Iranian journal of kidney diseases最新文献

{"title":"Anti-Glomerular Basement Membrane Disease Combined with IgA Nephropathy: A Case Report and Literature Review.","authors":"Limin Wang, Meichun Huang, Jun Liu, Xiuxiu Li, Liping Zhao","doi":"10.61882/89vw9y26","DOIUrl":"10.61882/89vw9y26","url":null,"abstract":"<p><p>Anti-glomerular basement membrane (GBM) disease is a rare autoimmune disease characterized by injury to small blood vessels in the kidneys and/or lungs. IgA nephropathy is the most common primary glomerular disease diagnosed in China. However, the co-occurrence of anti-GBM illness and IgA nephropathy has been infrequently documented in the literature. At present, its mechanism is unknown, the treatment approach is unclear, and the prognosis remains poor. Herein, we report a case of anti-GBM disease complicated with IgA nephropathy, in which renal function returned to normal state following treatment with a combination of prednisone and cyclophosphamide. This case suggests that in patients with anti-GBM disease and IgA nephropathy, the combination of glucocorticoids and cyclophosphamide may serve as an optimum treatment choice.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"19 6","pages":""},"PeriodicalIF":0.9,"publicationDate":"2026-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156662","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":"Bibliometric Analysis of Uremia Research: 2021-2025.","authors":"Jie Wei, Pan Gao, Zhi Wang, Lei Fan, Jin Zhao","doi":"10.61882/pjm29b56","DOIUrl":"10.61882/pjm29b56","url":null,"abstract":"<p><strong>Introduction: </strong>This study aimed to perform a bibliometric analysis of academic research on uremia, identifying research hotspots, development trends, and international collaboration networks in this field.  Methods. Relevant publications related to uremia were retrieved from Web of Science database. Bibliometric analysis was conducted using R software. Co-occurrence analysis of keywords, co-citation network analysis, and author/country collaboration analysis were performed to explore research focuses and academic influence.</p><p><strong>Results: </strong>From 2021 to 2022, research on uremia mainly concentrated on traditional areas such as chronic kidney disease, hemodialysis, and patient management. Since 2023, the focus has gradually shifted toward emerging topics including plasma biomarkers, inflammation mechanisms, and precision patient care. Co-citation network analysis revealed that studies related to chronic kidney disease and hemodialysis had the strongest academic influence, while research on inflammation and uremia also occupied central positions in the network. International collaboration was most active in China, the United States, and Europe, reflecting a trend toward cross-border and interdisciplinary research. Meanwhile, keywords such as gut microbiota, metabolomics, and precision medicine are increasingly frequent, highlighting new research frontiers.</p><p><strong>Conclusion: </strong>This study demonstrates that uremia research is shifting from traditional areas such as chronic kidney disease and hemodialysis toward emerging themes including inflammation, biomarkers, and patient management. These findings provide objective evidence of research hotspots and trends in uremia and underscore the importance of enhancing international and interdisciplinary collaboration.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"19 6","pages":"311-321"},"PeriodicalIF":0.9,"publicationDate":"2026-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156597","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":"Predictors of Kidney and Patient Survival in Monoclonal Gammopathy-Associated Kidney Disease: A Single-Center Cohort from Colombia.","authors":"Sebastian Gomez Jimenez, Simon Cano Rodas, Jose Nelson Carvajal Quiroz, Luis Fernando Arias Restrepo, Joaquín Roberto Rodelo-Ceballos","doi":"10.61882/ijkd.20.01.8912","DOIUrl":"10.61882/ijkd.20.01.8912","url":null,"abstract":"<p><strong>Introduction: </strong>Monoclonal gammopathies can induce various kidney disorders through the deposition of monoclonal immunoglobulin. Precise recognition and classification are essential for predicting outcomes and customizing treatment. However, data on prognostic factors in Hispanic and Latin American populations remain scarce. This study aimed to determine the predictors of kidney and patient survival in adults with biopsy-proven monoclonal gammopathy-associated kidney disease.