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.

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

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

CONCLUSION: This study represents the largest cohort of pediatric LN in AD population. Younger patients exhibited more frequent kidney flares, particularly within the first two years of diagnosis. Overall outcomes in pediatric LN showed a higher rate of dialysis and kidney failure than in Caucasian series.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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.

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

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

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

CONCLUSION: Life expectancy of 18-year-old kidney transplant recipients was lower compared with the general population, yet having a functioning kidney graft at age 18 years resulted in better outcomes than being on dialysis. Nevertheless, between ages 18 and 23 years, about one-fifth of the kidney grafts failed and one-third of the patients remained on dialysis.

CONCLUSIONS: The case demonstrates how important it is in these subjects to evaluate not only the kidneys but also the liver which could present polycystosis and cause liver failure, affecting the severity of the pathology and death. This data is important to emphasize in the clinical management of these patients a close monitoring of liver function also from a preventative perspective in life.

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...

CONCLUSIONS: Rescaled serum creatinine (SCr/Q) slightly increases during multidiscipline lifestyle intervention in this cohort of children with overweight and obesity. This effect seems to be independent from change in BMI z-score. Whether this minor decrease in estimated kidney function has clinical consequences in the long term remains to be seen in trials with a longer follow-up period.

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...

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

CONCLUSION: Protein-supportive therapy can improve hypoalbuminemia and fetal outcomes, but it may increase urinary protein excretion and edema resolution time. Thus, the benefits and risks should be carefully weighed in clinical practice.

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

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

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

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

CONCLUSION: GAMOS1 is the most prevalent form of GAMOS in Kuwait, likely due to the presence of two WDR73 regional founder variants. These findings contribute to the global understanding of GAMOS and highlight the important contribution of regional genetic studies in diverse populations to rare disease research.

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

INTRODUCTION: Pediatric steroid-sensitive nephrotic syndrome (pSSNS) is a common childhood glomerular disorder characterized by corticosteroid responsiveness, yet frequent relapses and steroid dependence lead to long-term complications. While GWAS have identified genetic risk loci for pSSNS, its shared genetic architecture with immune-mediated glomerulopathies like IgA nephropathy (IgAN) remains unclear.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and primary focal segmental glomerulosclerosis (FSGS) are distinct glomerular diseases that rarely occur together. When present concurrently, they may produce overlapping nephritic and nephrotic features that complicate diagnosis and management. We report a case of a 63-year-old man who presented with four weeks of progressive anasarca and several months of cognitive changes, blurry vision, and paresthesia. Investigations...

CONCLUSION: Short-term oral CYC combined with GC may be a safe alternative to LD-CYC regimens for achieving clinical remission in pMN. Although these findings align with recent evidence supporting the efficacy of low-dose CYC in pMN, well-designed clinical trials are still needed for more robust clinical validation.

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

Biallelic ITGA3 variants cause a rare subtype of junctional epidermolysis bullosa named interstitial lung disease, nephrotic syndrome, and epidermolysis bullosa (ILNEB). ILNEB is characterized by extensive clinical heterogeneity, suggesting the existence of modifier genes. We studied a family with two siblings affected with ILNEB due to a homozygous ITGA3 variant. The disease severity was strikingly different in the two siblings. Whole exome sequencing revealed the presence of two different...

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by diverse etiologies and a high risk of misdiagnosis. Autoinflammatory disorders, such as adult-onset Still's disease (AOSD), are often underrecognized as contributing factors. Minimal change disease (MCD) represents an exceedingly rare renal complication in the context of HLH. This report describes the case of an adult female diagnosed with Epstein-Barr virus (EBV)-associated HLH. Initial...

BACKGROUND: Volume assessment in cardiac amyloid light chain (AL) amyloidosis is challenging during high-dose chemotherapy and autologous stem cell transplantation (SCT), as clinical findings and body weight may not accurately reflect intravascular congestion.

CONCLUSIONS: Discontinuation of MMF may be considered in selected patients with sustained remission after rituximab treatment, particularly those with identified low-risk factors for relapse. The timing of discontinuation should be individualized according to these factors and patient-specific considerations because resumption of immunosuppressants or additional rituximab treatment is frequently required after relapse following MMF discontinuation.

The research focused on investigating the function and underlying mechanisms of Danggui Shaoyao San (DSS) in nephrotic syndrome (NS). The NS rat model was established using doxorubicin (Dox), and 24-hour urinary protein (UP) level was measured in NS rats. Blood urea nitrogen (BUN) and serum creatinine (SCr) levels were detected using an automatic biochemical analyzer; morphological changes of renal tissues were observed by HE staining; serum interleukin-6 (IL-6), interleukin-1β (IL-1β), tumor...

We aimed to evaluate the outcomes, antibiotic susceptibility of non-typhoidal Salmonella (NTS), and risk factors of invasive NTS (iNTS) infections in immunocompromised children. This is a retrospective observational study of immunocompromised children under 15 years of age with microbiologically proven NTS infections at Songklanagarind Hospital from January 2007 to December 2021. Among the 96 patients with NTS, 41 were diagnosed with hematologic malignancies, 26 with solid tumors, 6 with primary...

Waldenström macroglobulinaemia (WM) is a rare lymphoplasmacytic lymphoma that produces monoclonal IgM, and renal amyloidosis is an uncommon complication. Light-chain amyloidosis occurs more frequently than heavy-chain or combined heavy- and light-chain (AHL) amyloidosis. This report describes a woman with proteinuria in the nephrotic range, elevated IgM, and IgM kappa monoclonal protein in serum and urine. Kidney biopsy revealed AHL amyloidosis with IgM and kappa deposition. Laser...

CONCLUSION: This study shows IgMN is mainly presented with SDNS clinic and MesPGN pathology. Evolution to FSGS may be related to steroid resistance, MesPGN, high IgM intensity, and C3 co-deposition.

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

CONCLUSION: This case represents the first reported instance of IgM-κ MIDD presenting with both crescentic GN and TMA in the setting of WM, defining a uniquely aggressive dual-injury kidney phenotype. It highlights the diagnostic challenge of seronegative RPGN, in which MIDD should be included in the differential diagnosis. The case underscores the importance of integrated histopathology and hematologic genomics in identifying rare MGRS entities, recognizing that severe kidney injury may evolve...

CONCLUSION: The implementation of family-centered care, combined with the transtheoretical model, in the nursing of children with nephrotic syndrome was associated with significant improvements in health behaviors and compliance rates, potentially optimizing disease control and contributing to a lower risk of complications, and enhancing the satisfaction of children's family members with nursing.

CONCLUSION: The present study found a significant association between elevated CAR levels and early in-hospital mortality from all causes in patients with acute stroke. It also demonstrated that CAR may be an independent predictor of early in-hospital mortality from all causes in all patients hospitalized with acute stroke, both in the general ward and intensive care unit.

Scabies, caused by Sarcoptes scabiei var. hominis, is a common parasitic infestation in tropical regions. Although primarily dermatological, it can rarely trigger systemic complications such as immune complex-mediated membranoproliferative glomerulonephritis (MPGN). A 14-year-old boy presented with bilateral pedal edema, facial puffiness, and reddish urine for one week. Examination revealed pruritic papular lesions over the interdigital spaces, and microscopy confirmed Sarcoptes scabiei...

Chronic kidney disease (CKD) is a major global health burden that disproportionately impacts people of recent African ancestry. The discovery of risk variants in the apolipoprotein L1 (APOL1) gene has transformed the understanding of racial disparities in CKD. In particular, APOL1 variants have been associated with increased risk of focal segmental glomerulosclerosis, virus-associated nephropathy, and other glomerular diseases in Black adults. While approximately 10-15% of Black Americans have a...

