Search

Your search keyword '"Le Marec H"' showing total 20 results
Start Over Author "Le Marec H" Language undetermined
20 results on '"Le Marec H"'

1. MIBG Scintigraphy of a Patient with Pheochromocytoma on Labetalol Therapy

Author

A. Chetanneau, Bardies M, Le Marec H, Chatal Jf, M. Kremer, J. Morais, and Peltier P

Subjects
Adult, Male, medicine.medical_specialty, Adrenal Gland Neoplasms, Pheochromocytoma, Scintigraphy, Iodine Radioisotopes, Text mining, Internal medicine, Humans, Medicine, Labetalol, Radiology, Nuclear Medicine and imaging, Radionuclide Imaging, medicine.diagnostic_test, Iodobenzenes, business.industry, Imaging study, General Medicine, medicine.disease, 3-Iodobenzylguanidine, Endocrinology, Serial imaging, business, Nuclear medicine, medicine.drug
Abstract

A patient with pheochromocytoma was studied by MIBG scintigraphy while on labetalol therapy, which has been reported to interfere with imaging. Serial imaging was performed at 2, 18, 25, and 90 hours to obtain time/activity curves. Blood samples were taken at each imaging study to determine activity counts. Tumor activity per gram of tissue (surgery 116 hours after injection) was compared with blood activity. Tumor and blood activity decreased concurrently, whereas the decrease in peritumoral activity was faster, thus providing transiently improved contrast on 18- and 25-hour images. The diagnostic implications of these results are discussed.

Published
1992
Full Text
View/download PDF

2. Endomyocardial biopsies: a new approach for studying the electrical and mechanical properties of human ventricular myocardium

Author

E. Drouin, Charpentier F, Chevallier Jc, Laurent K, C. Gauthier, and Le Marec H

Subjects
medicine.medical_specialty, Pathology, business.industry, Biopsy, Heart Ventricles, Isoproterenol, Action Potentials, Myocardial Contraction, Endomyocardial biopsy, Ventricular myocardium, Internal medicine, Calcium concentration, medicine, Cardiology, Ventricular Function, Right, Humans, Cardiology and Cardiovascular Medicine, business, Molecular Biology, Endocardium
Abstract

The novel endomyocardial biopsy approach described here could facilitate the study of some human pathologies for which specimens are currently unavailable. Electrical and mechanical characteristics such as rate responses, effect of extracellular calcium concentration changes and β-adrenergic tone were similar to those determined for other human ventricular tissues, indicating that endomyocardial biopsies are suitable for physiological studies. This new source of cardiac tissues should facilitate the investigation of cellular mechanisms involved in the development of previously inaccessible human diseases.

Published
1994

7. EVADEF registry, main data,Le registre EVADEF: Principales données

Author

Le Heuzey, J. -Y, Ponsart, Z., Foudali, L., Dewailly, J., Lemaître, A., Trinquart, L., Chatellier, G., Durand-Zaleski, I., Clémenty, J., Haissaguerre, M., Jais, P., Reuter, S., Garrigue, S., Blanc, J. J., Mansourati, J., L Her, C., Ponsonnaille, J., Mansour, M., Lamaison, D., Tauriac, O., Defaye, P., Kacet, S., Lacroix, D., Klug, D., Zghal, N., Touboul, P., Kirkorian, G., Breyene, B., Chevalier, P., Lévy, S., Djiane, P., Deharo, J. C., Davy, J. M., Rodier, V., Maury, P., Cung, T., Aliot, E., Sadoul, N., Chilliou, C., Godin, J. F., Le Marec, H., Chevallier, J. C., Leenhardt, A., Extramiana, F., Lellouche, D., Elbaz, N., Lacotte, J., Frank, R., Hidden-Lucet, F., Himbert, C., Guize, L., Paziaud, O., Ait Said, M., Jouven, X., Lavergne, T., Daubert, J. C., Mabo, P., Leclercq, C., Pavin, D., Anselme, F., sana ouali, Chauvin, M., Delay, M., Prouteau, N., Salvador-Mazenq, M., Fauchier, J. P., Cosnay, P., Babuty, D., Fauchier, L., Otmani, A., Waintraub, X., and Marijon, E.

