Pediatric Nephrology最新文献

{"title":"Cystic fibrosis-related kidney disease-emerging morbidity and disease modifier.","authors":"Merrill Hart, Manish Kumar, Himanshu Ballav Goswami, William Tom Harris, Sladjana Skopelja-Gardner, Agnieszka Swiatecka-Urban","doi":"10.1007/s00467-025-06715-3","DOIUrl":"10.1007/s00467-025-06715-3","url":null,"abstract":"<p><p>Cystic fibrosis (CF) is a life-shortening multisystem disease resulting from mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, causing the most devastating phenotypes in the airway and pancreas. Significant advances in treatment for CF lung disease, including the expanded use of high-efficiency modulator therapies (HEMT) such as Trikafta, have dramatically increased both quality of life and life expectancy for people with CF (PwCF). With these advances, long-term extrapulmonary manifestations are more frequently recognized. Pseudo-Barter syndrome, acute kidney injury (AKI) induced by medications or dehydration, amyloidosis, nephrolithiasis, and IgA and diabetic nephropathies have been previously reported in PwCF. Newer data suggest that chronic kidney disease (CKD) is a new morbidity in the aging CF population, affecting 19% of people over age 55. CKD carries a high risk of premature death from cardiovascular complications. Studies suggest that CFTR dysfunction increases kidneys' vulnerability to injury caused by the downstream effects of CF. Improving the mutant CFTR function by HEMT may help to tease apart the kidney responses resulting from extrinsic factors and those intrinsically related to the CFTR gene mutations. Additionally, given the novelty of HEMT approaches, the potential off-target effects of their long-term use are currently unknown. We review the evolving kidney complications in PwCF and propose the term CF-related kidney disease. We hope this review will increase awareness about the changing phenotype of kidney dysfunction in PwCF and help prevent morbidity related to this condition.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3605-3614"},"PeriodicalIF":2.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143649774","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk profile of promoters and inhibitors of kidney stone formation in paediatric inflammatory bowel disease.","authors":"Britta Zobel, Burkhard Rodeck, Michael van Husen","doi":"10.1007/s00467-025-06851-w","DOIUrl":"10.1007/s00467-025-06851-w","url":null,"abstract":"<p><strong>Background: </strong>Nephrolithiasis is a well-known extraintestinal complication of adult inflammatory bowel disease (IBD), mainly attributed to hyperoxaluria. In contrast, data for paediatric IBD are limited. The objective of this study was to evaluate renal stone risk in children and adolescents with IBD by analysing urine composition concerning stone promoters and inhibitors and their possible influencing factors.</p><p><strong>Methods: </strong>In this cross-sectional study, the 24-h urine composition of 107 children and adolescents with surgically untreated IBD compared to 27 healthy controls was analysed regarding the risk of kidney stone development, including an evaluation using the CMC index [(citrate × magnesium)/calcium]. Disease activity, as assessed by the Paediatric Crohn Disease Activity Index (PCDAI), Paediatric Ulcerative Colitis Activity Index (PUCAI), and faecal calprotectin (FC) was investigated as influencing factor.</p><p><strong>Results: </strong>Stone inhibitors were lower in active IBD than in remission (citrate, P = 0.001; magnesium, P = 0.004). The CMC index was also decreased in active disease (P = 0.001), indicating increased urinary lithogenicity in these children. PCDAI and PUCAI were predictors of the CMC index. This proved relevant in the groups IBD, IBD with active disease (B = - 0.072, P = 0.029, adjusted R² = 0.114), ulcerative colitis, and ulcerative colitis with elevated FC (B = - 0.095, P = 0.039, adjusted R² = 0.388). In patients in remission/with inactive disease and in those with low FC, urine composition did not differ from that of healthy controls in terms of kidney stone risk. Hyperoxaluria was not associated with paediatric IBD. The prevalence of nephrolithiasis was 0%.</p><p><strong>Conclusions: </strong>Monitoring urine for lithogenic alterations and calculating CMC index in paediatric active IBD could contribute to identifying at-risk patients for later nephrolithiasis early, although childhood stone prevalence is low.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3675-3685"},"PeriodicalIF":2.