Immunoglobulin A-dominant infection-related glomerulonephritis (IgA-DIRGN) is a rare variant of acute postinfectious glomerulonephritis, typically seen in elderly individuals with comorbid conditions. IgA-DIRGN is characterized by diffuse endocapillary proliferation and the formation of mesangial deposits of IgA and C3, leading to inflammation and subsequently resulting in acute kidney injury, proteinuria, hematuria, and hypocomplementemia. In this report, we describe an immunocompromised...

Serum protein electrophoresis (SPEP) is commonly used in the diagnostic evaluation of nephrotic syndrome (NS) to rule out monoclonal gammopathies. However, NS itself and various other factors may cause transient bisalbuminemia, resulting in albumin fraction irregularities in capillary SPEP and complicating interpretation. This study proposes a stepwise approach for interpreting albumin fraction irregularities, illustrated through three representative cases of NS. Case 1: a 44-year-old woman with...

Older adult patients with acute-onset minimal change nephrotic syndrome (MCNS) often have difficulties with exercise therapy due to uremia caused by severe generalized edema and acute kidney injury (AKI). Rehabilitation treatment targeting increased light-intensity physical activity (LPA) is typically implemented to address this limitation. An 81-year-old man with suspected MCNS complicated by stage I AKI was admitted to our hospital. The patient's short physical performance battery (SPPB) score...

Minimal change disease (MCD) is a common cause of nephrotic syndrome in adults. Rituximab, a type I anti-CD20 antibody, is effective in many cases, but up to 40% of patients show an insufficient or transient response. Obesity, a frequent comorbidity, is associated with chronic low-grade inflammation and B-cell dysfunction, which may contribute to suboptimal treatment outcomes. Obinutuzumab, a type II anti-CD20 antibody, induces more profound B-cell depletion than rituximab. We report two obese...

PURPOSE OF REVIEW: The purpose of this commentary is to review the 2025 Kidney Disease Improving Global Outcomes (KDIGO) clinical practice guidelines on the management of childhood nephrotic syndrome (NS) within the Canadian context and based on current practices across the country.

Proteinuria in children is most often transient or orthostatic. However, the diagnosis and treatment of persistent proteinuria may significantly impact long-term health outcomes, as persistent proteinuria is both a sign of underlying kidney pathology as well as a modifiable risk factor for progression of kidney disease. The differential diagnosis of proteinuria differs depending on whether proteinuria is glomerular or tubular. This article reviews the pathophysiology, evaluation, and management...

CONCLUSIONS: Limited by retrospective case report methodology, this largest cohort to date suggests IgAN complicating SLE manifests a distinctive phenotype that bridges features of primary IgAN and lupus nephritis. Although immunosuppression is effective, long-term renal risk remains non-negligible. Early recognition and targeted therapy may improve outcomes.

CONCLUSION: SQ protects against IMN podocyte injury not via generalized anti-apoptosis, but through targeted PI3K/AKT/mTOR orchestration, where cytoskeletal stability intersects with mitochondrial death control. This work establishes a hybrid computational-experimental framework for decoding multi-target herbal mechanisms.

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CONCLUSION: We report for the first time that biallelic FLNB pathogenic variants are associated with paediatric SRNS by disrupting Filamin B expression, cytoskeletal integrity and podocyte function, providing evidence that FLNB is a novel monogenic cause of SRNS.

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Sphingosine-1-phosphate lyase insufficiency syndrome (SPLIS) is a rare condition causing nephrotic syndrome, neuropathy, and other manifestations. SPLIS is caused by mutations in SGPL1, which encodes sphingosine-1-phosphate lyase (SPL), a pyridoxal 5'-phosphate (PLP)-dependent enzyme needed to degrade the bioactive sphingolipid sphingosine-1-phosphate (S1P). Supplementation with the PLP precursor pyridoxine benefits some individuals with PLP-dependent enzymopathies. We sought to establish...

CONCLUSION: This case expands the clinical spectrum of CRB2-related kidney disease and highlights the importance of genetic testing in adults with unexplained CKD. Identifying genetic forms of CKD may refine diagnostic and therapeutic approaches in nephrology.

Autosomal dominant Alport syndrome results from heterozygous pathogenic variants in COL4A3 or COL4A4, and is the commonest monogenic kidney disease, affecting about 1% of the population. It is characterised by persistent glomerular hematuria, a thinned glomerular basement membrane and a family history of kidney disease. The typical Alport hearing loss and ocular abnormalities are not present. Autosomal dominant Alport syndrome is common in cohorts with hematuria, proteinuria or steroid-resistant...

CONCLUSION: This liquid biopsy strategy holds significant potential for non-invasive diagnosis, subtype classification, and personalized treatment decisions in LN.

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CONCLUSION: In conclusion, we demonstrated, in unrelated LPG patients with LPG carrying the same ApoE mutation, a protective role of LA on proteinuria and progression toward CKD. This LA-associated protective effect may be at least in part related to the modulation of biologically active EVs in patients' plasma.

The glomerular podocyte is the final barrier preventing urinary protein loss, relying on a resilient ultrastructure exposed to shear stress and hydrostatic forces. While often affected secondarily by systemic disease, podocytes are primary targets in conditions such as nephrotic syndrome. S100 proteins, involved in diverse intra- and extracellular functions, are linked to several diseases, yet their roles in podocytes remain poorly defined. The available podocyte-specific literature remains...

Few cases of immunoglobulin A nephropathy (IgAN) associated with tuberculosis have been described in the literature. We report an atypical case of extracapillary IgA glomerulonephritis discovered during the course of pulmonary tuberculosis. The patient, a 45-year-old diagnosed with pulmonary tuberculosis, presented with mixed nephrotic syndrome, characterized by hypertension, hematuria, and acute renal failure. We completed a renal biopsy which showed IgAN with diffuse crescents, classified as...

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

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

We report an 11-year-old boy with chronic kidney disease (CKD) stage 3 due to right multicystic dysplastic kidney and contralateral dysplasia who developed poststreptococcal acute glomerulonephritis (PSAGN) that rapidly progressed to kidney failure despite intensive immunosuppressive treatment. He presented with oliguria, macrohematuria, heavy proteinuria, hypocomplementemia, and an elevated antistreptolysin O titer. Initial kidney biopsy revealed diffuse endocapillary proliferation with...

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

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

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

CONCLUSIONS: The formal consensus reached is particularly significant in cases of weak or inconclusive evidence and where recommendations are based solely on expert opinion.

CONCLUSIONS: MMDx correlates with histologic assessment and can provide diagnostic clarification in ambiguous pediatric kidney allograft biopsies. Complementary use of histology and molecular diagnostics may inform clinical decision making; prospective studies are needed to determine effects on long-term outcomes.

CONCLUSION: The prevalence of pediatric AKI and RRT after hematopoietic cell transplantation is significant, and is 34.18% and 3.54%, respectively. The highest prevalence of AKI among the different sources of hematopoietic stem cells was observed in cord blood transplantation. However, the available evidence on the prevalence of AKI following the transplantation of hematopoietic stem cells derived from cord blood is insufficient, and more research is needed in this field. As a suggestion, it is...

Complement dysregulation may occur secondary to systemic immune activation, but its relationship with inflammatory bowel disease (IBD) remains poorly defined. We report a 10-year-old boy presenting with severe anemia and newly diagnosed IBD who developed acute kidney injury with microscopic hematuria and proteinuria. Complement studies showed markedly reduced C3 and CH50 with normal C4. Kidney biopsy demonstrated diffuse proliferative glomerulonephritis with dominant C3 deposition and...

