BMC Nephrology最新文献

{"title":"Early mortality in multiple myeloma patients with renal impairment: a nested case-control study.","authors":"Yan Li, Wenjiao Tang, Bing Xiang, Ling Pan, Yuhuan Zheng, Ting Niu, Li Zhang","doi":"10.1186/s12882-026-05018-5","DOIUrl":"https://doi.org/10.1186/s12882-026-05018-5","url":null,"abstract":"<p><strong>Background: </strong>Renal impairment (RI) in multiple myeloma (MM) patients significantly impacts outcomes, yet the prevalence and causes of early mortality within 12 months of diagnosis in newly diagnosed MM (NDMM) with RI are understudied in the novel agent era.</p><p><strong>Methods: </strong>This study conducted a nested case-control analysis including 199 NDMM patients with RI from West China Hospital from June 2016 to November 2022. Among this cohort, we identified 19 cases of early mortality (death within 12 months of diagnosis) and selected 76 matched controls from the remaining patients at a 1:4 ratio based on age, gender, and diagnosis date. All controls were followed for over one year. Potential risk factors including elevated N-terminal pro-brain natriuretic peptide (NT-proBNP), heart failure at diagnosis, and other clinical indicators were analyzed.</p><p><strong>Results: </strong>The incidence of early death was determined to be 9.5% (19/199) in MM with RI, with pneumonia as the leading cause. Compared to controls, the case group exhibited a higher prevalence of elevated lactate dehydrogenase (LDH) levels and pneumonia. Multivariable logistic regression identified elevated LDH levels and pneumonia at diagnosis as independent risk factors for early mortality, whereas NT-proBNP elevation and heart failure at onset did not show statistically significant associations. The 1-year overall survival rates for MM with RI were 95.3% with 0 risk factors, 85.2% with 1 risk factor, and 68.8% with 2 risk factors (p < 0.001).</p><p><strong>Conclusions: </strong>In this nested case-control study of NDMM patients with RI, elevated LDH levels and pneumonia at diagnosis were identified as independent risk factors for early mortality in NDMM patients with renal impairment.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833155","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric to adult transition in chronic kidney disease: a scoping review.","authors":"Zachariah Alkhayri, Rama Safadi, Andrea Varghese, Mustafa Husein, Shay Tanna, Sahithi R Mallyala, Nikhil Rayarakula, Carol L Vincent, Rupesh Raina","doi":"10.1186/s12882-026-05019-4","DOIUrl":"https://doi.org/10.1186/s12882-026-05019-4","url":null,"abstract":"<p><strong>Introduction: </strong>Chronic kidney disease (CKD) presents unique medical and psychosocial challenges in children and adolescents that differ from adult patients with CKD. Transitioning from pediatric to adult care is a critical period. Insufficient planning and inadequate preparation increase the risk of medication nonadherence, loss to follow-up, graft failure in transplant patients, and increased morbidity. Transition models address barriers to successful transition, yet vary in their approach.</p><p><strong>Methods: </strong>We conducted a scoping review of the transition of care in pediatric CKD in accordance with PRISMA-ScR guidelines. We included PubMed/MEDLINE full-text articles, KDIGO guidelines, and grey literature on transition models between 1st January 2000 and 31st August 2025. Eligible studies included original research articles in English, human clinical trials, and guidelines specifically addressing pediatric-to-adult CKD transition of care.</p><p><strong>Results: </strong>We identified barriers to effective transition at the patient, provider, and system levels. Patients often lacked health literacy, emotional readiness, or independence. Providers faced challenges with communication across pediatric and adult care teams. System-level obstacles included gaps in insurance coverage and limited resources. To assess readiness, models such as ON TRAC, TRAQ, RTQ, and the UNC TRxANSITION offer valuable questionnaires. Early preparation between the ages of 11-14, a gradual increase in patient autonomy, multidisciplinary teams' involvement, and structured transfer clinics are recommended for proper CKD transition.