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144485372","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ferric carboxymaltose use in pediatric kidney transplant recipients with iron deficiency.","authors":"Diletta Domenica Torres, Luigi Antonio Moscogiuri, Giulia Fontò, Paolo Giordano, Vincenza Carbone, Marida Martino, Luisa Santangelo, Mario Giordano","doi":"10.1007/s00467-025-06869-0","DOIUrl":"10.1007/s00467-025-06869-0","url":null,"abstract":"<p><strong>Background: </strong>Anemia is a common complication in pediatric kidney transplant recipients (KTR). Post-transplantation anemia (PTA) is multifactorial with iron deficiency being a frequent cause. Oral iron supplementation is often ineffective. Ferric carboxymaltose (FCM) is an intravenous iron formulation but its safety and efficacy in pediatric KTR has not yet been established.</p><p><strong>Methods: </strong>This is a single-center, retrospective cohort study evaluating all consecutive KT patients diagnosed with iron deficiency (ID) and/or iron deficiency anemia (IDA), defined by KDIGO guidelines, who received FCM between December 2016 and November 2022.</p><p><strong>Results: </strong>Fifteen patients had ID and ten of them also showed IDA. Hemoglobin, transferrin saturation percentage (TSat) and ferritin levels were assessed at baseline and at one, three, six and twelve months, after a median (IQR) post-transplant follow-up of 41.8 months (11.3-72.6). At each follow-up time point, the median increase of Hb levels ranged from 1.2 to 1.4 g/dl. Ferritin levels significantly increased up to six months post-treatment, while TSat improved at 30 days. Phosphate levels did not show any decrease throughout follow-up and no adverse reactions were observed in our study population.</p><p><strong>Conclusions: </strong>Our experience suggests that FCM is an effective and well-tolerated treatment for pediatric kidney transplant recipients with ID and/or IDA and highlights the need for larger, well-powered trials to definitively test FCM in this population.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3755-3760"},"PeriodicalIF":2.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144718275","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy and safety of telitacicept in IgA nephropathy and IgA vasculitis nephropathy in adolescents: a case series.","authors":"Jingyi Wu, Pei Chen, Lijun Liu, Sufang Shi, Fang Wang, Xuhui Zhong, Jicheng Lv, Hong Zhang","doi":"10.1007/s00467-025-06905-z","DOIUrl":"10.1007/s00467-025-06905-z","url":null,"abstract":"<p><strong>Background: </strong>IgA nephropathy (IgAN) and IgA vasculitis nephritis (IgAVN) are among the most common glomerulopathies in adolescents, yet treatment options remain limited. Telitacicept (TACI), a dual-target fusion protein, has shown therapeutic potential in adult patients. This study aimed to evaluate the efficacy and safety of telitacicept in adolescents with IgAN and IgAVN.</p><p><strong>Methods: </strong>This retrospective observational study included 15 adolescent patients (11 with IgAN and 4 with IgAVN) who received telitacicept between January 2023 and January 2025. Patients received weekly subcutaneous injections of 80 mg or 160 mg. The primary efficacy outcome was proteinuria from baseline to follow-up. Secondary outcomes included changes in eGFR, serum albumin, and hemoglobin.</p><p><strong>Results: </strong>The median age of the patients was 16.91 years. After TACI treatment, the median proteinuria decreased from 2.0 g/day at baseline to 1.0 g/day at month 3 (P = 0.074) and further to 0.5 g/day at month 12 (P = 0.015), corresponding to median reductions of 31.2% and 59.0%, respectively. Median eGFR remained stable, showing an 1.1% increase at month 12 (P = 0.912). At month 12, serum albumin and hemoglobin increased by 5.6 g/L (5.8%) and 12.5 g/L (10.6%), respectively. Subgroup analyses showed consistent proteinuria reduction across baseline characteristics. Most patients tolerated telitacicept well, with no serious adverse events or treatment discontinuations reported.</p><p><strong>Conclusions: </strong>Telitacicept appeared to be well-tolerated and was associated with substantial reductions in proteinuria in adolescent patients with IgAN and IgAVN. Larger studies are needed to validate these findings.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3663-3673"},"PeriodicalIF":2.