Optimizing ADAMTS13 prophylaxis to reduce relapse and organ failure in congenital thrombotic thrombocytopenic purpura.

Congenital thrombotic thrombocytopenic purpura (cTTP) is caused by a severe inherited ADAMTS13 deficiency. While acute episodes are life-threatening, long-term burden of ischemic complications and effectiveness of prophylactic strategies remain underexplored. We conducted a 25-year national, multicenter study of 88 cTTP patients enrolled in the French Thrombotic Microangiopathy (TMA) registry.

Hemodynamic Effect of Different Replacement Fluid Protocols During Therapeutic Apheresis Evaluated With CRIT-LINE.

In therapeutic apheresis (TA) procedures, it is essential to administrate human albumin solution (HAS) to prevent hypotensive episodes. The CRIT-LINE monitor is used in hemodialysis to estimate changes in relative blood volume (RBV) to prevent such hypotensive episodes. The aim of this study was to evaluate the change in RBV during therapeutic plasma exchange (TPE) and double filtration plasmapheresis (DFPP) according to different fluid replacement protocols in order to identify the best protocol.

Erratum to "Discordant Results Between Creatinine- and Cystatin C-based Equations for Estimating GFR" [ Volume 10, Issue 4, April 2025, Pages 1248-1259].

[This corrects the article DOI: 10.1016/j.ekir.

Anti-ADAMTS13 Antibodies Trajectory is Associated With ADAMTS13 Recovery in Immune-Mediated TTP.

Current triplet regimens associating therapeutic plasma exchange (TPE), immunosuppression with corticosteroids and rituximab, and caplacizumab have dramatically improved the outcome of immune-mediated thrombotic thrombocytopenic purpura (iTTP). However, nearly half of the patients require extended caplacizumab treatment (i.e.

Performance of the Kidney Failure Risk Equation according to diabetic status in the CKD-CAREMEAU cohort of patients with chronic kidney disease.

Background: 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.

Discordant Results Between Creatinine- and Cystatin C-based Equations for Estimating GFR.

Introduction: Discordant results between cystatin C and creatinine in estimating glomerular filtration rate (GFR) are an important medical issue. However, the equation that should be used when GFR estimates are discordant remains unclear.

Methods: This cross-sectional analysis included 15,485 participants with GFR measured by the clearance of an exogenous marker, serum creatinine, and cystatin C.

A systematic review on the determinants of long-term kidney sequelae after hypertensive diseases of pregnancy.

Introduction: Preeclampsia is a pregnancy syndrome defined by high blood pressure (≥140/90 mmHg) and at least one other associated complication, including proteinuria, maternal organ dysfunction, or uteroplacental dysfunction, after 20 weeks of gestation. The long-term effects of this disease on the kidneys are still not fully understood. This systematic review aimed to evaluate the risk of chronic kidney disease after preeclampsia and the determinants of these sequelae.

Phenotypic Heterogeneity of ADTKD-MUC1 Diagnosed Using VNtyper, a Novel Genetic Technique.

Rationale & Objective: Molecular diagnosis of autosomal dominant tubulointerstitial kidney disease (ADTKD) due to variants in the MUC1 gene has long been challenging because variants lie in a large variable number of tandem repeat (VNTR) region, making identification impossible using standard short-read techniques. Previously, we addressed this diagnostic limitation by developing a computational pipeline named VNtyper for easier reliable detection of MUC1 VNTR pathogenic variants from short-read sequences. This led to unexpected diagnoses of ADTKD-MUC1 among patients with kidney disease referred for genetic testing, which we report here.

Obstacles and Opportunities for Albuminuria Testing On the Basis of the Perspective of Primary Care: A Qualitative Study.

Key Points: Pay-for-performance indicators and lack of knowledge about new drugs limit general practitioners' ability to identify target populations and perform urinary protein assays. Choosing between several possible assays is associated with confusion and exposes general practitioners to the risk of inappropriate referrals to nephrologists. Revising pay-for-performance indicators, drafting multidisciplinary guidelines, raising multiprofessional collaboration, and patient awareness should be considered.

Older people predialysis care pathways and early morbidity-mortality upon start of dialysis.

