J E Wolf

University of Burgundy, Dijon, Bourgogne, France

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Publications (144)239.66 Total impact

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    ABSTRACT: OBJECTIVES: Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder with manifestations mainly involving the skeletal, ocular, and cardiovascular systems. The phenotypic variability observed in MFS makes genetic counselling difficult. Prenatal diagnosis (PND) and preimplantation genetic diagnosis are technically feasible when a causal mutation is identified, but both raise many ethical questions in this condition. Little is known about opinions and practices in such reproductive issues in MFS. The goal of this study was to report on patients' points of view and geneticists' standard practices. METHODS: Two different questionnaires were produced. RESULTS: Fifty geneticists filled in the questionnaire. Twenty-two per cent thought that PND was acceptable, 72% debatable and 6% not acceptable. Preimplantation genetic diagnosis was more often reported acceptable (34% of answers). Results varied according to the physician's experience with the disease. Fifty-four answers were collected for patients' questionnaires. Most of them (74%) were favourable to the development of prenatal testing, and believed that the choice should be given to parents. However, only a minority would opt for prenatal diagnosis for themselves. CONCLUSION: This study showed that the majority of patients were in favour of PND and that opinions among practitioners varied widely, but that overall, practitioners favoured a systematic multidisciplinary evaluation of the couple's request. © 2012 John Wiley & Sons, Ltd.
    Prenatal Diagnosis 11/2012; · 2.68 Impact Factor
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    ABSTRACT: The diagnosis of Marfan syndrome (MFS) is challenging and international criteria have been proposed. The 1996 Ghent criteria were adopted worldwide, but new diagnostic criteria for MFS were released in 2010, giving more weight to aortic root aneurysm and ectopia lentis. We aimed to compare the diagnosis reached by applying this new nosology vs the Ghent nosology in a well-known series of 1009 probands defined by the presence of an FBN1 mutation. A total of 842 patients could be classified as MFS according to the new nosology (83%) as compared to 894 (89%) according to the 1996 Ghent criteria. The remaining 17% would be classified as ectopia lentis syndrome (ELS), mitral valve prolapse syndrome or mitral valve, aorta, skeleton and skin (MASS) syndrome, or potential MFS in patients aged less than 20 years. Taking into account the median age at last follow-up (29 years), the possibility has to be considered that these patients would go on to develop classic MFS with time. Although the number of patients for a given diagnosis differed only slightly, the new nosology led to a different diagnosis in 15% of cases. Indeed, 10% of MFS patients were reclassified as ELS or MASS in the absence of aortic dilatation; conversely, 5% were reclassified as MFS in the presence of aortic dilatation. The nosology is easier to apply because the systemic score is helpful to reach the diagnosis of MFS only in a minority of patients. Diagnostic criteria should be a flexible and dynamic tool so that reclassification of patients with alternative diagnosis is possible, requiring regular clinical and aortic follow-up.
    Clinical Genetics 05/2011; 81(5):433-42. · 4.25 Impact Factor
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    Archives of Cardiovascular Diseases - ARCH CARDIOVASC DIS. 01/2011; 104(4):297-298.
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    ABSTRACT: Mutations in the FBN1 gene cause Marfan syndrome (MFS) and have been associated with a wide range of milder overlapping phenotypes. A proportion of patients carrying a FBN1 mutation does not meet diagnostic criteria for MFS, and are diagnosed with “other type I fibrillinopathy.” In order to better describe this entity, we analyzed a subgroup of 146 out of 689 adult propositi with incomplete “clinical” international criteria (Ghent nosology) from a large collaborative international study including 1,009 propositi with a pathogenic FBN1 mutation. We focused on patients with only one major clinical criterion, [including isolated ectopia lentis (EL; 12 patients), isolated ascending aortic dilatation (17 patients), and isolated major skeletal manifestations (1 patient)] or with no major criterion but only minor criteria in 1 or more organ systems (16 patients). At least one component of the Ghent nosology, insufficient alone to make a minor criterion, was found in the majority of patients with isolated ascending aortic dilatation and isolated EL. In patients with isolated EL, missense mutations involving a cysteine were predominant, mutations in exons 24–32 were underrepresented, and no mutations leading to a premature truncation were found. Studies of recurrent mutations and affected family members of propositi with only one major clinical criterion argue for a clinical continuum between such phenotypes and classical MFS. Using strict definitions, we conclude that patients with FBN1 mutation and only one major clinical criterion or with only minor clinical criteria of one or more organ system do exist but represent only 5% of the adult cohort. © 2009 Wiley-Liss, Inc.
