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...

No abstract

No abstract

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...

No abstract

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.

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

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

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

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

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

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

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

CONCLUSION: Childhood HUS in this cohort is dominated by aHUS and secondary types. Early etiological differentiation, comprehensive laboratory assessment and targeted therapy improve outcomes, with findings aligning with global data but showing a more pronounced female bias due to high SLE-related cases.

PURPOSE OF THE PROGRAM: Children with chronic kidney disease (CKD) experience significant physical and psychological symptoms, necessitating patient-reported outcome (PRO) measurement tools to quantify symptoms, and to improve communication between children with CKD and their health care providers. This study aimed to implement the novel PRO-Kid tool into pediatric CKD and dialysis programs in Canada.

Children of transplanted mothers are at increased risk of adverse birth outcomes, but childhood health outcomes are undefined. Using linked data from the Australia and New Zealand Dialysis and Transplant Registry, perinatal and hospital datasets, admissions were compared between children of transplanted mothers and mothers not exposed to kidney replacement therapy. From 2 067 661 babies, 137 children of transplanted mothers (137 birth admissions) were identified; 93 had 444 subsequent...

CONCLUSION: We report a rare case of focal myocardial calcification with pathological Q waves in a maintenance dialysis patient. Chronic kidney disease (CKD)-related disturbances of calcium-phosphate metabolism can cause metastatic myocardial calcification. Severe focal calcification may produce mechanical compression and cell necrosis, disrupt electrical coupling, create electrically silent zones, and result in pathological Q waves. In CKD patients with abnormal ECG findings, myocardial...

Proteinuria is a common laboratory finding in adolescents. It is often benign and due to transient causes or orthostatic proteinuria. However, it can also be an early sign of underlying conditions that may lead to long-term kidney damage. Early recognition and appropriate diagnostic evaluation are crucial to preventing or slowing disease progression. In this age group, proteinuria may result from newly diagnosed diseases, pre-existing conditions that become clinically evident during adolescence,...

Background and objective Kidney transplantation is the preferred treatment for children with end-stage kidney disease (ESKD), offering superior survival, quality of life, and growth outcomes compared with dialysis. Achieving successful outcomes requires thorough preparation and strict adherence to standardized protocols. This study aimed to report the quality measures and standardized preparation protocol for pediatric kidney transplantation at Prince Sultan Military Medical City (PSMMC),...

CONCLUSION: This study suggests that in young patients with SCD without known nephropathy, the CKiDU25 equation using serum cystatin C, provides GFR estimates close to the gold standard isotopic measurement. Early tubular dysfunction is prevalent and may justify therapeutic interventions. These findings warrant confirmation in larger cohorts.

CONCLUSIONS: Chronic HCV infection may contribute to immune tolerance and reduced allergic expression in BT patients, potentially modulated by IL10 and TLR7 genotypes. Further studies with functional immune profiling and larger cohorts are required.

CONCLUSION: High index of suspicion is important in diagnosing inborn errors of metabolism. Even in resource-limited setting, a multidisciplinary team with international partnership can optimize the care for patients with rare inborn errors of metabolism. There is also a need to increase awareness, improve diagnostic capacity and establish standardized treatment protocols for rare metabolic disorders in low-resource settings like Tanzania.

CONCLUSIONS: Rituximab reduced the risk for INS relapse, and maintenance treatment between 6 and 12 months was associated with further reduction in relapses. Prospective studies are required to better specify the benefit of rituximab maintenance therapy.

CONCLUSION: For children and young adults with LN requiring CYC, use of the EuroLupus regimen increased over time and is associated with demographic and clinical factors such as race or Hispanic ethnicity, renal impairment, and absence of neuropsychiatric involvement. The differences in regimen use with severe renal impairment and neuropsychiatric lupus highlight areas for future study in CYC dosing.

IgA nephropathy (IgAN) is the most common primary glomerulonephritis, typically presenting early in life, often in young adults but also frequently in childhood. This chronic disease can account for up to 50% of cases progressing to kidney failure, particularly when it clinically begins at a young age. Currently validated treatments, such as renin-angiotensin blockers, SGLT-2 inhibitors, and corticosteroids, can slow disease progression, but with limited efficacy. In light of this, novel...

TSC2/PKD1 contiguous gene deletion syndrome (PKDTS) is characterized by poor renal prognosis. We encountered a female patient with a history of facial angiofibromas since childhood who developed seizures and was subsequently diagnosed with tuberous sclerosis complex. The patient later progressed to kidney failure requiring replacement therapy at 23 years of age. Imaging studies showed polycystic kidney disease (PKD) and angiomyolipoma (AML), followed by renal hemorrhage in both kidneys. Genetic...

Advancements in pediatric cancer treatment protocols have significantly improved long-term survival. This has been accompanied by a growing recognition of morbidity and mortality associated with late effects of treatment, including kidney disease. Surviving cancer in childhood implies exposure to multiple nephrotoxic insults, some of which carry a greater risk for the development of chronic kidney disease and progression to kidney failure than others. In childhood cancer survivors who develop...

Rituximab maintains remission of complicated frequently relapsing or steroid-dependent nephrotic syndrome (FRNS/SDNS) by depleting peripheral B cells, but most patients eventually experience relapses after B cell recovery. We performed a multicenter, double-blind, randomized, placebo-controlled trial to assess rituximab's efficacy and safety for childhood-onset uncomplicated FRNS/SDNS (without prior treatment with glucocorticoid-sparing immunosuppressive agents) with a follow-up study to assess...

No abstract

IgA nephropathy (IgAN) is the most common glomerulonephritis worldwide, with significant implications for adults and children. The disease progresses variably, from asymptomatic hematuria to severe glomerulonephritis, and around 10-20% of children diagnosed in childhood develop stage 5 chronic kidney disease (CKD 5) within 20 years. Identifying reliable prognostic markers is crucial for early intervention and long-term management. The International IgAN Prediction Tool combines clinical,...

BACKGROUND: While testicular germ cell tumors (TGCT) survival exceeds 90%, many survivors of adult TGCT are at risk for treatment toxicities. Less is known about physical morbidities in children, adolescents, and young adults (CAYA) with TGCT.

Background and Objectives: The nephrotic syndrome (NS) is the most common acquired childhood kidney disease. Steroids represent the cornerstone of the therapeutic strategy, representing the first-line approach, but optimal therapeutic management is debated. This study aimed to compare different steroid therapeutic management protocols. Patients and Methods: A total of 140 NS pediatric patients were enrolled retrospectively. All the kids were divided among three different groups according to the...

CONCLUSION: Individuals with XLH often experience unmet needs throughout life; a multidisciplinary approach involving different specialists, is recommended. The new treatment with burosumab can provide an effective and safety therapeutic option in reducing the burden of the disease in both children and adults. Therefore, awareness about the XLH disease should be increased among stakeholders. The criteria and reimbursement policies of burosumab should be revised.

A young woman with a history of thrombocytopenia was treated for idiopathic thrombocytopenic purpura (ITP) with splenectomy, intravenous immunoglobulin, steroids and chemotherapeutic agents. The patient experienced hearing loss during childhood and, as a teenager, was diagnosed with hypertension and nephrotic-range proteinuria, which progressed to renal failure requiring dialysis. On presentation to our institution, her platelet count was 13×10⁹ /L. Peripheral blood smear showed giant platelets...

CONCLUSION: The finding of this MYH9 p.R424Q variant confirmed a diagnosis of MYH9-RD in these patients. MYH9 variants affecting the head domain typically result in severe thrombocytopenia. This recently reported head domain variant caused severe renal manifestations with mild thrombocytopenia and no manifestations of SNHL or cataracts in both patients, suggesting that this variant causes a renal-predominant form of MYH9-RD.

CONCLUSIONS AND RELEVANCE: In this population-based study, CCS were at increased risk for CKD and hypertension, which are associated with mortality, suggesting that early detection and treatment of these conditions in CCS may decrease late complications and mortality.

ANCA-associated vasculitis (AAV) is a group of rare small vessels vasculitis that preferentially affect the kidneys, lungs and upper airways. Although the detailed pathophysiology remains unclear, genetic background has been shown to play a role in sporadic forms of AAV. The discovery of these susceptibility genes (and associated biological pathways) involved in AAV have shaped the current understanding of AAV pathophysiology. In addition to common genetic polymorphisms, specific rare inborn...

BACKGROUND: Fabry disease (FD) is a rare, X-linked lysosomal storage disorder that affects both males and females. It is caused by pathogenic variants in the gene that encodes the enzyme α-galactosidase A, GLA. The classic form of the disease begins in childhood, presenting with a range of signs and symptoms that can lead to severe complications such as stroke, as well as cardiac and renal failure. In the late-onset form, the disease appears in adulthood, often with signs of cardiac involvement.

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

Data on the presentation of Autosomal Dominant Polycystic Kidney Disease (ADPKD) in children have been based on small/regional cohorts and practices regarding both asymptomatic screening in minors and genetic testing differ greatly between countries. To provide a global perspective, we analyzed over 2100 children and adolescents with ADPKD from 32 countries in six World Health Organization regions: 1060 children from the multi-national ADPedKD registry were compared to 269 pediatric patients...

