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1.
Animal ; 18(6): 101174, 2024 Apr 25.
Artigo em Inglês | MEDLINE | ID: mdl-38761441

RESUMO

The dynamics of cattle body chemical composition during growth and fattening periods determine animal performance and beef carcass quality. The aim of this study was to estimate the empty body (EB) and carcass chemical composition of growing beef-on-dairy crossbred bulls (Brown Swiss breed as dam with Angus, Limousin or Simmental as sire) using three-dimensional (3D) imaging. The 3D images of the cattle's external body shape were recorded in vivo on 48 bulls along growth trajectory (75-520 kg BW and 34-306 kg hot carcass weight [HCW]; set 1) and on 70 bulls at target market slaughter weight, including 18 animals from set 1 (average 517 ± 10 kg BW and 289 ± 10 kg HCW; set 2). The linear, circumference, curve, surface and volume measurements on the 3D body shape were determined. Those predictive variables were used in partial least square regressions, together with the effect of the sire breed whenever significant (P < 0.05), with leave-one-out cross-validation to estimate water, lipid, protein, mineral and energy mass or proportions in the EB and carcass. Mass and proportions were determined directly from postmortem grinding and chemical analyses (set 1) or indirectly using the 11th rib dissection method (set 2). In set 1, bulls' BW and HCW were estimated via 3D imaging, with root mean square error of prediction (RMSEP) of 12 kg and 6 kg, respectively. The EB and carcass chemical component proportions were estimated with RMSEP from 0.2% for EB minerals (observed mean 3.7 ± 0.2%) to 1.8% for EB lipid (11.6 ± 4.2%), close to the RMSEP found for the carcass. In set 2, the RMSEP for estimation via 3D imaging was 9 kg for BW and 6 kg for HCW. The EB energy and protein proportions were estimated, with RMSEP of 0.5 MJ/kg fresh matter (10.1 ± 0.8 MJ/DM) and 0.2% (18.7 ± 0.7%), respectively. Overall, the estimations of chemical component proportions from 3D imaging were slightly less precise for both sets than the mass estimations. The morphological traits from the 3D images appeared to be precise estimators of BW, HCW as well as EB and carcass chemical component masses and proportions.

2.
Rev Med Interne ; 44(12): 632-640, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37923588

RESUMO

INTRODUCTION: Several studies suggest the relevance of healthcare simulation to prepare future doctors to deliver bad news. A such, we designed a role-play workshop to train first-year residents enrolled in Lille University School of Medicine to break bad news. The objective of this work is to report on our experience of this training and to assess its educational value through its capacity to satisfy residents' expectations, to induce a feeling of ease towards bad news disclosure, and to change trainees' preconceptions regarding these situations. METHODS: The training consisted of a 45-minute heuristic reflective activity, aimed at identifying residents' preconceptions regarding bad news disclosure, followed by 4 30-min role-plays in which they played the parts of the physician, the patient and/or their relatives. Trainees were asked to answer 2 questionnaires (pre- and post-training), exploring previous experiences, preconceived ideas regarding bad news disclosure and workshop satisfaction. RESULTS: Almost all residents felt very satisfied with the workshop, which they regarded as formative (91%) and not too stressful (89%). The majority felt "more capable" (53% vs. 83%) and "more comfortable" (27% vs. 62%) to deliver bad news, especially regarding "finding the right words" (12% vs. 22%). Trainees tended to overestimate their skills before the workshop and lowered their assessment of their performance after attending the training, especially when they played the role of a patient in the simulation. CONCLUSION: Healthcare role-play seems an interesting technique for training to breaking bad news. Placing residents in the role of patients or relatives is an active approach that encourages reflexivity.