    American Journal of Medical Genetics Part A 04/2009; 149A(5):854 - 860. · 2.30 Impact Factor
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    ABSTRACT: Objectifs Les télomères sont des structures nucléoprotéiques situées à l’extrémité des chromosomes. Leur fonction est de maintenir l’intégrité du génome au cours des divisions successives de la cellule. Du fait de leur forte proportion en guanine, ces structures sont très sensibles aux atteintes oxydantes et leur implication est démontrée dans les pathologies cardiovasculaires où une composante inflammatoire existe. Les objectifs notre étude ont été de déterminer les liens pouvant exister entre la longueur des télomères et certains paramètres géniques, métaboliques et cliniques chez des patients admis en Unité de Soins Intensifs Cardiologiques pour un infarctus du myocarde. Matériels et Méthodes Sur une cohorte de 67 patients, des données biologiques et cliniques ont été recueillies, dans le cadre de l’obseRvatoire des Infarctus de Côte-d’Or: RICO. La longueur des télomères, ainsi que le niveau d’expression de 2 gènes impliqués dans les phénomènes d’adaptation de la cellule aux processus inflammatoires (c-Fos) et, dans la réparation de l’ADN (OGG1) suite à des dommages oxydatifs, ont été évalués sur l’ADN de leucocytes de patients par la technique de PCR quantitative en temps réel. Résultats Nos résultats montrent une forte corrélation entre la longueur des télomères et le facteur âge (r = 0,374, p = 0,001). Après avoir ajusté les données au facteur âge, il est démontré une corrélation positive entre la longueur des télomères, la clairance à la créatinine (r = −0,711, p < 0,001) et la Fraction d’Ejection Ventriculaire Gauche (r = -0,329, p = 0,008). De même, des corrélations négatives entre l’hypertension artérielle (HTA) (p = 0,023) et le niveau d’expression du gène c-Fos (r = 0,242, p = 0,045) ont été mises en évidence. Une augmentation du niveau d’expression du gène c-Fos est retrouvée chez les patients présentant les télomères les plus courts. Aucune corrélation n’a été retrouvée entre la longueur des télomères et le niveau d’expression d’OGG1 (p = 0,636). Conclusion Nos résultats confortent les données de la littérature selon lesquelles, les processus inflammatoires, rencontrés notamment au cours de l’HTA, sont associés à un raccourcissement des télomères par le biais d’atteintes d’ordre radicalaire.
    Archives of Cardiovascular Diseases - ARCH CARDIOVASC DIS. 01/2009; 102.
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    ABSTRACT: IntroductionEndomyocardial fibrosis with apical obliteration is the best known involvement among heart lesions induced by hypereosinophilia. However, hypereosinophilic heart disease may involve all three heart layers, with a polymorphic clinical and echocardiographic presentation.
    Revue De Medecine Interne - REV MED INTERNE. 01/2009; 30(12):1011-1019.
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    ABSTRACT: To evaluate aortic elasticity with MRI on young asymptomatic individuals with mutation of the smooth muscle myosin heavy chain in whom aortic enlargement is not present. Aortic compliance, aortic distensibility, and pulse wave velocity (PWV) were semiautomatically measured from MRI in 8 asymptomatic subjects having a mutation of the MYH11 gene (M+) and 21 nonmutated relatives (M-) of similar age, sex, and blood pressure characteristics. Despite a similar aortic diameter in both groups, the aortic compliance and distensibility were significantly lower in M+ subjects compared with M- (0.84+/-0.33 versus 2.03+/-0.54 mm2/mmHg, 1.18+/-0.62 10(-3) versus 5.11+/-1.58 10(-3) mmHg(-1), respectively), and PWV was significantly higher (5.35+/-1.53 versus 3.60+/-0.64 m.s(-1)). A threshold aortic compliance value of 1.3 mm2/mmHg separated the two groups. The receiver operating characteristics curve analysis indicated an optimal threshold of 2.9 10(-3) mmHg(-1) for aortic distensibility (sensitivity: 87.5%, specificity: 90%), and of 4.4 m.s(-1) for PWV (sensitivity: 75%, specificity: 100%). Young asymptomatic adults with MYH11 mutation have an aortic compliance impairment which is not detectable by the sole measurement of the aortic size. Aortic compliance measurement might be part of routine examination in patients suspected of inherited aortic disease even with a normal aortic diameter.
    Journal of Magnetic Resonance Imaging 12/2008; 28(5):1180-7. · 2.57 Impact Factor
  • European Journal of Heart Failure Supplements 06/2007; 6(S1).