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144708413","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Growth in neonates with congenital kidney failure requiring continuous kidney replacement therapy.","authors":"Kara Short, Janelle Hanick, Perrin Bickert, Jessica Potts, David Askenazi","doi":"10.1007/s00467-025-06887-y","DOIUrl":"10.1007/s00467-025-06887-y","url":null,"abstract":"<p><strong>Background: </strong>With advanced technology, survival of neonates with congenital kidney failure (CKF) requiring continuous kidney replacement therapy (CKRT) has improved. Nutrition is essential but difficult to attain as CKRT removes proteins and micronutrients, and many patients have multiple co-morbidities. Scant data exist to guide clinicians on appropriate energy requirements for growth.</p><p><strong>Methods: </strong>We performed a retrospective study of neonates with CKF admitted to Children's of Alabama between 2016 and 2022 who required KRT within 10 days. We evaluated risk factors and growth in the 18/24 (75%) infants who survived to 90 days. Our primary and secondary outcomes were length z-score ≥  - 2 vs. <  - 2 at 90 days and weight z-score ≥  - 2 vs. <  - 2 at 90 days, respectively. Demographics, comorbidities, CKRT Dose Eras (1-body surface area (2000/1.73/m²/hr) vs. 2-weight-based era (24 ml/kg/hr)), and Nutrition Era 1 vs. 2 were evaluated.</p><p><strong>Results: </strong>At 90 days, 7/18 (38.9%) had length z-score ≥  - 2 while 10/18 (55.6%) had a weight z-score ≥  - 2. Factors for weight z-score ≥  - 2 include time to PD transition and CKRT Dose Era 2. Factors for length z-score ≥  - 2 included Era with higher calorie and protein goal targets (both p < 0.01).</p><p><strong>Conclusions: </strong>Malnutrition in neonates with CKF on CKRT is high. More studies are needed to better understand optimal strategies to ensure adequate growth. Until then, we recommend 24 ml/kg/hr clearance dose and prescribing at least 130 kcal/kg/day and 4 g/kg/day amino acids to target higher actual intake to start for these patients.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3733-3741"},"PeriodicalIF":2.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144799793","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Reclassifying hypertension phenotypes in adolescents by ambulatory blood pressure monitoring: implications of the 2022 AHA guidelines in a Brazilian birth cohort.","authors":"Ivan Coelho Machado, Inalda Facincani, Mariana Cachero Lino, Gabriela Pap, Eduardo Barbosa Coelho, Viviane Cunha Cardoso, Fabio Carmona","doi":"10.1007/s00467-025-06917-9","DOIUrl":"10.1007/s00467-025-06917-9","url":null,"abstract":"<p><strong>Background: </strong>Hypertension in adolescents is a growing public health concern, with ambulatory blood pressure monitoring (ABPM) playing a key role in diagnosis. The 2022 American Heart Association (AHA) guidelines introduced substantial changes to ABPM classification criteria, with the exclusion of the blood pressure (BP) load criterion and the alignment of diagnostic thresholds with adult values. This study aimed to assess the impact of guideline revisions on the prevalence of hypertension phenotypes among Brazilian adolescents.</p><p><strong>Methods: </strong>We conducted a cross-sectional study of 1006 adolescents (aged 11-13 years) from the BRISA cohort in Ribeirão Preto, Brazil. Participants underwent 24-h ABPM and clinic BP (cBP) assessment. Hypertension phenotypes were classified according to both the 2014 and 2022 AHA guidelines. Statistical analysis included prevalence comparisons, reclassification patterns, and centroid-based phenotypic analysis.</p><p><strong>Results: </strong>The prevalence of cBP hypertension was 3.4%. Using either the 2014 or 2022 AHA criteria, around 13% of adolescents were classified as hypertensive. However, under the 2014 guidelines, nearly 20% of the cohort remained unclassified, primarily due to elevated BP load alone. These individuals were reclassified as normotensive under the 2022 criteria, leading to a 20% increase in this group. One in ten adolescents was diagnosed with masked hypertension. Among hypertensive individuals, half had elevated nighttime BP only.</p><p><strong>Conclusions: </strong>The 2022 AHA guideline revisions improved the clarity of hypertension classification in this prenatal cohort. These findings support the clinical utility of the updated criteria and highlight the importance of adapting ABPM interpretation to current standards.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3717-3723"},"PeriodicalIF":2.