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

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

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

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

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

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

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

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

CONCLUSIONS: ISW after LT may improve metabolic comorbidities. Mild histological graft changes may occur, but do not appear to affect long-term outcomes. Future studies should focus on safely expand ISW to a broader subset of LT patients.

CONCLUSION: The French version of the SONG-LP instrument is a valid and reliable tool for assessing life participation in KTRs.

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

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

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

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

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

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

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

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

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

No abstract

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

Kidney allograft failure is a devastating event often marked by return to dialysis and is associated with increased morbidity and mortality. Given the young age at transplant for pediatric recipients, they are likely to require retransplantation during their lifetime. Optimal immunosuppression (IS) management in the setting of a failing allograft is crucial to preserve residual renal function, prevent graft intolerance, and limit the development of anti-HLA antibodies. The management of IS...

Renal fibrosis is a common pathway of chronic kidney injury in children and an important determinant of long-term outcomes in both native and transplanted kidneys. Current assessment relies heavily on biopsy, which is invasive, prone to sampling error, and challenging to repeat in pediatric patients. Magnetic resonance elastography (MRE) is a noninvasive imaging technique that quantifies tissue stiffness and has become an established biomarker for hepatic fibrosis; however, its application to...

CONCLUSION: This 15-year retrospective analysis confirms the heterogeneous diagnoses and prognoses associated with FM when PUV are excluded. FM in females may indicate a poorer prognosis, while VUR is common in males. Detailed prenatal ultrasound findings may help distinguish cases with favourable outcomes from those with poorer prognosis. Postnatal imaging and renal assessment remain essential.

RATIONALE: Steroid-sparing strategies aim to reduce the adverse effects associated with steroid use. The main concern has been a potential increase in the risk of rejection, as reported in previous systematic reviews. In recent years, improvements in immunosuppressive regimens and a significant decline in the incidence of acute rejection have renewed interest in steroid-sparing strategies. More contemporary trials suggest that reducing steroid exposure might be safe. This is an updated review,...

Noncirrhotic portal hypertension has historically been described using heterogeneous and region-specific terminology-such as idiopathic portal hypertension (IPH), noncirrhotic portal fibrosis (NCPF), obliterative portal venopathy, and nodular regenerative hyperplasia-leading to substantial variability in diagnosis, reporting, and international research collaboration. Differences in guideline definitions from major societies (AASLD, EASL, and APASL), together with the presence of characteristic...

The field of nephrology is rapidly expanding and evolving with advancements in the areas of diagnostics including biomarkers, molecular genetics and imaging modalities, and therapeutics. As acute kidney injury affects a substantial proportion of hospitalized patients with considerable morbidity and mortality risk and chronic kidney disease is among the leading causes of morbidity and mortality globally, the advancements in the field of nephrology are worth focusing on and highlighting. Novel...

CONCLUSIONS: Combined IMT + EMT integrated with chest physiotherapy significantly improved pulmonary function, respiratory muscle strength, and functional capacity in pediatric kidney transplant recipients, suggesting it is a valuable adjunct in post-transplant rehabilitation.

CONCLUSIONS: Radiation exposure from medical imaging was low-to-moderate for most pediatric solid organ transplant recipients. Emphasis on optimizing ionizing modalities to reduce radiation dose and the promotion of non-ionizing imaging modalities remains important.

CONCLUSIONS: Among patients receiving dialysis who are diagnosed with localized or regional cancer, the risk of cancer-related death is low and may be exceeded by non-cancer mortality within 1 year of the cancer diagnosis. These findings support consideration of earlier transplantation for some patients with cancer and highlight the need for individualized approaches to defining transplant eligibility.

No abstract

CONCLUSIONS: This study reveals that FMT may exert neuroprotective effects by ameliorating lipid metabolic defects and replenishing precursors of the cholinergic anti-inflammatory pathway (CAP). These findings delineate an ASD-specific gut-kidney metabolic axis, providing a preliminary theoretical basis for precision ASD treatment strategies targeting the microbiota-metabolism axis.

Based on data from large international registries, the clinical presentation of fibromuscular dysplasia (FMD) in adult patients is now well established. By contrast, characteristics of FMD in children and adolescents remain poorly described. We present the first systematic review and meta-analysis focused on pediatric FMD. Seven databases were searched for studies published since 2000. Data were pooled using random-effects models. Subsequently, the characteristics of pediatric FMD patients were...

CONCLUSIONS: Despite concerns related to small caliber vessels and donor/recipient size mismatch, pediatric donors are associated with excellent mid-term outcomes in SPKT recipients and may not represent a contraindication to pancreas utilization in solitary pancreas transplantation.

Acute symptomatic seizures (ASS) represent one of the most frequent and clinically significant neurological complications following pediatric liver and kidney transplantation. Despite their clinical relevance, available evidence remains fragmented; pediatric-specific data are limited, and no standardized diagnostic or management framework has been established. To summarize current evidence regarding the epidemiology, etiological mechanisms, diagnostic evaluation, risk factors, and management...

CONCLUSION: This family demonstrates intrafamilial phenotypic variability of TBS but with strikingly consistent renal involvement linked to a familial SALL1 variant. TBS should be considered in patients with congenital anomalies of the kidney and Urinary tract (CAKUT) even in the absence of typical limb or anorectal anomalies. Early molecular diagnosis enables appropriate surveillance and timely management of progressive kidney disease.

The preoperative evaluation and postoperative management of kidney transplantation are fundamentally tailored to the distinct needs of adult and pediatric populations. The pediatric assessment prioritizes growth, congenital anomalies (like CAKUT), neurodevelopmental progress, and achieving pre-emptive transplantation. In contrast, adult evaluation focuses on comprehensive cardiovascular screening, malignancy risk, and managing accumulated comorbidities, with a growing emphasis on frailty...

CONCLUSION: This case series highlights the critical role of unconventional dialysis access methods in resource-constrained settings, where advanced interventional radiology options may be unavailable. While IVC cannulation for haemodialysis is more commonly described in adults, these are the first reported cases of vascath insertion into the IVC in paediatric patients. Additionally, these findings underscore the importance of innovative strategies to manage paediatric CKD in low-resource...

CONCLUSIONS: There is significant variability in practice patterns regarding the management of hypotension in pediatric transplant recipients. This highlights the need for further research to evaluate the prevalence of chronic hypotension in the pediatric ESKD population and to assess its impact on transplant outcomes.

Cyclosporine levels vary considerably in several case reports and series. This variability may be partially due to inconsistent sampling times among the reports and probably related to the fat content of the milk at the time of sampling. With typical maternal cyclosporine blood levels, a completely breastfed infant would usually receive no more than about 2% of the mother's weight-adjusted dosage or pediatric transplantation maintenance dosage, and often less than 1%. In most breastfed infants,...

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

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

CONCLUSION: Our findings highlight the importance of the systematic assessment including spatial and temporal variation of a broad range of air pollution components to determine the impact of exposure on short- and long-term outcomes in patients with primary glomerular disease (word count: 368).

CONCLUSIONS: Several years of follow-up are necessary to accurately estimate long-term eGFR slopes in patients with glomerular disease, but moderately sized gaps between eGFR measurements are acceptable.

CONCLUSIONS: The coexistence of membranous and IgA nephropathy may have contributed to the rapid progression of the disease. Clinicians should consider IPEX syndrome in children with kidney disease accompanied by autoimmune endocrinopathies or allergic features, even if the classic gastrointestinal involvement is missing.