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    ABSTRACT: Heart failure with conserved systolic function is frequent and attributed to the diastolic dysfunction. The diagnosis of diastolic heart failure requires the association of clinical signs of heart failure, a conserved left ventricular systolic function and a diastolic dysfunction. Objective: To determine the proportion of cases of isolated diastolic heart failure among patients hospitalized for acute pulmonary edema. The left ventricular ejection fraction (LVEF), the diastolic function and levels of NT-proBNP have been assessed at admission of 145 patients hospitalized for acute pulmonary edema. 49% of patients included were older than 80 years (mean age 78.6 + 0.9 years). Among the 83 patients with conserved LVEF, 25% had an ischemic heart disease, 24% a severe valvular disease, 22% an atrial fibrillation, 5% a severe bradycardia, 2% a severe hypertrophic obstructive cardiomyopathy. Only 15 patients presented an isolated diastolic heart failure. The level of NT-proBNP was correlated to LVEF but was not able to identify those with isolated diastolic heart failure in the group with "conserved systolic function". Among patients hospitalized for acute pulmonary edema, the prevalence of heart failure with conserved systolic function is high, but only 10% of them presented an isolated diastolic heart failure. The NT-proBNP levels do not permit to identify them.
    Archives des maladies du coeur et des vaisseaux 01/2007; 99(12):1178-83. · 0.40 Impact Factor
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    ABSTRACT: IntroductionPacemaker implantation is a usual technique in cardiology which may be followed by acute pleural effusion and delayed unusual pericarditis.Case reportWe reported the case of a 67 year-old man hospitalized for faintness. Rhythmical auricular disease was diagnosed and pacemaker was implanted without immediate complication. Though pericarditis with tamponade at the day 21 will required emergency péricardotomy surgery. A recurrent pericarditis at day 45 was treated with anti-inflammatory drugs without relapse at the end of the treatment.DiscussionRepeated delayed pericarditis after pacemaker surgery may be compared to the Dressler syndrome which occurs after myocardial infarction.
    Revue De Medecine Interne - REV MED INTERNE. 01/2006; 27.
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    ABSTRACT: The aim of this work is to evaluate the relationship between improvement of regional myocardial function and visual analysis of contrast-enhanced (CE) MRI in patients after acute myocardial infarction. MRI was performed on 19 patients 1 and 11 weeks after a reperfused acute myocardial infarction. Perfusion data (first-pass images [FPI] and delayed CE images) were acquired after an intravenous bolus of gadolinium-DTPA and visually analyzed using a 17 segment model. Each segment was then classified in 3 groups, according to the presence or absence of FPI and CE patterns at baseline study: group 0: normal-appearing segments; group 1: segments with delayed hyper-enhancement but no early hypo-enhancement; group 2: segments with early hypoenhancement. Relative Wall thickening (RWT) was analyzed in each segment and its improvement evaluated in each group. Between first MRI and follow-up study, a significant improvement of RWT occurred in group 1 (mean +/- SD) [from 43.43 +/- 26.59% to 76.71 +/- 47.38%; p = 0.001] but not in group 2 (from 32.73 +/- 25.58% to 39.57 +/- 30.57%; p = NS). In group 0, RWT despite normal value at baseline study exhibited a significant improvement at follow-up (from 65.23 +/- 46.52% to 79.73 +/- 48.46%; p = 0.0015). In conclusion, the combined analysis of early and delayed perfusion abnormalities in MRI in the week following myocardial infarction can predict myocardial viability and allows in the future an evaluation of the efficacy of perfusion therapy.
    Archives des maladies du coeur et des vaisseaux 01/2006; 98(12):1199-205. · 0.40 Impact Factor
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    ABSTRACT: The aims of this study were to evaluate new tools of risk stratification in an unselected population of myocardial infarction (MI), usable in a pre-hospital situation, and to compare the risk profile of these patients with those of other clinical trials or myocardial infarction registries. The risk scores of death at 30 days (TIMI score and TIMI risk index) based on data available in the context of coronary emergencies, were applied to the population base of the MI observatory of myocardial infarction in the Côte d'Or (RICO). The risk profile was expressed by the smoothed graph of frequency distribution of each score. The TIMI score applied to the RICO population had a high discriminating power (c = 0.80) for mortality whereas TIMI risk index was less powerful (c = 0.57). The risk profile of the RICO population was comparable to that of InTIME II, ASSENT 2 and the NRMI with reperfusion registry. The NRMI without reperfusion and the MAGIC studies had different profiles characterised by a shift in the graph towards high risk patients. The authors conclude that risk stratification scores, like the TIMI score, are valuable tools for early triage in the management of MI patients. The risk profiles allow comparative analysis of risk levels of populations notably with respect to other registries and also with respect to randomised clinical trials.
