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https://www.readbyqxmd.com/read/28446387/risk-factors-for-persistent-methicillin-resistant-staphylococcus-aureus-infection-in-cystic-fibrosis
#1
Mark T Jennings, Elliot C Dasenbrook, Noah Lechtzin, Michael P Boyle, Christian A Merlo
BACKGROUND: Methicillin-resistant Staphylococcus aureus (MRSA) has emerged as an important pathogen in cystic fibrosis (CF). Over 25% of individuals in the United States with CF are found to have MRSA in respiratory culture specimens, and persistent MRSA infection has been associated with more rapid decline in lung function and increased mortality. The objective of this study was to investigate clinical and demographic characteristics that are associated with the development of persistent MRSA infection in a CF population...
April 23, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28442759/a-statistical-algorithm-showing-coenzyme-q10-and-citrate-synthase-as-biomarkers-for-mitochondrial-respiratory-chain-enzyme-activities
#2
D Yubero, A Adin, R Montero, C Jou, C Jiménez-Mallebrera, A García-Cazorla, A Nascimento, M M O'Callaghan, J Montoya, L Gort, P Navas, A Ribes, M D Ugarte, R Artuch
Laboratory data interpretation for the assessment of complex biological systems remains a great challenge, as occurs in mitochondrial function research studies. The classical biochemical data interpretation of patients versus reference values may be insufficient, and in fact the current classifications of mitochondrial patients are still done on basis of probability criteria. We have developed and applied a mathematic agglomerative algorithm to search for correlations among the different biochemical variables of the mitochondrial respiratory chain in order to identify populations displaying correlation coefficients >0...
December 5, 2016: Scientific Reports
https://www.readbyqxmd.com/read/28440853/antibiotic-strategies-for-eradicating-pseudomonas-aeruginosa-in-people-with-cystic-fibrosis
#3
REVIEW
Simon C Langton Hewer, Alan R Smyth
BACKGROUND: Respiratory tract infection with Pseudomonas aeruginosa occurs in most people with cystic fibrosis. Once chronic infection is established, Pseudomonas aeruginosa is virtually impossible to eradicate and is associated with increased mortality and morbidity. Early infection may be easier to eradicate.This is an update of a Cochrane review first published in 2003, and previously updated in 2006, 2009 and 2014. OBJECTIVES: To determine whether antibiotic treatment of early Pseudomonas aeruginosa infection in children and adults with cystic fibrosis eradicates the organism, delays the onset of chronic infection, and results in clinical improvement...
April 25, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28437538/effects-of-diagnosis-by-newborn-screening-for-cystic-fibrosis-on-weight-and-length-in-the-first-year-of-life
#4
Daniel H Leung, Sonya L Heltshe, Drucy Borowitz, Daniel Gelfond, Margaret Kloster, James E Heubi, Michael Stalvey, Bonnie W Ramsey
Importance: Since the implementation of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or its association with correlates of disease among infants with CF who underwent NBS has not been well described. Objective: To examine incremental weight gain, linear growth, and clinical features in the first year of life among infants with CF who underwent NBS. Design, Setting, and Participants: The Baby Observational and Nutrition Study (BONUS), a multicenter, longitudinal, observational cohort study, was conducted during regular CF clinic visits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from January 7, 2012, through May 31, 2015...
April 24, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28433350/open-repair-of-chronic-thoracic-and-thoracoabdominal-aortic-dissection-using-deep-hypothermia-and-circulatory-arrest
#5
Joel Corvera, Hannah Copeland, David Blitzer, Adam Hicks, Joshua Manghelli, Philip Hess, John Fehrenbacher
BACKGROUND: Chronic dissection of the thoracic and thoracoabdominal aorta as sequela of a prior type A or B dissection is a challenging problem that requires close radiographic surveillance and prompt operative intervention in the presence of symptoms or aneurysm formation. Open repair of chronic thoracic and thoracoabdominal aortic dissection using deep hypothermia has been our preferred method to treat this complex pathology. The advantages of this technique include organ and spinal cord protection, the flexibility to extend the repair proximally into the arch, and the ability to limit ischemia to all vascular beds...