CONCLUSION: Structural inequities in area-level child opportunity may contribute to disparities in both cSLE severity and disease control. Tailoring interventions for communities with low levels of child opportunity may improve access to pediatric subspecialty care and cSLE outcomes.

CONCLUSIONS: Identifying VUS is a recurring challenge in routine clinical genetics, particularly for patients with rare diseases or atypical phenotypes in underrepresented populations. This case underscores the benefit of timely genetic diagnosis taking into account the patient's request. VUS reassessment becomes more relevant when considering a kidney transplant not only as an appropriate procedure, but as the therapy of choice, especially considering the patient's history of complications with...

CONCLUSIONS AND RELEVANCE: In this case-control study, age-related neurodevelopmental differences were observed in pediatric patients with CKD compared with healthy peers. Reductions in cerebellar volume were associated with cognitive deficits and lower kidney function. These findings underscore the importance of monitoring neurodevelopmental trajectories in children with CKD, as early interventions may be necessary to mitigate cognitive impairments associated with CKD.

Fabry disease (FD) belongs to the group of lysosomal storage diseases (LSD), characterized by insufficient enzyme activity responsible for the intra-lysosomal breakdown of various substrates. The result is an uncontrolled accumulation of by-products of cellular metabolism. Lysosomal storage diseases are inherited and transmitted mainly in an autosomal recessive fashion. Without a positive family history, an early diagnosis can often be missed. In addition, the age of clinical manifestation can...

Introduction. Health-related quality of life (HRQL) and its social consequences have not been evaluated in adults who started renal replacement therapy (RRT) in childhood/adolescence and are currently on hemodialysis. Population and methods. We compared 26 patients who started their RRT at 50 indicate good HRQL. Results. The study was conducted in 2018....

Background/Objectives: Hemolytic uremic syndrome (HUS) is a known cause of acute kidney injury in children, but there are few recent reports on its epidemiology and outcome. We aimed to investigate trends in the incidence and the long-term outcomes of both Shiga toxin-producing Escherichia coli -HUS (STEC-HUS) and atypical HUS (aHUS) in Poland over the last 12 years (2012-2023), based on the Polish Pediatric HUS and Pediatric Renal Replacement Therapy (RRT) Registries. Methods: A total of 436...

The efficacy and safety of rituximab in childhood steroid-resistant nephrotic syndrome (SRNS) remains unclear. Therefore, we conducted a retrospective cohort study at 28 pediatric nephrology centers from 19 countries in Asia, Europe, North America and Oceania to evaluate this. Children with SRNS treated with rituximab were analyzed according to the duration of calcineurin inhibitors (CNIs) treatment before rituximab [6 months or more (CNI-resistant) and under 6 months]. Primary outcome was...

Paroxysmal cold hemoglobinuria (PCH) is among the rarest forms of autoimmune hemolytic anemia, most often seen in young children. PCH is caused by a biphasic immunoglobulin G antibody that binds to red cells at low temperatures and causes complement-mediated lysis as the temperature is raised. Diagnosis is based on high clinical suspicion followed by confirmation of the presence of Donath-Landsteiner antibodies. We have described 3 cases diagnosed with PCH over a span of 1 year, 2 cases...

To promote improved trial design in upcoming randomized clinical trials in childhood chronic kidney disease (CKD), insight in the within- and inter-patient variability of uremic toxins with its nutritional, treatment- and patient-related confounding factors is of utmost importance. In this study, the within- and inter-patient variability of a selection of uremic toxins in a longitudinal cohort of children diagnosed with CKD was assessed, using the intraclass correlation coefficient (ICC) and the...

Early-onset systemic lupus erythematous (SLE) is a distinct clinical entity characterized by the onset of disease manifestations during childhood. Despite some similarities to patients who are diagnosed during adulthood, early-onset SLE typically displays a greater disease severity, with aggressive multiorgan involvement, lower responsiveness to classical therapies, and more frequent flares. Lupus nephritis is one of the most severe complications of SLE and represents a major risk factor for...

CONCLUSION: Concomitant inheritance of ADPKD and development of WT are extremely rare, and manifestations of ADPKD may not present until late childhood or adulthood. ADPKD is not a known predisposing condition for WT. When ADPKD diagnosis is made by family history, imaging, and/or genetic testing before WT diagnosis and treatment, the need for extensive preoperative characterization of cystic kidney lesions in children and increased risk of post-nephrectomy kidney failure warrant further...

While it is widely accepted that the nutritional management of the infant with chronic kidney disease (CKD) is paramount to achieve normal growth and development, nutritional management is also of importance beyond 1 year of age, particularly in toddlers, to support the delayed infantile stage of growth that may extend to 2-3 years of age. Puberty is also a vulnerable period when nutritional needs are higher to support the expected growth spurt. Inadequate nutritional intake throughout childhood...

CONCLUSION: In our cohort of patients with cloacal malformations following a strict renal protection protocol, incidence of progressive renal dysfunction is low at 2.9%. Most who go on to renal dysfunction present with impaired renal function.

CONCLUSION: The present study demonstrated that, especially IxS contributes to a lower height velocity in (pre)school children, whereas we could not find a role for uremic toxins with height velocity during pubertal stages.

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

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

CONCLUSION: Elevated ApoB levels lead to a dose-dependent overestimation of albumin concentration when measured using BCG method. Thus, it is advisable to utilize the ITA method for the accurate determination of serum albumin levels in PNS patients. Meanwhile, when ITA is not readily available, the bromocresol purple (BCP) method can serve as a more practical alternative. This ensures a proper assessment of colloid osmotic pressure and nutritional status, thereby facilitating clinical diagnosis...

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...

CONCLUSIONS: PCDH7-positive MN shows clinicopathological heterogeneity. PCDH7 can be detected in both PLA2R-positive and PLA2R-negative MN patients, with similar positive rates between the two groups. No consistent clinical presentation was observed in PCDH7-positive patients; instead, this group included a variety of potential secondary conditions. Additionally, anti-PCDH7 antibody levels did not correlate with clinical manifestations and outcomes in our preliminary observation.

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

No abstract

CONCLUSION: Primary glomerulopathies were predominant, while oedema and nephrotic syndrome were common findings.

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...

BACKGROUND: Kidney xenotransplantation has re-emerged as a viable therapeutic strategy to address the global shortage of donor organs, driven by substantial advances in porcine genetic engineering and immunomodulatory therapies. Clinical experiences in brain-dead decedents and, more recently, in living recipients have demonstrated that genetically modified pig kidneys can provide life-sustaining renal function in humans, marking a pivotal milestone in the field.

CONCLUSIONS: When considered necessary to establish a diagnosis during pregnancy, kidney biopsy performed in the first two trimesters appears to be safe, with a high diagnostic yield and a significant impact on therapeutic decision-making and patient management.

CONCLUSIONS: SGLT2i therapy showed acceptable safety and a possible reno-metabolic benefit in non-diabetic KT recipients. Controlled trials with longer follow-up are required to determine the robustness of this indication.

CONCLUSIONS: Qualitative methods can provide patient-informed meaningful change thresholds on global impression anchors, although patients may struggle to identify meaningful change for performance anchors. Nonetheless, qualitative patient-informed thresholds can inform quantitative COA interpretation and strengthen endpoint development and regulatory dialogue in rare disease clinical trials.

BACKGROUND: Parkinson's disease (PD) is a type of neurodegenerative diseases with the characterized by static tremors, bradykinesia, and muscle rigidity. This is mainly caused by the degradation and death of dopaminergic neurons in the substantia nigra, severely affects the health of the elderly no matter in developing or developed countries. In recent years, Traditional Chinese medicine (TCM) has made significant advances in the treatment and application of PD with its unique advantages in...

A previously healthy 44-year-old man presented with acute severe dyspnea. Laboratory evaluation revealed severe hypoalbuminemia, elevated low-density lipoprotein cholesterol and D-dimer levels, and marked proteinuria, suggesting nephrotic syndrome (NS). Imaging demonstrated bilateral pulmonary emboli with right ventricular strain. Since he developed progressive shock, veno-arterial extracorporeal membrane oxygenation (VA ECMO) and thrombolytic therapy were initiated. Persistent thrombi and...

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

Monoclonal gammopathy of renal significance (MGRS) is a clonal cell proliferative disorder, characterized by the production of monoclonal immunoglobulins in patients that do not meet hematological criteria for a specific malignancy. It can be present in B cell and plasma cell clonal proliferative diseases and accounts for 10% of monoclonal gammopathy of undetermined significance (MGUS) cases. We present a case of a patient presenting with acute kidney injury, hematuria, and nephrotic syndrome,...

This study aimed to investigate regional epidemiological trends of chronic kidney disease (CKD) to elucidate their etiologies and inform the development of preventive and diagnostic strategies. We retrospectively analyzed 2100 hospitalized patients undergoing native renal biopsy from January 1, 2012, to December 31, 2021. The clinical presentation, the prevalence of nephropathy revealed by biopsy, the age-distribution differences of main pathological types, and changes in the prevalence of...