</p><p><strong>Methods: </strong>We conducted a retrospective cohort involving 98 individuals with biopsy-confirmed disease evaluated between 2011 and 2022. Kidney and patient survival were estimated using Kaplan-Meier analysis, and differences across histopathologic subtypes were assessed with the log-rank test. Predictors of end-stage kidney disease (ESKD) and mortality were identified using univariate and multivariable Cox regression after verification of the proportional hazards' assumption.</p><p><strong>Results: </strong>Approximately one third of patients required kidney replacement therapy (KRT) at presentation. The need for KRT (hazard ratio [HR] 4.86, 95% confidence interval [CI] 2.01-11.79) and an estimated glomerular filtration rate (eGFR) < 30 mL/min/1.73 m² (HR 4.02, 95% CI 1.38-11.71) independently predicted progression to ESKD. Amyloidosis (HR 2.38, 95% CI 1.22-4.86) and age > 60 years (HR 1.96, 95% CI 1.06-3.61) were associated with higher mortality. The median follow-up was 41 months (interquartile range 24-68); 31% progressed to ESKD and 46% died.</p><p><strong>Conclusions: </strong>Severe kidney dysfunction and the need for replacement therapy at diagnosis are strong predictors of poor renal outcomes. Amyloidosis and older age significantly affect overall survival. Early recognition of high-risk patients and access to effective, clone-directed therapy are essential to improve prognosis in resource-limited settings.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"19 6","pages":"341-350"},"PeriodicalIF":0.7,"publicationDate":"2026-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156642","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":"The Efficacy of Mumijo Extract in Protecting Against Amoxicillin/Clavulanate-Induced Apoptotic Damage in Renal Tissues of Rats.","authors":"Hasan Mansur Durgun, Eda Yildizhan","doi":"10.61882/66mbsb92","DOIUrl":"10.61882/66mbsb92","url":null,"abstract":"<p><strong>Background: </strong>Amoxicillin/Clavulanate (A/C) is a widely used antibiotic that can induce oxidative stress and renal apoptosis through reactive oxygen species (ROS). Mumijo extract (PS), known for its antioxidant and anti-inflammatory properties, has shown promise in mitigating such damage. This study aims to evaluate the efficacy of PS in protecting against A/C-induced renal tissue damage in rats.</p><p><strong>Materials and methods: </strong>The study included 28 Wistar albino rats, divided into four groups: Sham (saline), A/C (10 mg/kg/day), PS (100 mg/kg/day), and A/C+PS. A/C and PS were administered orally for 21 days. Renal function markers (urea, creatinine), oxidative stress parameters (TAS, TOS, MDA), and histopathological findings were analyzed. Bax and Bcl-2 protein expressions were assessed to evaluate apoptosis.</p><p><strong>Results: </strong>A/C administration significantly increased renal urea, creatinine, TOS, and MDA levels while reducing TAS. Histopathological analysis revealed tubular dilatation, degeneration, and inflammation in the A/C group. PS supplementation in the A/C+PS group significantly reduced oxidative stress markers and improved TAS levels, histopathological scores, and apoptosis indicators (increased Bcl-2 and reduced Bax expressions). PS alone demonstrated no adverse effects on renal parameters.</p><p><strong>Conclusion: </strong>Mumijo extract effectively mitigates A/C-induced oxidative stress and renal apoptosis, preserving tissue integrity and function. These findings suggest PS as a potential therapeutic agent for managing drug-induced nephrotoxicity.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"19 6","pages":"351-360"},"PeriodicalIF":0.7,"publicationDate":"2026-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156609","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":"The Association Between Serum Sodium Variation During Pediatric Intensive Care Unit Admission and the Risk of Clinical Outcomes in Critically Ill Children: A Retrospective Cohort Study.","authors":"Zahra Pournasiri, Ali Nikparast, Mahsa Bakhtiary, Masumeh Hashemi, Seyyedeh Narjes Ahmadizadeh, Azita Behzad, Golaleh Asghari","doi":"10.61882/ijkd.19.06.8917","DOIUrl":"10.61882/ijkd.19.06.8917","url":null,"abstract":"<p><strong>Introduction: </strong>Dysnatremia at Pediatric Intensive Care Unit (PICU) has been associated with adverse outcomes; however, the impact of sodium variation during PICU stay remains underexplored. We aimed to assess the association between dysnatremia and sodium fluctuations during PICU admission and the probability of prolonged PICU stay (≥7days) and mortality.