</p><p><strong>Discussion: </strong>An effective transition is vital for improving both pediatric and adult health outcomes. Future research should focus on standardized protocols such as the Six Core Elements of Health Care Transition and the integration of technology-based tools to better engage adolescent patients. Structured and evidence-based transition models supported by healthcare systems can improve transition.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833151","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Associations between CT radiomics analyses and kidney biopsy in patients with kidney disease.","authors":"Jacob Jalil Hassan, Fabian Baalmann, Jakob Leonhardi, Timm Denecke, Tom H Lindner, Uwe Scheuermann, Kerstin Amann, Silke Zimmermann, Jonathan de Fallois, Hans-Jonas Meyer","doi":"10.1186/s12882-026-05023-8","DOIUrl":"https://doi.org/10.1186/s12882-026-05023-8","url":null,"abstract":"<p><strong>Background: </strong>Kidney disease is characterized by microstructural alterations that currently require invasive biopsy for definitive assessment. However, it remains unclear to what extent radiomics features extracted from contrast-enhanced CT can non-invasively reflect kidney function and histopathological changes.</p><p><strong>Methods: </strong>Between October 2020 and May 2025 all patients undergoing kidney biopsies and having CT scans prior to biopsy were retrospectively analyzed. A total of 49 patients (59% female, median age 60 years) were included. Of the included patients, 35 biopsies were performed in native kidneys (71%) and 14 in kidney allografts (29%). Contrast-enhanced CT images were used to extract radiomics parameters of the kidney. Kidney segmentation was performed using TotalSegmentator and radiomics feature extraction was conducted with PyRadiomics.</p><p><strong>Results: </strong>Several associations were identified between the extracted radiomics features and kidney function as well as kidney tissue alterations. For the eGFR (CKD-EPI) the highest association was found for the radiomics feature Energy, which is a measurement of the intensity uniformity (ρ = 0.51, p < 0.001), while the first-order feature 90th Percentile showed the best performance in discriminating patients above and below an eGFR threshold of 15 mL/min/1.73 m² with an AUC of 0.83 (95% CI: 0.67-0.98, p = 0.001). Busyness correlated negatively with glomerulosclerosis (ρ = -0.38, p = 0.007), and Coarseness was positively associated with interstitial inflammation (ρ = 0.37, p = 0.008).</p><p><strong>Conclusions: </strong>CT radiomics features are associated with kidney function as well as histopathological alterations observed in kidney biopsies. Further validation is needed in future studies.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"27 1","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833439","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"X-Linked immune dysregulation, polyendocrinopathy, and enteropathy (IPEX) syndrome with concurrent membranous and IgA nephropathy: a case report.","authors":"Eman Nooreddeen, Abdulrahman Alabdulsalam, Ibrahim Sandokji, Fayhan Alroqi","doi":"10.1186/s12882-026-05015-8","DOIUrl":"https://doi.org/10.1186/s12882-026-05015-8","url":null,"abstract":"<p><strong>Background: </strong>X-linked immune dysregulation, polyendocrinopathy, and enteropathy (IPEX) syndrome is a rare X-linked disorder caused by mutations in the forkhead box P3 (FOXP3) gene. It typically presents very early in life with the classic triad of intractable diarrhea, type 1 diabetes mellitus, and dermatitis. Kidney involvement has been reported in a substantial minority of patients with IPEX syndrome, with membranous nephropathy being the most frequent biopsy pattern.</p><p><strong>Case presentation: </strong>In this case report, we describe a 15-year-old boy who developed type 1 diabetes mellitus at age one year and later developed difficult-to-control asthma, eczema-like skin rashes, and food allergies. At nine years of age, he was noted to have increasing creatinine levels and proteinuria. Kidney biopsy revealed overlapping features of membranous and immunoglobulin A (IgA) nephropathy. Genetic testing identified a hemizygous pathogenic variant in FOXP3 (c·1040 G > A, p.Arg347His), establishing a diagnosis of IPEX syndrome. Despite supportive management, his kidney function continued to decline, progressing to stage V chronic kidney disease, and he is now awaiting hematopoietic stem cell transplantation. This case highlights an unusual kidney presentation of IPEX syndrome.