Journal of Nephrology最新文献

{"title":"Immunotherapy-induced acute interstitial nephritis with neutrophil infiltrate, looking like pyelonephritis.","authors":"Mathis Michel, Adrien Flahault, Antoine Max, Jerome Olagne, Hervé Sartelet","doi":"10.1007/s40620-025-02332-3","DOIUrl":"https://doi.org/10.1007/s40620-025-02332-3","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144608570","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":"Lumasiran at birth changes the trajectory of primary hyperoxaluria type 1: same disease, different outcomes in two affected siblings.","authors":"Licia Peruzzi, Marta Leporati","doi":"10.1007/s40620-025-02325-2","DOIUrl":"https://doi.org/10.1007/s40620-025-02325-2","url":null,"abstract":"<p><p>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 comorbidities. These challenges informed the decision to initiate immediate treatment for the younger sibling. The child was treated at 6 h of life with lumasiran 6 mg/kg subcutaneously, in combination with pyridoxin 10 mg/kg/day. Lumasiran 6 mg/kg was repeated at 30 and 60 days, then was reduced to 3 mg/kg every month. Intravenous hyperhydration (240 mL/kg/day) was maintained for 16 days, together with oral water and potassium citrate (500 mg in 500 mL/day) in addition to breastfeeding. Although gycolate oxidase inhibition was started immediately after birth in the absence of previous deposits, it showed a latency of at least 15 days. Over this period of time, dangerous levels of blood and urinary oxalate were reached, due to the physiological low glomerular filtration rate in the perinatal period, as demonstrated by the increasing levels of endogenous oxalate production until day 6. Blood oxalate supersaturation 30 days after the first dose of treatment was never reached again. No adverse events occurred. In this report, early treatment with lumasiran, coupled with hyperhydration and supportive therapy, was able to ensure the absence of primary hyperoxaluria type 1 symptoms throughout the 24 months of follow-up.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144591476","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":"Postpartum renal cortical necrosis:  experience from a tertiary care center in India.","authors":"Adarsh Kumar, Meenakshi Rajput, Rajesh Kumar, Pallavi Prasad, Himanshu Verma, Sanjiv Mahajan, Jyotsna Suri, Vinneta Batra","doi":"10.1007/s40620-025-02337-y","DOIUrl":"https://doi.org/10.1007/s40620-025-02337-y","url":null,"abstract":"<p><strong>Background: </strong>Renal cortical necrosis is a rare but severe form  of acute kidney injury (AKI), frequently seen in obstetric settings. This study examines the clinical features, laboratory trends, and renal outcomes of postpartum renal cortical necrosis.</p><p><strong>Methods: </strong>We retrospectively reviewed the medical records of patients who developed postpartum renal cortical necrosis over two years, analyzing their clinical profiles, hematological data at various time points, and kidney outcomes, at three months postpartum.</p><p><strong>Results: </strong>Among 88 patients with post partum AKI requiring dialysis, 25 were diagnosed with renal cortical necrosis (23 via kidney biopsy, 2 via contrast-enhanced computed tomography). The causes included puerperal sepsis (9), atypical hemolytic-uremic syndrome (aHUS) (8), placental abruption (7), postpartum hemorrhage (7), and lupus nephritis (2). All patients exhibited signs of  hemolysis, thrombocytopenia, and elevated liver enzymes immediately postpartum. Over 11.84 ± 2.76 days, the mean hemoglobin level, mean platelet count, and mean liver enzyme levels (SGPT and SGOT) improved significantly, while the mean serum creatinine level remained unchanged (p = 0.27). Of the 23 cases with a kidney biopsy, 19 had diffuse renal cortical necrosis and 4 had partial necrosis; 52.2% showed signs of thrombotic microangiopathy (TMA). Only 4 patients had partial renal recovery at discharge, with improved estimated glomerular filtration rate (eGFR) at 3 months; the remaining 21 showed no recovery. Diffuse cortical necrosis, persistent anuria, dialysis dependence at discharge, and elevated follow-up lactate dehydrogenase (LDH) and D-dimer were associated with no kidney recovery.</p><p><strong>Conclusions: </strong>In this recent series, postpartum renal cortical necrosis is associated with microangiopathic hemolysis, thrombocytopenia, and elevated liver enzymes, with over half of biopsies showing TMA. Renal outcomes were poor. Further research is needed to explore the pathogenesis of TMA and exploring eventual genetic factors.