11. Genetic aspects of valvulopathies

Author

Kyndt, F., Le Scouarnec, S., Jaafar, P., Gueffet, J. P., Legendre, A., Trochu, J. N., Jousseaume, V., Chaventre, A., Schott, J. J., Le Marec, H., and Vincent Probst

14. Interspecies differences in the cardiac negative inotropic effects of beta(3)-adrenoceptor agonists

Author

Gauthier C, Geneviève Tavernier, Jn, Trochu, Leblais V, Laurent K, Langin D, Escande D, and Le Marec H

Subjects
Male, Reverse Transcriptase Polymerase Chain Reaction, Guinea Pigs, Ferrets, Adrenergic beta-Agonists, In Vitro Techniques, Papillary Muscles, Myocardial Contraction, Rats, Dogs, Species Specificity, Depression, Chemical, Receptors, Adrenergic, beta-3, Receptors, Adrenergic, beta, Animals, Humans, RNA, Messenger, Rats, Wistar
Abstract

The aim of the present study was to compare the effects of three preferential (BRL 37344, SR 58611, CL 316 243) and a partial (CGP 12177) beta-adrenoceptor (beta(3)-AR) agonists on the contractility of ventricular strips sampled from various mammalian species including humans. In the human heart, all beta(3)-AR agonists tested decreased contractility by 40 to 60% below control with an order of potency: BRL 37344CL 316 243 = SR 58611CGP 12177. In the dog, the negative inotropic effects produced by beta(3)-AR stimulation were less pronounced than in humans, approximately 30% below control. The order of potency of beta(3)-AR agonists was CGP 12177BRL 37344 = SR 58611CL 316 243; i.e., very different from that observed in humans. In rat, only BRL 37344 was efficient to decrease contractility. In guinea pig, only CL 316 243 significantly reduced peak tension. In both species, the reduction in peak tension did not exceed 20 to 30%. Finally, in the ferret, none of the agonists tested induced a negative inotropic effect. In dog, the negative inotropic effects of CGP 12177 were not modified by nadolol, but were abolished by bupranolol, a beta(1-3)-AR. beta(3)-AR transcripts were detected in the dog but not in the rat ventricle by using a reverse transcription-polymerase chain reaction assay. We conclude that cardiac negative inotropic effects related to beta(3)-AR agonist stimulation vary markedly depending on the species. A comparable interspecies variation previously has been reported concerning the lipolytic effects of beta(3)-AR agonist stimulation. Our study demonstrates that the pharmacological profile of a beta(3)-AR agonist on the human myocardium cannot be extrapolated from usual animal models.

19. SCN5A mutations in 442 neonates and children: genotype-phenotype correlation and identification of higher-risk subgroups

Author

Michael J. Ackerman, Sonia Franciosi, Carla Spazzolini, Peter J. Schwartz, Laura Zahavich, Annika Winbo, Anna Joong, Hervé Le Marec, Arja S. Vink, Andrew D. Krahn, Isabelle Denjoy, Mangesh Jadhav, Béatrice Guyomarc’h-Delasalle, Virginie Beauséjour-Ladouceur, Johan M Bos, Nico A. Blom, Alban-Elouen Baruteau, Matthias Lachaud, Claudine Rieubland, Jean Marc Lupoglazoff, Minoru Horie, Yanushi D. Wijeyeratne, Peter C. Ruben, Mena Abdelsayed, Jonathan R. Skinner, Elijah R. Behr, George F. Van Hare, Dominic Abrams, Takeshi Aiba, Jean-Baptiste Gourraud, Arthur A.M. Wilde, Junichi Ozawa, Tak-cheung Yung, Wataru Shimizu, Jacob Tfelt-Hansen, Leonardo Liberman, Lia Crotti, Sit Yee Kwok, Anne M. Dubin, David J. Tester, Shubhayan Sanatani, Vincent Probst, Juan Pablo Kaski, Andrew M. Davis, Federica Dagradi, Elizabeth A. Stephenson, Véronique Fressart, Boris Rudic, Leonie C.H. Wong, F. Kyndt, Jean-Jacques Schott, Baruteau, A, Kyndt, F, Behr, E, Vink, A, Lachaud, M, Joong, A, Schott, J, Horie, M, Denjoy, I, Crotti, L, Shimizu, W, Bos, J, Stephenson, E, Wong, L, Abrams, D, Davis, A, Winbo, A, Dubin, A, Sanatani, S, Liberman, L, Kaski, J, Rudic, B, Kwok, S, Rieubland, C, Tfelt-Hansen, J, Van Hare, G, Guyomarc'h-Delasalle, B, Blom, N, Wijeyeratne, Y, Gourraud, J, Le Marec, H, Ozawa, J, Fressart, V, Lupoglazoff, J, Dagradi, F, Spazzolini, C, Aiba, T, Tester, D, Zahavich, L, Beauséjour-Ladouceur, V, Jadhav, M, Skinner, J, Franciosi, S, Krahn, A, Abdelsayed, M, Ruben, P, Yung, T, Ackerman, M, Wilde, A, Schwartz, P, Probst, V, Amsterdam Cardiovascular Sciences, Graduate School, ACS - Heart failure & arrhythmias, Cardiology, and Paediatric Cardiology