This case report describes a rare instance of minimal change disease (MCD) associated with Mycobacterium marinum infection. MCD is typically idiopathic but can occasionally occur secondary to infections. The patient was a 61-year-old fisherman with a history of relapsing MCD who developed a subcutaneous abscess in the lower leg while undergoing rituximab and glucocorticoid therapy. Ziehl-Neelsen staining revealed acid-fast bacilli, and culture at 30 °C isolated M. marinum, a slow-growing...

CONCLUSION: Bailing Capsule may serve as a safe and effective adjunctive therapeutic option for adult patients with PNS, with positive effects on improving renal function indicators, regulating immune function and enhancing clinical efficacy. However, further high-quality, large-scale and multicenter RCTs are still required to validate the long-term efficacy and safety of Bailing Capsule for adult PNS.

CONCLUSIONS: This work presents the first machine learning-based early prediction model for SDNS/FRNS using clinical big data from the acute phase of the condition. Additionally, it introduces an online prediction tool for paediatric SDNS/FRNS.

Lymphomatoid granulomatosis is a rare lymphoproliferative disease caused by dysfunction of host surveillance of Epstein-Barr virus (EBV)-infected B cells, which predominantly affects male adults, with a median onset at 46 years. Diagnosis can be challenging due to its nonspecific symptoms and the need for careful histopathological evaluation, particularly in atypical presentations. Here, we present a case of a 42-year-old Black man from Senegal with no past medical history who presented with a...

CONCLUSIONS: Collectively, these insights inform the Prepare-NS initiative's ongoing development and validation efforts. Incorporating standardized COAs in NS clinical trials could expand efficacy endpoints and pave the way for patient-centered NS drug development.

BACKGROUND Rituximab (RTX) is a chimeric IgG monoclonal antibody directed against the CD20 antigen expressed on normal and malignant B cells. It is used for treating a variety of neoplastic, autoinflammatory, and autoimmune pathologies, including disorders occurring in the pediatric population. It has been proven that rituximab can induce pulmonary complications including interstitial lung disease, bronchiectasis and pulmonary toxicities, predominantly in adults. By describing this case, we aim...

CONCLUSION: TKI-associated renal TMA presents as hypertension-proteinuria-AKI rather than classic hemolysis. Sunitinib confers highest risk, baseline hypertension is a key modifiable factor, and combination therapy accelerates onset. Lifelong monitoring and timely TKI discontinuation are critical, though residual CKD is common.

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.

Herein, we report a case of nephrotic syndrome developed during chemotherapy with an anti-EGFR antibody. The patient was a 72-year-old man. He underwent surgery for sigmoid colon cancer and liver metastasis. Two months later, lung and lymph node metastases were observed, and CAPOX+anti-VEGF antibody therapy was initiated. The patient was switched to CPT-11+panitumumab due to disease progression. After 3 courses, he was diagnosed with nephrotic syndrome due to rapid weight gain with diuretic...

Nephrotic syndrome is one of the most common causes of kidney disease in children. The glomerular and tubulointerstitial changes that occur in the kidney due to this state of high-grade proteinuria are incompletely understood. Here we report a mouse model of congenital nephrotic syndrome, induced by deletion of transcription factor 21 (Tcf21) from podocyte precursors, that can be used to study the injury sustained by young kidneys in response to nephrotic range proteinuria. Tcf21 is required for...

CONCLUSION: Since several diseases, including MN, IgA nephropathy and AIN, are frequently treatable or even curable, our study demonstrated the need for early suspicion and diagnosis of NDKD.

Monoclonal gammopathy of renal significance (MGRS) refers to a spectrum of kidney disorders caused by monoclonal immunoglobulins produced by small, often clinically silent clones of B-cells or plasma cells that do not meet the diagnostic criteria for overt haematological malignancy. These entities are frequently overlooked, as their clinical presentation often mimics more common renal diseases and typical markers of monoclonal protein may be absent. We describe the case of a 49-year-old woman...

Background and Objectives: Acute kidney injury (AKI) is a frequent and life-threatening complication in patients with liver cirrhosis (LC). Nephrotic-range proteinuria may reflect underlying structural renal vulnerability; however, its association with AKI severity in cirrhosis remains unclear. Materials and Methods: We conducted a retrospective cohort study of 408 adults with LC admitted to a tertiary referral hospital between January 2016 and December 2025. Nephrotic-range proteinuria was...

CONCLUSIONS: Increased doses/sessions or additional therapies for rFSGS associated with more favorable outcomes. Non-linear modelling identified when further increases did not improve outcomes.

CONCLUSION: MCD-IgAN behaves as a steroid-sensitive podocytopathy in which CS monotherapy is generally sufficient, whereas DFPE-IgAN, embedded in a heavier immune complex and chronic lesion burden, appears to benefit from early combination immunosuppression.

Osteoarthritis is a chronic disabling disease characterized by progressive degeneration of articular cartilage. The condition is characterized by an imbalance in the inflammatory response, the degradation of the extracellular matrix, the apoptosis of chondrocytes, and the disorder of the immune microenvironment. The present clinical efficacy of the treatment is unsatisfactory due to the limited self-healing ability of the articular cartilage. In recent years, there has been a growing body of...

Waldenström macroglobulinemia (WM) is a rare CD20-positive B-cell non-Hodgkin lymphoma. It is characterized by lymphoplasmacytic infiltration in the bone marrow and abnormal monoclonal IgM secretion. WM complicated by renal amyloidosis is uncommon but associated with rapid progression of organ damage. Accurate identification of pathogenic factors and individualized treatments are essential to improve prognosis. This paper reports the case of a 75-year-old female who initially presented with...

CONCLUSION: Repeat kidney biopsy detects pathological progression (e.g., increased chronicity) and guides treatment modifications in over 60% of pediatric LN cases. However, pathological class change alone does not dictate short-term outcomes, highlighting initial treatment response as a stronger prognostic indicator. Individualized use of repeat biopsies is recommended for children with proteinuria relapse to optimize management while mitigating procedural invasiveness.

Libman-Sacks endocarditis (LSE), characterized by sterile valvular vegetations, is a recognized complication of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). However, its occurrence as the initial manifestation of previously undiagnosed autoimmune disease is uncommon and may create significant diagnostic uncertainty. A 27-year-old female presented to the emergency department with acute-onset exertional dyspnea, cough, and severe anemia, with a history of three...

The proprotein convertase subtilisin/kexin 9 (PCSK9) protein is well known for its role in the regulation of plasma cholesterol levels. We previously showed that the initiation of hypercholesterolemia in nephrotic syndrome is related to PCSK9 secreted by the cortical collecting duct (CCD), whereas the established phase is due to PCSK9 secreted by the liver. In this study, we investigated whether early neutralization of circulating CCD-secreted PCSK9 could prevent sustained hypercholesterolemia....

CONCLUSION: RISS is a delayed reaction occurring after a free interval of several days, but it may clinically present shortly after a subsequent infusion in repeated dosing regimens. Its main features include the classic triad of fever, rash, and arthralgia or arthritis, commonly accompanied by elevated inflammatory markers and hypocomplementemia. Most patients improve rapidly after drug withdrawal, supportive care, and short-course corticosteroid therapy; however, rechallenge carries a...

ABSTRACT: Nuclear pore complexes serve as essential gatekeepers of the nuclear envelope, playing crucial roles in regulating transport across the nuclear envelope and maintaining compartmentalization between the nucleus and cytoplasm. While they are fundamental to all nucleated cells, the nucleoporins that make up these complexes are associated with various inherited diseases, often affecting specific cells, tissues, or organs. In this overview, we describe the clinical features, summarize...

B-lymphocyte proliferative disorder have a certain incidence rate among the elderly. Whether these conditions require treatment is closely related to the presence of concurrent organ damage. This case report details the diagnostic journey and treatment process of a 76-year-old male patient who presented with nephrotic syndrome and acute kidney injury. His condition was further complicated by the presence of monoclonal M proteinemia and the malignant B-lymphocytes in the bone marrow. Through a...

Cirrhosis is a known cause of secondary IgA nephropathy (IgAN), often presenting with a membranoproliferative glomerulonephritis (MPGN) pattern and poor renal outcomes. We report a case of IgAN with an MPGN pattern associated with autoimmune hepatitis (AIH) in a 74-year-old woman. Although the patient had liver cirrhosis, her AIH was in clinical remission following prior corticosteroid therapy. She presented with nephrotic-range proteinuria and lower leg edema. Renal biopsy confirmed IgAN with...

mRNA-based COVID-19 vaccines have been associated with rare renal complications, including podocytopathies. We report a 72-year-old Japanese man with a 32-year history of type 2 diabetes and an 11-year history of stable membranous nephropathy (MN) who developed nephrotic syndrome (NS) after receiving his third dose of the BNT162b2 mRNA vaccine. His proteinuria, which had been stable at approximately 0.5 g/gCr for years, acutely increased to 14.3 g/gCr. A kidney biopsy revealed diffuse thickening...

Treatment for adult IgA vasculitis (IgAV), particularly severe cases, is not well established, although recent reports have suggested potential efficacy of rituximab (RTX). Here, we report a case highlighting the effectiveness of RTX based on pathophysiological and pathological considerations of IgAV, as well as a treatment protocol that has not been previously described. A 50-year-old man receiving glucocorticoids for cutaneous IgAV developed nephrotic syndrome and acute nephritic syndrome....