CONCLUSIONS: Kidney biopsy did not significantly alter immunosuppressive management in newly diagnosed patients with SRNS at our center. Larger multicenter studies are needed to confirm these findings and evaluate whether more selective biopsy criteria could spare patients from a potentially avoidable invasive procedure, improve clinical management, and reduce healthcare costs.

Although membranous nephritis represents the classic presentation of hepatitis B virus--associated glomerulonephritis (HBV-GN) in children, adult cases can exhibit quite different features. In 1992, a 41-year-old man with recurrent nephrotic syndrome since 20 years was admitted for renal evaluation and underwent kidney biopsy. Light microscopy showed periodic acid methenamine silver staining with spike formation consistent with membranous nephropathy. However, electron microscopy demonstrated...

B-cell depletion with the chimeric anti-CD20 monoclonal antibody rituximab has revolutionized the treatment of glomerular diseases. Obinutuzumab, a type II glycoengineered anti-CD20 humanized monoclonal antibody, is increasingly being employed as an alternative to rituximab in the management of difficult-to-treat cases, due to deeper and more persistent B-cell depletion. However, its safety profile, especially in pediatric and young adults with glomerular diseases, remains to be fully...

CONCLUSION: Pediatric CNTN1-AN exhibits a low incidence, requiring ongoing renal function monitoring in affected cases. In CNTN1-AN with MN, neurological symptoms respond well to low-dose rituximab, whereas renal manifestations often require intensified regimens. The mechanisms linking CNTN1-AN and MN remain elusive, highlighting the need for further studies to optimize rituximab therapeutic protocols.

CONCLUSIONS: MN is a disease of autoimmunity directed against podocyte antigens, but some patients may also produce autoantibodies that target antigens on glomerular endothelial cells. The level of these antibodies correlates with adverse clinical findings.

Membranous-like glomerulopathy with masked Immunoglobulin G (IgG) kappa deposits (MGMID) is a recently described rare entity. MGMID is characterized by a membranous pattern of kidney injury with monoclonal IgG kappa restriction and is recognized and "unmasked" by pronase digestion on formalin-fixed paraffin-embedded tissue using immunofluorescence staining. This technique is necessary to identify peculiar forms of glomerular immune complex deposition, which is essential for diagnosing MGMID....

Podocytopathies are glomerular diseases caused by initial podocyte injury or dysfunction that lead to proteinuria and often nephrotic syndrome. The term encompasses characteristic histological patterns, most commonly focal segmental glomerulosclerosis, minimal changes, membranous nephropathy, diffuse mesangial sclerosis and collapsing glomerulopathy. However, proteinuria of glomerular origin is frequently managed without biopsy; importantly, when the protein loss is mostly albumin, it is a...

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...

CONCLUSION: This study developed a personalized risk prediction model for VTE in PMN patients using machine learning techniques. Additionally, a web-based tool for this predictive model was created. The model demonstrates strong predictive performance and can assist in clinical decision-making for the prevention and treatment of VTE in PMN patients.

CONCLUSION: Nephrotic syndrome was leading kidney biopsy indication. IgAN was the most common histological finding, followed by lupus nephritis. Primary GN was more prevalent than secondary GN. IgAN had a distinct clinical and laboratory profile. The findings emphasize establishing national kidney biopsy registry in Nepal to standardize data and track longitudinal outcomes.

CONCLUSION: A subgroup of pregnant patients can be managed without exposing the mother-child pair to adverse effects related to immunosuppression when preeclampsia is detected in the third trimester of gestation.

CONCLUSIONS: This case underscores the diverse clinical spectrum of primary Sjögren's syndrome and highlights the potential for rare glomerular involvement in children. It emphasizes the need for heightened awareness among pediatric healthcare providers regarding the systemic manifestations of primary Sjögren's syndrome to prevent delayed diagnosis.

MIRAGE syndrome is a rare multisystem disorder caused by gain-on-function SAMD9 variants. Kidney biopsies in some MIRAGE syndrome patients have shown glomerular sclerosis or interstitial nephritis. A boy with genetically confirmed MIRAGE syndrome, who showed microhematuria and nephrotic range proteinuria, underwent kidney biopsy at 18 months, revealing diffuse mesangial proliferation and partial segmental lobular accentuation associated with mesangial cell proliferation with neither crescentic...

Membranous-like glomerulopathy with masked IgG-kappa deposits (MGMID) is a rare entity described primarily among young females with previously diagnosed autoimmune diseases. We present a 12-year-old female with systemic juvenile idiopathic arthritis (sJIA) with persistent non-nephrotic range proteinuria despite normal kidney function. She underwent two kidney biopsies with the second ultimately confirming her diagnosis. The initial biopsy was suggestive of mild C3 glomerulonephritis (C3GN). She...

Electron microscopy (EM) has been essential for the diagnosis of dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). Recent research showed significantly higher accumulation of apolipoprotein E (ApoE) in DDD compared with C3GN and tested the use of ApoE immunohistochemistry for DDD diagnosis. We aimed to investigate the diagnostic value of ApoE in DDD and C3GN using 3 distinct ApoE clones-D719N, EP1373Y, and 1B2C9. Kidney biopsies of 26 DDD and 18 C3GN, diagnosed based on EM findings,...

CONCLUSION: A substantial proportion of patients were not in remission and had persistent proteinuria despite being on IST 3 years after their first biopsy.

We present a 12-year-old boy with acute onset sensorimotor neuropathy and membranous glomerulonephritis associated with contactin-1 antibodies. This prompted us to explore the clinical characteristics of this condition and assess whether its presentation differs between pediatric and adult patients. A comprehensive search was conducted across multiple online databases, including PubMed and EMBASE, using MeSH terms such as "chronic inflammatory demyelinating polyradiculopathy", "acute...

CONCLUSIONS: Participants with MN face the burden of living with a chronic relapsing disease and associated fatigue, swelling, and substantial treatment harms with the risk of kidney failure that impact life participation and relationships. Awareness and management of these burdens and psychological support may inform care and improve outcomes among patients living with MN.

Air pollution is a global problem and a major contributor to adverse health outcomes in patients of all ages. Most research has focused on the adverse effects of air pollution on cardiopulmonary events such as myocardial infarction, stroke and lung disease, with less attention given to kidney outcomes. In recent years, there is emerging evidence that air pollution contributes to the onset and progression of chronic kidney disease and, specifically, glomerular disease. This has been confirmed in...

CONCLUSIONS: PAC was used more conservatively than guidelines suggest and was mainly driven by hypoalbuminaemia severity in both adults and children. Although not included in the guidelines practice points, DOACs were used as often as coumarins in adults.

No abstract

CONCLUSIONS: Our study indicated that children affected by MN had a specific B-cell profile and that high levels of memory B-cell subsets are specific to INS pediatric patients independently of proteinuria intensity.

CONCLUSIONS: Circulating anti-nephrin antibody may be a potential biomarker of MCD and may play a role in the MCD diagnosis.

CONCLUSIONS: Peptide vaccination induces CD8^(+) Tregs that ameliorate induction of experimental membranous nephropathy which may represent a further peripheral regulation of autoimmunity.

CONCLUSIONS: Approximately 25% of each age cohort reached the composite eGFR decline outcome within 5 years. As more glomerular disease clinical trials become available, we must consider opening these trials to people with childhood and adolescent onset disease since like adults they are at high risk of progressive kidney function decline.