Assuntos
Internato e Residência , Relações Médico-Paciente , Humanos , Revelação da Verdade , Universidades , Escolaridade
3.
Rev Med Interne ; 44(8): 394-401, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37088663

RESUMO

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. Corticosteroid combined with tocilizumab from the outset resulted in improvement. Angioplasty was performed at 6months with good outcome. The second patient presented with asymptomatic tritruncular ostial coronaritis. Corticosteroid allowed clinic-biological improvement of GCA. Two years later, he presented relapse with an acute coronary syndrome, with favorable evolution after angioplasty, increase of corticosteroids and addition of tocilizumab. CONCLUSION: Patients presented were successfully treated with corticosteroids combined with tocilizumab and angioplasty of their coronary stenoses. Efficacy of tocilizumab in GCA has not been evaluated especially on coronaritis due to the rarity of this complication. Our experience and the cases reported in the literature suggest good results of angioplasty in this indication. Studies with long-term follow-up will be necessary to evaluate the risk of restenosis.


Assuntos
Arterite de Células Gigantes , Humanos , Masculino , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/terapia , Angioplastia , Corticosteroides/uso terapêutico
4.
Rev Med Interne ; 42(9): 608-615, 2021 Sep.
Artigo em Francês | MEDLINE | ID: mdl-34158189

RESUMO

INTRODUCTION: Hereditary angioedema (HAE) is characterized by recurrent attacks of swelling of various locations and severity. An impaired quality of life of patients with HAE has been reported by several studies. We aimed at examining the overall impact of the disease in patients followed for type I HAE, particularly its impact on daily life activities, emotions and quality of life. METHODS: A questionnaire was distributed to patients consulting for type I HAE, collecting demographics, disease characteristics, impact on professional life, Hospital Anxiety and Depression score (HAD), SF-36 score and the McMaster Toronto Arthritis Patient Preference Disability Questionnaire (MACTAR). RESULTS: The 33 patients included reported an average of 5.17 attacks over the last year. Stress was the main trigger A long-term treatment was reported by 58% of patients, 72% received specific treatment in the event of a serious attack. Sick days were reported by 33% of patients during their studies, and by 34% during work. One patient suffered from depressive symptoms and ten from anxious symptoms, according to the HAD score. The areas most impacted on the SF-36 score were general health and vitality. The mean score for MACTAR was low. CONCLUSION: HAE still has a significant impact on the daily and emotional lives of patients, despite the availability of prophylactic and crisis treatments.


Assuntos
Angioedemas Hereditários , Qualidade de Vida , Angioedemas Hereditários/diagnóstico , Angioedemas Hereditários/epidemiologia , Ansiedade/epidemiologia , Ansiedade/etiologia , Emoções , Humanos , Inquéritos e Questionários
6.
Dig Liver Dis ; 53(1): 72-78, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33221330

RESUMO

BACKGROUND: The pivotal clinical trials have largely demonstrated the efficacy and safety of ustekinumab in Crohn's disease. Real-life cohorts published so far only include very few bio-naïve patients. This study assesses effectiveness and safety of ustekinumab in bio-naïve and bio-failure patients treated with ustekinumab in routine practice and look for predictors of response. METHODS: We performed a retrospective monocentric study. Initial response was assessed by maintenance therapy beyond week 16. Sustained response was assessed by the continuation or cessation of therapy over time for another reason than stopping in sustained remission. Treatment persistence was assessed by Kaplan Meier curves and predictors of treatment persistence were studied by univariate and multivariate Cox model. RESULTS: Out of 156 recorded patients, three patients were still in their induction phase at time of analysis and 5 patients were lost to follow-up, leaving 148 patients for clinical effectiveness analyses, including 35 bio-naïve when starting ustekinumab. A maintenance therapy was initiated in 79.7%. At one year, the probability to be still treated with ustekinumab was 73.8%. Treatment cessation increased with smoking in multivariate analysis. Previous biologic failure (as a whole), CRP and fecal calprotectin baseline levels did not influence initial response and treatment persistence. CONCLUSION: A large proportion of CD patients initially respond to ustekinumab and continue this treatment beyond one year. Treatment persistence is as high in bio-failure as in bio-naïve patients.