Background. Membranous nephropathy (MN) is generally primary, but it can also occur as a secondary form in association with infections, neoplasms or autoimmune diseases. Systemic Sclerosis (SSc), especially in its sine scleroderma forms or in its early stages, rarely manifests itself as MN. Case report. A 60-year-old woman with onset of nephrotic syndrome and histological picture of MN, in the absence of systemic manifestations. The patient subsequently developed episodes of acute renal failure,...

Monoclonal gammopathy of renal significance (MGRS) encompasses renal diseases caused by nephrotoxic monoclonal immunoglobulins produced by small B-cell or plasma cell clones that do not meet the criteria for overt hematologic malignancy. Renal amyloid light-chain (AL) amyloidosis is a recognized lesion within the MGRS spectrum. We report the case of a 66-year-old woman who presented with nephrotic syndrome and renal dysfunction associated with monoclonal lambda light chains. Renal biopsy...

This case highlights the importance of considering primary glomerular diseases in the differential diagnosis of nephrotic-range proteinuria during pregnancy, particularly when clinical features are atypical for Preeclampsia. The use of angiogenic biomarkers, such as the sFlt-1/PlGF ratio, proved valuable for excluding Preeclampsia and guiding further diagnostic evaluation.

CONCLUSION: This review highlights the evolving landscape of GDs in Saudi Arabia, emphasizing the need for early diagnosis, standardized biopsy protocols, and multidisciplinary management. A national renal biopsy registry is recommended to improve surveillance and research collaboration.

CONCLUSION: Mice harboring the RKRK186QQQQ mutation of the distal polybasic tract develop hyperaldosteronism under a low sodium diet, pointing to the relevance of this tract for sodium preservation. However, proteolytical processing of γENaC in these mice appears to be compensated for by the involvement of other adjacent cleavage sites.

CONCLUSION: The lower the E-cadherin/vimentin ratio, the greater the degree of chronic renal damage, suggesting that partial EMT is associated with an increased risk of progression to ESRD. Moreover, partial EMT may be more present in FSGS, since vimentin and E-cadherin were the markers most associated with the development of fibrosis. The identification of these markers may assist in prognosis and in the development of antifibrotic therapies.

CONCLUSION: One course of felzartamab was safe and well-tolerated, but ineffective in patients with MN and rituximab-resistant NS, possibly because of the inability to persistently deplete nephritogenic autoantibody-producing CD38-expressing B cells.

Nephrotic syndrome is associated with a hypercoagulable state and can lead to serious thromboembolic complications. In patients presenting with pulmonary embolism without an identifiable provoking factor, hypoalbuminaemia and significant proteinuria should prompt consideration of underlying nephrotic syndrome. Awareness of this link is important to avoid delay in diagnosis.A woman in her 60s presented with exertional dyspnoea and pleuritic chest pain. CT pulmonary angiography confirmed bilateral...

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.

Regulatory T cells (Tregs) play a pivotal role as key modulators of immune tolerance in kidney transplantation, a critical therapeutic approach for end-stage renal disease. Despite advances in surgical techniques and immunosuppressive regimens, kidney transplantation continues to face significant challenges, including immune rejection and complications arising from long-term immunosuppression. In recent years, Tregs have garnered considerable attention due to their potential to promote graft...

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...

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...

CONCLUSIONS: Although necessary, the provision of universal health care coverage alone is insufficient to ensure long-term donor health and inform future practice. A new national model of long-term donor follow-up informed by the lived experience of past donors and input from primary care and transplant providers was proposed.

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...

CONCLUSIONS: CKD is associated with a profound systemic reduction in acetate and propionate, supporting the model of a compromised gut-kidney axis based on converging evidence. The divergent results for butyrate in paediatric versus adult populations suggest that SCFA metabolism is influenced by age-related factors or compensatory mechanisms. These findings highlight the potential for SCFA monitoring as a candidate or emerging markers for detecting early renal damage and stratifying risk.

CONCLUSION: Utilization of en bloc kidneys has declined in pediatric recipients but can have good outcomes in a select population. More information is needed to understand whether and how best to use en bloc kidney transplantation for adolescents with reduced access to transplant.

CONCLUSIONS: The reduced-dose VGCV prophylaxis regimen was associated with a high incidence of CMV infection and AEs in high-risk patients, indicating a suboptimal balance between efficacy and safety. In intermediate-risk patients, the regimen appeared effective and tolerable. Further optimization of prophylactic strategies is required, particularly for high-risk pediatric recipients.

Two areas of overlap between kidney transplantation and oncology that remain fraught with uncertainty and bias are (1) transplant candidacy for elderly patients with historic or active cancers or premalignant conditions, and (2) managing cancer in elderly kidney transplant recipients. These have led to inadvertent inequities in the care of elderly kidney transplant candidates with historic or active malignancies. Incorporating a patient's viewpoint and an oncologist's perspective, we present a...

Extracorporeal membrane oxygenation (ECMO) is increasingly being utilized for life-threatening cardiac and/or respiratory failure refractory to conventional treatment. Acute kidney injury (AKI) and fluid balance disorders commonly occur both before and during ECMO, with approximately half of cases receiving treatment with continuous renal replacement therapy (CRRT). Acute kidney injury, fluid balance disorders, and CRRT influence both short- and long-term outcomes in this population. The 36th...

Genetic testing is becoming a routine-and increasingly time-sensitive-tool in nephrology, with implications for diagnosis, prognosis, targeted therapy, transplant planning, and family counselling. However, many nephrologists remain uncomfortable with genetic concepts in general, and variant interpretation specifically. This gap will increasingly limit their ability to fully leverage genetic testing in routine nephrology care-and to translate results into management decisions that improve patient...

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.

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.

CONCLUSIONS: In a modern tacrolimus-based cohort, g+ptc≥2 without DSA was not associated with worse outcomes after adjustment for serial or concomitant TCMR and dnDSA events.

BACKGROUND IgA-associated vasculitis (IgAV), although relatively uncommon in adults, is frequently associated with increased vasculitis severity. Sudden abdominal pain and bloody diarrhea are among the usual manifestations of IgAV in children. In older patients, clinical manifestations of the disease may be typical, but advanced age and comorbidities can make the final diagnosis unexpected. CASE REPORT A 67-year-old man with multiple underlying medical conditions, including diabetes mellitus and...

CONCLUSIONS: In this Quality Improvement (QI) analysis, pediatric patients receiving compounded liquid tacrolimus demonstrated flatter mini-PK profiles than contemporaneous capsule recipients when evaluated within the same analytical platform. These findings support closer attention to PK shape (not trough alone) in clinically complex children requiring liquid formulations and motivate prospective studies that control for gastrointestinal comorbidity, co-medications, and administration route.

CONCLUSIONS: These findings reveal a reversal in ACGS gains in the contemporary era, highlighting the need for precision immunosuppression strategies tailored to the aging, comorbid transplant population.

Despite modest improvement, the lifespan of a child on dialysis continues to be 40 years shorter compared to healthy children. Cardiovascular disease (CVD) is the leading cause of morbidity and mortality in these patients. Risk factors for CVD are present even in early stages of chronic kidney disease (CKD) and accelerate as the child's renal function deteriorates. As a result, the highest burden of CVD exists in patients on chronic dialysis. Although dialysis is life-sustaining, the dialysis...

CONCLUSIONS: STAT3-HIES is a multisystem disorder that requires multidisciplinary care. Early diagnosis and supportive measures have altered its natural history. Recognition and improved understanding of the nonimmunologic manifestations of this disorder will further improve patient outcomes.

No abstract

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

CONCLUSIONS: VST administered as an ATMP-HE are feasible in routine clinical practice and demonstrate rapid antiviral activity in heavily pretreated pediatric patients. However, advanced viral disease and competing complications limit overall survival, underscoring the need for earlier intervention and advocating for treatment within the ongoing ALLOVISTA trial.

No abstract

CONCLUSIONS: These findings indicate that graft loss associated with DGF, independent of donor characteristics, is limited to the early posttransplant period. Future trials should therefore be cautious in adopting DGF as a primary surrogate endpoint for medium- and long-term graft survival.

Burosumab is effective for treatment of rickets in children with X-linked hypophosphatemia (XLH). The effects of burosumab on markers and regulators of osteoblast and osteoclast acitivity are largely unknown. In this cross-sectional observational study, we investigated 7 key markers and regulators of osteoblast and osteoclast activity in 107 burosumab-treated children with XLH. We calculated z-scores for bone-specific alkaline phosphatase (BAP), procollagen-1-N-terminal propeptide (P1NP),...

No abstract

CONCLUSIONS: Adolescents and young adults with chronic kidney disease and their parents had low awareness of peritoneal dialysis and low levels of engagement in the search for donors. Some barriers were unique to this population and differed from those previously identified among older adults with kidney failure. Providers should be aware of these differences when counseling this younger population on kidney replacement therapy options.

CONCLUSIONS: Many ideal pediatric-quality kidneys are allocated to adults, primarily multiorgan transplant recipients. We anticipate no meaningful change in the proportion of ideal pediatric-quality kidneys prioritized for children using the revised KDPI-8 calculation.