</p><p><strong>Methods: </strong>This retrospective cohort study included 966 critically ill children (2019-2022) with at least two serum sodium measurements during PICU admission. Patients were classified as normonatremic, hyponatremic, hypernatremic, or mixed dysnatremic based on all sodium values. Sodium fluctuation was defined as the difference between the highest and lowest sodium levels during admission and categorized into quartiles. Associations of dysnatremia categories and sodium fluctuation with prolonged PICU stay and PICU mortality were evaluated using multivariable logistic regression.</p><p><strong>Results: </strong>During the PICU admission, 542 patients developed dysnatremia. The PICU-acquired dysnatremia was independently associated with higher odds of prolonged PICU stay after adjusting for major confounders. The PICU-acquired hypernatremia (OR:5.23;95%CI:1.72-15.92) and mixed dysnatremia (OR:2.89;95%CI:1.14-7.29) had significantly higher odds for PICU mortality. Even mild sodium fluctuations (4-8mmoL/L) during PICU admission were significantly associated with higher odds of prolonged PICU stay. The highest (≥15mmol/L) fluctuations in serum sodium levels during PICU admission were also significantly associated with higher odds of PICU mortality (OR:2.74;95%CI:1.11-6.79).</p><p><strong>Conclusion: </strong>Our findings underscore the importance of careful monitoring of serum sodium levels during PICU admission to improve clinical outcomes. These results should be interpreted in light of the retrospective design and the unavailability of standardized illness-severity scores (e.g., PRISM or PIM) in the dataset.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"19 6","pages":"331-340"},"PeriodicalIF":0.7,"publicationDate":"2026-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156593","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":"A Report on Nephropathic Cystinosis in Iran- A Scoping Review.","authors":"Nakysa Hooman, Soudabeh Hosseini","doi":"10.61186/","DOIUrl":"10.61186/","url":null,"abstract":"<p><p>Cystinosis  is  a  rare  lysosomal  disease  affecting  all  organs.  The outcome  and  the  quality  of  life  varies  depending  on  the  early diagnosis, patient adherence to medication, and regular follow up. There is lack of enough evidence about cystinosis patients in Iran. In this review, we are going to find the incidence and prevalence, the  age  at  diagnosis,  clinical  presentation,  the  comorbidities, and  the  outcome  of  disease  in  Iranian  children.  We  searched  all available  database  of  Iran  published  between  January  1994  and January 2023. The search was filtered for the Iranian population. Totally  1125  cystinosis  patients  over  37  years  (between  1985  to 2023)  was  identified.  The  estimated  incidence  rate  was  1.03  per 100000 live births. The pooled prevalence was 28.3 (95%CI:14.9- 46) per  1000000  populations.  Median  age  at  presentation,  diagnosis, and  occurrence  of  end  stage  kidney  disease  were  0.73,  1.5,  and 8.3 years, respectively. The most frequent presentation that led to evaluation was failure to thrive (94%). Ophthalmic exam and bone marrow  aspiration  were  the  mostly  used  methods  for  diagnosis. The  most  common  detected  mutation  was  c.681G4A/c.681G4A. The  cysteine  level  represent  that  more  than  half  of  the  cases  did not receive correct dosage of medication. Almost 8.7% of cystinosis patients  reached  adulthood. Launching  to  transition  protocol  is mandatory  for  surviving  to  adulthood.  There  were  scant  data  in regard to extrarenal outcomes, systemic and ophthalmic medications side effects, longitudinal ophthalmic involvement reports, and the quality of life.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"19 5","pages":"260-271"},"PeriodicalIF":0.9,"publicationDate":"2025-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156661","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":"Examining the Association between Oxidative Stress Markers and the Severity of Symptoms in Individuals with COVID-19 and Healthy Individuals.","authors":"Fatemeh Pourrezagholi, Masoumeh Ebrahimi, Yadollah Shakiba, Hossein Amini, Mohammad Rahmanian, Tayebe Zomorrodi Jangaee, Zahra Golkar, Shideh Anvari, Nooshin Dalili","doi":"10.61186/","DOIUrl":"10.61186/","url":null,"abstract":"<p><strong>Introduction: </strong>The association between inflammation and oxidative stress  in  COVID-19  patients  is  well  documented.  