Assuntos
Doença de Crohn/tratamento farmacológico , Indução de Remissão/métodos , Ustekinumab/administração & dosagem , Produtos Biológicos/uso terapêutico , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Estudos Retrospectivos
7.
Orphanet J Rare Dis ; 15(1): 159, 2020 06 23.
Artigo em Inglês | MEDLINE | ID: mdl-32576213

RESUMO

BACKGROUND: As lack of awareness of rare diseases (RDs) among healthcare professionals results in delayed diagnoses, there is a need for a more efficient approach to RD training during academic education. We designed an experimental workshop that used role-play simulation with patient educators and focused on teaching "red flags" that should raise the suspicion of an RD when faced with a patient with frequently encountered symptoms. Our objective was to report our experience, and to assess the improvement in learners' knowledge and the satisfaction levels of the participants. RESULTS: The workshop consisted of 2 simulated consultations that both started with the same frequent symptom (Raynaud phenomenon, RP) but led to different diagnoses: a frequent condition (idiopathic RP) and an RD (systemic sclerosis, SSc). In the second simulated consultation, the role of the patient was played by a patient educator with SSc. By juxtaposing 2 seemingly similar situations, the training particularly highlighted the elements that help differentiate SSc from idiopathic RP. When answering a clinical case exam about RP and SSc, students that had participated in the workshop had a higher mean mark than those who had not (14 ± 3.7 vs 9.6 ± 5.5 points out of 20, p = 0.001). Participants mostly felt "very satisfied" with this training (94%), and "more comfortable" about managing idiopathic RP and SSc (100%). They considered the workshop "not very stressful" and "very formative" (both 71%). When asked about the strengths of this training, they mentioned the benefits of being put in an immersive situation, allowing a better acquisition of practical skills and a more interactive exchange with teachers, as well as the confrontation with a real patient, leading to a better retention of semiological findings and associating a relational component with this experience. CONCLUSIONS: Through the use of innovative educational methods, such as role-play simulation and patient educators, and by focusing on teaching "red flags", our workshop successfully improved RP and SSc learning in a way that satisfied students. By modifying the workshop's scenarios, its template can readily be applied to other clinical situations, making it an interesting tool to teach other RDs.


Assuntos
Doença de Raynaud , Escleroderma Sistêmico , Humanos , Doenças Raras , Escleroderma Sistêmico/diagnóstico
8.
Ann Pharm Fr ; 77(3): 241-249, 2019 May.
Artigo em Francês | MEDLINE | ID: mdl-30799017

RESUMO

OBJECTIVES: The pharmaceutical analysis of drug prescriptions is one of the key steps in the drug circuit. This mandatory regulatory practice in France and Quebec is based on national standards. The main objective of this work was to compare the practical methods of pharmaceutical analysis performed in French and Quebec university hospitals. METHODS: This is a prospective comparative survey conducted in 2 French and Quebec university hospital centres among pharmacists and pharmacy residents. RESULTS: The response rate to the survey was 60% (45/75). Between 16 and 22 elements were deemed necessary to structure the centralized, decentralized or mixed pharmaceutical analysis. The chronological ranking of these elements was comparable between the French and Quebec participants. All participants were in favour of the development of initial and continuing training in pharmaceutical analysis. Finally, the majority of participants were against using individual pharmaceutical analysis performance indicators to optimize the process (82%; 37/45). CONCLUSIONS: The French-Quebec practice of prescription analysis by a ward-pharmacist complies with national standards. The main differences in the practice of pharmaceutical analysis are related to the types of organization, the tools available and the length of time pharmacists have been deployed in care units in France and Quebec.


Assuntos
Prescrições de Medicamentos/normas , Instalações de Saúde/estatística & dados numéricos , Sistemas de Medicação no Hospital , Atitude do Pessoal de Saúde , França , Hospitais Universitários , Humanos , Farmacêuticos , Serviço de Farmácia Hospitalar , Estudos Prospectivos , Quebeque , Inquéritos e Questionários
9.
Rev Med Interne ; 40(8): 501-507, 2019 Aug.
Artigo em Francês | MEDLINE | ID: mdl-30551890