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833192","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between blood pressure and clinical outcomes in patients undergoing maintenance hemodialysis.","authors":"Seok Hui Kang, So Young Park, Yu Jeong Lim, Ji Young Choi, Bo Yeon Kim, Jun Young Do","doi":"10.1186/s12882-026-04954-6","DOIUrl":"https://doi.org/10.1186/s12882-026-04954-6","url":null,"abstract":"<p><strong>Background: </strong>Despite the clinical importance of blood pressure control in patients undergoing hemodialysis (HD), evidence on the blood pressure ranges associated with favorable outcomes remains limited and inconsistent.</p><p><strong>Methods: </strong>We retrospectively analyzed the data from the HD quality assessments in South Korea. Systolic blood pressure (SBP) was classified into 6 groups: VL-sys group (< 100, n = 230), L-sys (100-120, n = 2,272), R-sys (120-140, n = 17,004), H-sys (140-160, n = 17,493), VH-sys (160-180, n = 4,627), and EH-sys (≥ 180, n = 632). Diastolic blood pressure (DBP) was divided into 6 groups: EL-dia (< 60, n = 1,349), VL-dia (60-70, n = 5,460), L-dia (70-80, n = 13,361), R-dia (80-90, n = 17,442), H-dia (90-100, n = 4,211), and VH-dia (≥ 100, n = 435). Outcomes include all-cause mortality, cardiovascular events (CVE), dementia, atrial fibrillation (Afib), and fracture risk.</p><p><strong>Results: </strong>Higher SBP was associated with increased risks of all-cause mortality, CVE, dementia, and fractures compared to those of the R-sys group. Conversely, lower SBP was associated with reduced risks for all-cause mortality and CVE. The L-sys group had an adjusted hazard ratio of 0.92 (95% confidence interval [CI], 0.86-0.99) for all-cause mortality and 0.84 (95% CI, 0.76-0.93) for CVE compared with the R-sys group. Regarding DBP, in univariable analysis, survival rate was higher in the VH-dia group compared to R-dia group, but contradictive results were shown in multivariable analysis. Overall, in multivariable analysis, higher levels were associated with increased risks of all-cause mortality, CVE, and dementia, while lower DBP, particularly in the VL-dia and L-dia groups, was associated with reduced risks.</p><p><strong>Conclusions: </strong>In our study, a SBP of 100-119 mmHg and a DBP of 60-79 mmHg were associated with improved overall survival, reduced incidence of CVE, and lower risk of dementia and fractures in patients undergoing HD.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833551","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Renal hemodynamic alterations assessed by doppler ultrasonography in familial mediterranean fever patients without overt renal disease: a cross-sectional study.","authors":"Zeynep Tüzün, Hasan Abbasguliyev, Ekin Başak Doğancı, Muhammed Recai Akdoğan, Gökhan Polat, Meltem Alkan Melikoğlu","doi":"10.1186/s12882-026-05027-4","DOIUrl":"https://doi.org/10.1186/s12882-026-05027-4","url":null,"abstract":"<p><strong>Objective: </strong>Familial Mediterranean fever (FMF) is an autoinflammatory disease that may lead to renal parenchymal involvement and amyloidosis over time. Experimental and biopsy-based data from various renal diseases suggest that renal parenchymal injury may be accompanied by early alterations in renal hemodynamics. This study aimed to evaluate Doppler ultrasonography-derived renal hemodynamic parameters in adult FMF patients without overt renal involvement and to compare these findings with those of healthy controls.</p><p><strong>Methods: </strong>This cross-sectional observational case-control study included 77 adult FMF patients without significant proteinuria (> 500 mg/g) and 75 age- and gender-matched healthy controls. Bilateral renal resistance index (RRI) and pulsation index (PI) measurements were performed in all participants using Doppler ultrasonography. In subgroup analysis, FMF patients were divided into two groups based on urinary protein excretion: those with normal or minimal proteinuria (< 150 mg/g) and those with low-grade proteinuria (150-500 mg/g).