Publications by authors named "Maillard H"

Background: Patients with chronic illnesses, especially rare autoimmune and/or systemic diseases associated with significant diagnostic uncertainty, have a representation of their illness and a sometimes prolonged hospitalization experience that can be traumatic and anxiety-provoking.

Objective: The aim of this study was to evaluate the impact of a non-medicinal medical hypnosis intervention in reducing the stress state and improving the experience of patients hospitalized in an internal medicine department.

Methods: We conducted a prospective study of 24 patients hospitalized in the Internal Medicine Department of Lille University Hospital in 2023.

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  • * A study analyzed immune cells from 41 male Fabry patients and 20 controls, revealing that FD patients show higher expression of specific markers (CD27 and CD28) in certain T cells, which correlates with the levels of harmful lipids (lysoGb3 and Gb3) in the blood.
  • * Treatment with agalsidase beta was found to increase Natural Killer cell proportions, suggesting potential new biomarker correlations that could enhance understanding and management of Fabry disease.
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Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is a dominant inherited vascular disorder. The clinical diagnosis is based on the Curaçao criteria and pathogenic variants in the ENG and ACVRL1 genes are responsible for most cases of HHT. Four families with a negative targeted gene panel and selected by a multidisciplinary team were selected and whole-genome sequencing was performed according to the recommendations of the French National Plan for Genomic Medicine.

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Background: Nailfold capillaroscopy is recommended to diagnose primary or secondary Raynaud's phenomenon (RP). Capillaroscopy is normal in primary RP, which is the most frequent. Screening for RP capillary anomalies with nailfold dermoscopy has been promising.

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Background: Primary plantar hyperhidrosis has a severe impact on quality of life and conservative treatments are only effective in the short term. Thoracic sympathectomy has proven its effectiveness in the treatment of palmar hyperhidrosis, but lumbar sympathectomy for plantar hyperhidrosis remains poorly described.

Objectives: To report the satisfaction of patients with primary plantar hyperhidrosis treated with mechanical lumbar sympathectomy as well as their postoperative complications.

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  • Calcific uremic arteriolopathy (CUA) is a serious condition mainly affecting patients with end-stage renal disease (ESRD) on dialysis, marked by calcium deposits in the skin.
  • A study compared the elastic fiber structure and skin calcifications in 14 ESRD patients with CUA to 12 without using advanced imaging techniques.
  • The findings revealed significant differences in elastic fiber fragmentation and the presence of massive circumferential vascular calcifications in CUA patients, suggesting these features might indicate a more specific link to CUA compared to general calcifications seen in other ESRD patients.
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Introduction: Rare disease referral centres are entrusted with missions of clinical expertise and research, two activities that have to contend with numerous obstacles. Providing specialist opinions is time-consuming, uncompensated and limited by difficulties in exchanging medical data. Clinical research is constrained by the need for frequent research protocol visits.

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  • Bevacizumab, a monoclonal antibody, is used to treat hereditary hemorrhagic telangiectasia (HHT), but this is the first randomized trial investigating its effectiveness.
  • In a double-blind study involving 24 patients, those treated with bevacizumab showed a greater decrease in blood transfusions compared to the placebo group, with significant improvements in hemoglobin levels over six months.
  • Despite some positive results, the trial was underpowered, meaning more research is needed to confirm that bevacizumab helps reduce the need for blood transfusions in HHT patients, especially in those exposed to higher doses.
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Pressure ulcers (PUs) are one of the most important care-related ailments, making their prevention a pressing concern for hospital structures. Epidemiology of PU is interesting to adapt human resources and observe the impact of targeted public health measures. Very few data are presented in the French literature.

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Background: It is unclear whether hereditary hemorrhagic telangiectasia (HHT) patients can tolerate antithrombotic therapies (AT) including antiplatelet (AP) and/or anticoagulant (AC) agents.

Objectives: Primary endpoint was tolerance to AT in HHT. Secondary endpoints were to identify factors associated with major bleeding events (MBE) and premature discontinuation of AT.

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Objectives: Sjögren's syndrome (SS) is an autoimmune disease with an impact on quality of life (QoL). The aim of patient education (PE) is to improve patients' QoL. The main objective of this study was to describe the medico-psycho-social characteristics defining the six spheres of an allosteric educational model in order to characterise clusters of patients with SS and intentionality for patients to participate in a programme of patient education.

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Background: In areas of low physician density, especially as regards dermatologists in France, there is an increasing interest in tele-expertise. This is particularly the case in the Sarthe department, where the number of physicians continues to decline and access to care was further limited by the COVID 19 epidemic.

