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Si Houn Hahn, David Kronn, Nancy D Leslie, Loren D M Pena, Pranoot Tanpaiboon, Michael J Gambello, James B Gibson, Richard Hillman, David W Stockton, John W Day, Raymond Y Wang, Kristina An Haack, Raheel Shafi, Susan Sparks, Yang Zhao, Catherine Wilson, Priya S Kishnani
PurposePompe disease results from lysosomal acid α-glucosidase (GAA) deficiency and its associated glycogen accumulation and muscle damage. Alglucosidase alfa (recombinant human GAA (rhGAA)) received approval in 2006 as a treatment for Pompe disease at the 160 L production scale. In 2010, larger-scale rhGAA was approved for patients up to 8 years old without cardiomyopathy. NCT01526785 evaluated 4,000 L rhGAA efficacy/safety in US infantile- or late-onset Pompe disease (IOPD, LOPD) patients up to 1 year old transitioned from 160 L rhGAA...
March 22, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
Melissa Preissner, Rhiannon P Murrie, Isaac Pinar, Freda Werdiger, Richard P Carnibella, Graeme R Zosky, Andreas Fouras, Stephen Dubsky
We have developed an X-ray imaging system for <i>in vivo</i> four-dimensional computed tomography (4DCT) of small animals for pre-clinical lung investigations. Our customized laboratory facility is capable of high resolution <i>in vivo</i> imaging at high frame rates. Characterization using phantoms demonstrate a spatial resolution of slightly below 50 μm at imaging rates of 30 Hz, and the ability to quantify material density differences of at least 3%...
March 22, 2018: Physics in Medicine and Biology
Fabienne D Simonis, Carmen S V Barbas, Antonio Artigas-Raventós, Jaume Canet, Rogier M Determann, James Anstey, Goran Hedenstierna, Sabrine N T Hemmes, Greet Hermans, Michael Hiesmayr, Markus W Hollmann, Samir Jaber, Ignacio Martin-Loeches, Gary H Mills, Rupert M Pearse, Christian Putensen, Werner Schmid, Paolo Severgnini, Roger Smith, Tanja A Treschan, Edda M Tschernko, Marcos F Vidal Melo, Hermann Wrigge, Marcelo Gama de Abreu, Paolo Pelosi, Marcus J Schultz, Ary Serpa Neto
BACKGROUND: The majority of critically ill patients do not suffer from acute respiratory distress syndrome (ARDS). To improve the treatment of these patients, we aimed to identify potentially modifiable factors associated with outcome of these patients. METHODS: The PRoVENT was an international, multicenter, prospective cohort study of consecutive patients under invasive mechanical ventilatory support. A predefined secondary analysis was to examine factors associated with mortality...
March 21, 2018: Annals of Intensive Care
S Blecha, H J Schlitt, B M Graf, M Leitzmann, T Bein
BACKGROUND: In critical illnesses low socioeconomic status (SES) is associated with higher morbidity and mortality. In addition to the SES, further factors at an individual level (e.g., sex, health insurance status and place of residence) may influence the severity of illness and medical treatment. We investigated these additional parameters in a secondary analysis of the ECSSTASI data. METHODS: Within the framework of the ECSSTASI study, 996 patients were recruited from a surgical intensive care unit...
March 21, 2018: Der Anaesthesist
Ji Hye Jo, Yong Hee Choi, Jeong Ha Wie, Hyun Sun Ko, In Yang Park, Jong Chul Shin
Objective: To evaluate the significance of fetal Doppler parameters in predicting adverse neonatal outcomes and the risk of cesarean delivery due to non-reassuring fetal status, in severe small for gestational age (SGA) fetuses of late preterm and term gestation. Methods: Fetal brain and umbilical artery (UmA) Doppler parameters of cerebroplacental ratio (CPR) and UmA pulsatility index (PI) were evaluated in a cohort of 184 SGA fetuses between 34 and 41 weeks gestational age, who were less than the 5th percentile...
March 2018: Obstetrics & Gynecology Science
Asit Kumar Sikary, Sumit Dixit, Om P Murty
Background: Carbon monoxide (CO) is a colorless, odorless, tasteless, and nonirritating gas which makes it difficult for those who are exposed, to detect it, leading to unexpected death. This study was undertaken to see the pattern of fatal CO poisoning and to discuss preventive aspect. Materials and Methods: It was a retrospective descriptive study of fatal CO cases which were autopsied at All India Institute of Medical Sciences, New Delhi, from the year 2010 to the year 2015...
