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Pierre Robin

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https://www.readbyqxmd.com/read/29777780/pierre-robin-sequence-a-comprehensive-narrative-review-of-the-literature-over-time
#1
Amerigo Giudice, Selene Barone, Kahina Belhous, Anne Morice, Véronique Soupre, Francesco Bennardo, Nathalie Boddaert, Marie-Paule Vazquez, Véronique Abadie, Arnaud Picard
Pierre Robin syndrome (PRS) is characterized of a triad of clinical signs: micrognathia, glossoptosis and obstruction of the upper airways frequently associated with palatal cleft. It is a heterogenic pathological entity and it can be found as isolated disease (nsPRS) or in association with other syndromes (sPRS), with more pronounced symptoms and systemic involvement. This review aims to summarize the principal features of PRS, analysing the different aspects of the disease. Epidemiological data highlight incidence, severity and mortality of PRS; pathophysiological mechanism reports the etiology and pathogenesis of the disease distinguishing between isolated and syndromic form...
May 16, 2018: Journal of Stomatology, Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29757793/technique-utilizing-a-modified-oral-ring-adair-elwyn-tube-to-provide-continuous-oxygen-and-sevoflurane-delivery-during-nasotracheal-intubation-in-an-infant-with-a-difficult-airway-a-case-report
#2
Janice Y Man, John E Fiadjoe, Grace Hsu
Managing the airway of an infant with Pierre Robin sequence (PRS) is particularly challenging for anesthesiologists. Patients with PRS have the triad of micrognathia, glossoptosis, and airway obstruction that potentially and frequently leads to difficulty with both ventilation and intubation. Thus continuous oxygenation and spontaneous ventilation during intubation are essential. We describe a new method to deliver continuous oxygen and volatile anesthetic during nasotracheal intubation in an infant with PRS...
May 15, 2018: A&A practice
https://www.readbyqxmd.com/read/29728421/glossoptosis-in-pierre-robin-sequence
#3
Kevin C Cahill, David J A Orr
No abstract text is available yet for this article.
May 4, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29727222/mandibular-catch-up-growth-in-pierre-robin-sequence-a-systematic-review
#4
Chad A Purnell, Lindsay E Janes, Julian L Klosowiak, Arun K Gosain
OBJECTIVE: The concept of mandibular catch-up growth is often quoted in the literature regarding Pierre Robin sequence (PRS). We endeavored to perform a systematic review of whether the literature supports this concept. DESIGN: Systematic review. INTERVENTIONS: A PubMed-based systematic review of the English literature was performed of articles objectively measuring mandibular growth or position after nonoperative management of PRS. MAIN OUTCOME MEASURES: Rate and end point of mandibular length, ramus length, gonial angle, and maxillomandibular discrepancy...
January 1, 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29721538/upper-airway-obstruction-in-neonates-does-sleep-exacerbate-symptoms
#5
Tyler Van Heest, Bridget Ebert, Brianne Barnett Roby, James Sidman
Objective: Describe the factors that exacerbate upper airway obstructions (UAOs) in neonates. Study Design: Retrospective chart review. Setting: Pediatric tertiary care hospital. Subjects and Methods: All neonates hospitalized between 1/1/2010 and 12/31/2014 diagnosed with either: 1) laryngomalacia, 2) Pierre Robin sequence, or 3) vocal cord paralysis were included in this study. Patient charts were reviewed to determine factors that exacerbated symptoms of airway obstruction...
April 2018: Laryngoscope Investigative Otolaryngology
https://www.readbyqxmd.com/read/29709343/increased-risk-of-thrombosis-associated-with-peripherally-inserted-central-catheters-compared-with-conventional-central-venous-catheters-in-children-with-leukemia
#6
Pierre-Amaël Noailly Charny, Nathalie Bleyzac, Robin Ohannessian, Edouard Aubert, Yves Bertand, Cecile Renard
OBJECTIVE: To study the risk of catheter-associated thrombosis (CAT) between peripherally inserted central catheters (PICCs) and tunneled central venous catheters in children with leukemia. STUDY DESIGN: We analyzed all PICCs and conventional tunneled catheters placed in patients aged <18 years and admitted to our institute for leukemia treatment between February 2008 and April 2014. Cases of symptomatic CAT were confirmed by ultrasound and treated with low-molecular-weight heparin...