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144584105","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":"Kidney transplant from donation after circulatory death: a novel pre-transplant combined clinico-pathological predictive score.","authors":"Leonardo Bizzo, Nadia Mansour, Alessandra Maria Storaci, Maria Carmela Rossi, Tullia De Feo, Stefano Ferrero, Alessandro Del Gobbo, Umberto Gianelli","doi":"10.1007/s40620-025-02336-z","DOIUrl":"https://doi.org/10.1007/s40620-025-02336-z","url":null,"abstract":"<p><strong>Background: </strong>Kidney donation after circulatory death is a valid alternative to donation after brain death. However, ischemia-reperfusion injury can damage the graft and make it non-viable. The Karpinski score, currently used to evaluate kidney quality, does not account for this type of damage and is, therefore, insufficient for predicting expected functional recovery.</p><p><strong>Methods: </strong>Sixty-seven kidney biopsies from donors  after circulatory death and seventy-two kidney biopsies from donors after brain death were analyzed histologically. A Karpinski score was assigned to all samples. Additionally, biopsies obtained from donation after circulatory death were reviewed for the presence of ischemia-reperfusion injury defined as brush border loss within the tubular epithelium as well as acute tubular injury. Clinical data were also retrieved and analysed, in an attempt to find a statistically significant correlation between histological/clinical findings and graft function.</p><p><strong>Results: </strong>The mean Karpinski scores of samples obtained on the occasion of donation after circulatory death and after brain death showed no significant difference. Ischemia-reperfusion injuries were two times more frequent in samples obtained after donation after circulatory death compared to donation after brain death. Statistical analysis revealed that a cutoff of 15% in brush border loss and 0.9 mg/dL in serum creatinine before death allowed to predict functional recovery, especially delayed functional recovery. A composite predictive score summing these two parameters was therefore created.</p><p><strong>Conclusions: </strong>Donation after circulatory death is associated with unique patological characteristics compared to donation after brain death and requires different considerations. This study proposes a simple and clinically applicable tool for predicting the delayed functional recovery in the case of kidneys donated after circulatory death; the novelty lies in combining morphological (brush border loss) and biochemical (serum creatinine before death) into a new predictive score that may provide support for pre-implantation decision-making and potentially improve transplant outcomes.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144567489","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":"Pulmonary-renal syndrome under Dostarlimab, a recent PD-1 inhibitor, in a patient with Endometrial Cancer.","authors":"Decaestecker Antoine, Balzer Geoffrey, Gnemmi Viviane, Pagniez Marie-Sophie, Ydee Amandine","doi":"10.1007/s40620-025-02358-7","DOIUrl":"https://doi.org/10.1007/s40620-025-02358-7","url":null,"abstract":"<p><p>Immune checkpoint inhibitors (ICIs) have been increasingly used in the treatment of various cancers since 2011. Dostarlimab is a recent anti-PD-1 agent, approved notably as a first-line treatment for endometrial cancer. The renal toxicity of anti-PD-1 agents is rare, with a prevalence ranging from 1 to 5% depending on the studies, mainly manifesting as acute interstitial nephritis and, much more rarely, as glomerulopathies. In this article, we report a case of pulmonary-renal syndrome occurring under dostarlimab in a 77-year-old woman treated for metastatic endometrial cancer. After three treatment cycles, she developed a nephrotic syndrome with KDIGO stage 3 acute kidney injury (AKI). Immunological testing revealed positive anti-nuclear antibodies at a titer of 1:80 and positive MPO-ANCA at 5.8 U/mL. Kidney biopsy revealed necrotizing crescentic glomerulonephritis with 75% recent lesions. Immunofluorescence revealed IgG and C3 deposits. This condition was attributed to dostarlimab, which was discontinued, and IV corticosteroid therapy was initiated. After the third bolus, the patient experienced massive intra-alveolar hemorrhage, leading to a diagnosis of pulmonary-renal syndrome. She was treated with IV cyclophosphamide (500 mg). The evolution was satisfactory on the pulmonary level but without renal improvement, leading to discussion about kidney replacement therapy. To our knowledge, this is the first reported case of pulmonary-renal syndrome associated with dostarlimab, and we found it noteworthy to report, particularly given the favorable pulmonary evolution under cyclophosphamide IV.