RESUMO

PURPOSE: Therapeutic education (TE) intends to help patients with systemic lupus erythematosus to better understand their disease and to improve their quality of life. The objective of this study was to assess illness perceptions of the person to provide a motivational environment for TE. METHODS: Systemic lupus erythematosus patients followed in the department of internal medicine in Lille university hospital responded to a questionnaire assessing five dimensions of the person as proposed by Giordan: the cognitive (knowledge), perceptual (fatigue and pain), affective (anxious and depressive symptoms), infra-cognitive (intimate reasoning) and metacognitive (worldview) dimensions. The quality of life was also evaluated. RESULTS: One hundred and twenty-four patients (114 women (92%); mean age 44.3±14.3 years) responded to the questionnaire. Regarding the cognitive dimension: quantity of information at the time of diagnosis was considered insufficient for 57 patients (46%). The median adherence evaluated by a scale had a median 97mm [88-100]. Regarding the perceptual dimension: pain was assessed at 59mm [44-78] and fatigue at 66mm [50-79] at visual scales. Regarding the affective dimension: prevalence of anxiety symptoms was 67% (83/124) and 28% (35/124) for depressive symptoms. Regarding the infra-cognitive dimension 78 patients (63%) had an external control place. Concerning the metacognitive dimension, systemic lupus erythematosus had repercussions on professional and family life. The quality of life was impacted. CONCLUSION: Multiple dimensions of systemic lupus erythematosus patient have to be considered for an optimal motivational environment for the practice of TE.


Assuntos
Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/psicologia , Adulto , Ansiedade/etiologia , Estudos Transversais , Depressão/etiologia , Função Executiva , Fadiga/etiologia , Feminino , Humanos , Masculino , Dor/etiologia , Educação de Pacientes como Assunto , Qualidade de Vida , Inquéritos e Questionários , Escala Visual Analógica
10.
Arch Pediatr ; 2018 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-29914755

RESUMO

Inherited metabolic diseases (IMD) form a heterogeneous group of genetic disorders that surface primarily during childhood and result in significant morbidity and mortality. A prevalence of 1 in 2500-5000 live births is often reported. The transfer of adolescents from pediatric care to adult health facilities is often difficult for patients and their families and can lead to a breakdown in medical follow-up and therefore serious complications. Existing recommendations for the successful transition of patients with chronic disorders do not specifically address patients with IMDs associated with dietary treatment. Here, the French network for rare inherited metabolic diseases (G2M) presents its reflections and recommendations for a successful transition. Preparations for the transfer must be made well in advance. The transfer must aim for adolescents gaining autonomy by making them responsible and providing them with the knowledge that will enable them to manage their care themselves, know how to react appropriately if there is any change in their condition, and move comfortably within the adult healthcare system. This requires the active participation of the patient, his or her family, and pediatric and adult care teams. It involves multidisciplinary management plus the production and maintenance of an educational therapy program. Finally, the identification of physicians and dietitians trained in IMDs, relevant subspecialists, and even expert patients could improve the continuum of complete and appropriate care for these patients within adult medicine.

11.
Orphanet J Rare Dis ; 13(1): 21, 2018 01 25.
Artigo em Inglês | MEDLINE | ID: mdl-29370874

RESUMO

BACKGROUND: In fructose 1,6 bisphosphatase (FBPase) deficiency, management aims to prevent hypoglycaemia and lactic acidosis by avoiding prolonged fasting, particularly during febrile illness. Although the need for an emergency regimen to avoid metabolic decompensation is well established at times of illness, there is uncertainty about the need for other dietary management strategies such as sucrose or fructose restriction. We assessed international differences in the dietary management of FBPase deficiency. METHODS: A cross-sectional questionnaire (13 questions) was emailed to all members of the Society for the Study of Inborn Errors of Metabolism (SSIEM) and a wide database of inherited metabolic disorder dietitians. RESULTS: Thirty-six centres reported the dietary prescriptions of 126 patients with FBPase deficiency. Patients' age at questionnaire completion was: 1-10y, 46% (n = 58), 11-16y, 21% (n = 27), and >16y, 33% (n = 41). Diagnostic age was: <1y, 36% (n = 46); 1-10y, 59% (n = 74); 11-16y, 3% (n = 4); and >16y, 2% (n = 2). Seventy-five per cent of centres advocated dietary restrictions. This included restriction of: high sucrose foods only (n = 7 centres, 19%); fruit and sugary foods (n = 4, 11%); fruit, vegetables and sugary foods (n = 13, 36%). Twenty-five per cent of centres (n = 9), advised no dietary restrictions when patients were well. A higher percentage of patients aged >16y rather than ≤16y were prescribed dietary restrictions: patients aged 1-10y, 67% (n = 39/58), 11-16y, 63% (n = 17/27) and >16y, 85% (n = 35/41). Patients classified as having a normal fasting tolerance increased with age from 30% in 1-10y, to 36% in 11-16y, and 58% in >16y, but it was unclear if fasting tolerance was biochemically proven. Twenty centres (56%) routinely prescribed uncooked cornstarch (UCCS) to limit overnight fasting in 47 patients regardless of their actual fasting tolerance (37%). All centres advocated an emergency regimen mainly based on glucose polymer for illness management. CONCLUSIONS: Although all patients were prescribed an emergency regimen for illness, use of sucrose and fructose restricted diets with UCCS supplementation varied widely. Restrictions did not relax with age. International guidelines are necessary to help direct future dietary management of FBPase deficiency.