April 27, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29708799/combined-tongue-palate-fusion-with-alveolar-bands-in-a-patient-with-pierre-robin-sequence-and-van-der-woude-syndrome
#7
Alexa Robbins, Yuri A Zarate, Larry D Hartzell
This report describes the presentation of a newborn male with circumferential tongue-palate fusion associated with cleft palate and alveolar bands. After intraoral adhesions lysis, the patient was diagnosed with Pierre Robin sequence. A family history of cleft lip and palate was noted, and interferon regulatory factor 6 ( IRF6) sequencing revealed a heterozygous variant, confirming the diagnosis of van der Woude syndrome. The disruption of IRF6 resulted in abnormal orofacial development including micrognathia and intraoral adhesions as well as tongue-palate fusion, then resulting in glossoptosis with airway obstruction and cleft palate...
January 1, 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29707444/applications-of-computer-technology-in-complex-craniofacial-reconstruction
#8
Kristopher M Day, Kyle S Gabrick, Larry A Sargent
Background: To demonstrate our use of advanced 3-dimensional (3D) computer technology in the analysis, virtual surgical planning (VSP), 3D modeling (3DM), and treatment of complex congenital and acquired craniofacial deformities. Methods: We present a series of craniofacial defects treated at a tertiary craniofacial referral center utilizing state-of-the-art 3D computer technology. All patients treated at our center using computer-assisted VSP, prefabricated custom-designed 3DMs, and/or 3D printed custom implants (3DPCI) in the reconstruction of craniofacial defects were included in this analysis...
March 2018: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/29705219/multicentric-standardized-flow-cytometry-routine-assessment-of-septic-patient-to-predict-clinical-worsening
#9
Thomas Daix, Estelle Guerin, Elsa Tavernier, Emmanuelle Mercier, Valérie Gissot, Olivier Hérault, Jean-Paul Mira, Florence Dumas, Nicolas Chapuis, Christophe Guitton, Marie C Béné, Jean-Pierre Quenot, Cindy Tissier, Julien Guy, Gaël Piton, Anne Roggy, Grégoire Muller, Éric Legac, Nicolas de Prost, Mehdi Khellaf, Orianne Wagner-Ballon, Rémi Coudroy, Elodie Dindinaud, Fabrice Uhel, Mikaël Roussel, Thomas Lafon, Robin Jeannet, Frédéric Vargas, Catherine Fleureau, Mickaël Roux, Kaoutar Allou, Philippe Vignon, Jean Feuillard, Bruno François
BACKGROUND: In this study, we primarily sought to assess the ability of flow cytometry to predict early clinical deterioration and overall survival in septic patients admitted in the emergency department and intensive care unit. METHODS: Patients admitted for community-acquired acute sepsis from 11 hospital centers were eligible. Early (Day 7) and late (Day 28) deaths were notified. Levels of CD64pos granulocytes, CD16pos monocytes, CD16dim immature granulocytes (IG), T and B lymphocytes were assessed by flow cytometry, using an identical, cross-validated, robust and simple consensus standardized protocol in each center...
April 26, 2018: Chest
https://www.readbyqxmd.com/read/29695406/a-novel-association-of-campomelic-dysplasia-with-hydrocephalus-due-to-an-unbalanced-chromosomal-translocation-upstream-of-sox9
#10
Prince Antwi, Christopher S Hong, Daniel Duran, Jin Sheng Chih, Weilai Dong, Michael DiLuna, Kristopher T Kahle
Campomelic dysplasia is a rare skeletal dysplasia characterized by Pierre-Robin sequence, craniofacial dysmorphism, shortening and angulation of long bones, tracheobronchomalacia, and occasionally sex reversal. The disease is due to mutations in SOX9 or chromosomal rearrangements involving the long arm of chromosome 17 harboring the SOX9 locus. SOX9, a transcription factor, is indispensible in establishing and maintaining neural stem cells in the CNS. We present a patient with angulation of long bones and external female genitalia on prenatal ultrasound who was subsequently found to harbor the chromosomal abnormality 46, XY, t(6;17) (p21...
