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排序方式: 共有2308条查询结果,搜索用时 15 毫秒
91.
Gilles Morin Valrie Biancalana Andoni Echaniz‐Laguna Jean‐Baptiste Noury Xavire Lornage Maurizio Moggio Michela Ripolone Raffaella Violano Pascale Marcorelles Denis Marchal Florence Renaud Claude‐Alain Maurage Cline Tard Jean‐Marie Cuisset Jocelyn Laporte Johann Bhm 《Human mutation》2020,41(1):17-37
Calcium (Ca2+) acts as a ubiquitous second messenger, and normal cell and tissue physiology strictly depends on the precise regulation of Ca2+ entry, storage, and release. Store‐operated Ca2+ entry (SOCE) is a major mechanism controlling extracellular Ca2+ entry, and mainly relies on the accurate interplay between the Ca2+ sensor STIM1 and the Ca2+ channel ORAI1. Mutations in STIM1 or ORAI1 result in abnormal Ca2+ homeostasis and are associated with severe human disorders. Recessive loss‐of‐function mutations impair SOCE and cause combined immunodeficiency, while dominant gain‐of‐function mutations induce excessive extracellular Ca2+ entry and cause tubular aggregate myopathy (TAM) and Stormorken syndrome (STRMK). TAM and STRMK are spectra of the same multisystemic disease characterized by muscle weakness, miosis, thrombocytopenia, hyposplenism, ichthyosis, dyslexia, and short stature. To date, 42 TAM/STRMK families have been described, and here we report five additional families for which we provide clinical, histological, ultrastructural, and genetic data. In this study, we list and review all new and previously reported STIM1 and ORAI1 cases, discuss the pathomechanisms of the mutations based on the known functions and the protein structure of STIM1 and ORAI1, draw a genotype/phenotype correlation, and delineate an efficient screening strategy for the molecular diagnosis of TAM/STRMK. 相似文献
92.
Hamza Berguigua Ludovic Iche Philippe Roche Cyril Aubert Renaud Blond Antoine Legrand Brnice Puech Chlo Combe Charles Vidal Margot Caron Marie-Christine Jaffar-Bandjee Christophe Caralp Nora Oulehri Hugo Kerambrun Jrme Allyn Yvonnick Bou Nicolas Allou 《Medicine》2021,100(48)
In February 2021, an explosion of cases of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pneumonia overwhelmed the only hospital in Mayotte. To report a case series of patients with acute respiratory failure (ARF) due to SARS-CoV-2 who were evacuated by air from Mayotte to Reunion Island.This retrospective observational study evaluated all consecutive patients with ARF due to SARS-CoV-2 who were evacuated by air from Mayotte Hospital to the intensive care unit (ICU) of Félix Guyon University Hospital in Reunion Island between February 2, and March 5, 2021.A total of 43 patients with SARS-CoV-2 pneumonia were evacuated by air, for a total flight time of 2 hours and a total travel time of 6 hours. Of these, 38 patients (88.4%) with a median age of 55 (46–65) years presented with ARF and were hospitalized in our ICU. Fifteen patients were screened for the SARS-CoV-2 501Y.V2 variant, all of whom tested positive. Thirteen patients (34.2%) developed an episode of severe hypoxemia during air transport, and the median paO2/FiO2 ratio was lower on ICU admission (140 [102–192] mmHg) than on departure (165 [150–200], P = .022). Factors associated with severe hypoxemia during air transport was lack of treatment with curare (P = .012) and lack of invasive mechanical ventilation (P = .003). Nine patients (23.7%) received veno-venous extracorporeal membrane oxygenation support in our ICU. Seven deaths (18.4%) occurred in hospital.Emergency air evacuation of patients with ARF due to SARS-CoV-2 was associated with severe hypoxemia but remained feasible. In cases of ARF due to SARS-CoV-2 requiring emergency air evacuation, sedated patients receiving invasive mechanical ventilation and curare should be prioritized over nonintubated patients. It is noteworthy that patients with SARS-CoV-2 pneumonia related to the 501Y.V2 variant were very severe despite their young age. 相似文献
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95.
Umberto Volpe Hania Amin Olatunde O. Ayinde Alistair Burns Wai Chi Chan Renaud David Slavica Djukic Dejanovic Gorica Djokic Defne Eraslan Giulia A.L. Fischer Patricia Gracia-García Syed Usman Hamdani Changsu Han Hussain Jafri Roy A. Kallivayalil Roderick Leonard Kriekaart Ee Heok Kua Linda C.W. Lam Dusica Lecic-Tosevski Iracema Leroi Antonio Lobo Adriana Mihai Fareed Aslam Minhas Heena Mistry Afolakemi T. Ogundele Marcel G.M. Olde Rikkert Javier Olivera Claudia Palumbo Angela Parker Bojana Pejuskovic Florian Riese Philippe Robert Maya Semrau Gabriela Stoppe Sanu Sudhakar Andreea Raluca Tirintica Sehrish Tofique Chris Tsoi Lucas Wolski Irem Yalug Huali Wang Xin Yu Norman Sartorius 《International journal of geriatric psychiatry》2020,35(2):163-173
96.
97.