The  primary objective of the present study was to evaluate the levels of oxidative stress  markers  in  COVID-19  patients  compared  to  the  healthy individuals.</p><p><strong>Methods: </strong>In this case-control study, a comprehensive evaluation was conducted on 45 individuals, comprising 30 patients diagnosed with  COVID-19  and  15  in  a  healthy  control.  COVID-19  patients were divided into two groups: mild and severe, based on clinical severity. In addition to standard laboratory tests like ESR, serum levels of oxidative stress markers were measured, including total protein, total antioxidant capacity, total oxidant status, carbonylated protein content, glutathione reductase enzyme, and catalase activity.</p><p><strong>Results: </strong>ESR levels were significantly elevated in the severe group compared to the mild group. However, no significant differences were  observed  in  the  levels  of  oxidative  stress  markers  between mild and severe COVID-19 patients. Compared to healthy controls, COVID-19 patients exhibited a significant decrease in total protein levels (P < .0001), and significant increases were observed in serum total antioxidant capacity (TAC) (P < .0001) and total oxidant status(TOS) (P = .002).</p><p><strong>Conclusion: </strong>Oxidative  stress  markers  exhibit  a  substantial  rise in  patients  with  COVID-19, suggesting  a  potential  role  in  the progression  of  the  disease.  Implementing  strategies  to prevent oxidative  stress  may  offer  clinical  advantageous  in  managing COVID-19 patients.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"19 5","pages":"272-278"},"PeriodicalIF":0.9,"publicationDate":"2025-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156649","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":"Calf Bioimpedance Analysis: A Novel Approach for Evaluating Hydration Status in Pediatric Dialysis Patients Under High Dialysate Sodium.","authors":"Mohammad Firouzmand, Shahrokh Noori, Vahid Reza Nafisi, Nakysa Hooman","doi":"10.61186/","DOIUrl":"10.61186/","url":null,"abstract":"<p><strong>Introduction: </strong>Assessment  of  hydration  status  in  pediatric hemodialysis is complicated by dynamic fluid shifts and sodium variability.  Conventional  methods  such  as  clinical  evaluation and  whole-body  bioimpedance  analysis  (BIA)  lack  precision  for individual-level monitoring. This study introduces a calf impedance ratio (ΔZ₁₅ₖₕ / ΔZ₁₅₀ₖₕ) to classify fluid responses and evaluate the impact of high dialysate sodium (143 mEq/L) on fluid dynamics and blood pressure.</p><p><strong>Methods: </strong>A  cross-sectional  study  was  conducted  involving  14 children aged 5-12 years undergoing maintenance hemodialysis. Impedance was measured pre- and post-dialysis at two frequencies using  a  custom-built  device.  The  raw  impedance  changes  were analyzed  without  conversion  to  fluid  volumes,  allowing  for classification  into  three  physiological  response  groups  based  on the impedance ratio.</p><p><strong>Results: </strong>Three  distinct  physiological  profiles  emerged.  Group1  (ΔZΔZ150kh15kh > 1)  showed  effective  extracellular  fluid  removal  with stable  hemodynamics.  Group  2  (ratio  between  0  and  1)  reflected minimal  Extracellular  Water  (ECW)  ECW  changes,  potentially due  to  sodium-driven  redistribution,  with  additional  influence from  sodium-enhanced  tissue  conductivity.  Group  3  (ΔZ₁₅ₖₕ < 0) exhibited paradoxical impedance decreases despite fluid removal,along  with  sodium  increase  and  blood  pressure  elevation.  ΔZ₁₅ₖₕpositively correlated with ΔBP-sys (r = 0.650, P = .012).</p><p><strong>Conclusion: </strong>The impedance ratio (ΔZΔZ150kh15kh ) provides a sensitive index for classifying fluid responses and identifying sodium-related effects in pediatric hemodialysis. It may support individualized dialysis prescriptions by guiding adjustments in ultrafiltration and dialysate sodium to improve hemodynamic stability and fluid balance.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"19 5","pages":"294-303"},"PeriodicalIF":0.9,"publicationDate":"2025-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156672","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":"Efficacy and Safety of Oral Antidiabetic Drugs in People with Diabetes and Chronic Kidney Disease: A Systematic Review.","authors":"Hoda Borooghani, Mohsen Arabi, Mitra Kazemi Jahromi","doi":"10.61882/zwq4sh57","DOIUrl":"10.61882/zwq4sh57","url":null,"abstract":"<p><p>This study is a systematic review assessing the efficacy and safety of oral antidiabetic drugs in managing type 2 diabetes mellitus in people with chronic kidney disease (CKD), focusing on their dosing and effects on renal function and glycemic control. A systematic search of online databases was conducted for published from  January  2023  to September  2024.  