Primary membranous nephropathy remains a rare but challenging condition to manage in pregnancy. We present a case of an unplanned pregnancy in a 35-year-old woman with PLA(2)R-antibody positive membranous nephropathy, who had demonstrated serological response to rituximab given three months prior to pregnancy (PLA(2)R 115 IUmL reducing to 2 IU/mL, normal <13.9 IU/mL)). Throughout pregnancy, serial measurements of proteinuria and PLA(2)R-antibodies were used to understand disease activity and...

CONCLUSION: To address the gap in measure availability and fluid overload content, the Prepare-NS team has launched a set of qualitative studies for concept elicitation from the population of interest to inform development of new measures. The resulting measures subsequently will undergo psychometric evaluation and validation in a survey study.

A 3-year-old boy initially presented with purpura-like rashes and nephrotic syndrome, suspected to be IgA vasculitis nephritis (IgAVN). The suggestion of kidney biopsy was rejected. Although the patient responded well to glucocorticoids, they later developed recurrent proteinuria, refractory diarrhea, and subsequent metabolic acidosis. Kidney biopsy showed membranous nephropathy with positive semaphorin 3B expression, indicative of other kidney diseases rather than IgAVN. Although his kidney...

Membranous nephropathy is a major etiology of nephrotic syndrome in adults and less frequently in children. Circulating antibodies to intrinsic podocyte antigens, such as M-type phospholipase A2 receptor, or to extrinsic proteins accumulate beneath the podocyte to cause damage via complement activation and/or other mechanisms. The availability of clinical testing for autoantibodies to M-type phospholipase A2 receptor has allowed noninvasive diagnosis of this form of membranous nephropathy and a...

Kimura's disease (KD) is a chronic inflammatory disorder characterized by nontender lymphadenopathy involving the head and neck region. Renal involvement in KD is rare, especially in children. We report a 12-year-old boy who had been previously treated for classical KD and had presented with anasarca and oliguria after 4 years. There were no swellings or lymphadenopathy. The kidney biopsy revealed membranous nephropathy. Remission was achieved with oral prednisolone and tacrolimus therapy. This...

CONCLUSION: This study comprehensively elucidates the distinct attributes of renal damage related to Wilson's disease, while also speculating that renal dysfunction in Wilson's disease could be linked to immune complex deposition. Depending on the underlying pathogenesis, kidney injury associated with Wilson's disease can be classified as primary or secondary. To slow down the progression of renal impairment, it is essential to undergo a renal biopsy pathological examination as early as possible...

CONCLUSION: MGMID can affect both adult and pediatric patients. Further studies are needed to fully characterize its risk factors, optimal therapy, and outcomes.

CONCLUSIONS: Among adult patients in CureGN, class 2-3 obesity is associated with cardiovascular but not kidney outcomes when adjusted for potential confounding factors.

Nephrotic syndrome (NS) is one of the common presentations of kidney diseases both in children and adults. NS patients, particularly those with membranous nephropathy, have increased risk of thromboembolic events. Heparin and vitamin K antagonists (VKAs) continue to be commonly used as prophylactic and therapeutic agents, given the experience of use of these agents in NS and nonrenal indications of anticoagulation. The use of direct oral anticoagulants (DOACs) in NS is reported in some case...

No abstract

CONCLUSION: Low concentrations of anti-CysR-IgG4, anti-CTLD1-IgG4, and anti-CTLD6-7-8-IgG4 at initial diagnosis predict rapid remission after treatment. The use of specific IgG4 against PLA2R and its different epitopes combined with eGFR and urinary protein provides a better assessment of the prognostic outcome of IMN.

Primary glomerular diseases are rare entities. This has hampered efforts to better understand the underlying pathobiology and to develop novel safe and effective therapies. NEPTUNE is a rare disease network that is focused on patients of all ages with minimal change disease, focal segmental glomerulosclerosis, and membranous nephropathy. It is a longitudinal cohort study that collects detailed demographic, clinical, histopathologic, genomic, transcriptomic, and metabolomic data. The goal is to...

CONCLUSION: Nephrotic syndrome is a chronic disease that demands extensive treatment plans and strict monitoring. Medication errors are common among parents or caregivers of pediatric patients. This case is a take-home message emphasizing the significance of patient-centered communication in preventing medication errors. A clinical pharmacist can aid in conveying simple and unambiguous information to parents or caregivers.

CONCLUSION: The distribution of glomerular diseases showed age, sex and race differences. This research will be beneficial for providing epidemiological evidence for clinical diagnosis, disease prevention and public health decision-making.

Urinary biomarkers are a promising diagnostic modality whose role was explored in nephrotic syndrome (NS). We estimated urinary apolipoprotein A1 (Apo A1) and neutrophil gelatinase-associated lipocalin (NGAL) in children with first-episode NS (FENS) and controls with a longitudinal follow-up to see the serial changes during remission. The study groups comprised 35 children with FENS and an equal number of age- and sex-matched controls. Patients were followed up at regular intervals, and 32...

CONCLUSIONS: Our research demonstrated the cell type-specific molecular features in the circulation and kidney of the NEG pMN patient.

CONCLUSION: Approximately 60% of pediatric membranous cases are positive for a novel antigen on kidney biopsy and the clinical prognosis is generally favorable. More studies are needed to understand the clinical implications of each specific novel antigen.

Membranous nephropathy is an autoimmune disease affecting the glomeruli and is one of the most common causes of nephrotic syndrome. In the absence of any therapy, 35% of patients develop end-stage renal disease. The discovery of autoantibodies such as phospholipase A2 receptor 1, antithrombospondin and neural epidermal growth factor-like 1 protein has greatly helped us to understand the pathogenesis and enable the diagnosis of this disease and to guide its treatment. Depending on the...

Glomerular diseases are common causes of chronic kidney disease in childhood, adolescence, and adulthood. The epidemiology of glomerular diseases differs between different age groups, with minimal change disease being the leading cause of nephrotic syndrome in childhood, while membranous nephropathy and focal segmental glomerulosclerosis are more common in adulthood. IgA vasculitis is also more common in childhood. Moreover, there is a difference in disease severity with more children presenting...

Glomerulonephritis (GN) is a predominant cause of kidney failure in Africa. The prevalence of primary GNs varies widely across Africa depending on the relative proportion of secondary GNs and genetic predispositions. We assessed the overall and sub-regional prevalence of primary GN and its histologic subtypes in Africa. We searched PubMed, EMBASE and African Journals Online for studies of biopsy-proven primary GNs across all age groups in Africa published between 2010 and 2022. Data for primary...

INTRODUCTION: Childhood-onset systemic lupus erythematosus (cSLE) accounts for 15-20% of all forms of SLE. It is associated with greater disease severity and requires more aggressive treatment than adult-onset SLE. Despite therapeutic advances, many patients do not achieve sustained remission, highlighting the need for more effective treatment strategies.

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...

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

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.

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

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

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

CONCLUSION: Compared with traditional transfer to adult rheumatology, the YARC reduced acute care utilization and improved the no-show rate. Additionally, young adult rheumatology patients valued the PCMH model and were more likely to adhere to recommended preventive care practices.

No abstract

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

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

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

Lupus nephritis (LN) represents a severe renal complication of systemic lupus erythematosus (SLE). Myxovirus resistance 1 (MX1) has been reported to play an important role in the development of kidney injury, however, its specific function in pristane-induced LN remains unclear. In this study, we determined that pristane-treated MX1-knockout mice resulted in attenuated renal histopathological injury, reduced serum levels of anti-RNP antibody and IgG, inhibited concentrations of blood creatinine...