October 2017: Journal of Family Medicine and Primary Care
Toru Kotani, Masanori Hanaoka, Shinya Hirahara, Hisashi Yamanaka, Eckhard Teschner, Atsuko Shono
Background: Prone positioning may provide a uniform distribution of transpulmonary pressure and contribute to prevent ventilator-induced lung injury. However, despite moderate positive end-expiratory pressure and low tidal volumes, there is still a risk of regional overdistension. Case presentation: A man with refractory hypoxemia was mechanically ventilated with prone positioning. Although prone positioning with a plateau pressure of 18 cmH2 O and a positive end-expiratory pressure of 8 cmH2 O promptly improved oxygenation, regional ventilation monitoring using electrical impedance tomography initially detected decreased distribution in the dorsal region but increased in the ventral, suggesting overdistension...
2018: Journal of Intensive Care
Sangheun Lee, Heeyoung Lee, Lucy Youngmin Eun, Seung Woong Gang
Purpose: Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non-ventilator-assisted groups. Methods: We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital...
February 2018: Korean Journal of Pediatrics
Chih-Cheng Lai, Ya-Hui Wang, Cheng-Yi Wang, Hao-Chien Wang, Chong-Jen Yu, Likwang Chen
Objectives: This study aimed to compare the effects of angiotensin-converting-enzyme inhibitors (ACEis) and angiotensin receptor blockers (ARBs) on the risk of pneumonia and severe exacerbations in patients with COPD. Patients and methods: All patients with COPD who used ACEis and ARBs for >90 days between 2000 and 2005 were recruited. Pairwise matching (1:1) of the ACEi and ARB groups resulted in two similar subgroups, with 6,226 patients in each. The primary outcomes were pneumonia and COPD exacerbations, and the secondary outcome was death...
2018: International Journal of Chronic Obstructive Pulmonary Disease
Takamasa Kitajima, Satoshi Marumo, Hiroshi Shima, Masahiro Shirata, Satoru Kawashima, Daiki Inoue, Yuko Katayama, Ryo Itotani, Minoru Sakuramoto, Motonari Fukui
Purpose: Episodic nocturnal hypercapnia (eNH) caused by rapid eye movement (REM) sleep-related hypoventilation is often noted in patients with advanced COPD. The purpose of this study was to clarify the clinical significance of eNH and the effectiveness of eNH-targeted noninvasive positive pressure ventilation (NPPV). Patients and methods: We enrolled patients with stable, severe, or very severe COPD with daytime arterial partial oxygen pressure PaO2 ≥55 mmHg and daytime arterial partial carbon dioxide pressure PaCO2 <55 mmHg, who underwent overnight transcutaneous carbon dioxide pressure (PtcCO2 ) monitoring from April 2013 to April 2016...
2018: International Journal of Chronic Obstructive Pulmonary Disease
Bita Hajian, Jan De Backer, Wim Vos, Wouter H van Geffen, Paul De Winter, Omar Usmani, Tony Cahn, Huib Am Kerstjens, Massimo Pistolesi, Wilfried De Backer
Introduction: Severe exacerbations associated with chronic obstructive pulmonary disease (COPD) that require hospitalization significantly contribute to morbidity and mortality. Definitions for exacerbations are very broad, and it is unclear whether there is one predominant underlying mechanism that leads to them. Functional respiratory imaging (FRI) with modeling provides detailed information about airway resistance, hyperinflation, and ventilation-perfusion (V/Q) mismatch during and following an acute exacerbation...
2018: International Journal of Chronic Obstructive Pulmonary Disease
Stephen Dubsky, Jordan Thurgood, Andreas Fouras, Bruce R Thompson, Gregory J Sheard
The beating heart is known to produce pressure and airflow oscillations in the lungs of mammals. This phenomenon is often disregarded as detailed measurement of its effects in the lung have hitherto not been possible. Previous studies have attempted to measure the effect of these oscillations on gas mixing. However, the results have proven inconclusive, due to the lack of a direct measurement tool capable of flow measurement throughout the entire bronchial tree. Here we present the first detailed measurement of cardiogenic oscillations, using synchrotron-based dynamic lung imaging of live mechanically ventilated mice...
March 21, 2018: Scientific Reports
Ichiro Higashikubo, Heihachiro Arito, Kenji Ando, Akihiro Araki, Hidesuke Shimizu, Haruhiko Sakurai
OBJECTIVES: This study aimed to assess workers' exposure to indium and its compounds in 55 indium-handling operations among 13 Japanese plants. The surveyed plants were selected from indium-manufacturing plants whose annual indium production exceeded 500 kg. METHODS: The Control of Substances Hazardous to Health (COSHH) Essentials control banding toolkit, which contains simple scales for hazard levels, quantities in daily use, and "dustiness" characteristics, was used to assess generic risks of indium-handling operations...
March 20, 2018: Journal of Occupational Health
Javier Sayas Catalán, Manel Luján Torné
No abstract text is available yet for this article.