March 18, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28426447/measles-cases-in-children-requiring-hospital-access-in-an-academic-pediatric-hospital-in-italy-2008-2013
#6
Marta Ciofi Degli Atti, Antonietta Filia, Antonino Bella, Annamaria Sisto, Maria Antonietta Barbieri, Antonino Reale, Massimiliano Raponi
BACKGROUND: The Lazio Region is one of the Italian regions where sustained measles transmission continues to occur. We investigated measles cases reported by the Emergency Department (ED) of the largest pediatric hospital in Italy, located in Lazio. METHODS: We reviewed clinical records of all measles cases from 0 to 18 years of age evaluated in the ED in 2008-2013. We compared demographic and clinical characteristics of patients admitted to the inpatient setting with those of patients discharged home, to assess possible determinants of hospital admission...
April 18, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28421973/-bitten-by-an-exotic-venomous-snake
#7
M A Dijkman, I de Vries, M van Dam, D W de Lange
BACKGROUND: Patients who have been bitten by an exotic venomous snake are at risk of severe morbidity and a fatal outcome following an incorrect risk-assessment. Treatment with an antivenom can be necessary and can turn out to be lifesaving. In the Netherlands there are only a few cases of bites from exotic venomous snakes each year. CASE DESCRIPTION: A 28-year-old man presented at the emergency department (ED) after having been bitten by a monocled cobra (Naja kaouthia)...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28416349/cytoplasmic-body-pathology-in-severe-acta1-related-myopathy-in-the-absence-of-typical-nemaline-rods
#8
Sandra Donkervoort, Sophelia H S Chan, Leslie H Hayes, Nathaniel Bradley, David Nguyen, Meganne E Leach, Payam Mohassel, Ying Hu, Mathula Thangarajh, Diana Bharucha-Goebel, Amanda Kan, Ronnie S L Ho, Christine A Reyes, Jessica Nance, Steven A Moore, A Reghan Foley, Carsten G Bönnemann
Mutations in ACTA1 cause a group of myopathies with expanding clinical and histopathological heterogeneity. We describe three patients with severe ACTA1-related myopathy who have muscle fiber cytoplasmic bodies but no classic nemaline rods. Patient 1 is a five-year-old boy who presented at birth with severe weakness and respiratory failure, requiring mechanical ventilation. Whole exome sequencing identified a heterozygous c.282C>A (p.Asn94Lys) ACTA1 mutation. Patients 2 and 3 were twin boys with hypotonia, severe weakness, and respiratory insufficiency at birth requiring mechanical ventilation...
March 2, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28416348/respiratory-function-in-facioscapulohumeral-muscular-dystrophy-1
#9
M Wohlgemuth, C G C Horlings, E L van der Kooi, H J Gilhuis, J C M Hendriks, S M van der Maarel, B G M van Engelen, Y F Heijdra, G W Padberg
To test the hypothesis that wheelchair dependency and (kypho-)scoliosis are risk factors for developing respiratory insufficiency in facioscapulohumeral muscular dystrophy, we examined 81 patients with facioscapulohumeral muscular dystrophy 1 of varying degrees of severity ranging from ambulatory patients to wheelchair-bound patients. We examined the patients neurologically and by conducting pulmonary function tests: Forced Vital Capacity, Forced Expiratory Volume in 1 second, and static maximal inspiratory and expiratory mouth pressures...
March 22, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28403885/lung-structure-and-function-similarities-between-primary-ciliary-dyskinesia-and-mild-cystic-fibrosis-a-pilot-study
#10
Marco Maglione, Silvia Montella, Carmine Mollica, Vincenzo Carnovale, Paola Iacotucci, Fabiola De Gregorio, Antonella Tosco, Mariarosaria Cervasio, Valeria Raia, Francesca Santamaria
BACKGROUND: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are increasingly compared. There are no chest magnetic resonance imaging (MRI) comparative studies of PCD and CF. We assessed clinical, functional, microbiological and MRI findings in PCD and mild CF patients in order to evaluate different expression of lung disease. METHODS: Twenty PCD (15.1 years) and 20 CF subjects with mild respiratory impairment (16 years, 70% with pancreatic insufficiency) underwent MRI, spirometry, and sputum cultures when clinically stable...