    Archives des maladies du coeur et des vaisseaux 12/2005; 98(11):1130-6. · 0.40 Impact Factor
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    ABSTRACT: To assess the mechanisms through which an enlarged aortic root may facilitate right to left shunting through a patent foramen ovale. 19 patients with the platypnoea-orthodeoxia syndrome (POS) were compared with 30 control patients without platypnoea. Multiplane transoesophageal echocardiography. The aortic root diameter, atrial septal dimension behind the aortic root, and amplitude of the phasic oscillation of the septum were measured. Four groups of patients were compared: 12 platypnoeic patients with a dilated aortic root (POS-D), 7 platypnoeic patients with a normal aortic root (POS-N), 15 control patients with a dilated aortic root (CONT-D), and 15 control patients with a normal aortic root (CONT-N). In POS-D and CONT-D patients, the apparent atrial septal dimension was 16.3 (2.7) mm and 17.4 (5.9) mm respectively, compared with 24.4 (5.2) mm in POS-N patients and 25 (4) mm in CONT-N (p < 0.005). Furthermore, the amplitude of septal oscillation was 14.7 (2.5) mm in the POS-D group versus 5.8 (2.4) mm in CONT-N (p < 0.001) compared with 23.3 (3) mm in seven patients with an atrial septal aneurysm (p < 0.001). Patients with an enlarged aorta have an apparently smaller dimension and increased mobility of the atrial septum. These findings appear to result from compression by the aortic root and decreased septal tautness. Consequently, a "spinnaker effect" with the inferior vena caval flow may take place, opening the foramen ovale and leading to sustained right to left shunting.
    Heart (British Cardiac Society) 08/2005; 91(8):1030-5. · 5.01 Impact Factor
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    ABSTRACT: The occurrence of atrial fibrillation (AF) in the acute phase of myocardial infarction with ST segment elevation is common and responsible for an excess hospital mortality. The aim of this work was to define the incidence, predictive factors, and the prognostic impact of AF during MI with and without raised ST segment in the RICO study. Between January 2001 and July 2003, 1701 patients were included in this study: 130 (7.6%) had AF in the first 24 hours of management (AF+ group); 1571 (92.4%) remained in sinus rhythm (AF- group). Among the 1701 patients included in this study, 1197 (70.4%) had MI with raised ST and 504 (29.6%) had MI without raised ST. The incidence of AF was identical whatever the type of MI (7.6% with raised ST versus 7.7% without, p=0.334). The presence of Killip class >2 on admission and chronic obstructive pulmonary disease were independent predictive factors for the occurrence of AF (OR=3.84, p=0.007, and OR=2.47, p=0.014 respectively). The presence of AF was significantly associated with the occurrence of ventricular arrhythmia and/or cardiovascular mortality during admission in the non-selected MI population whatever the type of MI (raised ST ; AF+; 34% and AF-; 18%, p<0.01 versus without raised ST; AF+; 36% and AF-; 16%, p = 0.01). This study provides evidence that the incidence of AF during the first 24 hours of MI, as well as its poor prognosis, are identical whether or not there is ST segment elevation.
    Archives des maladies du coeur et des vaisseaux 07/2005; 98(6):608-14. · 0.40 Impact Factor
  • European Journal of Heart Failure Supplements 06/2005; 4(S1).
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    ABSTRACT: Intravenous insulin therapy is used in diabetic patients at the acute phase of coronary syndrome (ACS). However, hyperglycemia in diabetic patients is a powerful predictive factor for patient outcome as it is associated with a doubling of in-hospital mortality and poor long-term prognosis. Recent studies involving non-diabetic patients show that even mild hyperglycemia in the setting of ACS is also a predictive factor of in-hospital mortality. Moreover, the new entity called impaired fasting glucose (IFG) (6.1 to 7 mmol/L) is not only an independent factor of mortality for coronary patients, but very recently has also been associated with a doubling of the risk of in-hospital mortality in the setting of ACS. Admission as well as follow-up glycaemia are fundamental parameters in ACS on the one hand for their prognostic value, and on the other end as a diagnostic tool in determining the presence of diabetes or IFG.
    Archives des maladies du coeur et des vaisseaux 01/2005; 97 Spec No 3:47-50. · 0.40 Impact Factor
  • Atherosclerosis Supplements - ATHEROSCLER SUPPL. 01/2005; 6(1):16-17.