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

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

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

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

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

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

Haploinsufficiency of A20 (HA20) is a primary immune regulation disease caused by heterozygous loss-of-function variants in TNFAIP3, resulting in unchecked inflammatory signaling. HA20 is a highly heterogeneous disorder with overlapping features of autoinflammation, autoimmunity, immunodeficiency, atopy, and lymphoproliferation. Most patients develop symptoms in early childhood mimicking Behcet's disease, inflammatory bowel disease, periodic fevers with aphthous stomatitis, pharyngitis, and...

CONCLUSIONS: Maternal AID may be relevant to the NIPT failure in this case, and excessive chromosomal aberrations detected by NIPT suggest considering AID in addition to malignancies.

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

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

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

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

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

A two-year-and-nine-month-old girl was referred for widespread bullous and herpetiform skin lesions associated with limb edema and asthenia, evolving over approximately seven months. The initial clinical evaluation raised suspicion of bullous systemic lupus erythematosus based on cutaneous findings and abnormal blood tests, including proteinuria, hypoalbuminemia, and low complement C4. Skin histopathology showed dermal edema with a neutrophilic infiltrate, and direct immunofluorescence revealed...

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

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

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

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

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,...

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

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

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

CONCLUSIONS: We suggest a detailed picture of the changes in the kidney immune mechanisms in LN as this disease progresses.

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

CONCLUSION: In pediatric SLE, add-on belimumab improves disease control and safety at 6 months and reduces flare risk at 12 months compared with standard immunotherapy.

Epstein-Barr virus (EBV) infects the vast majority of humans and establishes lifelong latency, yet causes disease in only a minority of individuals, underscoring the critical role of host determinants. This review examines the recent advances in how host genetics and immunological variation shape susceptibility to EBV across three major disease contexts. First, inborn errors of immunity have revealed essential CD8^(+) T-cell pathways governing control of EBV-driven B-cell lymphoproliferative...

ObjectiveTo evaluate national and regional trends in age-standardized mortality rates (ASMRs) from childhood-onset systemic lupus erythematosus (cSLE) in Mexico between 2000 and 2023, stratified by sex and region.MethodsThis cross-sectional, retrospective study used the INEGI national death registry via the Public Health Intelligence Unit (UISP-INSP). Deaths in children aged 1-14 years attributed to cSLE were identified using ICD-10 codes M32.1, M32.8, and M32.9, excluding M32.0. ASMRs per...

CONCLUSION: Autoimmune DAH in pediatric patients carries high morbidity, often requiring advanced respiratory support and multimodal immunosuppression. Early diagnostics work up and initiation of TPE may improve outcomes. Further prospective pediatric study would be necessary to guide standardized treatment protocols.

CONCLUSION: In this cohort of paediatric patients with rheumatic diseases, no significant association was observed between cumulative hydroxychloroquine use and QT interval prolongation.

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CONCLUSION: ACEs exposure was prevalent in this cSLE cohort, associated with NPSLE diagnosis, disease damage and comorbid psychiatric diagnoses. These findings suggest that early-life stressors may contribute to cSLE morbidity, particularly neuropsychiatric features, emphasising the importance of addressing psychosocial issues in the context of medical care.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare idiopathic inflammatory disorder characterized by low incidence and a propensity for misdiagnosis. Although its etiology remains elusive, KFD is often linked to underlying autoimmune conditions. Definitive diagnosis requires lymph node biopsy. Classically, patients present with cervical lymphadenopathy and fever; however, this report describes an atypical, severe manifestation in a patient whose...

Objective: Systemic lupus erythematosus (SLE), Sjögren syndrome (SS) and antiphospholipid syndrome (APS) are common autoimmune conditions in child-bearing aged women, but their influence on pregnancy and assisted reproductive outcomes remain controversial. We aimed to perform an umbrella review to summarize the current evidence to provide a reference for clinicians and future research. Methods: PubMed, Embase (Ovid) and Cochrane database were searched (inception to April 2025) for relevant...

CONCLUSION: Early diagnosis and timely intervention enhance outcomes in fetal congenital heart block, but many patients still require pacemakers, highlighting the importance of careful monitoring. Advancing risk stratification, exploring novel therapies, and conducting multicenter research are essential for optimizing management and further understanding the impact of maternal autoimmunity.

CONCLUSIONS: The CHSQ demonstrates strong psychometric properties for assessing perceived health status in Thai children with SLE, providing culturally appropriate measurement tools for nursing assessment and clinical practice.

Pediatric neurological autoimmune diseases (PNADs) are a result of immune system abnormalities that target the central and peripheral nervous systems, leading to various neurological dysfunctions in children. The limitations of current immunotherapies underscore the necessity for more efficacious treatment interventions. The objective of this review is to examine the fundamental principles, recent advancements, and clinical applications of chimeric antigen receptor (CAR) T cell therapy in the...

Gitelman syndrome (GS) is an autosomal recessive disorder caused by mutations in the SLC12A3 gene, characterized by hypomagnesemia, hypokalemia, alkalosis, and hypocalciuria. Finerenone, a non-steroidal mineralocorticoid receptor antagonist (MRA), has shown cardiorenal protective effects and improved prognoses in type 2 diabetes (T2DM) and chronic kidney disease (CKD). However, its use in non-diabetic nephrology has been infrequently reported. This report presents a case of a male child with GS...

CONCLUSION: Urinary ANGPTL3/Cre is a reliable, noninvasive biomarker for podocyte injury in pediatric glomerular diseases. Notably, it may have potential value in identifying subclinical podocyte injury in patients with normal protein excretion, thereby supporting its robust potential for longitudinal monitoring and risk stratification in this patient population.

CONCLUSIONS: This study confirmed that HRS promotes diabetic wound healing by alleviating oxidative stress and inflammation, and these effects may be mechanistically associated with the RAS pathway.

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

Chimeric antigen receptor T (CAR-T) cell therapy has achieved remarkable success in haematological malignancies, ushering in the era of adoptive cell therapy. In recent years, CAR-T cell therapy has been progressively explored in autoimmune diseases (AIDs), with the goal of inducing deep immune remodelling and durable drug-free remission in selected patients. This review evaluates CAR-T cell therapy in AIDs through three clinically relevant dimensions: depth, breadth, and length. We use this...

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...

CONCLUSIONS: ASM is associated significantly and positively with anthropometric and body composition parameters such as body mass, BMI, LTM and LTI both in patients with eGFR < 60 ml/min/1.73 m2 and in those with eGFR ≥ 60 ml/min/1.73 m2 which suggests that the determinants of ASM are similar in CKD and in the general population.

Median Arcuate Ligament Syndrome (MALS) typically causes gastrointestinal symptoms due to celiac artery compression. Rarely, extrinsic compression of the renal artery may lead to renovascular hypertension. We report a 15-year-old boy with progressive renal asymmetry and poorly controlled hypertension without abdominal symptoms. Imaging demonstrated compression of the celiac trunk and left renal artery by the median arcuate ligament and diaphragmatic crura. Open surgical decompression restored...

BACKGROUND: Blood purification techniques are rarely used in low-weight pediatric patients because extracorporeal circuits and devices designed for adults may expose neonates and infants to significant adverse events. Recently, hemoadsorption cartridges with low priming volumes (Jafron®) have become available.

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CONCLUSION: CKD screening uptake among adults on ART was high, but screening was influenced by clinical and service factors. Higher CD4 counts and more frequent clinic attendance were associated with increased screening, while provider recommendations were paradoxically linked to lower screening, highlighting gaps in translating advice into action. Strengthening integrated, patient-centered approaches and ensuring systematic CKD screening across all patient groups is essential to optimize early...