Philippe dAbadie Stephan Walrand Renaud Lhommel Michel Hesse Ivan Borbath Franois Jamar 《Current oncology (Toronto, Ont.)》2022,29(4):2422
Selective internal radiation therapy (SIRT) is part of the treatment strategy for hepatocellular carcinoma (HCC). Strong clinical data demonstrated the effectiveness of this therapy in HCC with a significant improvement in patient outcomes. Recent studies demonstrated a strong correlation between the tumor response and the patient outcome when the tumor-absorbed dose was assessed by nuclear medicine imaging. Dosimetry plays a key role in predicting the clinical response and can be optimized using a personalized method of activity planning (multi-compartmental dosimetry). This paper reviews the main clinical results of SIRT in HCC and emphasizes the central role of dosimetry for improving it effectiveness. Moreover, some patient and tumor characteristics predict a worse outcome, and toxicity related to SIRT treatment of advanced HCC patient selection based on the performance status, liver function, tumor characteristics, and tumor targeting using technetium-99m macro-aggregated albumin scintigraphy can significantly improve the clinical performance of SIRT. 相似文献
98.
Leo P. Renaud 《Trends in neurosciences》1987,10(12):498-502
Hypothalamic magnocellular neurosecretory cells (MNCs), which secrete vasopressin and oxytocin into the plasma, contain an array of intrinsic ionic and voltage-sensitive conductances that modulate action potential shapes, discharge frequencies and patterns. These conductances interact with osmotic and synaptically generated conductances, activated by a variety of transmitters and neuropeptides, which may retard spike generation or induce phasic and bursting discharge patterns, and thus provide more efficient hormone release in the neurohypophysis. In the rat, medullary noradrenergic and forebrain GABAergic neurons, as well as neurons located in a circumventricular structure, the subfornical organ, appear to constitute major pathways conveying visceral, osmotic and cardiovascular information to MNCs. 相似文献
99.
Test‐retest reliability of the default mode network in a multi‐centric fMRI study of healthy elderly: Effects of data‐driven physiological noise correction techniques
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Rocco Marchitelli Ludovico Minati Moira Marizzoni Beatriz Bosch David Bartrés‐Faz Bernhard W. Müller Jens Wiltfang Ute Fiedler Luca Roccatagliata Agnese Picco Flavio Nobili Oliver Blin Stephanie Bombois Renaud Lopes Régis Bordet Julien Sein Jean‐Philippe Ranjeva Mira Didic Hélène Gros‐Dagnac Pierre Payoux Giada Zoccatelli Franco Alessandrini Alberto Beltramello Núria Bargalló Antonio Ferretti Massimo Caulo Marco Aiello Carlo Cavaliere Andrea Soricelli Lucilla Parnetti Roberto Tarducci Piero Floridi Magda Tsolaki Manos Constantinidis Antonios Drevelegas Paolo Maria Rossini Camillo Marra Peter Schönknecht Tilman Hensch Karl‐Titus Hoffmann Joost P. Kuijer Pieter Jelle Visser Frederik Barkhof Jorge Jovicich 《Human brain mapping》2016,37(6):2114-2132
Understanding how to reduce the influence of physiological noise in resting state fMRI data is important for the interpretation of functional brain connectivity. Limited data is currently available to assess the performance of physiological noise correction techniques, in particular when evaluating longitudinal changes in the default mode network (DMN) of healthy elderly participants. In this 3T harmonized multisite fMRI study, we investigated how different retrospective physiological noise correction (rPNC) methods influence the within‐site test‐retest reliability and the across‐site reproducibility consistency of DMN‐derived measurements across 13 MRI sites. Elderly participants were scanned twice at least a week apart (five participants per site). The rPNC methods were: none (NPC), Tissue‐based regression, PESTICA and FSL‐FIX. The DMN at the single subject level was robustly identified using ICA methods in all rPNC conditions. The methods significantly affected the mean z‐scores and, albeit less markedly, the cluster‐size in the DMN; in particular, FSL‐FIX tended to increase the DMN z‐scores compared to others. Within‐site test‐retest reliability was consistent across sites, with no differences across rPNC methods. The absolute percent errors were in the range of 5–11% for DMN z‐scores and cluster‐size reliability. DMN pattern overlap was in the range 60–65%. In particular, no rPNC method showed a significant reliability improvement relative to NPC. However, FSL‐FIX and Tissue‐based physiological correction methods showed both similar and significant improvements of reproducibility consistency across the consortium (ICC = 0.67) for the DMN z‐scores relative to NPC. Overall these findings support the use of rPNC methods like tissue‐based or FSL‐FIX to characterize multisite longitudinal changes of intrinsic functional connectivity. Hum Brain Mapp 37:2114–2132, 2016. © 2016 Wiley Periodicals, Inc. 相似文献
100.
Severe infantile isolated exocrine pancreatic insufficiency caused by the complete functional loss of the SPINK1 gene
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Kareen Billiemaz Renaud Touraine Claire Gay Sylvie Destombe David N. Cooper Hugues Patural Jian‐Min Chen Claude Férec 《Human mutation》2017,38(12):1660-1665
Exocrine pancreatic insufficiency (EPI) is rare in children, with most if not all cases occurring as part of syndromic conditions such as cystic fibrosis and Shwachman–Diamond syndrome. Here we report two cases, both presenting with severe EPI around 5 months of age. Characterized by diffuse pancreatic lipomatosis, they otherwise exhibited no remarkable deficiencies in other organs. Novel non‐identical homozygous variants (a deletion removing the entire SPINK1 gene and an insertion of a full‐length inverted Alu element into the 3′‐untranslated region of the SPINK1 gene) resulting in the complete functional loss of the SPINK1 gene (encoding pancreatic secretory trypsin inhibitor) were identified in each patient. Having correlated our findings with current knowledge of SPINK1's role in exocrine pancreas pathophysiology, we propose that complete and partial functional losses of the SPINK1 gene are associated with quite distinct phenotypes, the former causing a new pediatric disease entity of severe infantile isolated EPI. 相似文献