April 2018: Respiratory Care
Huang-Kai Kao, Charles Yuen Yung Loh, Hao-Wei Kou, Kuo-Chin Kao, Han-Chung Hu, Chia-Ming Chang, Chia-Hui Lee, Hsiang-Hao Hsu
BACKGROUND: Majority of current research focuses on pre-hospital care in mass casualty incidents (MCI) whereas this study is the first to examine multifactorial aspects of intensive care unit (ICU) resource management during a surge in massive burn injury (MBI) patients whilst identifying key outcome predictors that resulted in successful disaster managements. METHODS: Both critical care, surgical parameters and cost-effectiveness are investigated in patients admitted with severe burns resulting from the explosion...
March 18, 2018: Burns: Journal of the International Society for Burn Injuries
Santhi Iyer Kumar, Kathleen Doo, Julie Sottilo-Brammeier, Christianne Lane, Janice M Liebler
BACKGROUND: Studies exploring the effect of body mass index (BMI) on outcomes in the intensive care unit (ICU) have yielded mixed results, with few studies assessing patients at the extremes of obesity. We sought to understand the clinical characteristics and outcomes of patients with super obesity (BMI > 50 kg/m2 ) as compared to morbid obesity (BMI > 40 kg/m2 ) and obesity (BMI > 30 kg/m2 ). METHODS: A retrospective review of patients admitted to the Los Angeles County + University of Southern California medical intensive care unit (MICU) service between 2008 and 2013 was performed...
January 1, 2018: Journal of Intensive Care Medicine
H Wei, T L Dong, X H Yang
Objective: To evaluate the effect of Penehyclidine Hydrochloride Injection on pulmonary ischemia-reperfusion in aortic dissection surgery. Methods: This study was a prospective randomized controlled trial. Between September 2015 and October 2017 in the Second Affiliated Hospital of Zhengzhou University, 60 patients with aortic dissection underwent total arch replacement surgery were randomly divided into penehyclidine hydrochloride group (group A 30 cases) and control group (group B 30 cases). Patients in group A were infused with penehyclidine hydrochloride 0...
March 13, 2018: Zhonghua Yi Xue za Zhi [Chinese medical journal]
J Q Shen, Q Ji, W J Ding, L M Xia, L Wei, C S Wang
Objective: To evaluate in-hospital and mid-term outcomes of sequential versus separate grafting of in situ skeletonized left internal mammary artery (LIMA) to the left coronary system in a single-center, propensity-matched study. Methods: After propensity score matching, 120 pairs of patients undergoing first, scheduled, isolated coronary artery bypass grafting (CABG) with in situ skeletonized LIMA grafting to the left anterior descending artery (LAD) territory were entered into a sequential group (sequential grafting of LIMA to the diagonal artery and then to the LAD) or a control group (separate grafting of LIMA to the LAD)...
March 13, 2018: Zhonghua Yi Xue za Zhi [Chinese medical journal]
L J Li, Y M Liu, Y M Wang, F Zhou, H Li, X Q Xing, X D Han, L Chen, C X Zhang, J X Wang, B Liu, L J Suo, G H Yu, G Q Wang, X X Yao, Y Xiao, X L Zhu, C X Xue, X J Cui, B Cao
Objective: To explore the clinical features, etiological features and prognostic risk factors of long-term glucocorticoid users with community-acquired pneumonia (CAP). Methods: A retrospective study included 100 long-term glucocorticoid users with CAP (G-CAP group) from 11 hospitals of China between January 2014 and December 2014, while 100 non-immunocompromised patients with community-acquired pneumonia were enrolled as controls (nICH-CAP group). Characteristics including age, gender, underlying diseases, corticosteroids, symptoms, disease severity, imaging manifestations, etiology, respiratory failure, mechanical ventilation, whether the application of vasoactive drugs, antibiotics application, hospital mortality rate between the two groups were compared, and the prognostic factors of G-CAP were investigated using Logistic regression...
March 13, 2018: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Li Dong, Yi-Kun Kang, Xiang-Guang An
BACKGROUND: Off-pump coronary artery bypass grafting (OPCAB) is one of the standard treatments for coronary artery disease (CAD) while hybrid coronary revascularization (HCR) represents an evolving revascularization strategy. However, the difference in outcomes between them remains unclear. OBJECTIVE: We performed a meta-analysis to compare the short-term and mid-term outcomes of HCR versus OPCAB for the treatment of multivessel or left main CAD. METHODS: We searched the PubMed, EMBASE, Web of Science and Cochrane databases to identify related studies and a routine meta-analysis was conducted...
March 19, 2018: Arquivos Brasileiros de Cardiologia
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