Study Design: We retrospectively collected data from tele-expertise requests submitted to Le Mans General Hospital by general practitioners via a dedicated platform between May 6, 2019, and April 9, 2021.

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  • Lentigo maligna (LM) is a skin condition that can develop into melanoma; surgery is typically the preferred treatment, but there is uncertainty about the best excision margins.
  • A clinical study tested the effectiveness of imiquimod, an immunomodulator, versus placebo over a month, followed by surgical removal of LM.
  • Results showed that while imiquimod significantly reduced the size of LM compared to placebo, there was no significant difference in successful surgical removal between the two groups, indicating its potential as an effective treatment option.
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Introduction: Coronaritis is a rare but serious complication of giant-cell arteritis (GCA), with an estimated prevalence of less than 1%, however difficult to establish, and of early onset.

Methods: We describe 2 cases of GCA presenting with coronaritis and present a review of the literature on this complication.

Results: The first patient presented with stable angina on common trunk coronaritis with ostial stenosis.

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Background: loss of function is associated with type 2 capillary malformation-arteriovenous malformation syndrome, an autosomal dominant vascular disorder. The phenotype partially overlaps with hereditary haemorrhagic telangiectasia (HHT) due to epistaxis, telangiectases and cerebral arteriovenous malformations, but a similar liver involvement has never been described.

Methods: Members of the French HHT network reported their cases of mutation identified after an initial suspicion of HHT.

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Importance: Catastrophic antiphospholipid syndrome (CAPS) is a severe, rare complication of antiphospholipid syndrome (APS), but cutaneous involvement has not yet been adequately described.

Objective: To describe cutaneous involvement during CAPS, its clinical and pathological features, and outcomes.

Design, Setting, And Participants: This cohort study was a retrospective analysis of patients included in the French multicenter APS/systemic lupus erythematosus register (ClinicalTrials.

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  • The study aimed to identify distinct subgroups among APS patients using an unsupervised hierarchical cluster analysis to understand the diverse phenotypes of the disease.
  • Researchers analyzed data from 509 APS patients who met specific criteria, identifying four patient clusters based on clinical and laboratory features: venous thromboembolism, arterial events with health issues, younger patients with additional autoimmune diseases, and severe cases with catastrophic symptoms.
  • The findings suggest that APS is a heterogeneous disease with different underlying mechanisms, indicating the need for tailored treatments for each patient subgroup.
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Background: Despite the poor prognosis associated with melanoma brain metastases (BM), data concerning these patients and their inclusion in clinical trials remains scarce. We report here the efficacy results of a subgroup analysis in patients with BRAFV600-mutant melanoma and BM treated with BRAF and MEK inhibitors dabrafenib (D) and trametinib (T).

Patients And Methods: This phase IIIb single-arm, open-label, multicenter, French study included patients with unresectable stage IIIc or IV BRAFV600-mutant melanoma with or without BM.

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The committee involved in transitional care within the French network on rare autoimmune and auto-inflammatory diseases has developed tools and published recommendations for the care during the transition of adolescents and young adults suffering from these chronic diseases which often demonstrate flares. As the challenge is the compliance to the continuation of care in the adult world, the therapeutic alliance between the young patient and his/her pediatrician and then his/her adult doctor is particularly important. The working group thus carried out a survey on how doctors and young patients perceive their relationship during the period of transition in medical supervision.

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(1) Background: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease with a high mortality and morbidity rate. Identification of biomarkers that can predict the evolution of SSc is a key factor in the management of patients. The aim of this study was to assess the association of routine laboratory parameters, widely used in practice and easily available, with the severity and progression of SSc.

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Objective: To determine whether a single session of botulinum toxin type A (BTA) injections into both hands more effectively decreases the frequency of systemic sclerosis-associated Raynaud's phenomenon (SSc-RP) episodes than placebo.

Methods: This multicenter, randomized, double-blind, placebo-controlled, parallel-group phase III trial in patients with SSc-RP assessed the effect of 50-unit BTA or placebo injections into the palms of both hands around each neurovascular bundle during 1 session in winter. The primary end point was the between-group difference in the median change in the number of RP episodes from baseline (day 0) to 4 weeks postinjection.

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Background: First-degree relatives (FDRs, defined as parents, children, and siblings) of melanoma patients are at a two-to-fivefold increased risk of developing melanoma themselves. FDRs are advised to perform self-skin examination (SSE) and annual medical total cutaneous examination (TCE) performed either by a dermatologist or a general practitioner, and to change their sun-related behavior. This advice is given orally to melanoma patients who are asked to relay the information to their FDRs.

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