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144567490","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":"Ketogenic diet as rescue therapy to access transplantation in obese and advanced stage IV-CKD patients: a case report and literature review.","authors":"Sara Caissutti, Gisella Vischini, Maria Letizia Petroni, Sofia Collini, Anna Laura Croci Chiocchini, Gaetano La Manna","doi":"10.1007/s40620-025-02335-0","DOIUrl":"https://doi.org/10.1007/s40620-025-02335-0","url":null,"abstract":"<p><p>Kidney transplantation represents the best therapy for kidney failure, but obesity can limit access to this therapeutic option. Therapeutic strategies, such as diet or bariatric surgery, should be considered for obese patients with kidney failure. Unfortunately, there are no guidelines available to manage obesity in Chronic Kidney Disease (CKD). Reducing energy intake is necessary to manage obesity. This usually entails a protein intake of at least 1 g/kg of body weight, contrary to what is recommended by CKD guidelines. To date, bariatric surgery is the best strategy for weight loss, but is not indicated in some conditions, such as in patients with major mood disorders. We present the case of a woman with CKD stage G4A2, metabolic syndrome, and anxiety-depressive disorder. She was not eligible for transplantation because of morbid obesity, or for bariatric surgery due to her major mood disorder. An individualised ketogenic diet was prescribed to give her a chance to access the kidney transplantation waitlist. During the dietary treatment, there was no worsening of kidney function or any adverse effects, and the patient lost 11.7 kg. In selected cases, ketogenic diet could represent a rescue therapy for obese CKD patients allowing them access to the kidney transplantation program.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144560423","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":"Impact of sodium bicarbonate locking solution on catheter-related blood stream infection in hemodialysis patients: comparative effectiveness of three locks.","authors":"Tamar Kasradze, Tamar Didbaridze, Irma Tchokhonelidze","doi":"10.1007/s40620-025-02315-4","DOIUrl":"10.1007/s40620-025-02315-4","url":null,"abstract":"<p><strong>Background: </strong>The primary challenge for hemodialysis (HD) patients using permanent tunneled cuffed catheters is to prevent catheter-related bloodstream infections. This study assessed the effectiveness of sodium bicarbonate in preventing catheter-related bloodstream infections and compared its efficacy to antibiotic-containing locks.</p><p><strong>Design, materials, and methods: </strong>We conducted a prospective single-center, open-label, cohort study over 30 months with the aim to compare three cohorts: cohort I employed 8.4% sodium bicarbonate solution lock, cohort II employed 0.5 mg/ml gentamicin/acid citrate dextrose solution lock, and cohort III employed unfractionated conventional heparin 5000 U/ml solution lock. The primary endpoint was the first episode of catheter-related bloodstream infection, while the secondary endpoint was catheter loss due to catheter-related bloodstream infection.</p><p><strong>Results: </strong>This study involved 204 HD patients with permanent tunneled cuffed central venous catheters (CVCs). A total of 58 cases of catheter-related bloodstream infection were documented. In the sodium bicarbonate-lock cohort, infection occurred in 17.24%, in the gentamicin/acid citrate-lock cohort in 36.21%, and in the heparin-lock cohort in 46.55%. Patients with sodium bicarbonate and gentamicin/acid citrate locks exhibited a statistically significant lower risk of developing infections (0.4/1000 catheter days and 0.7/1000 catheter days, respectively) compared to patients with heparin-lock (1.4/1000 catheter days). Patients with heparin lock faced a higher risk of losing a catheter due to infection compared to those with gentamicin/acid citrate lock and sodium bicarbonate-lock (Hazard Ratio (HR) = 1.26, 95% Confidence Interval (CI) [1.09-1.46], P = 0.001) and (HR = 1.10, 95% CI [1.01-1.19], P = 0.024).