</p><p><strong>Results: </strong>Both RRI and PI scores were significantly elevated in FMF patients than in controls (p < 0.05 for both). Subgroup analysis in FMF patients further revealed that RRI scores and PI scores were significantly higher in patients with low-grade proteinuria than in patients with normal or minimal proteinuria (p < 0.05 for both).</p><p><strong>Conclusions: </strong>Renal hemodynamic changes detected by Doppler ultrasonography can be identified in FMF patients, even when there is no apparent renal involvement. The greater increase in RRI and PI values was found in patients with low-grade proteinuria, indicating that changes in renal hemodynamics may be present at an early stage of the disease. These changes detected by Doppler ultrasonography may be used as an adjunctive tool for early evaluation of renal involvement in FMF.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833212","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Difference in risk profiles for pulmonary hypertension between non-elderly and elderly patients undergoing maintenance hemodialysis.","authors":"Zhen Ai, Qingling Jia, Suiqin Wen, Ying Zhu, DiHua Zhang, Raoping Wang, Jianhua Wu, Yuanwen Xu","doi":"10.1186/s12882-026-05020-x","DOIUrl":"https://doi.org/10.1186/s12882-026-05020-x","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary hypertension (PH) is highly prevalent and associated with increased mortality in patients undergoing maintenance hemodialysis (MHD). This study aimed to explore the disparities in the prevalence, risk factors, and prognostic impacts of PH between non-elderly and elderly MHD patients.</p><p><strong>Methods: </strong>This study included 179 MHD patients with complete clinical records. All patients were evaluated using Doppler echocardiography, and PH was defined as a pulmonary artery systolic pressure > 35 mmHg.</p><p><strong>Results: </strong>The prevalence rates of PH were 24.8% in non-elderly patients and 33.8% in elderly patients. Serum N-terminal pro-brain natriuretic peptide (NT-proBNP) was a predominant risk factor for PH in hemodialysis patients. A lower ratio of uric acid to high-density lipoprotein (UHR) was associated with PH among non-elderly patients, whereas diabetes mellitus served as a specific risk factor for elderly patients. The areas under the receiver operating characteristic curves of these risk factors identified in non-elderly and elderly patients were 0.86 (bootstrap 95% confidence interval (CI) 0.78-0.93) and 0.90 (bootstrap 95% CI 0.82-0.97), respectively. During a median follow-up duration of 32.90 (4.00-61.50) months, the presence of PH notably elevated the risk of all-cause mortality and cardiovascular hospitalization in both non-elderly and elderly patients. Meanwhile, it significantly augmented the risk of all-cause hospitalization (HR: 2.24, 95% CI 1.26-3.98, P = 0.006) in the non-elderly.</p><p><strong>Conclusions: </strong>Distinct risk profiles for PH were identified between non-elderly and elderly MHD patients, offering valuable clinical data for the development of early detection and prevention strategies based on age stratification.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833593","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hydroxychloroquine-induced renal phospholipidosis manifesting as proximal tubulopathy in systemic lupus erythematosus.","authors":"Shun Manabe, Momoko Seki, Yusuke Ushio, Moeko Ochiai, Rina Takahashi, Shizuka Kobayashi, Shiho Makabe, Naoko Ito, Hiroshi Kataoka, Sekiko Taneda, Kazuho Honda, Kosaku Nitta, Junichi Hoshino","doi":"10.1186/s12882-026-05021-w","DOIUrl":"https://doi.org/10.1186/s12882-026-05021-w","url":null,"abstract":"<p><strong>Background: </strong>Hydroxychloroquine (HCQ)-induced renal phospholipidosis typically manifests as glomerular \"zebra bodies\", \"myeloid bodies\", and \"curvilinear bodies\" and is generally considered a benign histological mimic of Fabry disease. We report a case of HCQ-induced renal phospholipidosis with proximal tubulopathy presenting as slowly progressive kidney dysfunction and Fanconi syndrome, challenging the notion that renal phospholipidosis is clinically silent.