April 12, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28403100/delayed-bilateral-vocal-cord-paresis-after-a-continuous-interscalene-brachial-plexus-block-and-endotracheal-intubation-a-lesson-why-we-should-use-low-concentrated-local-anesthetics-for-continuous-blocks
#11
Hee-Sun Park, Ha-Jung Kim, Young-Jin Ro, Hong-Seuk Yang, Won-Uk Koh
RATIONALE: Recurrent laryngeal nerve block is an uncommon complication that can occur after an interscalene brachial plexus block (ISB), which may lead to vocal cord palsy or paresis. However, if the recurrent laryngeal nerve is blocked in patients with a preexisting contralateral vocal cord palsy following neck surgery, this may lead to devastating acute respiratory failure. Thus, ISB is contraindicated in patients with contralateral vocal cord lesion. To the best of our knowledge, there are no reports of bilateral vocal cord paresis, which occurred after a continuous ISB and endotracheal intubation in a patient with no history of vocal cord injury or surgery of the neck...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28400726/review-of-epidural-spinal-cord-stimulation-for-augmenting-cough-after-spinal-cord-injury
#12
REVIEW
Jan T Hachmann, Jonathan S Calvert, Peter J Grahn, Dina I Drubach, Kendall H Lee, Igor A Lavrov
Spinal cord injury (SCI) remains a debilitating condition for which there is no cure. In addition to loss of somatic sensorimotor functions, SCI is also commonly associated with impairment of autonomic function. Importantly, cough dysfunction due to paralysis of expiratory muscles in combination with respiratory insufficiency can render affected individuals vulnerable to respiratory morbidity. Failure to clear sputum can aggravate both risk for and severity of respiratory infections, accounting for frequent hospitalizations and even mortality...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28399050/veptr-are-we-reducing-respiratory-assistance-requirements
#13
Sarah B Nossov, Evan Curatolo, Robert M Campbell, Oscar H Mayer, Sumeet Garg, Patrick J Cahill
BACKGROUND: The assisted ventilation rating (AVR) indicates the degree of external respiratory support required in children with thoracic insufficiency syndrome (TIS) and early onset scoliosis. For skeletally immature patients with TIS, the vertical expandable prosthetic titanium rib (VEPTR) device can be used to improve lung volume and growth. We hypothesized that patients who underwent early thoracic reconstruction by VEPTR treatment had an improved respiratory status. METHODS: Preoperative and postoperative AVR ratings were prospectively collected in a multicenter study group and compared with determine change after VEPTR treatment...
April 10, 2017: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/28398936/endogenous-glucocorticoid-response-to-single-dose-dexamethasone-for-croup-in-children-a-pharmacodynamic-study
#14
Natasha Gill, Natalie Sirizzotti, David Johnson, Gary Joubert, Andrew S Kucey, Alvin Tieu, Brad L Urquhart, Melanie Columbus, Rodrick Lim, Michael Rieder, Shruti Mehrotra, Emily D Hartjes, Naveen Poonai
OBJECTIVES: Dexamethasone is associated with adrenal insufficiency in adults and children with chronic disease. This association has not been studied after single-dose oral dexamethasone, the standard of care for children with croup. We hypothesized that single-dose oral dexamethasone in children with croup is associated with a transient decrease in endogenous glucocorticoids. METHODS: We conducted a prospective, 2-arm, pharmacodynamic study of single-dose oral dexamethasone 0...
April 11, 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28395030/clinical-features-molecular-heterogeneity-and-prognostic-implications-in-yars2-related-mitochondrial-myopathy
#15
Ewen W Sommerville, Yi Shiau Ng, Charlotte L Alston, Cristina Dallabona, Micol Gilberti, Langping He, Charlotte Knowles, Sophie L Chin, Andrew M Schaefer, Gavin Falkous, David Murdoch, Cheryl Longman, Marianne de Visser, Laurence A Bindoff, John M Rawles, John C S Dean, Richard K Petty, Maria E Farrugia, Tobias B Haack, Holger Prokisch, Robert McFarland, Douglass M Turnbull, Claudia Donnini, Robert W Taylor, Gráinne S Gorman
Importance: YARS2 mutations have been associated with a clinical triad of myopathy, lactic acidosis, and sideroblastic anemia in predominantly Middle Eastern populations. However, the identification of new patients expands the clinical and molecular spectrum of mitochondrial disorders. Objectives: To review the clinical, molecular, and genetic features of YARS2-related mitochondrial disease and to demonstrate a new Scottish founder variant. Design, Setting, and Participants: An observational case series study was conducted at a national diagnostic center for mitochondrial disease in Newcastle upon Tyne, England, and review of cases published in the literature...