Circulating anti-nephrin antibodies have recently been identified as mediators in many cases of childhood nephrotic syndrome. After binding of the antibody to nephrin, a key component of the slit diaphragm, different intracellular signaling mechanisms lead to foot process effacement. These new insights into the pathophysiology of nephrotic syndrome may have the potential to influence clinical treatment strategies. First, the measurement provides a pathomechanism-specific serological biomarker of...

CONCLUSIONS: EV proteomics is a promising approach for detecting and monitoring AR in PKT recipients and warrants larger multicenter studies.

CONCLUSION: Our study indicates that girls predominate among children with anti-GBM disease and that children have a better outcome in terms of eGFR than adults, which is at least partly because of better eGFR values at diagnosis. The need for dialysis is a strong predictor of outcome, regardless of age.

CONCLUSIONS: These two cases represent the first report of transient NDM due to HNF1B deletion. Our findings broaden the molecular and clinical spectrum of HNF1B-related diabetes, and emphasize the importance of considering HNF1B defects in children with transient neonatal hyperglycemia.

CONCLUSIONS: In this real-world pediatric cohort, temporary MMF discontinuation, when clinically indicated, closely monitored, and followed by cautious reintroduction, was not associated with adverse long-term graft outcomes. Younger age at transplantation was associated with a higher likelihood of MMF discontinuation, supporting individualized immunosuppression strategies and proactive efforts to identify children at risk for viral-driven immunosuppression intolerance.

No abstract

CONCLUSION: These evidence-based, locally adapted guidelines aim to standardize and optimize the management of cLN in Saudi Arabia, improving early diagnosis, guiding treatment selection by LN class, and promoting supportive strategies to enhance renal outcomes, survival, and quality of life.

Living donor liver transplantation (LDLT) reduces wait‑list mortality in children, but its long‑term advantages over deceased donor liver transplantation (DDLT) and how socioeconomic context shapes outcomes in a universal healthcare system remain uncertain. We compared long‑term outcomes after pediatric LDLT versus DDLT and evaluated modification by socioeconomic status (SES). We linked clinical data for pediatric liver transplants in Ontario, Canada, from 1991 to 2021 to provincial health...

Children after kidney transplantation (KTx) are prone to mineral and bone disease (MBD) including growth restriction, bone pain, skeletal deformities, fractures, and vascular calcifications. We present a position paper on the diagnosis and management of post-transplant MBD in this population based on the available evidence and the opinion of experts from the European Society for Paediatric Nephrology (ESPN) CKD-MBD, Dialysis and Transplantation Working Groups. PICO (Patient, Intervention,...

CONCLUSIONS: Although DGF risk was associated with older donor age, RR technique, and longer ischemia times, it did not affect one-year graft function. Graft survival with DCD donors did not appear inferior to that with DBD. DCD KT appears feasible in pediatric recipients and may help expand donor availability under carefully selected conditions.

CONCLUSIONS: Overall, no clear excess risk of pre-eclampsia was found in post-donation pregnancies. However, modestly increased risks of low-birth-weight and small-for-gestational-age infants were observed, although absolute risks remained low. A possible trend towards preterm birth compared with matched controls was observed. These findings support individualized pre-donation counselling and highlight the need for enhanced monitoring in pregnancies following kidney donation.

CONCLUSION: Understanding factors that result in non-transmission of CMV to seronegative recipients despite receiving a seropositive organ may guide more targeted use of antiviral prophylaxis in pediatric SOT recipients.

CONCLUSIONS: NGAL concentration differences may facilitate identification of patients at risk of AKI or with suspected AKI stage 1S at admission. Heterogeneity and variability across studies, specimen types, and settings emphasize the importance of interpreting NGAL values within the specific clinical context and patient population.

CONCLUSION: In Türkiye, peritoneal dialysis has been shown to be associated with better survival rates than haemodialysis in children with stage 5 CKD, particularly among younger patients.

CONCLUSIONS: LAVV was immunogenic and generally well tolerated in our cohort of patients. Post-SOT LAVV should be considered for select VZV seronegative children with close follow-up.

CONCLUSION: Acute rejection remains significantly associated with adverse long-term graft outcomes in pediatric kidney transplantation. CI ≥ 4 was associated with long-term functional decline, whereas AI primarily reflected short-term functional impairment.

CONCLUSIONS: Longer time spent with low eGFR is associated with greater odds of preemptive and living donor transplantation. Earlier transplant referral for all children, especially Black youth, may help improve access to preemptive and living donor transplantation.

CONCLUSIONS: Overall, this commentary offers practical strategies to help teams overcome key ethical, methodological, and operational challenges to implementing pediatric TwiCs.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

No abstract

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

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

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

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

No abstract

CONCLUSIONS: Among patients with primary GN, COVID-19 infection was severe for 1 in 8 cases and was associated with subsequent worsening of GN disease activity, as defined by proteinuria. By contrast, vaccination against COVID-19 was not associated with change in disease activity or kidney function decline. These results support COVID-19 vaccination for patients with GN.

BACKGROUND: Membranous nephropathy is a glomerular disease characterized by the presence of immune-complexes deposited in the subepithelial space of the glomerular basement membrane. It is the main cause of nephrotic syndrome in adults, while in children it is very infrequent. Anti-CD20 monoclonal antibodies, mainly rituximab, represent a specific treatment for this disease.

CONCLUSION: The KB is an essential step in the diagnosis of nephropathies. Focal segmental glomerulosclerosis is frequent in our study. The establishment of a Kidney registry would allow better knowledge of renal pathologies in sub-Saharan Africa.

CONCLUSIONS: Belimumab combined with conventional therapy may enhance NK cell counts, improve serological and clinical outcomes, and facilitate steroid tapering in cSLE. Further prospective controlled studies are needed to confirm these findings.

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...

BACKGROUND: Libman-Sacks endocarditis (LSE) is a noninfective thrombotic endocarditis frequently associated with antiphospholipid syndrome (APS) and/or systemic lupus erythematosus. Valvular obstruction, if it occurs, may require urgent surgery.

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

Transposable elements (TEs) make up almost half of the human genome and are among its most densely methylated regions. Their epigenetic silencing is crucial for genomic stability and immune homeostasis, and accumulating evidence indicates that dysregulated TE methylation and expression contribute to autoimmune disease pathogenesis. Hypomethylation of selected TE families can permit transcriptional reactivation, production of immunostimulatory nucleic acids and peptides, and engagement of...

CONCLUSION: Vitamin D deficiency was prevalent in newly diagnosed c-SLE and was associated with LN, proteinuria, and moderate to high disease activity.

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.

Genetic contributions to systemic autoimmunity are often considered more significant in children than in adults. As such, genetic evaluation may be more frequently pursued in pediatric rheumatology patients. Motivated by the discovery of a STING-associated vasculopathy with onset in infancy (SAVI) mutation in a patient with adult-onset relapsing polychondritis and systemic lupus erythematosus, we hypothesized that STING gain-of-function mutations might underlie a broader spectrum of autoimmune...

CONCLUSIONS: BAFF inhibition in paediatric LN is associated with rebalancing of innate and adaptive immunity through enhancement of Breg function and suppression of ILC1-driven inflammation.

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

Dysregulated B lymphocyte activation plays a pivotal role in the pathogenesis of systemic lupus erythematosus (SLE), making it a promising target for therapeutic intervention. Chimeric antigen receptor T cell (CAR-T) therapy, specifically targeting CD19-positive B cells, has shown potential in selectively eliminating aberrant B cells, offering a path toward sustained remission and even complete eradication of SLE. However, the broad immunosuppressive effects and cytotoxicity associated with...

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.

CONCLUSION: GPIHBP1 autoantibody-related HTG is important and potentially treatable severe pediatric HTG, representing an early manifestation of autoimmune dysregulation that requires an accurate diagnosis and longitudinal surveillance.

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...

No abstract

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

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

No abstract

CONCLUSIONS: This case highlights that severe invasive bacterial infection may occasionally be the clinical circumstance that leads to the diagnosis of pediatric systemic lupus erythematosus. Persistent systemic inflammation or evolving multisystem involvement despite appropriate antimicrobial therapy should prompt consideration of an underlying autoimmune disease, even in patients without a prior history of immune dysfunction.

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...

PFAS are a group of persistent organic pollutants, that bioaccumulate and are associated with negative health effects. Reviews have suggested that the most critical effects of PFAS are on the immune system, but little is known of effects on development of autoimmunity. Our objective was to map and summarize available evidence concerning exposure to any PFAS and development/presence of autoimmunity, in humans and animals. We assessed studies reporting potential associations between PFAS exposure...

No abstract

No abstract

CONCLUSION: Brain injury marker levels were high and associated with disease activity and CD in this cSLE adolescent cohort, suggesting a link between systemic inflammation and clinically under-detected neuronal/glial injury. Larger, longitudinal studies should explore the potential clinical utility of brain injury markers for clinical assessment of brain involvement in cSLE.

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...

No abstract

CONCLUSION: Our findings suggest that women with chronic hypertension who have evidence of fetal growth restriction at 35-36 weeks' gestation (but not those with only an estimated fetal weight <10th percentile) more frequently develop maternal manifestations of superimposed preeclampsia and should be considered for enhanced maternal and fetal surveillance. These findings should be replicated at earlier gestational ages.

