Publications by authors named "Boffa Jean-Jacques"

BACKGROUND Focal segmental glomerulosclerosis (FSGS) very rarely occurs in patients with multiple myeloma. Much more common are renal impairments secondary to monoclonal light-chain tubulopathy, AL amyloidosis, light-chain deposition disease, and the so-called monoclonal gammopathy of renal significance. CASE REPORT We report the case of a 79-year-old myeloma patient without noticeable medical problems but with a long history of myeloma treatment beginning 13 years ago.

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  • AA amyloidosis (AAA) is a serious multisystem disease stemming from chronic inflammation, commonly affecting the kidneys and posing risks during pregnancy.
  • A study reviewed cases of AAA in pregnant women, identifying three new patients and highlighting that familial Mediterranean fever was the leading cause of AAA in this group.
  • The research found that pregnancies in AAA patients often led to complications like growth retardation and preterm birth, indicating the need for heightened medical monitoring during pregnancy.
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Background And Study Aims: Chronic kidney disease (CKD) is a well-known risk factor of gastrointestinal angiectasia (GIA). The aim was to compare this population with CDK patients without GIA.

Methods: Patients followed in the Nephrology Department of Tenon Hospital for which an endoscopy was performed between 2012 and 2022 were identified.

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Introduction: The objective of this study was to describe kidney involvement in patients with myelodysplastic syndromes (MDS), their treatments, and outcomes.

Methods: We conducted a multicenter retrospective study in seven centers, identifying MDS patients with acute kidney injury (AKI), chronic kidney disease (CKD), and urine abnormalities.

Results: Fifteen patients developed a kidney disease 3 months after MDS diagnosis.

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Key Points: We evidenced terminal pathway activation (C5b-9 deposits) in most of the glomeruli on kidney biopsy of C3 glomerulopathy. The amount of C5b-9 deposits correlated with disease prognosis in C3 glomerulopathy. Increased terminal pathway activation was found predominantly in a subgroup exhibiting an immuno-fibroblastic signature.

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Kidney Biopsy (KB) is a crucial diagnostic tool in the field of renal diseases and is routinely performed in nephrology departments. A previous survey conducted by the Société Francophone de Néphrologie Dialyse Transplantation (SFNDT) revealed significant disparities in clinical practices, sometimes conflicting with the existing literature and recently published recommendations. In response, the SFNDT wished to promote the development of best practice guidelines, under the auspices of the French National Authority for Health (HAS), to establish a standardized framework for performing kidney biopsies in France.

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  • The study focuses on the characteristics and outcomes of 31 patients with ANCA vasculitis who experienced acute kidney injury, revealing that renal failure is common in these cases and linked to higher mortality rates, particularly with chronic kidney disease and lung involvement.
  • Among the patients studied, 32.2% required haemodialysis, but those on dialysis had a higher chance of progressing to end-stage renal failure and overall mortality rates were similar when compared to those not on dialysis.
  • Initial treatments included cyclophosphamide for the majority and rituximab for a smaller portion, with a low relapse rate of 6.5% and significant complications affecting more than half of the patients, resulting
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  • Atypical anti-glomerular basement membrane (GBM) nephritis is a rare kidney condition that presents differently than typical cases, showing bright linear immunoglobulin staining without the usual serum anti-GBM antibodies.
  • In a study involving patients diagnosed from 2003 to 2022, 25 out of 38 potential cases were confirmed, with a majority exhibiting symptoms like hematuria and varying forms of glomerulonephritis.
  • The findings suggest that atypical anti-GBM nephritis often progresses slower than typical cases, but further research is necessary to understand its complete nature and implications.
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  • The study looked at how two substances, copeptin and iFGF23, in patients with chronic kidney disease (CKD) might predict bad health outcomes.
  • It involved 329 CKD patients and found that higher levels of iFGF23 were linked to worse kidney health and more deaths, while copeptin did not show this link.
  • The researchers suggest that checking iFGF23 levels could help doctors better monitor CKD patients, but copeptin levels may not be as helpful.
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  • * In a study of 50 patients with severe renal impairment, only one death occurred within the first 100 days post-transplant, with most patients showing significant improvements in hematological responses.
  • * Overall, after two years, 84% of patients were alive, 70% were free from disease progression, and 59% saw an improvement in kidney function, indicating that the procedure is both safe and effective for this population.
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Especially exposed to frequent cardiovascular events and its related mortality, some cardiovascular therapies of the hemodialyzed population as well as coronary investigations remain controversial. We have collected data and discussed recent trials and guidelines dedicated to this patient subset.