April 10, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28394553/drug-induced-oral-ulcerations-case-report
#16
Vanja Vučičević Boras, Snježana Židovec-Lepej, Branka Marinović, Sven Seiwerth, Ivana Škrinjar, Dražen Pulanić, Danica Vidović Juras
A 70-year-old patient was admitted to the Department of Oral Medicine for multiple oral ulcerations on the left buccal mucosa, around 0.5 cm in diameter, as well as on the gingiva. Otherwise, the patient suffered from chronic lymphocytic leukemia, hypogammaglobulinemia, chronic renal insufficiency, with complete afunction of the right kidney, asthma, hypertension, gastritis and prostate hyperplasia. Differential diagnosis of oral ulcerations included drug induced oral ulcerations, paraneoplastic pemphigus, viral ulcerations (cytomegalovirus, herpes simplex viruses), fungal ulcerations (candidiasis, aspergillosis, histoplasmosis, cryptococcosis) and bacterial ulcerations, as well as neutropenic ulcers...
June 2016: Acta Clinica Croatica
https://www.readbyqxmd.com/read/28393008/an-unusual-cause-of-ventilatory-failure-in-motor-neurone-disease
#17
Epaminondas Markos Valsamis, Ian Smith, Adri De Sousa
A patient previously diagnosed with motor neurone disease (MND) and gastrostomy-fed was under surveillance for ventilatory decline via our respiratory centre. At a planned review she was found to be hypercapnic, which would usually prompt an offer of non-invasive ventilation for home use. However, she was alkalotic and not acidotic as we might expect. Her serum potassium was checked urgently and confirmed as low. It was established that the community team had prescribed a feeding regime with insufficient potassium...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28392237/routine-molecular-point-of-care-testing-for-respiratory-viruses-in-adults-presenting-to-hospital-with-acute-respiratory-illness-respoc-a-pragmatic-open-label-randomised-controlled-trial
#18
Nathan J Brendish, Ahalya K Malachira, Lawrence Armstrong, Rebecca Houghton, Sandra Aitken, Esther Nyimbili, Sean Ewings, Patrick J Lillie, Tristan W Clark
BACKGROUND: Respiratory virus infection is a common cause of hospitalisation in adults. Rapid point-of-care testing (POCT) for respiratory viruses might improve clinical care by reducing unnecessary antibiotic use, shortening length of hospital stay, improving influenza detection and treatment, and rationalising isolation facility use; however, insufficient evidence exists to support its use over standard clinical care. We aimed to assess the effect of routine POCT on a broad range of clinical outcomes including antibiotic use...
April 6, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28392046/efficacy-and-safety-of-tumor-necrosis-factor-antagonists-in-refractory-sarcoidosis-a-multicenter-study-of-132-patients
#19
Yvan Jamilloux, Fleur Cohen-Aubart, Catherine Chapelon-Abric, Delphine Maucort-Boulch, Alicia Marquet, Laurent Pérard, Laurence Bouillet, Alban Deroux, Sébastien Abad, Philip Bielefeld, Diane Bouvry, Marc André, Nicolas Noel, Boris Bienvenu, Alice Proux, Sandra Vukusic, Bahram Bodaghi, Françoise Sarrot-Reynauld, Jean Iwaz, Zahir Amoura, Christiane Broussolle, Patrice Cacoub, David Saadoun, Dominique Valeyre, Pascal Sève
INTRODUCTION: The off-label use of TNF antagonists in refractory sarcoidosis is increasingly reported but data on their efficacy and safety are still insufficient. OBJECTIVE: To report on efficacy and safety of TNF antagonists in severe and refractory sarcoidosis. METHODS: Examination of retrospective demographic, clinical, therapeutic, and adverse event data on 132 sarcoidosis patients (58% women; mean (min-max) age = 45.5 (14-78) years) given TNF antagonists (mainly infliximab, 91%) and investigation of response-linked factors...
March 8, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28391180/classifying-oxidative-stress-by-f2-isoprostane-levels-across-human-diseases-a-meta-analysis
#20
Thomas J van 't Erve, Maria B Kadiiska, Stephanie J London, Ronald P Mason
The notion that oxidative stress plays a role in virtually every human disease and environmental exposure has become ingrained in everyday knowledge. However, mounting evidence regarding the lack of specificity of biomarkers traditionally used as indicators of oxidative stress in human disease and exposures now necessitates re-evaluation. To prioritize these re-evaluations, published literature was comprehensively analyzed in a meta-analysis to quantitatively classify the levels of systemic oxidative damage across human disease and in response to environmental exposures...
March 28, 2017: Redox Biology
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