Assuntos
Deficiência de Frutose-1,6-Difosfatase/dietoterapia , Acidose Láctica/etiologia , Acidose Láctica/prevenção & controle , Estudos Transversais , Carboidratos da Dieta , Suplementos Nutricionais , Jejum , Deficiência de Frutose-1,6-Difosfatase/complicações , Humanos , Hipoglicemia/etiologia , Hipoglicemia/prevenção & controle , Inquéritos e Questionários
12.
Lupus ; 27(3): 357-364, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28728511

RESUMO

Background/Purpose Long-term anticoagulation is the standard treatment for thrombotic antiphospholipid syndrome (APS). However, in daily practice, the question of withdrawing anticoagulation may arise, without any evidence-based recommendations. This study aimed to assess outcomes in APS patients after anticoagulation withdrawal. Methods Thrombotic APS patients followed in our centre, whose anticoagulation was withdrawn after APS diagnosis, were retrospectively selected, and were match-controlled with patients under anticoagulation, based on sex, age, APS clinical phenotype and disease duration. Results Thirty cases with anticoagulation withdrawal were included. Median follow-up was 51 months (12-124). The risk of thrombotic relapse was higher in cases compared to controls (7.3% versus 1.5% patient-year ( p = 0.01); hazard ratio 4.8; 95% confidence interval (1.4-16.7)). Male gender, anti-ß2GP1 and triple positivity at inclusion were predictive factors for thrombotic relapse. Conversely, aspirin prescription was a protective factor against relapses. Persistence of LA, anti-ß2GP1 and triple positivity over time were associated with a higher risk of thrombosis and aPL disappearance with a lower risk. Conclusion In our study, anticoagulation withdrawal was associated with an increased risk of thrombotic relapse. Our findings emphasize the influence of anti-ß2GP1 and triple positivity persistence over time on the risk of relapse and the benefit of aspirin prescription when anticoagulation has been withdrawn.


Assuntos
Anticoagulantes/administração & dosagem , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/tratamento farmacológico , Trombose/complicações , Trombose/tratamento farmacológico , Adulto , Anticorpos Antifosfolipídeos/imunologia , Anticoagulantes/uso terapêutico , Aspirina/uso terapêutico , Coagulação Sanguínea/efeitos dos fármacos , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Recidiva , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
13.
Psychol Med ; 48(7): 1128-1138, 2018 05.
Artigo em Inglês | MEDLINE | ID: mdl-28893331

RESUMO

BACKGROUND: Previous research has shown relatively diminished medial prefrontal cortex activation and heightened psychophysiological responses during the recollection of personal events in post-traumatic stress disorder (PTSD), but the origin of these abnormalities is unknown. Twin studies provide the opportunity to determine whether such abnormalities reflect familial vulnerabilities, result from trauma exposure, or are acquired characteristics of PTSD. METHODS: In this case-control twin study, 26 male identical twin pairs (12 PTSD; 14 non-PTSD) discordant for PTSD and combat exposure recalled and imagined trauma-unrelated stressful and neutral life events using a standard script-driven imagery paradigm during functional magnetic resonance imaging and concurrent skin conductance measurement. RESULTS: Diminished activation in the medial prefrontal cortex during Stressful v. Neutral script-driven imagery was observed in the individuals with PTSD, relative to other groups. CONCLUSIONS: Diminished medial prefrontal cortex activation during Stressful v. Neutral script-driven imagery may be an acquired characteristic of PTSD. If replicated, this finding could be used prospectively to inform diagnosis and the assessment of treatment response.


