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Jiang Zhang, Fangrui Lin, Liuying Chai, Lichun Wei, Tongsheng Chen
No abstract text is available yet for this article.
October 1, 2016: Journal of Biomedical Optics
Ji-Hong Chen
Ineffective esophageal motility (IEM) is characterized by low to very low amplitude propulsive contractions in the distal esophagus, hence primarily affecting the smooth muscle part of the esophagus. IEM is often found in patients with dysphagia or heartburn and is commonly associated with gastroesophageal reflux disease. IEM is assumed to be associated with ineffective bolus transport; however, this can be verified using impedance measurements or evaluation of a barium coated marshmallow swallow. Furthermore, water swallows may not assess accurately the motor capabilities of the esophagus, since contraction amplitude is strongly determined by the size and consistency of the bolus...
2016: Clinical and Experimental Gastroenterology
B Stawarczyk, D Awad, N Ilie
Determining the amount of blue light (360-540nm) passing through nine monolithic computer-aided design/computer-aided manufacturing (CAD/CAM) materials depends on material thickness, initial irradiance, and the distance between the curing unit and the specimen's surface. A total of 180 specimens of two thicknesses (1 mm and 2 mm, n=10/subgroup) were fabricated from TelioCAD, VITA CAD-Temp (VCT), experimental nanocomposite, LAVA Ultimate (LU), VITA ENAMIC (VE), VITA MarkII (VM), IPS EmpressCAD (IEC), IPS e.maxCAD (IEM), and CELTRA DUO (CD)...
September 2016: Operative Dentistry
Maryam Abiri, Saeed Talebi, Jouni Uitto, Leila Youssefian, Hassan Vahidnezhad, Tina Shirzad, Shadab Salehpour, Sirous Zeinali
Most inborn errors of metabolism (IEMs) are inherited in an autosomal recessive manner. IEMs are one of the major concerns in Iran due to its extensive consanguineous marriages. Herein, we report two patients with two co-existent IEMs: a girl affected by classic phenylketonuria (PKU) and maple syrup urine disease (MSUD) and a male patient affected with Sandhoff disease and PKU, where Sandhoff disease was suspected due to the presence of a cherry-red spot in the eyes at 6 months which is unrelated to PKU. Sequencing of candidate genes in the first patient revealed one novel and three recurrent compound heterozygous mutations of p...
October 1, 2016: Journal of Pediatric Endocrinology & Metabolism: JPEM
Julia Hamonet-Torny
OPINION/FEEDBACK: INTRODUCTION: The Motor Education Institute (IEM) of Couzeix set up in June 2015 workshops of therapeutic education, with the aim of helping adolescents to better understand their motor disabilities and to reinforce their motivation for the rehabilitation. METHOD: Between March and April 2015, the educational supports of this workshop entitled "understanding my motor disability" were elaborated in collaboration by the paramedical and the educative staffs, with the participation of volunteer teenagers...
September 2016: Annals of Physical and Rehabilitation Medicine
Robert Chiesa, Robert F Wynn, Paul Veys
PURPOSE OF REVIEW: This review summarizes the main results of haematopoietic stem cell transplantation (HSCT) in selected inborn errors of metabolism (IEMs). RECENT FINDINGS: Early diagnosis and immediate referral to an IEM specialist is of paramount importance to improve clinical outcome: patients who are transplanted early or in their presymptomatic phase generally achieve better correction of their somatic symptoms and neurocognitive development. Long-term outcome in children with Hurler syndrome is influenced by age at HSCT, baseline clinical status and post-HSCT enzyme levels...
November 2016: Current Opinion in Hematology
Abdellah Tebani, Carlos Afonso, Stéphane Marret, Soumeya Bekri
The rise of technologies that simultaneously measure thousands of data points represents the heart of systems biology. These technologies have had a huge impact on the discovery of next-generation diagnostics, biomarkers, and drugs in the precision medicine era. Systems biology aims to achieve systemic exploration of complex interactions in biological systems. Driven by high-throughput omics technologies and the computational surge, it enables multi-scale and insightful overviews of cells, organisms, and populations...