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...

CONCLUSIONS: This study found that jSLE mainly affects female patients, with musculoskeletal, renal, and hematologic involvement being the most common manifestations. Renal involvement is associated with initial anti-dsDNA positivity and SLEDAI-2K scores. The study also found that better remission outcomes are linked to lower initial disease activity and longer follow-up periods.

Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disorder characterized by chronic inflammation, oxidative stress, and multi-organ damage, with lupus nephritis (LN) constituting a major cause of morbidity and mortality. Dysregulated iron metabolism and insufficient expression of heme oxygenase-1 (HO-1), a pivotal antioxidant enzyme regulating iron homeostasis and immune responses, have been implicated in LN pathogenesis. However, therapeutic strategies targeting HO-1 and iron...

Lupus nephritis (LN), a severe manifestation of Systemic Lupus Erythematosus (SLE), is a heterogeneous disease driven by diverse immune and tissue cell types. We obtained 538K single-cell and 140K single-nuclear profiles from kidney biopsies of 155 LN patients and 30 pre-implantation transplant biopsy controls, along with 325K single-cell blood profiles overlapping many of these patients. We identified key tissue cell types and cell states, and immune cell states; we were able to determine cell...

Juvenile Systemic Lupus Erythematosus (JSLE) is a rare and chronic multisystem autoimmune condition with an accelerated course compared with adult-onset SLE. Its heterogeneity of presentation and overlap with infectious and inflammatory conditions frequently hinders diagnosis, especially in areas where tuberculosis is endemic. A 15-year-old male patient came to the Emergency Department with his sixth attack of generalized tonic-clonic seizures within 20 days. He had a history of 8 months with...

Suicide attempts (SA) and suicidal ideation (SI) are major public health concerns with incompletely understood underlying genetic and molecular mechanisms. We investigated the shared genetic architecture of SA and SI with 13 genetically correlated psychiatric, behavioural, and somatic phenotypes using summary statistics from 15 genome-wide association studies (N=46,350-975,353). Local Analysis of [co]Variant Association (LAVA) quantified locus-specific genetic covariance, while conjunctional...

Autoantibody flares are important drivers of pathology in systemic lupus erythematosus (SLE), highlighting the pivotal role of B cells in initiating and propagating chronic autoimmunity. Although autoreactive specificities are a normal feature of the naive B cell repertoire, these cells are normally suppressed by layered tolerance checkpoints that limit inappropriate activation. Autoimmune-prone environments can lower these tolerance thresholds, rendering naive autoreactive B cells more...

We studied 132,525 Danish general population individuals to examine whether risk of osteoarthritis and/or use of pain-relieving medication was increased in haemochromatosis C282Y homozygotes with normal or low plasma iron, transferrin saturation, or ferritin. We genotyped all 132,525 individuals for the HFE C282Y and H63D variants. During a median follow-up of 40 years, 31,636 individuals had osteoarthritis. Risk of osteoarthritis was increased even in C282Y homozygotes with normal or low plasma...

COPA (coatomer protein complex subunit α) syndrome is a rare inborn error of immunity associated with constitutive activation of type I interferon signaling. Organ inflammation and damage (eg, lungs, kidneys, joints) usually develops early in life. Kidney involvement occurs in a subset of COPA syndrome patients, with the potential for rapid progression to kidney failure. Through an international collaboration, we identified COPA syndrome patients who developed kidney failure and underwent a...

BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic, multi-organ autoimmune disease characterised by a highly heterogeneous presentation. Specific genetic variations predispose patients to the disease, and rare monogenic forms caused by single-gene variations have been identified in a small percentage of patients, often with early disease onset. In this study, we used exome sequencing in a large cohort of patient with juvenile-onset SLE to gain insight into the genetic basis of juvenile...

CONCLUSIONS: Weight negatively impacts body esteem in adolescents with rheumatic conditions, particularly those with SLE. Findings underscore the need for targeted interventions addressing weight-related HRQOL in this population.

CONCLUSION: Paediatric rheumatic diseases can present as life-threatening emergencies, frequently as the first manifestation of illness. Systemic lupus erythematosus was the most common underlying diagnosis in our cohort. Early recognition and timely initiation of immunosuppression may improve outcomes.

CONCLUSION: Autoantibody-based stratification effectively delineates two cSLE subgroups with distinct genetic, immunological, and clinical trajectories. The anti-Sm/RNP positive subgroup, defined by HLA-DRB1*15/09 risk alleles and a high IFN-α signature, represents a more severe phenotype with a higher risk of flare, potentially explaining the suboptimal response to belimumab in this group.

Traditional treatment regimens for rheumatism and autoimmune diseases are associated with significant adverse effects, and some patients have a limited response, leading to poor prognosis. Telitacicept, a biologic agent and fusion protein targeting B cell activating factor (BAFF) and a proliferation inducing ligand (APRIL), offers a novel therapeutic strategy for refractory rheumatism and autoimmune diseases. Clinical trials have shown promising efficacy and safety. In systemic lupus...

Chronic urticaria (CU) is characterized by the recurrent occurrence of wheals and/or angioedema for more than six weeks. CU comprises two principal subtypes: chronic spontaneous urticaria (CSU) and chronic inducible urticaria (CIndU). Increasing evidence suggests that CSU has an autoimmune basis, and patients with CSU exhibit a higher prevalence of concomitant autoimmune disorders. While this association has been well documented in adults, data in pediatric populations remain limited. Available...

Background/Objectives: Childhood-onset Sjögren disease (cSjD) is a rare autoimmune disorder with heterogeneous manifestations and ongoing diagnostic challenges, as there are no validated paediatric criteria. Our study aims to characterise the clinical, laboratory, and imaging features of children diagnosed with cSjD at a single Romanian paediatric rheumatology centre between 2015 and 2025 and contextualise these findings within the most recent literature. Methods: A retrospective review of 15...

CONCLUSION: Parents of children with SLE exhibit a moderate level of PTSD-related symptoms, with arousal and reactivity symptoms being the most prominent. Prominent arousal and reactivity symptoms suggest a potential link to chronic caregiver stress, which warrants further investigation.

CONCLUSIONS: Our findings suggest that TNFSF4 and IKZF1 polymorphisms affect vulnerability to juvenile SLE.

CONCLUSION: Both sCD163 levels were associated with LN activity and differed from levels in extrarenal SLE and non-inflammatory CKD, suggesting that sCD163 may serve as a potential biomarker of kidney inflammation in pediatric SLLE patients.

Epstein-Barr virus (EBV) is an important environmental risk factor in the development of several autoimmune conditions, with the mechanisms still to be fully elucidated. EBV primarily infects memory B cells, transitioning between lytic (active) and latent (dormant) phases of infection. Our group has previously proposed two molecular mechanisms linking EBV pathogenesis to autoimmunity: one indicates that EBV lytic switching contributes to systemic lupus erythematosus (SLE) pathogenesis, while...

Autoimmune rheumatic diseases (ARDs) are a diverse group of chronic disorders characterized by immune dysregulation and multi-organ inflammation. B cell receptor (BCR) signaling emerges as a shared, yet heterogeneously regulated, pathogenic axis across these diseases. This dysregulation drives B cell activation, autoantibody production, and ultimately tissue damage. Recent research highlights its involvement in both common and disease-specific mechanisms, which helps explain the wide variation...

Considerable advances have been made in our understanding of Bardet-Biedl syndrome (BBS), particularly in its core clinical features and molecular genetics, warranting an update to the existing diagnostic criteria framework. Using a rigorous, evidence-based, and consensus-driven process, a multidisciplinary group of international experts and patient-led organizations developed an updated diagnostic algorithm. This algorithm provides practical, updated guidance for clinicians, including a pathway...

Alpha-mannosidosis (AM, OMIM# 248500), an ultra-rare lysosomal storage disorder (LSD), is caused by insufficient activity of alpha-mannosidase, an enzyme involved in degradation of N-glycan oligosaccharides. Hence, specific oligosaccharides accumulate in tissues, resulting in a progressive multi-organ disease. Therapeutic options exist but treatment outcome depends on early diagnosis. Thus, multiple methods have been developed for analysis of these oligosaccharides in urine that are qualitative...

Acute-on-chronic liver failure (ACLF) is associated with high mortality and multi-organ damage in chronic liver disease. This study investigated the role of CD45^(+) erythroid progenitor cells (EPCs) in ACLF progression and underlying mechanisms. CD45^(+) EPCs from 22 patients with hepatitis B virus-related hepatocellular carcinoma (HCC) were analyzed by flow cytometry. Immunofluorescence was used to visualize cell distribution in liver tissue. Mitochondrial function assays and RNA sequencing...

Metastasis represents a major challenge in the treatment of clinical colorectal cancer (CRC), and anoikis resistance enables cancer cell dissemination. This study identifies Calcium/calmodulin-dependent protein kinase I (CAMK1) as a crucial regulator of anoikis in CRC progression. We analyzed CAMK1 expression in CRC tissues and its correlation with patient prognosis. In vitro, CAMK1 was overexpressed in CRC cell lines to assess its effects on proliferation, migration, invasion, and anoikis...