Subjects
0301 basic medicine, Male, medicine.medical_specialty, BIO/18 - GENETICA, 610 Medicine & health, 030204 cardiovascular system & hematology, Lower risk, Asymptomatic, NAV1.5 Voltage-Gated Sodium Channel, 03 medical and health sciences, Electrocardiography, 0302 clinical medicine, Cardiac Conduction System Disease, Interquartile range, Loss of Function Mutation, Risk Factors, Internal medicine, Genotype, Cardiac conduction, Medicine, Humans, Child, Genetic Association Studies, Brugada syndrome, Brugada Syndrome, Retrospective Studies, business.industry, Age Factors, Infant, Newborn, Infant, Retrospective cohort study, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, medicine.disease, Brugada syndrome, Genotype–phenotype correlation, Long QT syndrome, Progressive cardiac conduction disorders, SCN5A, Sodium channelopathy, Long QT Syndrome, 030104 developmental biology, Child, Preschool, Gain of Function Mutation, Cohort, Asymptomatic Diseases, Female, medicine.symptom, Cardiology and Cardiovascular Medicine, business, Follow-Up Studies
Abstract

Aims To clarify the clinical characteristics and outcomes of children with SCN5A-mediated disease and to improve their risk stratification. Methods and results A multicentre, international, retrospective cohort study was conducted in 25 tertiary hospitals in 13 countries between 1990 and 2015. All patients ≤ 16 years of age diagnosed with a genetically confirmed SCN5A mutation were included in the analysis. There was no restriction made based on their clinical diagnosis. A total of 442 children {55.7% boys, 40.3% probands, median age: 8.0 [interquartile range (IQR) 9.5] years} from 350 families were included; 67.9% were asymptomatic at diagnosis. Four main phenotypes were identified: isolated progressive cardiac conduction disorders (25.6%), overlap phenotype (15.6%), isolated long QT syndrome type 3 (10.6%), and isolated Brugada syndrome type 1 (1.8%); 44.3% had a negative electrocardiogram phenotype. During a median follow-up of 5.9 (IQR 5.9) years, 272 cardiac events (CEs) occurred in 139 (31.5%) patients. Patients whose mutation localized in the C-terminus had a lower risk. Compound genotype, both gain- and loss-of-function SCN5A mutation, age ≤ 1 year at diagnosis in probands and age ≤ 1 year at diagnosis in non-probands were independent predictors of CE. Conclusion In this large paediatric cohort of SCN5A mutation-positive subjects, cardiac conduction disorders were the most prevalent phenotype; CEs occurred in about one-third of genotype-positive children, and several independent risk factors were identified, including age ≤ 1 year at diagnosis, compound mutation, and mutation with both gain- and loss-of-function.

Published
2017
Full Text
View/download PDF

20. Common variants at SCN5A-SCN10A and HEY2 are associated with Brugada syndrome, a rare disease with high risk of sudden cardiac death