</p><p><strong>Case presentation: </strong>A 36-year-old woman with systemic lupus erythematosus (SLE) treated with HCQ for 18 months presented with slowly progressive kidney dysfunction. Urinalysis showed minimal proteinuria and no active sediment suggestive of a lupus nephritis flare; however, urinary markers of tubular injury were markedly elevated. She exhibited normoglycemic glycosuria, pan-aminoaciduria, hypophosphatemia, hypouricemia, and metabolic acidosis, consistent with mild but distinct Fanconi syndrome. Her estimated glomerular filtration rate (eGFR) slope rapidly declined at - 11.2 mL/min/1.73 m²/year during HCQ treatment. Kidney biopsy revealed glomerular z\"zebra bodies\", \"myeloid bodies\", and \"curvilinear bodies\" characteristic of HCQ-induced renal phospholipidosis, as well as lysosomes filled with electron-dense granules within glomeruli. Notably, lysosomes filled with electron-dense granules were also abundant in proximal tubular epithelial cells, resembling the \"lysosomal accumulation of light chains\" seen in light chain proximal tubulopathy (LCPT) without crystal formation and \"lysosomes containing dark electron-dense aggregates\" of chronic interstitial nephritis in agricultural communities (CINAC). Extensive clinical, biochemical, genetic, and histological evaluations excluded Fabry disease. Immunofluorescence demonstrated globotriaosylceramide (Gb3) minor and patchy positivity accumulation in both glomeruli and proximal tubules, suggesting that lysosomal metabolic dysfunction occurred similarly in glomerular cells and tubular epithelial cells. Based on these findings, a diagnosis of proximal tubulopathy secondary to HCQ-induced renal phospholipidosis was made. HCQ discontinuation resulted in the resolution of Fanconi syndrome and improvement of the eGFR slope to + 0.9 mL/min/1.73 m²/year.</p><p><strong>Conclusions: </strong>This case indicates that HCQ-induced renal phospholipidosis is not merely a silent histological finding but can manifest as clinically significant proximal tubulopathy. The pathophysiology likely involves active tubular secretion of HCQ causing rapid lysosomal and transporter dysfunction analogous to LCPT without crystal formation and CINAC. While standard monitoring for lupus nephritis focuses on glomerular markers, monitoring tubular function markers in HCQ-treated patients may enable early detection of this potentially underdiagnosed complication.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147810496","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Case report of a child with kidney disease: consideration of the risk of a single APOL1 G2 allele with a protective N264K variant on the G0 parental chromosome.","authors":"Meidad Greenberg, Orly Tabachnikov, Dror Ben-Ruby, Asaf Vivante, Karl Skorecki","doi":"10.1186/s12882-026-05006-9","DOIUrl":"https://doi.org/10.1186/s12882-026-05006-9","url":null,"abstract":"<p><strong>Background: </strong>Apolipoprotein L1 (APOL1)-mediated kidney disease is causally associated with the G1/G2 risk alleles of the APOL1 gene, and shows incomplete penetrance shaped by environmental and genomic modifiers. A rare coding variant, N264K, observed on G0 or G2 variants, has been associated with protection in high-risk genotypes. Importantly, emerging data indicate that in some settings a single APOL1 risk allele can confer risk, including reports of end stage kidney disease in G0/G2 individuals, underscoring the need to determine whether N264K protection requires cis configuration with G2 allele (on the same polypeptide), or can also act in trans (on the opposite allele), an issue of potential future clinical diagnostic and therapeutic relevance when phase is unknown for certain genotypes.</p><p><strong>Case presentation: </strong>A previously healthy 10-year-old Muslim Arab male developed edema during laboratory-confirmed influenza. He had sub-nephrotic-range proteinuria with hypoalbuminemia, preserved kidney function, and normal blood pressure. Treatment with prednisone according to Kidney Disease: Improving Global Outcomes (KDIGO) pediatric guidelines did not induce remission. Kidney biopsy showed diffuse mesangial hypercellularity with prominent IgM deposition. In patients with steroid non-remitting disease, this finding is compatible with IgM nephropathy that can herald focal segmental glomerulosclerosis. Tacrolimus was started, and later pulse methyl-prednisolone was administered, achieving