Publications by authors named "Jean Francois Alexandra"

Article Synopsis
  • Fabry disease results from a deficiency in the enzyme alpha-galactosidase A, leading to a buildup of glycosphingolipids and causing various health issues, including neurologic symptoms like neuropathic pain and strokes, with aseptic meningitis being a potential complication.
  • A study reviewed patients from 1995 to 2023, finding that 3.7% experienced aseptic meningitis, with additional data from published cases leading to a total of 29 patients analyzed, predominantly young adults.
  • The findings indicate that many of these patients were unaware they had Fabry disease before their meningitis episode, and the underlying causes of the inflammation in these cases are still not fully understood.
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Introduction: Steroids and anti-IL6 biotherapy are highly effective in obtaining remission in patients with giant cell arteritis (GCA) but the risk of relapses remains high. We aimed to identify predictors of relapse in GCA.

Methods: All consecutive patients admitted with a new diagnosis of GCA - according to the 2022 American College of Rheumatology/EULAR (ACR/EULAR) classification criteria - between May 2011 and May 2022 were eligible for this study.

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Introduction: Giant cell arteritis (GCA) is complicated in 10 to 20% of cases by permanent visual ischemia (PVI). International guidelines advocate the use of intravenous pulse of methylprednisolone from 250 to 1000mg per day, for three days, followed by oral prednisone at 1mg/kg per day. The aim of this study is to assess whether this strategy significantly reduces the risk of early PVI of the second eye, compared with direct prednisone at 1mg/kg per day.

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Background: Prognosis data on giant-cell arteritis (GCA)-associated aortitis are scarce and heterogeneous. The aim of this study was to compare the relapses of patients with GCA-associated aortitis according to the presence of aortitis on CT-angiography (CTA) and/or on FDG-PET/CT.

Methods: This multicenter study included GCA patients with aortitis at diagnosis; each case underwent both CTA and FDG-PET/CT at diagnosis.

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Objectives: Myelodysplastic syndromes (MDS) and chronic myelomonocytic leukaemia (CMML) are associated with systemic inflammatory and autoimmune diseases (SIADs) in 10-30% of cases. The aims of this study were (i) to evaluate the prevalence of venous thromboembolism VTE in patients presenting with both MDS/CMML and SIADs, (ii) to describe risk factors associated with thrombosis, and (iii) to analyse the impact of VTE on overall survival and transformation to acute myeloid leukaemia in comparison to patients with MDS/CMML-associated SIADs without VTE.

Methods: This retrospective multicentre case-control study was conducted among patients with MDS/CMML and dysimmune disorders and featured in the French retrospective database of the French Network of Dysimmune Disorders Associated with Hemopathies (MINHEMON), diagnosed with MDS/CMML and dysimmune disorders.

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Article Synopsis
  • * Out of 218 patients with AM, 15 (6.9%) were diagnosed with various AIIDs, including systemic lupus erythematosus and adult onset Still's disease, with some presenting severe heart dysfunction but ultimately responding well to treatment.
  • * Key indicators for AIID in AM cases included pericardial effusion, absence of chest pain, and elevated CRP levels at the time of admission, suggesting a better prognosis with appropriate treatment.
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Background: The aim of this study was to evaluate the prognostic value of persistent retroperitoneal fibrosis FDG uptake using FDG/PET CT in patients with idiopathic retroperitoneal fibrosis (IRF).

Methods: In this monocentric retrospective cohort study, all patients admitted for IRF from January 2009 to December 2017 underwent a FDG/PET CT at diagnosis and during follow up. Metabolic activity of IRF was assessed by retroperitoneal fibrosis FDG uptake measured as maximal standardized uptake value (SUVmax).

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Article Synopsis
  • The study aimed to assess whether increased FDG pericardial uptake observed through FDG-PET/CT can predict relapse in patients with acute pericarditis and pericardial effusion.
  • Conducted retrospectively, it analyzed patients treated between 2009 and 2016, measuring pericardial FDG uptake and tracking relapses during follow-up.
  • Results indicated that patients showing higher FDG uptake at diagnosis had a significantly greater risk of experiencing a relapse of pericarditis later on.*
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Aims: Acute pericarditis may be the heralding manifestation of various systemic inflammatory diseases (SIDs). The aim of this study was to identify clinical indicators for SIDs in patients admitted for acute pericarditis with pericardial effusion.

Methods: All consecutive adult patients hospitalized in a Department of Internal Medicine over a 10-year period for acute pericarditis with pericardial effusion were retrospectively reviewed.

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Objectives: To analyze the prevalence, characteristics and outcome of cystic lung disease associated with Sjögren's syndrome (SS).

Methods: From June 2010 to February 2015, 90 consecutive SS patients [60.1±14.

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The use of 18F-fluoro-deoxyglucose positron emission tomography scan (FDG-PET) and computed tomography angiography (CTA) to improve accuracy of diagnosis of giant cell arteritis (GCA) is a very important clinical need. We aimed to compare the diagnostic performance of FDG-PET and CTA in patients with GCA.FDG-PET and CTA were acquired in all consecutive patients suspected for GCA.

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Patients with a cancer at time of first venous thromboembolism (VTE) have not been thoroughly analyzed. Our study aimed to (1) determine the frequency of cancer diagnosed in patients hospitalized for a first VTE episode, (2) investigate the characteristics of VTE and cancer in such patients. All consecutive adults patients hospitalized over a 6-years period for a first VTE episode in a tertiary care hospital were considered.

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Background: Medication reconciliation is a powerful process to correct medication errors (ME) resulting from miscommunicated information at transitions of care. This study aims to develop and evaluate a scoring method for assessing the severity of potential harm of ME intercepted by medication reconciliation at hospital admission in elderly.

Methods: The development of the scoring method was based on a literature search and the creation of a list of high-risk drugs used in outpatient care.

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Background: Medication reconciliation has proved its effectiveness at improving drug-prescription safety. This study was undertaken to assess the impact of an intervention aimed at decreasing the discrepancies between a patient's usual treatment(s) and medications prescribed at admission.

Methods: Our study was conducted from November 2010 to May 2011.

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Objectives: Familial Mediterranean fever (FMF) is an autosomal-recessive autoinflammatory disorder common in Mediterranean populations. FMF is associated with mutations of the MEFV gene, which encodes pyrin. Functional studies suggest that pyrin is implicated in the maturation and secretion of IL-1.

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Susac syndrome is characterized by the clinical triad of encephalopathy, hearing loss, and retinal artery branch occlusions, mostly in young women. To our knowledge, long-term outcome and impact of pregnancy have not been specifically addressed. We report a series of 9 patients (7 female, 2 male) followed at the same institution, with special emphasis on clinical outcome including pregnancy and long-term sequelae.

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