of sepsis-related deaths with co-occurring infectious syndromes.

BACKGROUND: Timely and comprehensive analyses of causes of death stratified by age, sex, and location are essential for shaping effective health policies aimed at reducing global mortality. The Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2023 provides cause-specific mortality estimates measured in counts, rates, and years of life lost (YLLs). GBD 2023 aimed to enhance our understanding of the relationship between age and cause of death by quantifying the probability of...

BACKGROUND: Comprehensive, comparable, and timely estimates of demographic metrics-including life expectancy and age-specific mortality-are essential for evaluating, understanding, and addressing trends in population health. The COVID-19 pandemic highlighted the importance of timely and all-cause mortality estimates for being able to respond to changing trends in health outcomes, showing a strong need for demographic analysis tools that can produce all-cause mortality estimates more rapidly with...

BACKGROUND: Cancer is a leading cause of death globally. Accurate cancer burden information is crucial for policy planning, but many countries do not have up-to-date cancer surveillance data. To inform global cancer-control efforts, we used the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2023 framework to generate and analyse estimates of cancer burden for 47 cancer types or groupings by age, sex, and 204 countries and territories from 1990 to 2023, cancer burden...

Coronavirus disease 2019 (COVID-19) affected billions of individuals globally, with symptoms ranging from isolated blood clotting to severe acute hypoxemic respiratory failure requiring intensive respiratory support ventilators. Those with advanced chronic kidney disease (CKD stage 5) were at high risk of severe disease faced a particularly heightened risk of severe illness. Inflammation and associated immune-thrombotic events in CKD stage 5 have attracted increasing attention, yet remain poorly...

Objective: The COVID-19 pandemic disrupted the seasonal pattern of RSV infections, increasing cases outside the typical epidemic season. This study aimed to assess the pandemic's impact on the clinical characteristics of RSV infections in children hospitalized at the Polish Mother's Memorial Health Institute in Łódź, based on a 9-year observation period from 2016 to 2024. Methods: A retrospective analysis was conducted on 330 children hospitalized for RSV between 2016 and 2024. Patients were...

CONCLUSION: This case report highlights the importance of considering DD in differential diagnoses of children with the pseudo-Bartter syndrome, that is, renal salt and potassium wasting, with or without hypercalciuria and nephrocalcinosis. Additionally, in children with rickets and proteinuria, urinary low-molecular-weight protein measurement could assist in screening for the possibility of DD, particularly in low-resource settings.

CONCLUSIONS: Local and systemic hyperactivation of innate immunity characterizes acute pyelonephritis, a common and severe bacterial infection in childhood and a significant cause of urosepsis and mortality in adults. The results define a transient cytokine storm response, resembling that induced during severe acute respiratory syndrome coronavirus 2 infection, as characteristic of acute pyelonephritis, rather than individual protein biomarkers.

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)...

CONCLUSIONS: This study demonstrates that agalsidase beta is safe and effective in Chinese patients with Fabry disease, and suggestes that COVID-19 infection may potentially impact the renal prognosis for Fabry disease.

CONCLUSION: Adolescent candidates evaluated during the COVID-19 pandemic had significantly higher executive functioning and mental health concerns compared to those evaluated before the pandemic; however, no significant differences were found in the mean scores for preadolescent candidates.

In the original publication [...].

The safety of XBB.1.5-containing COVID-19 mRNA vaccines warrants investigation. We assessed the relative risk of 15 adverse events following the XBB.1.5 vaccination using a self-controlled case series study design with data from the National COVID Cohort Collaborative (N3C) from September 11, 2023, to June 1, 2024 in the USA. Based on a baseline population of 244,494 patients, adverse events included Guillain-Barré syndrome, seizure, non-hemorrhagic stroke and transient ischemic attack,...

CONCLUSION: Hybrid meetings, allowing for more flexibility and better utilization of resources, were the preferred modality for scientific meetings, regardless of the number of participants. A targeted survey could further explore how to optimize meeting attendance and participation in scientific discussions.

CONCLUSION: Dipstick hemoglobinuria is associated with histologic markers of active disease in IgAN and may provide clinically relevant information to complement current assessment of disease activity in IgAN.

CONCLUSION: Our study is the first to analyze the prevalence of GLA variants in patients with parapelvic cysts, showing results that are significantly higher compared to CKD-ND patients and the general population. Parapelvic cyst identification, even before clinical manifestations, is crucial for early Fabry disease diagnosis and treatment.

Patients with rare diseases are particularly vulnerable during emergencies due to dependence on specialised care pathways, medicines, and expertise. European Reference Networks (ERNs) for rare and complex diseases have operated since 2017 and were confronted with two major crises: the COVID-19 pandemic and the war in Ukraine. To assess ERN experiences, responses, and preparedness for crisis and disaster situations, we conducted a cross-sectional survey of all 24 ERN coordinators between July and...

C3 glomerulopathy (C3G) and immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) are severe complement-mediated kidney diseases. In a substantial proportion of these patients, C3 nephritic factors (C3NeFs) are detected; these autoantibodies stabilize the complement alternative pathway (AP) C3 convertase. Previous studies have investigated and distinguished properdin-dependent and properdin-independent C3NeFs. In this study, we investigated a distinct subset of C3NeFs that...

Immune checkpoint inhibitors (ICIs) have transformed cancer therapy but are associated with immune-related adverse events, including ICI-associated acute kidney injury (ICI-AKI). ICI-AKI presents diagnostic and management challenges and can influence decisions regarding immunosuppression and ICI rechallenge, with important implications for both kidney and cancer outcomes. An international, multidisciplinary panel convened at the 34th Acute Disease Quality Initiative (ADQI) consensus conference...

The objective of this study was to establish a French National Diagnosis and Care Protocol (PNDS: Protocole National de Diagnostic et de Soins), with the aim of providing health professionals with free, open access synthesis on optimal management and care of patients with Infantile Idiopathic Hypercalcemia (IIH) (https://www.has-sante.fr/jcms/p_3522489/fr/hypercalcemie-infantile-idiopathique-hii). The process involved a critical review of the literature and a multidisciplinary expert consensus....

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 primary 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 AND IMPLICATIONS OF KEY FINDINGS: Patients managed with PEX achieved hematological remission faster than those on ECZ; the time to renal recovery was similar. Given the precautions and vigilance necessary with complement blockade, PEX appears to be a satisfactory initial choice for managing anti-FH associated HUS, particularly in low-resource settings. Prospective trials should compare the efficacy, safety and healthcare costs of these strategies in managing patients with anti-FH...

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.

No abstract

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.

No abstract

Lumasiran, an RNA interference therapeutic, demonstrated effectiveness in clinical trials, leading to approval for primary hyperoxaluria type 1 management in all age groups. To date, little is known about its use in newborns. This study assesses, for the first time, the oxalate and glycolate metabolism in a newborn affected by primary hyperoxaluria type 1 treated at birth. His older brother, also affected by primary hyperoxaluria type 1, experienced severe disease progression and significant...

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...