only transient reduction in proteinuria; therapy was complicated by reversible insulin-requiring hyperglycemia and subsequently withdrawn. Subsequent administration of Rituximab in combination with angiotensin-converting enzyme (ACE) inhibition resulted in significant reduction of proteinuria. Trio exome sequencing demonstrated APOL1 G0/G2 with N264K on the paternal G0 allele (trans to G2) and no other monogenic cause of steroid-resistant nephrotic syndrome. At follow-up, the child has preserved kidney function with normal plasma albumin level and without proteinuria. In human embryonic kidney (HEK-293) cultured cells co-transfection assays modeling heterozygosity, N264K reduced G2-dependent cytotoxicity only in cis (same construct as G2) and not in trans (on G0), despite comparable expression; the effect was stronger on an EIK than a KIK haplotype backbone.</p><p><strong>Conclusions: </strong>Taken together the clinical findings and the in vitro experimental laboratory results suggest that APOL1 allele phasing may be informative in selected cases where both G2 and N264K are reported, and support development of APOL1-mediated kidney disease biomarkers and genotype-informed therapies.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833645","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Delayed graft function and its impact on short- and long-term outcomes after kidney transplantation.","authors":"Jehat Kiliç, Rengin Esen, Caner Varhan, Ömer Faruk Alakuş, Delyadil Karakaş Kiliç, Ramazan Daniş","doi":"10.1186/s12882-026-05029-2","DOIUrl":"https://doi.org/10.1186/s12882-026-05029-2","url":null,"abstract":"<p><strong>Introduction: </strong>Kidney transplantation is an effective treatment for end-stage renal disease, markedly improving survival and quality of life. However, delayed graft function (DGF) remains a notable early post-transplant complication. This study examine the impact of DGF on mortality, hypertension, graft survival, and other post-transplant complications.</p><p><strong>Methods: </strong>This retrospective study included adult recipients (≥ 18 years) who underwent kidney transplantation at the Organ Transplant Unit of Diyarbakır Gazi Yaşargil Training and Research Hospital between 1 January 2013 and 31 December 2023. A total of 285 patients with at least 12 months of follow-up were analyzed. Demographic characteristics, presence of post-transplant hypertension, immunosuppressive regimens, and laboratory parameters related to graft function were extracted from the Hospital Information Management System (HIMS) using a standardized abstraction form. Post-transplant hypertension was defined according to the KDIGO 2021 guideline; details of the analytic approach are described in the Statistics section.</p><p><strong>Results: </strong>A total of 285 kidney transplant recipients were included, with DGF observed in 31 patients (10.9%). Baseline characteristics such as recipient age, sex, BMI, and pre-transplant hypertension were comparable between groups (p > 0.05). Patients with DGF had significantly older donors (p = 0.006), a higher proportion of non-related donors (p = 0.001), longer dialysis duration (p = 0.008), and more frequent deceased donor transplantation (35.5% vs. 5.9%, p < 0.001). Postoperative complications and mortality were also higher in the DGF group (p = 0.003 and p < 0.001, respectively). In univariate analysis, non-related donor status, postoperative events, discharge protein levels, and dialysis duration were associated with DGF. In multivariate analysis, deceased donor status (OR = 4.883, p = 0.015), postoperative events (OR = 3.336, p = 0.011), and discharge protein levels (OR = 1.007, p = 0.033) remained independent predictors.</p><p><strong>Conclusion: </strong>DGF was associated with adverse outcomes after kidney transplantation. Deceased donor status, postoperative events, and higher discharge protein levels were identified as independent predictors, underscoring the multifactorial nature of DGF. Early identification of high-risk patients and close post-transplant monitoring may improve clinical outcomes.</p><p><strong>Clinical trial number: </strong>Not applicable.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147833567","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}