CONCLUSIONS: Our study confirms the severely impaired developmental potential and frequent neurological and medical complications in lissencephaly patients from an early age. The psychomotor prognosis in LIS1/PAFAH1B1-related lissencephaly is significantly worse compared to DCX-related lissencephaly. Supportive therapies are used intensively and are considered to be very effective. The disease puts a high burden on caregivers and the entire family. This emphasizes the need for appropriate...

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

CONCLUSION: The use of AVAC in a case series with mainly GPA patients led to rapid clinical improvement, reduction of corticosteroid doses, and improvement in fatigue. The findings demonstrate beneficial effects of AVAC as add-on therapy in severe AAV.

Cardiorenal disease reflects a tightly interconnected pathophysiological continuum driven by neurohormonal activation, inflammation, oxidative stress, and progressive fibrosis. Among these mechanisms, mineralocorticoid receptor (MR) overactivation has emerged as a central mediator of cardiac and renal injury independent of blood pressure effects. Finerenone, a non-steroidal selective MR antagonist, exhibits distinct pharmacological properties compared with steroidal MR antagonists, including...

CONCLUSIONS: CYP with persistent hypertension had worst rates of LVH and indexed LV mass. A correlation between changes in NT Pro-BNP and LVMI highlights a probable role of fluid overload. Further research into using NT Pro-BNP as a biomarker to improve hypertension management is warranted.

CONCLUSION: AKI is frequent in a semirural setting in Cameroon but seems to remain largely invisible to clinicians. Implementation of the ISN 5R strategy, clinician training on KDIGO criteria and improved laboratory access may increase AKI recognition.

No abstract

Interleukin-1 receptor-associated kinase 2 (IRAK2) is essential for the Myddosome complex formation downstream of Toll-like receptors. We identify twelve patients with a homozygous loss-of-function copy number variant in IRAK2, designated IRAK2-∆ex2. Most patients present with recurrent infections, autoantibody production, and gastrointestinal ulceration. Two patients were clinically diagnosed with primary immunodeficiency, while the majority fulfill diagnostic criteria for autoimmune or...

CONCLUSIONS: LINC00152 serves as a promising prognostic biomarker and therapeutic target in TGCT. It promotes tumour malignancy via YBX1/AKT signaling and drives immune evasion by inducing HAVCR2-mediated T cell exhaustion, providing new insights for combination immunotherapy in TGCT.

CONCLUSION: Low numbers of pHLTx has been performed in Scandinavia. Long-term outcomes were unfavorable, but comparable to other larger international and North American registries. The declining number of performed pHLTx may result from a combination of poor prognosis, declining incidence of Eisenmenger´s syndrome, improved surgical techniques for cardiac repair combined with lung transplantation, and increasing use of lung transplantation for isolated pulmonary vascular disease.

CONCLUSIONS: Child health threats from non-optimal temperatures are cause-specific, geographically clustered, and inequitable. Although the global burden has decreased, it is worsening in specific regions and for certain diseases. Future interventions should be precisely tailored and integrate health systems with climate adaptation.

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...

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

No abstract

CONCLUSION: Preeclampsia is associated with increased long-term maternal risk of hypertension, CKD, and CVD. Higher UPE levels correlate with higher risk of later hypertension and CKD.

Diabetic ketoacidosis (DKA) is a life-threatening complication of type 1 diabetes, and cerebral edema is well described in children; it is rare in adults and carries a high mortality. We present the case of a 47-year-old male with poorly controlled type 1 diabetes who was brought to the ED unresponsive. On arrival, he was obtunded, required intubation, and vasopressor support. Laboratory evaluation was consistent with severe DKA, including marked hyperglycemia, high anion gap metabolic acidosis,...

CONCLUSIONS: Increased inorganic sulfate loss is prevalent in children with proximal tubular defects and sulfate stores can be repleted by oral drugs like cysteamine.

Chronic kidney disease affects 850 million people worldwide and places a disproportionate burden on low-income and middle-income countries where access to timely diagnosis, treatment, and life-sustaining kidney replacement therapy (KRT) is restricted. In May, 2025, the 78th World Health Assembly adopted a resolution on kidney health that called on all member states to integrate kidney care into national strategies; enhance prevention, early detection, and timely management; strengthen primary...

To evaluate the bidirectional causal relationship between gut microbiota and Rheumatoid arthritis using Mendelian randomization (MR). Using gut microbiota data from the IEU OpenGWAS database (n = 5959, Finnish cohort) and rheumatoid arthritis genetic data from the Finnish database (6236 cases and 1,47,221 controls), forward and reverse MR analyses were conducted with gut microbiota and rheumatoid arthritis as exposure factors and outcomes, respectively. The significance threshold for forward MR...

Cystinosis is a rare lysosomal storage disorder that affects approximately 1 in 100,000 to 200,000 live births worldwide. Long-term graft success in cystinosis depends on cysteamine adherence and structured multidisciplinary transition, supported by protocols developed by both pediatric nephrologists and hospital organizations. This review explores institutional and regional care models, structured programs such as the RISE protocol and Got Transition's Six Core Elements, and major barriers...

CONCLUSION: An AP diameter ≥17 mm, decreased differential kidney function, UTI history, and family history of congenital anomalies of the kidney and urinary tract are strong indicators for surgical intervention.

To retrospectively review the indications, timing, outcomes, and complications of membrane-based therapeutic plasma exchange (mTPE) in critically ill children at a single-center pediatric intensive care unit (PICU). Retrospective observational study. A tertiary-care PICU at All-India Institute of Medical Sciences (AIIMS), Raipur. A total of 106 patients aged 1 month to 18 years who underwent mTPE between January 2022 and December 2024. Patients had a median age of 144 months (IQR: 13-216). Renal...

Intracranial aneurysms are a prevalent cerebrovascular condition and a leading cause of aneurysmal subarachnoid hemorrhage (aSAH). Beyond established risk factors, increasing attention has been focused on metabolic conditions that may modulate vascular inflammation and endothelial function. In this context, obesity has been proposed as potential modifiers of intracranial aneurysm vulnerability. However, clinical evidence remains inconsistent and, in some cases, counterintuitive. Most studies...

CONCLUSIONS: Umbilical venous access using the 6Fr dELC may represent a viable alternative for neonatal CKRT. Coil reinforcement within the catheter may support patency and better functionality. Additional studies are needed to assess safety and broader applicability.

CONCLUSION: Our results unveil a novel Insig1/Dapk3 axis as a critical regulator of AKI progression, highlighting its potential as a therapeutic target in clinic.

CONCLUSIONS: A stepwise incorporation of interventions, including education, the development of a novel exit-site scoring tool, and a treatment-based algorithm, resulted in a 78% decrease in IV vancomycin use in our pediatric chronic HD unit within 1 year and an 82% decrease in associated costs.

Chronic kidney disease (CKD) is characterized by fibrosis. Alport syndrome, a common monogenic form of CKD caused by collagen type IV variants, leads to basement membrane defects and progressive fibrosis. Understanding extracellular matrix dynamics is crucial for identifying disease-specific biomarkers and optimizing therapeutic timing. We applied a discovery approach combining stable ^(13)C-lysine metabolic labeling with proteomics, RNA sequencing, super-resolution imaging, and bioinformatics...

CONCLUSIONS: In this population-based study LTFR occurred in many cases well beyond puberty, in both groups, favoring the role for disease length on LTFR, probably due to maturational immune system changes.

OBJECTIVE: Eating disorders (EDs) are frequently underdiagnosed in transgender and gender-diverse (TGD) adults. Many existing ED screeners were developed in cisgender, White, female samples and may miss symptom patterns relevant to TGD adults. This study evaluated internal consistency and screening accuracy of the Brief Assessment of Stress and Eating (BASE), a 10-item ED screener, in a national sample of TGD adults.

CONCLUSION: These consensus statements support the use of RCA during CRRT in critically ill patients with impaired citrate metabolism and provide practical guidance for monitoring and management of metabolic complications. However they reflect expert opinion, especially for questions with limited data and low-level evidence.

No abstract

CONCLUSION: This study added criteria PP1, PP4 and PS4 to c.1883_1884del [p. His628ArgfsTer126] according to the ACMG guidelines and also supports a potential reclassification of c.2145 C > T variant from variant of uncertain significance (VUS) to likely pathogenic by adding PP4 and PM3. These findings will significantly help specialists in their decision-making about these variants.

CONCLUSIONS: The reduced-dose VGCV prophylaxis regimen was associated with a high incidence of CMV infection and AEs in high-risk patients, indicating a suboptimal balance between efficacy and safety. In intermediate-risk patients, the regimen appeared effective and tolerable. Further optimization of prophylactic strategies is required, particularly for high-risk pediatric recipients.

CONCLUSIONS: This systematic review suggested that machine learning models for predicting postoperative AKI in pediatric cardiac surgery indicated good discriminative ability. However, the high risk of bias across all included studies and the significant heterogeneity in model performance indicated that the reported performance may be overestimated. The high heterogeneity observed highlights the substantial variability in model performance, which is likely driven by differences in study design...