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144822075","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The use of urinary biomarkers as prognostic tools: predicting kidney outcomes in pediatric acute kidney injury.","authors":"Wun Fung Hui, Renee Wan Yi Chan, Man Fung Tang, Tony Chun Hei Lei, Tsz Ki Liu, Kwok Hei Ho, Shu Wing Ku, Ting Fan Leung, Kam Lun Hon","doi":"10.1007/s00467-025-06920-0","DOIUrl":"10.1007/s00467-025-06920-0","url":null,"abstract":"<p><strong>Background: </strong>There is limited data on applying urinary biomarkers for prediction of kidney outcomes in pediatric acute kidney injury (AKI).</p><p><strong>Methods: </strong>We prospectively measured urinary neutrophil gelatinase-associated lipocalin (NGAL), tissue metalloproteinases-2 (TIMP-2), insulin-like growth factor-binding protein 7 (IGFBP-7) and C-C motif chemokine ligand 14 (CCL14), alongside serum kidney function test in critically ill children with AKI admitted to the pediatric intensive care unit. The primary outcomes included persistent AKI (lasting for ≥ 72 h) and prolonged AKI (lasting for ≥ 7 days).</p><p><strong>Results: </strong>There were altogether 134 patients (median age 4.3 years; 43.3% female; AKI severity stage 1: 44.8%, stage 2: 33.6% and stage 3: 21.6%). The incidence of persistent and prolonged AKI was 40.3% and 25.4%, respectively. All four biomarkers, either measured singly, simultaneously or serially, significantly predicted both outcomes, with NGAL demonstrating the best performance (areas under the curve [AUC] 0.72 [0.61, 0.83] for persistent AKI and 0.72 [0.61, 0.84] for prolonged AKI). Integrating the simultaneous AKI staging with biomarker levels significantly improved prediction (NGAL: AUC 0.86 [0.78, 0.94] for persistent AKI and 0.87 [0.79, 0.96] for prolonged AKI). Persistent AKI increased the risk of acute kidney disease (hazard ratios [HR]: 2.59 [1.55, 4.34]), which was associated with kidney function non-recovery 90 days after AKI (HR 7.73 [1.01, 59.03]).</p><p><strong>Conclusions: </strong>Urinary NGAL, TIMP-2, IGFBP-7 and CCL14 demonstrated promising performance of predicting kidney function non-recovery within 7 days of AKI onset. Integrating urinary biomarkers with concurrent clinical data substantially enhanced predictive performance.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3815-3823"},"PeriodicalIF":2.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144848236","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Erythropoietin resistance among pediatric patients on chronic hemodialysis: A cross-sectional study.","authors":"Rasha Helmy, Fatina I Fadel, Rasha E Galal, Amira M Mohammed, Shaimaa Sayed","doi":"10.1007/s00467-025-06776-4","DOIUrl":"10.1007/s00467-025-06776-4","url":null,"abstract":"<p><strong>Background: </strong>The erythropoietin resistance index (ERI) is an accurate indicator of erythropoietin (EPO) resistance and is related to a worse prognosis in patients on hemodialysis (HD). ERI is simple, cheap and could be calculated easily in children receiving HD. We aimed to assess the EPO resistance in children with kidney failure on regular HD.</p><p><strong>Methods: </strong>An analytical cross-sectional study was conducted on 80 children with kidney failure on regular HD. They were assessed by history taking and laboratory investigations including complete blood count, C- reactive protein (CRP), iron, ferritin, parathyroid hormone and serum electrolytes. ERI was calculated.</p><p><strong>Results: </strong>The study included 80 patients; 41 (51.2%) were male. The mean age of the study group was 8.86 ± 2.76 years. Sixty-three patients (78.8%) were on iron therapy. Mean ERI was 28.87 ± 10.62. The ERI was significantly positively correlated with age (r = 0.242; P = 0.031), EPO dose (r = 0.290; P = 0.001) and CRP (r = 0.219; P = 0.049). The ERI had a significantly negative correlation with KT/V (r = - 0.262; P = 0.019), hemoglobin level (r = - 0.265; P = 0.001) and platelet count (r = - 0.254; P = 0.023).</p><p><strong>Conclusions: </strong>Erythropoietin resistance is associated with many risk factors, including high CRP and low KT/V. Inadequate HD is the most important risk factor for EPO resistance in children on chronic HD. Adequate HD is considered as a protective measure against EPO resistance.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3725-3732"},"PeriodicalIF":2.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144035122","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Utilization of the modified Bosniak classification system for complex renal cysts.","authors":"Michael Frumer, David Ben-Meir, Chen Shenhar, Leslie Peard, Amanda F Buchanan, Adam B Hittelman, Israel Franco","doi":"10.1007/s00467-025-06848-5","DOIUrl":"10.1007/s00467-025-06848-5","url":null,"abstract":"<p><strong>Background: </strong>Solid renal masses are commonly managed through surgical intervention, while conservative management is the preferred approach for simple renal cysts. However, the optimal management strategies for complex renal cysts in children remain unresolved.