Cystic kidney diseases are an important cause of pediatric morbidity and mortality. Most genetic cystic kidney diseases are "ciliopathies", a group of disorders that share a common feature of abnormal structure or function of the primary cilia. Ciliopathies represent a broad range of clinically and genetically heterogenous disorders, which affect multiple organ systems. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are the 2 most...

Alport syndrome is a genetic kidney disease caused by variants in genes encoding the ⍺3, ⍺4, or ⍺5 isoforms of type IV collagen leading to hematuria, proteinuria, and CKD in many, but not all, affected patients. Variants in type IV collagen genes have been increasingly recognized and may affect up to 1 in 106 people. There is wide phenotypic variability depending on inheritance pattern (autosomal recessive vs autosomal dominant vs X-linked vs digenic), sex of the affected individual, specific...

CONCLUSIONS: PSC is a STAR prospective study which will address a major gap in our understanding of outcomes post-HCT specific to SCD. WeDecide, a larger study comparing HCT health-related quality of life outcomes to those who receive non-transplant disease modifying therapy (NT-DMT) is in development, and PSC will provide the HCT comparator data. These data will also be highly relevant as other curative and transformative therapies, such as gene therapy, become more widely used.

Congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of pediatric kidney failure, but predicting individual progression remains challenging. This multicenter study developed and validated POCC, a machine learning model for predicting kidney failure risk at 1, 3, and 5 years post-diagnosis in CAKUT patients. Two versions were created using data from 2249 children. The general model achieved internal AUCs of 0.93-0.99 and external AUCs of 0.89-0.98 and 0.81-0.90 in...

CONCLUSIONS: We provide a review of the literature on malaria-associated AAC, which identified 28 previously reported cases, only 7 in children. Most reported patients contracted the infection in malaria-endemic regions but were then managed in high-income countries. The majority were managed conservatively and had favorable outcomes. Including our series, 32 cases (11 pediatric) have now been reported. Our findings suggest that AAC, which can often occur in association with BWF and AKI, is...

CONCLUSION: Mortality among CKD with co-occurring depression has accelerated sharply since 2015, diverging markedly from the plateauing trend of CKD overall. Routine depression screening integration into nephrology care is urgently warranted, especially for high-risk populations.

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

Hughes-Stovin syndrome (HSS) is a rare and aggressive type of systemic vasculitis which is characterized by peripheral venous thrombosis and pulmonary artery aneurysms (PAAs) at the onset of the disease, as well as the absence of recent or previous attacks of uveitis. The onset of HSS and its dominant clinical disease presentations differ in many aspects from those observed in Behçet's disease (BD). The absence of uveitis is a mandatory exclusion criterion in the preliminary diagnostic criteria...

CONCLUSIONS: The SIRS and PSC identify partially overlapping populations with distinct risk stratification, showing poor diagnostic concordance. The PSC has superior independent predictive performance for PICU 28-day mortality and may be considered for prognostic assessment of infected children in the PICU setting. Importantly, historical study results based on the SIRS criteria should be extrapolated cautiously to PSC-defined populations.

CONCLUSIONS: Outpatient CGA did not improve goal attainment or clinical outcomes in frail older adults with CKD. These findings do not support routine implementation of single-episode outpatient CGA in this setting and suggest a need for alternative, integrated models of person-centered care.

CONCLUSIONS: Urinalysis parameter combinations may help predict urine culture positivity (≥ 10⁵ CFU/mL) in children undergoing CIC, though these findings cannot distinguish between asymptomatic bacteriuria and clinically significant UTI requiring treatment.

CONCLUSION: FC may serve as a useful non-invasive biomarker for predicting colonoscopic abnormalities in pediatric FMF patients. An FC level below 123 µg/g appears to reliably predict normal colonoscopic findings, suggesting that unnecessary invasive procedures may be avoided in this subgroup. Key Points • Fecal calprotectin levels correlated with the severity of colonoscopic abnormalities in FMF patients with gastrointestinal symptoms. • A fecal calprotectin level below 123 µg/g feces strongly...

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CONCLUSIONS: A five-gene signature was derived from m6A-related targets, which shows reliable diagnostic potential for SSc-PAH and correlates with the immune microenvironment. These findings provide the first integrative transcriptomic evidence connecting m6A-related regulation with immune dysfunction in SSc-PAH, offering mechanistic insight and potential diagnostic biomarkers.

CONCLUSION: Somatrogon's real-world AE pattern shows early-onset injection-site reactions, device-related issues, and endocrine signals, highlighting the need for early treatment monitoring and ongoing post-marketing surveillance as its use grows globally.

CONCLUSION: Moderate hepatic impairment has a minimal impact on the pharmacokinetics of Ritanine, suggesting that no dosage adjustment is necessary for patients with moderate hepatic impairment in clinical practice.

Mobile phone addiction is a significant public health issue among college students, associated with poor mental health and procrastination, yet the underlying mechanisms involving attention problems are not well understood. A cross-sectional survey was conducted among college students in Zhejiang Province. Linear regression models were employed to assess the relationships between mobile phone addiction, mental health, and procrastination. Statistical mediation analysis was performed to evaluate...

Point-of-care ultrasonography (POCUS) has revolutionized pediatric healthcare by offering immediate, bedside diagnostic capabilities that enhance clinical decision-making and patient management. This comprehensive review examines the impact and necessity of POCUS in assessing renal conditions in children. By synthesizing current literature, clinical applications, diagnostic accuracy, and patient outcomes associated with POCUS, this paper highlights its essential role in pediatric nephrology....

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

CONCLUSION: EIF5A and TGIF1 DNA methylation represent cross-tissue epigenetic signatures linking blood-based DNA methylation to adipose tissue dysfunction, adipocyte hypertrophy, and early metabolic risk. These findings support the potential of blood DNA methylation markers to reflect adipose tissue health and metabolic outcomes across the lifespan.

CONCLUSIONS: Semaglutide reduced lean body mass and fat mass in patients with CKD with overweight status or obesity. These changes did not correlate with changes in creatinine or cystatin C eGFR or mGFR, suggesting that body weight reductions of 10% with semaglutide do not influence GFR estimates.

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

Acute kidney injury (AKI) and fluid accumulation (FA) are common during extracorporeal membrane oxygenation (ECMO) support and are associated with increased morbidity and mortality. Despite their clinical importance, contemporary data describing the epidemiology and management of these conditions remain limited. Founded in 2021, the Worldwide Exploration of Renal Replacement Outcomes Collaborative in Kidney Disease (WE-ROCK) seeks to improve outcomes for children requiring extracorporeal organ...

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CONCLUSIONS: Current evidence underscores substantial gaps in understanding the natural history and optimal management of diabetic foot disease in dialysis-dependent ESKD. Future research should prioritise well-designed prospective studies to delineate dialysis-specific risk pathways, incorporate robust stratification by dialysis modality, and evaluate interventions targeting ischaemia and limb preservation. Standardisation of outcome reporting, particularly for healing durability and...

CONCLUSIONS: Despite increased BPV and arterial stiffness in children and adolescents with obesity, short-term BPV was not independently associated with LVMI or LVH. Further prospective studies are warranted to clarify the clinical relevance of BPV in pediatric obesity.

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

Mitochondrial protein import machineries are essential for organelle homeostasis. Metaxin-2 (Mtx2) is an evolutionarily conserved component of the mitochondrial sorting and assembly machinery, and its mutations are associated with a progeroid syndrome, named mandibuloacral dysplasia associated to Mtx2 (MADaM). To investigate the pathologic mechanisms of MADaM, we developed Mtx2 genetic models in Drosophila. Mtx2 null mutants are lethal at a preadult stage, and this phenotype can be rescued by...