April 25, 2018: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/29680814/spect-v-q-for-the-diagnosis-of-pulmonary-embolism-protocol-for-a-systematic-review-and-meta-analysis-of-diagnostic-accuracy-and-clinical-outcome
#11
Pierre-Yves Le Roux, Philippe Robin, Cécile Tromeur, Alexandra Davis, Helia Robert-Ebadi, Marc Carrier, Francis Couturaud, Grégoire Le Gal, Pierre-Yves Salaun
INTRODUCTION: Single photon emission computed tomography ventilation/perfusion (SPECT V/Q) imaging has many proponents within the nuclear medicine community and has already largely replaced planar V/Q scintigraphy in daily practice for the diagnosis of pulmonary embolism (PE). However, the test is still described in clinical guidelines as an experimental test because of insufficient evidence. METHODS AND ANALYSIS: We will conduct a systematic review and a meta-analysis of diagnostic accuracy and management outcome studies involving patients evaluated with V/Q SPECT for suspected acute PE...
April 21, 2018: BMJ Open
https://www.readbyqxmd.com/read/29664868/active-transcutaneous-bone-conduction-implant-middle-fossa-placement-technique-in-children-with-bilateral-microtia-and-external-auditory-canal-atresia
#12
Carolina Der, Sofía Bravo-Torres, Nicolás Pons
AIM: The aim of this study is to present the middle fossa technique (MFT) as an alternative for patients who cannot undergo traditional surgery for active transcutaneous bone conduction implants (ATBCI) due to their altered anatomy or desire for future aesthetic reconstruction. DESIGN: A case series descriptive study was designed. The MFT was developed. Preoperative and postoperative information from 24 patients with external auditory canal atresia (EACA) and implanted with ATBCI was reviewed...
April 16, 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29643641/pierre-robin-sequence-with-cervicothoracic-kyphoscoliosis-an-anesthetic-challenge
#13
V Abraham, S Grewal, G Bhatia, N Kaur, W Raghav, P Jain, N Gupta, M Singh, C George
No abstract text is available yet for this article.
January 2018: Journal of Anaesthesiology, Clinical Pharmacology
https://www.readbyqxmd.com/read/29642420/ebna1-oncogenic-activity-immune-evasion-and-biochemical-functions-provide-targets-for-novel-therapeutic-strategies-against-epstein-barr-virus-associated-cancers
#14
REVIEW
Joanna B Wilson, Evelyne Manet, Henri Gruffat, Pierre Busson, Marc Blondel, Robin Fahraeus
The presence of the Epstein-Barr virus (EBV)-encoded nuclear antigen-1 (EBNA1) protein in all EBV-carrying tumours constitutes a marker that distinguishes the virus-associated cancer cells from normal cells and thereby offers opportunities for targeted therapeutic intervention. EBNA1 is essential for viral genome maintenance and also for controlling viral gene expression and without EBNA1, the virus cannot persist. EBNA1 itself has been linked to cell transformation but the underlying mechanism of its oncogenic activity has been unclear...
April 6, 2018: Cancers
https://www.readbyqxmd.com/read/29627422/predictors-of-failure-in-infant-mandibular-distraction-osteogenesis
#15
Jeffrey A Hammoudeh, Artur Fahradyan, Colin Brady, Michaela Tsuha, Beina Azadgoli, Sally Ward, Mark M Urata
PURPOSE: Mandibular distraction osteogenesis (MDO) has been shown to be successful in treating upper airway obstruction caused by micrognathia in pediatric patients. The purpose of this study was to assess the success rate of MDO and possible predictors of failure. PATIENTS AND METHODS: The records of all neonates and infants who underwent MDO from 2008 to 2015 were retrospectively reviewed. Procedural failure was defined as patient death or the need for tracheostomy postoperatively...
March 15, 2018: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29620532/dopant-radial-inhomogeneity-in-mg-doped-gan-nanowires
#16
Alexandra-Madalina Siladie, Lynda Amichi, Nicolas Mollard, Isabelle Mouton, Bastien Bonef, Catherine Bougerol, Adeline Grenier, Eric Robin, Pierre-Henri Jouneau, Nuria Garro, Ana Cros, Bruno Daudin
Using atom probe tomography, it is demonstrated that Mg doping of GaN nanowires grown by Molecular Beam Epitaxy is resulting in marked radial inhomogeneities, namely a higher Mg content in the periphery of the nanowires. This spatial inhomogeneity is assigned to a preferential incorporation of Mg through the m-plane sidewalls of nanowires, in relation to the formation of a Mg-rich surface reconstruction stabilized by hydrogen. This scheme is further supported by Raman spectroscopy experiments putting in evidence Mg-H complexes in the doped nanowires...