2016: International Journal of Molecular Sciences
Sarah Van Lent, Winnok H De Vos, Heather Huot Creasy, Patricia X Marques, Jacques Ravel, Daisy Vanrompay, Patrik Bavoil, Ru-Ching Hsia
The polymorphic membrane protein (Pmp) paralogous families of Chlamydia trachomatis, Chlamydia pneumoniae and Chlamydia abortus are putative targets for Chlamydia vaccine development. To determine whether this is also the case for Pmp family members of C. psittaci, we analyzed transcription levels, protein production and localization of several Pmps of C. psittaci. Pmp expression profiles were characterized using quantitative real-time PCR (RT-qPCR), immunofluorescence (IF) and immuno-electron microscopy (IEM) under normal and stress conditions...
2016: PloS One
Majid Alfadhel, Mohammed Benmeakel, Mohammad Arif Hossain, Fuad Al Mutairi, Ali Al Othaim, Ahmed A Alfares, Mohammed Al Balwi, Abdullah Alzaben, Wafaa Eyaid
BACKGROUND: Inborn errors of metabolism (IEMs) are individually rare; however, they are collectively common. More than 600 human diseases caused by inborn errors of metabolism are now recognized, and this number is constantly increasing as new concepts and techniques become available for identifying biochemical phenotypes. The aim of this study was to determine the type and distribution of IEMs in patients presenting to a tertiary care center in Saudi Arabia. METHOD: We conducted a retrospective review of children diagnosed with IEMs presenting to the Pediatric Department of King Abdulaziz Medical City in Riyadh, Saudi Arabia over a 13-year period...
2016: Orphanet Journal of Rare Diseases
Saartje Bloemen, Xiao Xuan Wu, Katrien M Devreese, Bas de Laat, Jacob H Rand, Ljiljana V Vasovic
INTRODUCTION: The antiphospholipid syndrome (APS) is an acquired autoimmune disorder predisposing patients to thrombosis or pregnancy complications. Since inverted erythrocyte membranes (iEMs) might provide a physiologically relevant source of anionic phospholipids, we studied the interactions of phospholipid-binding proteins and APS antibodies using iEMs. MATERIALS & METHODS: iEMs were prepared from packed erythrocytes by hypotonic lysis. Phosphatidylserine (PS) exposure was confirmed by annexin A5 (A5) binding using fluorescence microscopy and flow cytometry...
October 2016: Thrombosis Research
Liuqin Jiang, Bixing Ye, Ying Wang, Meifeng Wang, Lin Lin
Background/Aims: Little data exists about esophageal body dysmotility and reflux patterns in refractory gastroesophageal reflux disease (RGERD) patients off therapy. We aimed to evaluate effects of esophageal body dysmotility on reflux parameters in RGERD patients by combining impedance-pH monitoring and high-resolution manometry (HRM). Methods: We retrospectively reviewed the impedance-pH data and HRM metrics in patients with refractory gastroesophageal reflux symptoms...
September 6, 2016: Journal of Neurogastroenterology and Motility
Sascha Ketelhut, Florian Milatz, Walter Heise, Reinhard G Ketelhut
BACKGROUND: Regular physical activity is known to reduce arterial pressure (BP). In a previous investigation, we could prove that even a single bout of moderate-intensity continuous exercise (MICE) causes a prolonged reduction in BP. Whether high-intensity interval training (HIIT) has a favourable influence on BP, and therefore may be followed subjects and methods by a prolonged BP reduction, should be examined on the basis of blood pressure response after exercise and during a subsequent stress test...
September 2016: VASA. Zeitschrift Für Gefässkrankheiten
Larisa Rudenko, Leena Yeolekar, Irina Kiseleva, Irina Isakova-Sivak
Influenza is a viral infection that affects much of the global population each year. Vaccination remains the most effective tool for preventing the disease. Live attenuated influenza vaccine (LAIV) has been used since the 1950s to protect humans against seasonal influenza. LAIVs developed by the Institute of Experimental Medicine (IEM), Saint Petersburg, Russia, have been successfully used in Russia since 1987. In 2006, the World Health Organization (WHO) announced a Global action plan for influenza vaccines (GAP)...