CONCLUSIONS: Given its strong performance and minimal computational overhead, we recommend augmenting TabPFN through sampling with replacement as the optimal approach for small-data binary classification tasks. This method achieves performance comparable to that of more complex SDG techniques while offering substantial computational advantages.

CONCLUSION: Compared with traditional transfer to adult rheumatology, the YARC reduced acute care utilization and improved the no-show rate. Additionally, young adult rheumatology patients valued the PCMH model and were more likely to adhere to recommended preventive care practices.

CONCLUSION: This best-evidence summary comprehensively synthesizes evidence-based health education for pediatric urinary tract infections. The rigorous methodology and broad content coverage provide valuable scientific guidance for healthcare professionals involved in UTI health education.

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

The 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,...

Medical ozone therapy has been increasingly explored as a potential adjunctive intervention across a range of clinical conditions. This narrative review aims to provide a critical overview of the biological mechanisms, biochemical interactions, and clinical applications of ozone therapy, with particular emphasis on the translational gap between experimental findings and clinical evidence. Ozone exerts its biological effects primarily through the generation of reactive oxygen species (ROS) and...

CONCLUSION: Some anti-inflammatory agents may reduce the risk of cardiovascular outcomes, but their effects appear to vary substantially across drug classes, with important differences in both efficacy and safety. These findings underscore the need for rigorous evaluation of new anti-inflammatory therapeutics to ascertain benefits and harms across different populations and therapeutic indications.

CONCLUSIONS: This study demonstrated APOL1 to be a membrane protein involved in the influx of Ca2+ from the extracellular medium. Increased activity of APOL1 led to markedly increased cytotoxicity, which supports the gain-of-function theory. This effect was prevented by the presence of N264K variant or treatment with the APOL1 inhibitor VX-147.

CONCLUSIONS: This study confirms the established epidemiologic and clinical profile of PFAPA syndrome in the pediatric population, highlighting its characteristic manifestations and the high effectiveness of glucocorticoid therapy and tonsillectomy. The findings may contribute to consolidating current knowledge about PFAPA and to reducing the inappropriate use of antibiotics.

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

CONCLUSION: Overall, PUV was not associated with lower QoL or family impact scores compared to comparator groups in this tertiary-care cohort; however, lower scores were observed among younger children with PUV, likely reflecting parent-perceived burden early in the disease course. This highlights the importance of age-tailored support and close monitoring, particularly for younger children and families navigating early post-diagnosis care.

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,...

BACKGROUND: Meningitis remains the leading infectious cause of neurological disabilities globally, disproportionately affecting children younger than 5 years and populations in the African meningitis belt. Whereas previous global estimates focused on ten pathogen categories, this study presents the most comprehensive analysis to date, assessing the meningitis burden attributable to 17 causative pathogens based on the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2023...

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)...

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...

CONCLUSIONS: Our results confirm the high transmission of subclinical disease among household contacts, which may vary due to psychosocial factors. This reinforces the importance of isolating cases to prevent transmission, regardless of vaccination status.

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

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: 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...

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

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: Coronavirus disease 2019 infection could be a possible trigger factor for acute interstitial nephritis and Vogt-Koyanagi-Harada disease. Early diagnosis and treatment of these autoimmune features with corticosteroids and other antiinflammatory agents can help in faster improvement in these patients. In addition, it is crucial for physicians to consider Vogt-Koyanagi-Harada disease in pediatrics as one of the coronavirus disease 2019 complications for early diagnose and current...

CONCLUSIONS: Electrocardiometry can assess the hemodynamic profile of children receiving CKRT. Compensatory cardiovascular changes remain intact in children receiving CKRT, as evidenced by correlations between SVI, SVRI, CI, and MAP. Future studies should investigate how this technology could enable more individualized CKRT prescriptions and improve patient outcomes.

CONCLUSION: In this study, despite facing the challenge of fear of death, nurses have tried to adhere to ethical principles, however, it is recommended to investigate other factors affecting the moral performance of nurses.

CONCLUSIONS: PIGN incidence decreased during the early COVID-19 pandemic (2020-2022), followed by a resurgence of cases with an altered seasonality pattern. During the pandemic, children with PIGN were younger and had milder disease severity.

CONCLUSIONS: The differential roles of IL-6, NLR, and WBC in predicting AKI onset highlight distinct physiopathological pathways influenced by COVID-19. In CKD+ patients, chronic inflammation and immune dysregulation are key drivers of AKI, with IL-6 and NLR serving as robust markers of this inflammatory state. In contrast, in CKD- patients, AKI may be more influenced by acute inflammatory responses and infectious factors, as reflected by WBC count.

Background/Objectives: COVID-19 has impacted Romania's healthcare, economy, society, and public health. This study aims to evaluate the financial impact of the COVID-19 pandemic in Romania by analyzing both hospital costs and key elements of economic costs. The assessment was conducted from the perspective of the national payer. Hospital costs were analyzed covering two distinct timeframes: Q4 2020-Q3 2021 and Q1 2022-Q4 2022. The estimation of economic costs covered Q4 2020-Q3 2021. Methods:...

CONCLUSION: In our small single-center cohort, SARS-CoV-2 vaccination or infection is unlikely to increase the risk for rejection or de novo DSA in pediatric kidney transplant recipients. Larger prospective studies with a control group are needed to further understand the immune effects of the COVID-19 vaccine and disease in this population.

The use of extracorporeal membrane oxygenation has been increasing over time, in part due to the COVID-19 pandemic. Whilst lifesaving, complications that must be managed are also associated with its use. AKI and fluid overload are complications of concern due to their associations with poor outcomes, and ability to be managed by additional interventions such as the use of kidney replacement therapy. Various modalities, timings, and types of kidney replacement therapy are currently being used and...

CONCLUSIONS: In a systematic pre/post comparison of individual-level relapse frequency, we found no significant difference in risk or rates of relapse after COVID-19 vaccination in children with NS.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

CONCLUSIONS: Muscle-specific complications are often overlooked in systemic cystinosis treatment. We show that defective CTNS function impairs effective cystine mobilization from lysosomes, thereby affecting the protein levels of myogenic regulators. A deeper understanding of the molecular mechanisms underlying cystinosis myopathy holds promise for the development of targeted, personalized therapies to improve the quality of life for patients living with cystinosis.

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.

CONCLUSIONS: All patients with iFH-N had similar clinical presentation, appeared to be refractory to aggressive IS, and had poor renal outcome.

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

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

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

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

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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 pharmacokinetics of eculizumab are similar in patients with atypical hemolytic uremic syndrome and patients with paroxysmal nocturnal hemoglobinuria, yet less variable in patients with paroxysmal nocturnal hemoglobinuria. Alternative dosing regimens can improve treatment in terms of efficacy and patient friendliness.

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

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

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

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

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

CONCLUSIONS: ChatGPT exhibited substantial potential in addressing patient inquiries regarding rare kidney diseases in a real-world context. While it demonstrated resilience against misinformation in this application, careful human oversight remains essential and indispensable.

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

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

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CONCLUSIONS: Significant histologic LN findings are observed in 30% of SLE patients without overt kidney disease; frequently associated with high-titer dsDNA, anti-Smith antibodies, and/or hypocomplementemia. Thus, baseline kidney biopsy in newly diagnosed SLE patients, irrespective of clinical and laboratory manifestations, may aid in guiding therapy.