Assuntos
Imageamento por Ressonância Magnética , Córtex Pré-Frontal/fisiopatologia , Transtornos de Estresse Pós-Traumáticos/fisiopatologia , Veteranos/psicologia , Idoso , Estudos de Casos e Controles , Humanos , Imaginação , Masculino , Rememoração Mental , Pessoa de Meia-Idade , Estados Unidos
14.
Mol Genet Metab Rep ; 13: 83-89, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29021961

RESUMO

BACKGROUND: The definitive dietary management of propionic acidaemia (PA) is unknown although natural protein restriction with adequate energy provision is of key importance. AIM: To describe European dietary practices in the management of patients with PA prior to the publication of the European PA guidelines. METHODS: This was a cross-sectional survey consisting of 27 questions about the dietary practices in PA patients circulated to European IMD dietitians and health professionals in 2014. RESULTS: Information on protein restricted diets of 186 PA patients from 47 centres, representing 14 European countries was collected. Total protein intake [PA precursor-free L-amino acid supplements (PFAA) and natural protein] met WHO/FAO/UNU (2007) safe protein requirements for age in 36 centres (77%). PFAA were used to supplement natural protein intake in 81% (n = 38) of centres, providing a median of 44% (14-83%) of total protein requirement. Seventy-four per cent of patients were prescribed natural protein intakes below WHO/FAO/UNU (2007) safe levels in one or more of the following age groups: 0-6 m, 7-12 m, 1-10 y, 11-16 y and > 16 y. Sixty-three per cent (n = 117) of patients were tube fed (74% gastrostomy), but only 22% received nocturnal feeds. CONCLUSIONS: There was high use of PFAA with intakes of natural protein commonly below WHO/FAO/UNU (2007) safe levels. Optimal dietary management can only be determined by longitudinal, multi-centre, prospective case controlled studies. The metabolic instability of PA and small patient cohorts in each centre ensure that this is a challenging undertaking.

15.
Rev Med Interne ; 38(11): 718-724, 2017 Nov.
Artigo em Francês | MEDLINE | ID: mdl-28705597

RESUMO

Between 9000 and 14000 people have systemic sclerosis (ScS) in France. The work is often affected. Our study aims to assess the frequency of professional difficulties (DP) of scleroderma patients, identify these DP, the symptoms involved and the solutions used. A heteroquestionnaire was offered to scleroderma patients the Lille University Hospital. Data were cross-checked with the clinical database of the National Observatory of scleroderma patients. We used 104 questionnaires of which 84% are women. A total of 62.5%, (95% CI [52.5 to 71.8]) patients had experienced DP related to SSc, 55% reported symptoms worsened at work, 41% lower efficiency and a 19% job loss. Symptoms responsible for their DP were mainly asthenia, Raynaud syndrome, arthralgias and finger ulcerations. Only 40% of patients in difficulty were initiating steps with the county home for disabled people and 45% in informing their doctor at work (MT). The MT information absence was related to a misunderstanding of possible aid in 45% of cases, 57% of patients had not found a solution, 41% of them have lost their jobs and 31% of patients in the cohort experienced a drop in income. The impact of SSc the work is important. The aid job retention devices are under-used due to lack of knowledge of steps to take and the role of MT. Taking into account the symptoms responsible for PD must be improved to participate in this retention.