</p><p><strong>Objective: </strong>To conduct a meta-analysis of the literature on the modified Bosniak (mBosniak) classification system to assess its diagnostic performance in pediatric complex renal cysts.</p><p><strong>Data sources and study eligibility criteria: </strong>A predefined database search (January 1986-December 2023), used the following keywords: Bosniak classification, modified Bosniak, pediatric renal cysts, complex renal cyst, and pediatric renal tumor. Two independent reviewers extracted data on mBosniak categories and their associated pathology diagnoses. The inclusion criteria comprised (1) English-language publications, (2) involving the pediatric population, (3) using the mBosniak classification and (4) incorporating histopathological data.</p><p><strong>Study appraisal and synthesis methods: </strong>The quality of studies was assessed using the QUADAS-2 tool. The analysis involved pooling data, performing subgroup analyses, and exploring potential publication bias.</p><p><strong>Results: </strong>A total of 7 retrospective studies, which included 154 lesions and 368 scan readings, were included. The overall prevalence of intermediate/malignant lesions was 70% (257/368). In distinguishing benign from intermediate/malignant pathology using the mBosniak classification (I-II vs. III-IV), the overall pooled sensitivity, specificity, PPV, and NPV were 0.88 (227/257), 0.74 (82/111), 0.89 (227/256), and 0.73 (82/112), respectively. mBosniak III category showed a PPV of 0.78 (70/90) and mBosniak II category showed a NPV of 0.59 (36/61). From the perspective of pathological diagnoses, 0.24 (29/122) of the readings for intermediate-risk lesions inaccurately categorized the lesions as mBosniak I-II. The diagnostic performance of the classification varies among distinct subgroups of patients and lesion characteristics.</p><p><strong>Limitations: </strong>We included studies with pathologically verified lesions only. In addition, aspects of the radiographic follow-up, such as the duration of follow-up, changes in cyst complexity or size over time, and the correlation between specific dynamics and malignancy, could not be evaluated based on the data provided in the included studies.</p><p><strong>Conclusions and implications of key findings: </strong>The mBosniak classification is a less precise tool for stratifying the challenging cases of intermediate-risk lesions, and the relatively low predictive values of the middle categories (II-III) cast doubt on its utility. Clinicians should incorporate additional clinical-epidemiological data for a more accurate assessment of these lesions. Registration number CRD42023493223.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3637-3645"},"PeriodicalIF":2.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144275544","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric anti-phospholipid syndrome nephropathy: a rare differential diagnosis for acute kidney injury in children.","authors":"Sanya Chopra, Thivya Sekar, Iona Madden, Madhuri Raja, Stephen D Marks, Matko Marlais","doi":"10.1007/s00467-025-06863-6","DOIUrl":"10.1007/s00467-025-06863-6","url":null,"abstract":"<p><p>Pediatric anti-phospholipid syndrome (APS) is a rarely diagnosed multisystem autoimmune inflammatory disorder resulting in recurrent vascular thrombosis with serious clinical implications. We highlight a case of an adolescent female who presented with Catastrophic APS (CAPS) in hypertensive emergency and end organ damage. She developed reduced kidney function, fluid overload and neurological manifestations. Neuroimaging and kidney biopsy confirmed micro-angiopathic thrombosis. Her blood results showed high titers of triple positive anti-phospholipid antibodies with negative serology for other immunological conditions. She was managed with dual anti-coagulation and immunosuppression and remains under pediatric subspecialist follow-up for ongoing multisystem involvement. APS should be considered as a differential diagnosis in children presenting with sepsis and multi-organ dysfunction. Pediatric focused diagnostic criteria and long-term management is needed to cater to needs of the pediatric population.</p>","PeriodicalId":19735,"journal":{"name":"Pediatric Nephrology","volume":" ","pages":"3655-3658"},"PeriodicalIF":2.6,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144497612","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}