April 5, 2018: Nanotechnology
https://www.readbyqxmd.com/read/29576976/proteomic-identification-of-galectin-11-and-14-ligands-from-haemonchus-contortus
#17
Dhanasekaran Sakthivel, Jaclyn Swan, Sarah Preston, M D Shakif-Azam, Pierre Faou, Yaqing Jiao, Rachael Downs, Harinda Rajapaksha, Robin Gasser, David Piedrafita, Travis Beddoe
Haemonchus contortus is the most pathogenic nematode of small ruminants. Infection in sheep and goats results in anaemia that decreases animal productivity and can ultimately cause death. The involvement of ruminant-specific galectin-11 (LGALS-11) and galectin-14 (LGALS-14) has been postulated to play important roles in protective immune responses against parasitic infection; however, their ligands are unknown. In the current study, LGALS-11 and LGALS-14 ligands in H. contortus were identified from larval (L4) and adult parasitic stages extracts using immobilised LGALS-11 and LGALS-14 affinity column chromatography and mass spectrometry...
2018: PeerJ
https://www.readbyqxmd.com/read/29559892/-in-utero-administration-of-drugs-targeting-microglia-improves-the-neurodevelopmental-outcome-following-cytomegalovirus-infection-of-the-rat-fetal-brain
#18
Robin Cloarec, Sylvian Bauer, Natacha Teissier, Fabienne Schaller, Hervé Luche, Sandra Courtens, Manal Salmi, Vanessa Pauly, Emilie Bois, Emilie Pallesi-Pocachard, Emmanuelle Buhler, François J Michel, Pierre Gressens, Marie Malissen, Thomas Stamminger, Daniel N Streblow, Nadine Bruneau, Pierre Szepetowski
Congenital cytomegalovirus (CMV) infections represent one leading cause of neurodevelopmental disorders. Recently, we reported on a rat model of CMV infection of the developing brain in utero , characterized by early and prominent infection and alteration of microglia-the brain-resident mononuclear phagocytes. Besides their canonical function against pathogens, microglia are also pivotal to brain development. Here we show that CMV infection of the rat fetal brain recapitulated key postnatal phenotypes of human congenital CMV including increased mortality, sensorimotor impairment reminiscent of cerebral palsy, hearing defects, and epileptic seizures...
2018: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/29554444/predicting-adverse-perioperative-events-in-patients-undergoing-primary-cleft-palate-repair
#19
Marten N Basta, John E Fiadjoe, Albert S Woo, Kenneth N Peeples, Oksana A Jackson
OBJECTIVE: This study aimed to identify risk factors for adverse perioperative events (APEs) after cleft palatoplasty to develop an individualized risk assessment tool. DESIGN: Retrospective cohort. SETTING: Tertiary institutional. PATIENTS: Patients younger than 2 years with cleft palate. INTERVENTIONS: Primary Furlow palatoplasty between 2008 and 2011. MAIN OUTCOME MEASURE(S): Adverse perioperative event, defined as laryngo- or bronchospasm, accidental extubation, reintubation, obstruction, hypoxia, or unplanned intensive care unit admission...
April 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29554066/an-algorithm-for-airway-management-in-patients-with-pierre-robin-sequence
#20
Katherine E Hicks, Kathleen R Billings, Chad A Purnell, John M Carter, Bharat Bhushan, Arun K Gosain, Dana M Thompson, Jeffrey C Rastatter
PURPOSE: Airway management in neonates with Pierre Robin sequence (PRS) can be challenging. The goal was to describe the algorithm developed by the authors over the past 8 years. METHODS: A retrospective case series analyzing airway management in neonates with PRS admitted to the neonatal intensive care unit at a tertiary care pediatric hospital was performed. The utility of the proposed algorithm for airway management incorporating more consistent use of polysomnography (PSG), and airway assessment was assessed...
March 16, 2018: Journal of Craniofacial Surgery
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