Assuntos
Estresse Ocupacional/epidemiologia , Ocupações/estatística & dados numéricos , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/epidemiologia , Adaptação Fisiológica , Adulto , Idoso , Astenia/epidemiologia , Estudos Transversais , Pessoas com Deficiência/estatística & dados numéricos , Feminino , França/epidemiologia , Humanos , Satisfação no Emprego , Masculino , Pessoa de Meia-Idade , Estresse Ocupacional/psicologia , Ocupações/normas , Qualidade de Vida , Inquéritos e Questionários
16.
Mol Genet Metab Rep ; 12: 16-22, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28275552

RESUMO

BACKGROUND: In Europe, dietary management of isovaleric acidemia (IVA) may vary widely. There is limited collective information about dietetic management. AIM: To describe European practice regarding the dietary management of IVA, prior to the availability of the E-IMD IVA guidelines (E-IMD 2014). METHODS: A cross-sectional questionnaire was sent to all European dietitians who were either members of the Society for the Study of Inborn Errors of Metabolism Dietitians Group (SSIEM-DG) or whom had responded to previous questionnaires on dietetic practice (n = 53). The questionnaire comprised 27 questions about the dietary management of IVA. RESULTS: Information on 140 patients with IVA from 39 centres was reported. 133 patients (38 centres) were given a protein restricted diet. Leucine-free amino acid supplements (LFAA) were routinely used to supplement protein intake in 58% of centres. The median total protein intake prescribed achieved the WHO/FAO/UNU [2007] safe levels of protein intake in all age groups. Centres that prescribed LFAA had lower natural protein intakes in most age groups except 1 to 10 y. In contrast, when centres were not using LFAA, the median natural protein intake met WHO/FAO/UNU [2007] safe levels of protein intake in all age groups. Enteral tube feeding was rarely prescribed. CONCLUSIONS: This survey demonstrates wide differences in dietary practice in the management of IVA across European centres. It provides unique dietary data collectively representing European practices in IVA which can be used as a foundation to compare dietary management changes as a consequence of the first E-IMD IVA guidelines availability.

17.
Sci Rep ; 7: 44322, 2017 03 13.
Artigo em Inglês | MEDLINE | ID: mdl-28287157

RESUMO

Microvascular dysfunction may have an early onset in type 1 diabetes (T1D) and can precede major complications. Our objectives were to assess the endothelial-dependent (acetylcholine, ACh; and post-occlusive hyperemia, PORH), non-endothelial-dependent (sodium nitroprusside, SNP) and neurovascular-dependent (local heating, LH and current induced vasodilation, CIV) microcirculatory vasodilation in T1D patients compared with matched control subjects using a laser speckle contrast imager. Seventeen T1D patients - matched with 17 subjects according to age, gender, Body-Mass-Index, and smoking status - underwent macro- and microvascular investigations. The LH early peak assessed the transient receptor potential vanilloid type 1 channels (TRPV1) mediated vasodilation, whereas the plateau assessed the Nitirc-Oxyde (NO) and endothelium-derived hyperpolarizing factor (EDHF) pathways. PORH explored sensory nerves and (EDHF), while CIV assessed sensory nerves (C-fibers) and prostaglandin-mediated vasodilation. Using neurological investigations, we observed that C-fiber and A-delta fiber functions in T1D patients were similar to control subjects. PORH, CIV, LH peak and plateau vasodilations were significantly decreased in T1D patients compared to controls, whereas there was no difference between the two groups for ACh and SNP vasodilations. Neurovascular microcirculatory vasodilations (C-fibers and TRPV 1-mediated vasodilations) are impaired in TD1 patients whereas no abnormalities were found using clinical neurological investigations. Clinicaltrials: No. NCT02538120.


Assuntos
Diabetes Mellitus Tipo 1/fisiopatologia , Microcirculação/fisiologia , Fibras Nervosas Amielínicas/fisiologia , Canais de Cátion TRPV/fisiologia , Vasodilatação/fisiologia , Acetilcolina/farmacologia , Adulto , Endotélio Vascular/fisiopatologia , Feminino , Humanos , Masculino , Nitroprussiato/farmacologia , Vasodilatação/efeitos dos fármacos , Vasodilatadores/farmacologia , Adulto Jovem
18.
Lupus ; 26(2): 163-169, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27432808