Background: The ageing of the population with advanced chronic kidney disease (CKD) increases the complexity of care pathways. Our aim was to identify subgroups of older people according to predialysis care pathways and describe their association with early morbidity-mortality after transition to dialysis.

Methods: This study included 22 128 incident dialysis patients aged ≥75 years during 2009-2017 from the French nationwide registry linked to the National Health Data System.

Performance of an interstitial glucose monitoring device in patients with type 1 diabetes during haemodialysis.

Background: The use of interstitial glucose monitoring devices such as flash glucose monitoring has been shown to be beneficial in patients with type 1 diabetes mellitus (T1DM). However, these devices have been little studied in patients with diabetes treated by chronic haemodialysis (HD).

Methods: The goal of this prospective, observational, multicentric study was to evaluate the analytical performance of the FreeStyle Libre 2 (FSL2) sensor in T1DM patients during HD sessions.

Pregnancy Among Women Receiving Chronic Dialysis in France (2006-2020).

Introduction: In women receiving chronic dialysis, fertility is impaired. The objectives of this study were to estimate the incidence rate of pregnancies among women of childbearing age (15-50 years) receiving chronic dialysis from 2006 to 2020 in France, to describe the pregnancy outcomes and renal management during pregnancy.

Methods: This national observational, retrospective study was based on data from the French REIN registry matched with the National Health Data System.

Avacopan for anti-neutrophil cytoplasm antibodies-associated vasculitis: a multicentre real-world study.

Objectives: Avacopan, a selective C5aR1 inhibitor, recently emerged as a glucocorticoid (GCs) sparing agent in anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitis (AAV). We aim to evaluate the tolerance and efficacy of avacopan given outside randomized clinical trials or with severe kidney involvement.

Methods: In this multicentre retrospective study, we reviewed the clinical charts of patients with AAV and contraindication to high dose of GCs who received avacopan 30 mg b.

A randomized crossover trial of regional anticoagulation modalities for intermittent haemodialysis.

Background: The optimal regional anticoagulation (RA) of dialysis filters in patients at risk of bleeding remains elusive. Inducing hypocalcaemia within the filter by using a calcium-free dialysate has emerged as an easy-to-use heparin-free RA, including in critically ill patients, but comparative studies are lacking.

Methods: We conducted a multicentre, randomized, crossover trial to compare the efficacy and tolerance of two RAs [heparin-coated membrane (HCM) and calcium-free dialysate (CFD) with calcium reinjection according to ionic dialysance] in patients requiring haemodialysis and at risk of bleeding.

Immunogenicity of SARS-CoV-2 vaccines in patients treated with chronic double filtration plasmapheresis.

Background: The impact of chronic therapeutic plasmapheresis on humoral response following COVID-19 vaccination is poorly documented, especially among patients treated with double filtration plasmapheresis (DFPP).

Objectives: This retrospective single-center study evaluated the humoral response after SARS-CoV-2 vaccination and studied anti-SPIKE seropositivity and antibody dynamics in patients with chronic DFPP at our institution.

Method: All patients undergoing chronic DFPP at a tertiary center in France from December 2020 to November 2022 were included.

Anti-GBM antibody in a patient with diabetic nephropathy; all that glitters is not gold.

We present the case of a 58-year-old male diabetic patient admitted to our department for a slight decrease in kidney function, with nephrotic range proteinuria, hematuria (16,000/ml) and positive anti-glomerular basement membrane antibodies. Kidney biopsy revealed diabetic nephropathy with no evidence of crescent formation or linear immunoglobulin deposits along the basement membrane. We discuss the various clinical settings involving positive anti-glomerular basement membrane in the absence of crescentic glomerulonephritis.

Successful pregnancy after 10 consecutive failures in a liver transplant patient with advanced kidney failure.

We report a successful, albeit complicated pregnancy with a live-born healthy baby at 28 weeks' gestation, after 10 pregnancy failures, in a 39-year-old patient with a history of liver transplantation and chronic kidney disease with hypertension and proteinuria. Multidisciplinary management (obstetrician, nephrologist and hepatology transplant specialist) allowed close monitoring, adaptation of immunosuppressive treatments and strict control of fetal growth. The onset of preeclampsia at 28 weeks' gestation led to a cesarean section, resulting in the birth of a healthy 830 g boy, with subsequent normal development.