Background/Objectives: A blunted growth hormone (GH) response in stimulation tests (GHSTs) in obese patients is well documented, with less evidence for insulin-like growth factor-1 (IGF-1) concentrations. The aim of this study was to assess the relationships between nutritional status, GH peak in GHST, and IGF-1 concentrations, and to develop machine learning prediction models of GH deficiency (GHD) in children with short stature. Methods: A case-control study included 1592 children with short...

CNS complications (CNSC) may occur in a significant proportion of patients after allogeneic haematopoietic cell transplantation (allo-HCT). They can be differentiated into infectious and non-infectious CNSC, such as vascular pathologies (e.g., bleeding, stroke), drug-related abnormalities, and CNS relapse of the underlying malignancy. Initiation of prompt and adequate diagnostics - mainly neuroimaging and cerebrospinal fluid (CSF) analyses - and treatment are crucial to improve the prognosis....

No abstract

The integration of artificial intelligence (AI) into clinical decision support (CDS) holds promise for proactive, personalized, and precision care. However, current understanding of how to establish trustworthy human-AI partnerships is in its infancy, despite its critical importance for implementing AI in healthcare. We present the Asthma-Guidance and Prediction System (A-GPS) as a case study of a practice-integrated AI platform that demonstrates how trustworthy, generalizable and sustainable AI...

Cardiovascular-kidney-metabolic (CKM) syndrome represents a continuum of interrelated adiposity, insulin resistance, cardiovascular disease, kidney dysfunction, and metabolic disturbances that evolve across the lifespan. Emerging evidence demonstrates that both biological sex and sociocultural gender significantly shape CKM risk, progression, and clinical expression. CKM syndrome pathogenesis reflects complex multisystem interactions involving adipose tissue dysfunction, neurohormonal...

BACKGROUND: Children and adolescents with chronic kidney failure undergoing hemodialysis are frequently physically inactive. Intradialytic exergaming may provide a motivating approach; however, it has not yet been evaluated in pediatric dialysis settings.

CONCLUSIONS: This work presents the first machine learning-based early prediction model for SDNS/FRNS using clinical big data from the acute phase of the condition. Additionally, it introduces an online prediction tool for paediatric SDNS/FRNS.

CONCLUSIONS: This multispecialty consensus-based checklist provides a pragmatic tool to support early suspicion of AAV in routine clinical practice and may help reduce diagnostic delay.

Extracorporeal membrane oxygenation (ECMO) is increasingly being utilized for life-threatening cardiac and/or respiratory failure refractory to conventional treatment. Acute kidney injury (AKI) and fluid balance disorders commonly occur both before and during ECMO, with approximately half of cases receiving treatment with continuous renal replacement therapy (CRRT). Acute kidney injury, fluid balance disorders, and CRRT influence both short- and long-term outcomes in this population. The 36th...

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

CONCLUSIONS: Eupalinolide B was identified as a novel CDC37-targeting agent with renoprotective and anti-inflammatory effects against DKD that functions by inhibiting CDC37-HSP90 interaction.

Mitochondrial dysfunction and insufficient mitophagy are central to cisplatin-induced acute kidney injury (AKI). Tirzepatide, a dual GLP‑1/GIP receptor agonist, exhibits reno-protective effects, but its mechanism related to mitochondrial homeostasis remains unclear. Here, we used metabolomics, in vivo mouse AKI model, and in vitro cisplatin-injured HK‑2 cells to explore the protective effects and underlying mechanisms. Tirzepatide pretreatment significantly alleviated renal dysfunction, tubular...

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

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

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

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

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

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.

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

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

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)...

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

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

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

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

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

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

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

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

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

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

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

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

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

CONCLUSION: In summary, ARPKD causes significantly impaired hrQOL, psychosocial problems and caregiver burden, which were equal to, if not greater than, that of controls with more advanced kidney failure. Treatment modality and developmental delay were the most important risk factors.

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

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

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

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

Pediatric solid organ transplant candidates and recipients remain undervaccinated and at higher risk of vaccine preventable illness (VPI) than the general population. An American Society of Transplantation Pediatric Community of Practice Controversies Conference was held in October 2023 to discuss opportunities to improve vaccine uptake and decrease rates of VPI in this population. Undervaccination results from failures at different levels. Clinician misconceptions about when vaccines may be...

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

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

No abstract

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

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

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

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

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

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

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.

Chronic care manages long-term, progressive conditions, while acute care addresses short-term conditions. Chronic conditions increasingly strain health systems, which are often unprepared for these demands. This study examines the burden of conditions requiring acute versus chronic care, including sequelae. Conditions and sequelae from the Global Burden of Diseases Study 2019 were classified into acute or chronic care categories. Data were analysed by age, sex, and socio-demographic index,...

CONCLUSIONS: LC pts show more microvascular alterations at NVC as compared with RC patients and CNT, which may contribute to the pathogenesis of persistent organ/systems dysfunction.

Maintenance hemodialysis patients are at increased risk of cardiovascular complications and mortality following COVID-19 infection due to compromised immune function. This study aims to evaluate the impact of the COVID-19 vaccine (CoronaVac) on cardiac function and survival in this population. Background/Objectives: We aimed to examine whether CoronaVac vaccination affects heart function and survival rates in maintenance hemodialysis patients. Specifically, we assessed changes in heart...

CONCLUSIONS: The finalized mortality dataset for Belgrade can be safely used in COVID-19 impact analysis. Belgrade experienced a significant increase in mortality during 2020 and 2021, with most of the excess mortality attributable to SARS-CoV-2. Concerns about increased mortality from causes other than COVID-19 in Belgrade seem misplaced as their impact appears negligible.

Solid organ transplantation (SOT) is a life-saving procedure for those with end-stage organ dysfunction. The main goals of SOT are to improve quality of life and daily function, which are supported by pre- and post-transplant rehabilitation. In-person rehabilitation programs have traditionally been the standard-of-care for delivering rehabilitation for SOT patients. Many programs have adopted a virtual delivery model [telerehabilitation (TR)], an approach that has become increasingly used given...

Glomerular diseases are complex conditions, many of which have a genetic basis. However, although some genetic variants can affect glomerular and thereby kidney function, not all identified variants are pathogenic. The process of evaluating genetic and experimental evidence to determine the validity of gene-disease relationships is known as gene curation, and it is critical for the identification of genes that should be examined in diagnostic tests and used to guide clinical management. Gene...

CONCLUSIONS: Serum IgG from both classified and non-classifiable APS may react with other β2GPI domains than DI and DIV-V. Anti-β2GPI domain selectivity can explain discordant results among diagnostic assays.

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

Cardiologists consider degenerative or infectious causes when evaluating valvular heart disease. However, the role of autoimmune disorders, though less frequent, remains clinically significant. This report describes a young male patient presenting with persistent coronary disease and a suspected valvular cusp perforation initially attributed to infective endocarditis, which ultimately proved to be a manifestation of IgG4-related disease. IgG4-related disease is a rare condition, more prevalent...

X-linked hypophosphatemia (XLH) is a rare genetic condition in which excess fibroblast growth factor 23 causes renal phosphate wasting, leading to skeletal morbidities. Patients experience musculoskeletal pain, stiffness, and fatigue, with impaired physical function and health-related quality of life (HRQL). Burosumab has been available in France for the treatment of XLH since 2021; European treatment guidelines suggest use in adults with pseudofractures or with insufficient response and/or...

CONCLUSION: Our study indicates that girls predominate among children with anti-GBM disease and that children have a better outcome in terms of eGFR than adults, which is at least partly because of better eGFR values at diagnosis. The need for dialysis is a strong predictor of outcome, regardless of age.

BACKGROUND: Education in medical and nursing curricula aims to build a strong theoretical foundation and practical skills, essential for addressing the complex challenges of healthcare delivery. Interprofessional learning fosters teamwork and improves patient care by enhancing collaboration across disciplines. Simulation-based education provides a safe environment for critical thinking and interprofessional collaboration, particularly in procedures like arterial blood gas (ABG) testing. Despite...

No abstract

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.

No abstract

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...

No abstract

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

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

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

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

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

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

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

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

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

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

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

No abstract

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

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

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.

No abstract

No abstract

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: In this study, we confirm that IFX and ADA are both effective in TAK, without significant differences in the risk of relapse and revascularizations.

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

No abstract

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

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

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

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

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

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

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

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

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

No abstract

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...

No abstract

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.

CONCLUSIONS: The CRM is representative of patient anti-β2GPI/CL heterogeneity and should improve anti-β2GPI IgG method harmonization. However, the level of achievable method harmonization is affected by differences in the selectivity among the assays.

Background/Objectives: Cryofibrinogenemia, characterized by plasma cryoprecipitation of fibrinogen and related proteins, is a rare and often under-recognized entity that can present with significant renal involvement. Methods: we describe a 66-year-old woman with progressive renal failure due to membranoproliferative glomerulonephritis driven by cryofibrinogen deposits. Her clinical course was marked by relapsing-remitting disease with limited response to high-dose corticosteroids but...

CONCLUSION: A unified commitment to prioritizing URDs on the global health agenda, paired with targeted funding, stipulated national strategies, and aligned international cooperation, is imperative to leveling the playing field for the diagnosis and management of URDs and capitalizing on the potential of Advocacy Groups as allies in this endeavor.