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Background: IgG4-related kidney disease is a major manifestation of IgG4-related disease, a systemic fibroinflammatory disorder. However, the clinical and prognostic kidney-related factors in patients with IgG4-related kidney disease are insufficiently defined.

Methods: We conducted an observational cohort study using data from 35 sites in two European countries.

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Objective: Antiphospholipid syndrome (APS) is defined by the association of thromboembolic and/or obstetrical clinical manifestations and the presence of antiphospholipid antibodies. The objective of our study was to evaluate the impact of the triple-positive profile in a cohort of 204 APS patients.

Methods: We conducted a retrospective study, including patients with primary or secondary APS, meeting the Sydney criteria with at least one thrombotic and/or obstetrical complication.

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Introduction: Exome sequencing (ES) has widened the field of nephrogenomics in adult nephrology. In addition to reporting the diagnostic yield of ES in an adult cohort study, we investigated the clinical implications of molecular diagnosis and developed a clinical score to predict the probability of obtaining positive result.

Methods: From September 2018 we have used ES to prospectively perform a first-tier liberal exploration of adult nephropathies of unknown origin and/or when a genetic kidney disease was clinically suggested.

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Introduction: Antiphospholipid syndrome (APS) is an autoimmune disease characterised by thrombosis (arterial, venous or small vessel) or obstetrical events and persistent antiphospholipid antibodies (aPL), according to the Sydney classification criteria. Many studies have performed cluster analyses among patients with primary APS and associated autoimmune disease, but none has focused solely on primary APS. We aimed to perform a cluster analysis among patients with primary APS and asymptomatic aPL carriers without any autoimmune disease, to assess prognostic value.

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Focal segmental glomerulosclerosis (FSGS) is a major cause of end-stage renal disease and remains without specific treatment. To identify new events during FSGS progression, we used an experimental model of FSGS associated with nephroangiosclerosis in rats injected with L-NAME (N-nitro-L-arginine methyl ester). After transcriptomic analysis we focused our study on the role of Isthmin-1 (ISM1, an anti-angiogenic protein involved in endothelial cell apoptosis.

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 Nephrotic syndrome (NS) is associated with an increased risk of thromboembolic events (TEs), due to hemostatic derangements. The use of direct oral anticoagulants (DOACs) in the prevention of TE has not been studied intensively in patients suffering from NS.  The method included retrospective analysis of consecutive incident patients with NS due to glomerular disease, receiving apixaban for thromboprophylaxis.

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  • * A Delphi consensus method was used, where a committee of 17 experts drafted 52 statements on managing treatment initiation, adverse effects, special populations, patient communication, and adherence, with 49 statements reaching consensus through voting.
  • * The findings aim to improve side effect management of PARP inhibitors, enhance patient adherence, and maintain quality of life, marking the first Delphi consensus on this topic.
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Background: We determine the benefit of pulsed methylprednisolone for improving kidney function in patients with sarcoidosis tubulointerstitial nephritis.

Methods: We conducted a multicenter, prospective, randomized, open-label, controlled trial in patients with biopsy-proven acute tubulointerstitial nephritis caused by sarcoidosis at 21 sites in France. Patients were randomly assigned to receive a methylprednisolone pulse 15 mg/kg/day for 3 days, then oral prednisone (MP group) or oral prednisone 1 mg/kg/day alone (PRD group).

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Background: Antiphospholipid syndrome (APS) nephropathy (APSN) is a rare pattern with specific features resulting from microvascular lesions. The prognosis of APSN, outside of lupus nephritis, is unknown. The aim of this study was to describe the renal, vascular and overall outcomes of patients with APSN.

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