Studies  that  focused  on oral  antidiabetic  drugs  for  individuals  with  diabetes and  CKD, and  reported  outcomes  such  as  glycemic  control,  renal  function, and adverse events were included. A risk-of-bias assessment was performed to evaluate study quality. A  total  of  21  studies  that  met  the  criteria  were  included.  The evidences indicate that SGLT2 (Sodium-Glucose Cotransporter-2) inhibitors and GLP-1 (Glucagon-like peptide-1) receptor agonists provide renal protective benefits. SGLT2 inhibitors are more effective especially in early-stages of CKD. DPP-4 (Dipeptidyl peptidase 4) inhibitors are safe across various CKD stages and require minimal dose adjustments. Metformin remains a popular drug for glycemic control but should be monitored and even discontinued in advanced stages of CKD because of the risk of lactic acidosis. Sulfonylureas were related to hypoglycemia risk. Oral  antidiabetic  drugs,  especially  SGLT2  inhibitors  and  GLP-1 receptor  agonists,  are  suggested  in  managing  blood  glucose  in CKD  patients  owing  to  their  renal  and  cardiovascular  benefits. Individualized therapy is an important factor as drug safety and efficacy  are  influenced  by  CKD  stage,  comorbid  conditions,  and hypoglycemia risk. Individualized therapy helps maximize renal and cardiovascular protection while minimizing adverse outcomes. Regular monitoring of HbA1c (hemoglobin A1c), GFR (glomerular filtration rate), and albuminuria is recommended.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"19 5","pages":"249-259"},"PeriodicalIF":0.9,"publicationDate":"2025-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156667","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":"A Rare Cause of Retroperitoneal Fibrosis in Adults: H Syndrome Presenting After 35 Years- A Case Report.","authors":"Türker Emre, Nazife Nur Özer Şensoy","doi":"10.61882/npz6gc25","DOIUrl":"10.61882/npz6gc25","url":null,"abstract":"<p><strong>Introduction: </strong>Retroperitoneal  fibrosis  (RPF)  is  a  rare  fibro- inflammatory  disorder  that  may  cause  urinary  tract  obstruction and renal failure. Although most RPF cases are IgG4-related, rare genetic conditions can also contribute to the disease. H syndrome, an  autosomal  recessive  disorder  caused  by  biallelic  SLC29A3 mutations, usually presents in childhood with cutaneous, endocrine, and systemic manifestations; adult onset is exceptionally rare.</p><p><strong>Case presentation: </strong>A 36-year-old woman was admitted to hospital with the complaint of nausea, vomiting, and oliguria. Laboratory tests showed urea 116 mg/dL, creatinine 5,8 mg/dL,. Non-contrast computed tomography imaging revealed bilateral hydronephrosis and a 2,5 cm retroperitoneal fibrotic mass encasing the abdominal aorta and iliac arteries. Extensive evaluation-including positron emission tomography, lymph-node histology, and autoimmune/ IgG4 serology-found no evidence of malignancy, infection, or IgG4- related  disease.  Family  history  disclosed  parental  consanguinity and  a  brother  with  H  syndrome.  Next-generation  sequencingidentified  a  homozygous  SLC29A3  (NM_018344.5):c.1339G > A (p.Glu447Lys) mutation, confirming H syndrome. Bilateral ureteral stenting  and  corticosteroid  therapy  led  to  full  renal  recovery (creatinine 0.7 mg/dL).</p><p><strong>Conclusion: </strong>This case expands the clinical spectrum of H syndrome by  documenting  late-onset  RPF  without  typical  dermatologic  or auditory  signs.  Recognition  of  familial  risk  and  timely  genetic testing  were  decisive  for  diagnosis.  Early  decompression  and immunosuppression  preserved  renal  function.  Reporting  suchatypical  presentations  increases  awareness  of  H  syndrome  as  a rare  genetic  cause  of  RPF  and  supports  comprehensive  genetic evaluation in adults with unexplained retroperitoneal fibrosis.</p>","PeriodicalId":14610,"journal":{"name":"Iranian journal of kidney diseases","volume":"19 5","pages":"304-309"},"PeriodicalIF":0.9,"publicationDate":"2025-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148156656","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}