RESUMO

Introduction The long-term risk of first thrombosis and benefit of prophylaxis in antiphospholipid antibody (aPL) carriers without history of thrombosis or obstetrical morbidity is poorly known. This study aimed to evaluate the long-term rate and risk factors associated with a first thrombosis in those patients. Patients and methods After a prior study ended in December 2005 and was already published, we extended the follow-up period of our cohort of aPL carriers. Results Ninety-eight of the 103 patients of the previous study were included. The annual first thrombosis rate was 2.3% per patient-year during a median of 13 years (6-17). None of the baseline characteristics was predictive of risk of first thrombosis, but persistent aPL over time were associated with an increased risk. The stronger association was found in triple aPL-positive carriers: OR 3.38 (95% CI: 1.24-9.22). Of note, conversely to our previous findings, no benefit of aspirin prophylaxis was observed. Conclusion The risk of first thrombosis in aPL carriers without history of thrombosis or obstetrical morbidity was significant, persisted linearly over time and was associated with persistent aPL. This risk was especially increased in triple aPL-positive carriers, in whom a close follow-up seems to be necessary. Nevertheless, the benefit of aspirin prophylaxis remained unclear.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Trombose/etiologia , Adulto , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/tratamento farmacológico , Aspirina/administração & dosagem , Biomarcadores/sangue , Distribuição de Qui-Quadrado , Intervalo Livre de Doença , Feminino , Fibrinolíticos/administração & dosagem , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Razão de Chances , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Trombose/sangue , Trombose/diagnóstico , Trombose/prevenção & controle , Fatores de Tempo , Resultado do Tratamento
19.
Ann Pharm Fr ; 75(2): 131-143, 2017 Mar.
Artigo em Francês | MEDLINE | ID: mdl-27423187

RESUMO

OBJECTIVES: The main objective of this study was to compare the responses of pharmacy residents regarding critical steps for medication order review, in the presence or absence of clinical pharmacists on patient care units, to describe the sequence of these steps and to compare them to an optimal sequence. The secondary objectives were to test this sequence in a simulation and to assess the residents' level of agreement on medication order review. METHODS: Twenty-two validation steps were selected from guidelines. A simulation on order review was organized in three steps: selecting elements judged to be necessary or not for the order review critical path, then organizing this sequence in chronological order, implementation of this critical path on two simulated practical cases, resident perceptions about order review in their training. RESULTS: Forty-one residents participated in the activity. Responses were heterogeneous regarding the elements' sequence and the time required for the review of a simulated case (3-13minutes). A majority of residents considered that their training was insufficient (29/41), that pharmacists validated differently (27/41), and that it was impossible to review the 22 proposed items for each prescription (30/41). CONCLUSIONS: This article highlights heterogeneous medication order review practices among pharmacy residents, due to a lack of training in their curriculum according to them. It is essential to acquire medication order review standard both locally and nationally.


Assuntos
Educação de Pós-Graduação em Farmácia/organização & administração , Internato não Médico , Conduta do Tratamento Medicamentoso/educação , Conduta do Tratamento Medicamentoso/organização & administração , Farmacêuticos , Serviço de Farmácia Hospitalar , Simulação por Computador , Humanos , Erros de Medicação/prevenção & controle
20.
Rev Med Interne ; 37(12): 849-853, 2016 Dec.
Artigo em Francês | MEDLINE | ID: mdl-27842952

RESUMO

INTRODUCTION: Hyperhomocysteinemia is a biological marker that could be identified in the venous thrombotic events and rarely during acute arterial thrombotic events. The consequences can be serious. Effective diagnostic strategy is needed to optimize the management. CASE REPORT: Following bariatric surgery, a 40-year-old patient was admitted with an acute encephalopathy associated with peripheral lower limb arterial ischemia. The diagnostic work-up identified a major hyperhomocysteinemia whose causes were several. Surgical treatment and anticoagulation was associated with vitamins and trace elements supplementation. Correcting deficiencies allowed delirium and hyperhomocysteinemia improvement. Once treatment established, the patient did not present a recurrent thrombotic episode. CONCLUSION: Major hyperhomocysteinemia seems to be associated with an increased risk of acute arterial thrombosis. This marker might be considered in nutritional deficiency situations with appropriate support on the vascular, metabolic and nutrition level.


Assuntos
Anticoagulantes/uso terapêutico , Hiper-Homocisteinemia/complicações , Trombose/etiologia , Adulto , Artérias/patologia , Suplementos Nutricionais , Feminino , Humanos
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