Author

Jade Violleau, Stefan Kääb, Susan Bartkowiak, Richard Redon, Antoine Leenhardt, Hanno L. Tan, Jean-Baptiste Gourraud, Peter Weeke, Rachel Bastiaenen, Rianne Wolswinkel, Federica Dagradi, Vincent M. Christoffels, Jacob Tfelt-Hansen, Jean-Jacques Schott, Hervé Le Marec, Manfred Gessler, Françoise Gros, Pascale Guicheney, Julien Barc, Arthur A.M. Wilde, Simon Lecointe, Akihiko Nogami, Tohru Minamino, Sven Zumhagen, Margherita Torchio, Elijah R. Behr, Rainer Schimpf, Carol Ann Remme, Florence Kyndt, Lia Crotti, Yuka Mizusawa, Morten S. Olesen, Vincent Probst, Naoto Endo, Naomasa Makita, Eric Charpentier, Philippe Froguel, Arie O. Verkerk, Minoru Horie, Takeshi Aiba, Christian Dina, Peter J. Schwartz, Kanae Hasegawa, Floriane Simonet, Véronique Fressart, Seiko Ohno, Cornelia Wiese, Eric Schulze-Bahr, Stéphane Bézieau, Hiroshi Watanabe, Vincent Portero, Wataru Shimizu, Beverley Balkau, Martin Borggrefe, Britt M. Beckmann, Charles Antzelevitch, Bas J. Boukens, Dan M. Roden, Pierre Lindenbaum, Aurore Despres, David Weber, Olivier Lantieri, Connie R. Bezzina, Stéphanie Chatel, Ruben Coronel, ACS - Amsterdam Cardiovascular Sciences, Cardiology, Medical Biology, Other departments, ARD - Amsterdam Reproduction and Development, Bezzina, C, Barc, J, Mizusawa, Y, Remme, C, Gourraud, J, Simonet, F, Verkerk, A, Schwartz, P, Crotti, L, Dagradi, F, Guicheney, P, Fressart, V, Leenhardt, A, Antzelevitch, C, Bartkowiak, S, Borggrefe, M, Schimpf, R, Schulze-Bahr, E, Zumhagen, S, Behr, E, Bastiaenen, R, Tfelt-Hansen, J, Olesen, M, Kääb, S, Beckmann, B, Weeke, P, Watanabe, H, Endo, N, Minamino, T, Horie, M, Ohno, S, Hasegawa, K, Makita, N, Nogami, A, Shimizu, W, Aiba, T, Froguel, P, Balkau, B, Lantieri, O, Torchio, M, Wiese, C, Weber, D, Wolswinkel, R, Coronel, R, Boukens, B, Bézieau, S, Charpentier, E, Chatel, S, Despres, A, Gros, F, Kyndt, F, Lecointe, S, Lindenbaum, P, Portero, V, Violleau, J, Gessler, M, Tan, H, Roden, D, Christoffels, V, Le Marec, H, Wilde, A, Probst, V, Schott, J, Dina, C, and Redon, R

Subjects
Male, Qrs Duration, Bioinformatics, Sodium Channels, NAV1.5 Voltage-Gated Sodium Channel, Sudden cardiac death, Genome-Wide Association, Mice, Atrial Gene-Expression, Basic Helix-Loop-Helix Transcription Factors, Odds Ratio, Brugada Syndrome, Brugada syndrome, Mice, Knockout, St-Segment Elevation, SECS-S/01 - STATISTICA, cardiovascular system, Cardiology, Chromosomes, Human, Pair 6, Female, Chromosomes, Human, Pair 3, medicine.medical_specialty, Bundle-Branch Block, BIO/18 - GENETICA, Locus (genetics), Biology, Polymorphism, Single Nucleotide, Article, NAV1.8 Voltage-Gated Sodium Channel, Internal medicine, Genetic variation, Cardiac conduction, Genetics, medicine, Animals, Humans, Genetic Predisposition to Disease, cardiovascular diseases, HEY2, Alleles, Heart-Rate, Genetic Variation, Cardiac arrhythmia, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, medicine.disease, Qt Interval Duration, Repressor Proteins, Ventricular-Fibrillation, Pr Interval, Death, Sudden, Cardiac, Case-Control Studies, Conduction System, Genome-Wide Association Study, Rare disease
Abstract

Brugada syndrome is a rare cardiac arrhythmia disorder, causally related to SCN5A mutations in around 20% of cases. Through a genome-wide association study of 312 individuals with Brugada syndrome and 1,115 controls, we detected 2 significant association signals at the SCN10A locus (rs10428132) and near the HEY2 gene (rs9388451). Independent replication confirmed both signals (meta-analyses: rs10428132, P = 1.0 × 10(-68); rs9388451, P = 5.1 × 10(-17)) and identified one additional signal in SCN5A (at 3p21; rs11708996, P = 1.0 × 10(-14)). The cumulative effect of the three loci on disease susceptibility was unexpectedly large (Ptrend = 6.1 × 10(-81)). The association signals at SCN5A-SCN10A demonstrate that genetic polymorphisms modulating cardiac conduction can also influence susceptibility to cardiac arrhythmia. The implication of association with HEY2, supported by new evidence that Hey2 regulates cardiac electrical activity, shows that Brugada syndrome may originate from altered transcriptional programming during cardiac development. Altogether, our findings indicate that common genetic variation can have a strong impact on the predisposition to rare diseases.

Published
2013
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results