</p><p><strong>Conclusion: </strong>The sodium bicarbonate locking solution demonstrated an infection-free catheter survival comparable to that of the gentamicin citrate solution and significantly decreased catheter-related bloodstream infection compared to heparin locks.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144540566","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":"Polycystic pancake kidney.","authors":"Riccio Eleonora, Bracale Umberto, Ivana Capuano, Pisani Antonio","doi":"10.1007/s40620-025-02326-1","DOIUrl":"https://doi.org/10.1007/s40620-025-02326-1","url":null,"abstract":"","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144528418","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":"Crescentic glomerulonephritis associated with myelodysplatic syndrome and visceral leishmaniasis.","authors":"Antoine Decaestecker, Adrien Daniel, Zoé Malbranque, Nicolas Wayolle, Amandine Ydee","doi":"10.1007/s40620-025-02329-y","DOIUrl":"10.1007/s40620-025-02329-y","url":null,"abstract":"<p><p>Membranoproliferative glomerulonephritis (MPGN) can be secondary to infections, autoimmune diseases, or hematological disorders. MPGN is uncommon and has a poor renal prognosis. We report the case of a patient with a myelodysplastic syndrome, presenting with autoimmune inflammatory manifestations and rapidly progressing kidney failure. The kidney biopsy revealed MPGN with extracapillary proliferation. The patient's condition rapidly improved under corticosteroid therapy, with normalization of the kidney function. However, during corticosteroid treatment, the patient developed severe visceral leishmaniasis with cutaneous, splenic, and osteo-medullary involvement. Upon review, we found that parasites were already present in minimal quantities in the bone marrow biopsy performed three months prior to corticosteroid treatment. We cannot conclusively determine whether the kidney lesions are linked to the autoimmune inflammatory manifestations of the myelodysplastic syndrome or to leishmaniasis. This case may be noteworthy due to the combination of two diseases that could be responsible for the histological lesions, and emphasizes the importance of a thorough evaluation in the case of MPGN.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144333324","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":"Performance of the Kidney Failure Risk Equation according to diabetic status in the CKD-CAREMEAU cohort of patients with chronic kidney disease.","authors":"Julien Prouvot, Pascal Reboul, Pedram Ahmadpoor, Edouard Clemmer, Marion Gerbal, Florian Garo, Sylvain Cariou, Emilie Pambrun, Ziyad Messikh, Olivier Moranne","doi":"10.1007/s40620-025-02286-6","DOIUrl":"https://doi.org/10.1007/s40620-025-02286-6","url":null,"abstract":"<p><strong>Background: </strong>The Kidney Failure Risk Equation (KFRE) estimates the risk of kidney replacement therapy (KRT) at 5 years. Patients with diabetes mellitus are at high risk of KRT and death, a competing event. This study assesses the performance of the 5-year KFRE in patients with diabetes mellitus, compared to non-diabetic patients and considering age, in a cohort of patients evaluated by a nephrologist at a tertiary care center.</p><p><strong>Methods: </strong>The CKD-CAREMEAU cohort included all patients who visited a nephrologist for chronic kidney disease (CKD) before KRT between 2008 and 2017. The 5-year KFRE was calculated for each patient, based on their baseline characteristics. The study evaluated performance regarding discrimination and calibration, taking the patient's diabetic status and age into account.</p><p><strong>Results: </strong>The study included 2935 patients with a median age of 73 [65-80] years, 1800 of whom were men (61%), and 1249 (43%) who were affected by diabetes. The incidence of KRT was higher in diabetic patients (167 (13%)) than in non-diabetic patients (184 (11%))(p = 0.04). Additionally, the death rate within five years after inclusion was not statistically higher for diabetic patients (351 (28%)) than non-diabetic patients (443 (26%))(p = 0.3). Diabetic status affected neither discrimination nor calibration. However, the equation overestimated the risk for higher-risk patients, especially among the elderly.</p><p><strong>Conclusions: </strong>Our study found no difference in the 5-year KFRE performance between diabetic and non-diabetic patients. However, performance was worse for patients over 75 years old.</p>","PeriodicalId":16542,"journal":{"name":"Journal of Nephrology","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144326077","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}