October 26, 2016: Vaccine
Alessandra Gorini, Ketti Mazzocco, Haridimos Kondylakis, Gordon McVie, Gabriella Pravettoni
The uniqueness of a patient as determined by the integration of clinical data and psychological aspects should be the aspired aim of a personalized medicine approach. Nevertheless, given the time constraints usually imposed by the clinical setting, it is not easy for physicians to collect information about the patient's unique mental dimensions and needs related to her illness. Such information may be useful in tailoring patient-physician communication, improving the patient's understanding of provided information, her involvement in the treatment process, and in general her empowerment during and after the therapeutic journey...
2016: Ecancermedicalscience
Christian Meisel, Dietmar Plenz, Andreas Schulze-Bonhage, Heinz Reichmann
Pathologic increases in excitability levels of cortical tissue commonly underlie the initiation and spread of seizure activity in patients with epilepsy. By reducing the excitability levels in neural tissue, antiepileptic drug (AED) pharmacotherapy aims to reduce seizure severity and frequency. However, AEDs may also bring about adverse effects, which have been reported to increase with higher AED load. Measures that monitor the dose-dependent effects of AEDs on cortical tissue and quantify its excitability level are therefore of prime importance for efficient clinical care and treatment but have been difficult to identify...
August 26, 2016: Epilepsia
Nora King, Romina Alvizures, Pablo García, Ann Wessel, Peter Rohloff
BACKGROUND: Argininemia is rare inborn error of metabolism which, when untreated, presents in late infancy with growth delay and developmental regression. In developed countries, argininemia is diagnosed early by newborn screening and is treated immediately with a protein-restricted diet. In developing countries, diagnosis may be delayed by the assumption that stunting is related to malnutrition alone. CASE PRESENTATION: We describe the diagnosis and treatment of argininemia in a 60-month-old Kaqchikel Maya girl in rural Guatemala...
2016: BMC Pediatrics
Hai-Xing Chang, Qian Fu, Yun Huang, Ao Xia, Qiang Liao, Xun Zhu, Ya-Ping Zheng, Chi-He Sun
To eliminate the negative impacts of pollutants in wastewater (such as suspended solids, excess N, P, heavy metals) on microalgae growth, an annular ion-exchange-membrane photobioreactor (IEM-PBR) was proposed in this study. The IEM-PBR could avoid direct mixing of algae cells with wastewater by separating them into two chambers. In the IEM-PBR, the nutrients (mainly N and P) in wastewater continuously permeated into microalgae cultures through the ion-exchange-membrane for microalgae growth, while the pollutants hardly permeated into microalgae cultures...
November 2016: Bioresource Technology
Zabedah Md Yunus, Salina Abdul Rahman, Yew Sing Choy, Wee Teik Keng, Lock Hock Ngu
BACKGROUND: The aim of this study was to determine the feasibility of performing newborn screening (NBS) of inborn errors of metabolism (IEMs) using tandem mass spectrometry (TMS) and the impact on its detection rate in Malaysia. METHODS: During the study period between June 2006 and December 2008, 30,247 newborns from 11 major public hospitals in Malaysia were screened for 27 inborn errors of amino acid, organic acid and fatty acid metabolism by TMS. Dried blood spot (DBS) samples were collected between 24 h and 7 days with parental consent...
September 1, 2016: Journal of Pediatric Endocrinology & Metabolism: JPEM
Kaleem Anwar Mir, Pallav Purohit, Gary A Goldstein, Rajasekhar Balasubramanian
This study aims to assess the current and future air pollution and associated health impacts in Pakistan. In this study, the Pakistan Integrated Energy Model (Pak-IEM) is used to assess current and future energy consumption in Pakistan. To assess air pollution levels and associated health impacts, we used the Greenhouse gas and Air pollution INteractions and Synergies (GAINS) model. A linkage has been established between both the models to feed the energy outputs from Pak-IEM into GAINS for exploring different scenarios...
August 14, 2016: Environmental Science and Pollution Research International
Tiago Alves Jorge de Souza, Gabriel Jose de Carli, Tiago Campos Pereira
An unconventional interaction between a patient and parasites was recently reported, in which parasitic cells invaded host's tissues, establishing several tumors. This finding raises various intriguing hypotheses on unpredicted forms of interplay between a patient and infecting parasites. Here we present four unusual hypothetical host-parasite scenarios with intriguing medical consequences. Relatively simple experimental designs are described in order to evaluate such hypotheses. The first one refers to the possibility of metabolic disorders in parasites intoxicating the host...
September 2016: Medical Hypotheses
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