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    ABSTRACT: Preventing coronary in-stent restenosis is a major challenge for physicians and industry. To assess new stent technologies, a comparative paired iliac artery model in rabbits is proposed. One tubular stent was implanted in each external iliac artery in 12 rabbits (i.e., 24 stents). An artery overdilatation level of 20% was strictly observed. Restenosis was examined at 30 days by angiography, intravascular ultrasound (IVUS) examination, and histomorphometry. On quantitative angiography, the mean loss of angiographic diameter was 9.8 +/- 4.4% in the right as compared to 9.3 +/- 55% in the left artery (p = 0.75). On IVUS, the volume of intrastent neointimal proliferation was 26.6 +/- 4.9 mm(3) in the right and 25.8 +/- 3.5 mm(3) in the left artery (p = 0.58). In histomorphometry, the neointimal proliferation area was 0.78 +/- 17 mm(2) in the right and 0.76 +/- 0.17 mm(2) in the left artery (p = 0.87). Intrastent neointimal proliferation was comparable between the left and right arteries of all rabbits. The model has three main advantages: (1) arterial dilatation and thus arterial wall aggression are controlled, (2) pairing makes each animal its own control subject, and (3) the statistical power for comparative testing is maximized. The model enables the effect of a new drug-delivery device to be assessed.
    Journal of Biomedical Materials Research Part B Applied Biomaterials 09/2004; 70(2):303-10. · 2.31 Impact Factor
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    ABSTRACT: National therapeutic strategies in acute coronary syndromes (ACS) required revaluation, especially with regards to reperfusion. RICO is an observatory of ACS in the Côte d'Or district. Between January 1st 2001 and April 31st 2003, the cases of 706 patients with ACS and persistent ST elevation or appearances of left bundle branch block eligible for revascularisation (admitted < 12 hours after onset of symptoms and no contra-indications to thrombolysis), were reviewed. The number of revascularised patients was 488 (69%) and 218 (31%) were not revascularised. Thrombolysis was the most commonly used method of revascularisation (66%) in this district: 34% underwent primary angioplasty. Multivariate analysis showed only three independent predictive factors of non-reperfusion during the acute phase. They were: age (> or = 75 years) (p < 0.001), left bundle branch block (p = 0.002) and hospital admission > or = 6 hours after onset of symptoms (p < 0.001). These results confirm the utility of developing networks to improve the efficacy of management and reduce the delay before hospital admission. They also identify specific population groups, the elderly for example, who require specific therapeutic strategies for coronary revascularisation in ACS.
    Archives des maladies du coeur et des vaisseaux 03/2004; 97(3):195-200. · 0.40 Impact Factor
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    ABSTRACT: METHODS AND RESULTS: 101 patients hospitalized for acute non-ST-elevation myocardial infarction (NSTEMI) were included in the study. Median N-terminal fragment of the brain natriuretic peptide (BNP) prohormone (Nt-proBNP) plasma level was 136 (40-335) pmol/l. Patients with increasing levels of troponin I [from low (0.1-10 ng/ml), intermediate (10-40 ng/ml) to high (> or =40 ng/ml) levels] had significantly increased levels of Nt-proBNP (p < 0.05). High-risk patients classified by a high PURSUIT score (i.e. supramedian) had significantly increased Nt-proBNP levels compared to patients with low scores (p < 0.001). Moreover, patients with in-hospital events (death, recurrent MI or clinical heart failure: 27%) had significantly increased median levels of Nt-proBNP compared to event-free patients (184 vs. 105 pmol/l, p = 0.02). CONCLUSION: Our data in an unselected population of NSTEMI patients indicate that high levels of circulating Nt-proBNP levels are associated with an increased risk of early cardiovascular events.
    Cardiology 02/2004; 102(1):37-40. · 1.52 Impact Factor

Publication Stats

726 Citations
239.66 Total Impact Points

Institutions

  • 2002–2008
    • University of Burgundy
      Dijon, Bourgogne, France
  • 1996–2005
    • Centre Hospitalier Universitaire de Dijon
      • Department of Infectious Diseases
      Dijon, Bourgogne, France
  • 2003
    • Masaryk University
      • Faculty of Medicine
      Brno, South Moravian Region, Czech Republic
  • 1998
    • CHRU de Strasbourg
      Strasburg, Alsace, France
  • 1996–1997
    • Centre Georges-François Leclerc
      Dijon, Bourgogne, France
  • 1990–1994
    • Centre Hospitalier Universitaire de Grenoble
      • Service de Cardiologie
      Grenoble, Rhône-Alpes, France
  • 1989–1992
    • Centre Hospitalier Régional et Universitaire de Besançon
      Becoinson, Franche-Comté, France
  • 1991
    • University Joseph Fourier - Grenoble 1
      Grenoble, Rhône-Alpes, France