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https://www.readbyqxmd.com/read/28105733/vitamin-d-supplementation-for-sickle-cell-disease
#1
REVIEW
Htoo Htoo Kyaw Soe, Adinegara Bl Abas, Nan Nitra Than, Han Ni, Jaspal Singh, Abdul Razzak Bin Mohd Said, Ifeyinwa Osunkwo
BACKGROUND: Sickle cell disease is a genetic chronic haemolytic and pro-inflammatory disorder. The clinical manifestations of sickle cell disease result from the presence of mutations on the beta globin genes that generate an abnormal haemoglobin product (called haemoglobin S) within the red blood cell. Sickle cell disease can lead to many complications such as acute chest syndrome, stroke, acute and chronic bone complications (including painful vaso-occlusive crisis, osteomyelitis, osteonecrosis and osteoporosis)...
January 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28104394/co-segregation-of-the-m-1555a-g-mutation-in-the-mt-rnr1-gene-and-mutations-in-mt-atp6-gene-in-a-family-with-dilated-mitochondrial-cardiomyopathy-and-hearing-loss-a-whole-mitochondrial-genome-screening
#2
Olfa Alila-Fersi, Imen Chamkha, Imen Majdoub, Lamia Gargouri, Emna Mkaouar-Rebai, Mouna Tabebi, Abdelaziz Tlili, Leila Keskes, Abdelmajid Mahfoudh, Faiza Fakhfakh
Mitochondrial disease refers to a heterogeneous group of disorders resulting in defective cellular energy production due to dysfunction of the mitochondrial respiratory chain, which is responsible for the generation of most cellular energy. Because cardiac muscles are one of the high energy demanding tissues, mitochondrial cardiomyopathies is one of the most frequent mitochondria disorders. Mitochondrial cardiomyopathy has been associated with several point mutations of mtDNA in both genes encoded mitochondrial proteins and mitochondrial tRNA and rRNA...
January 16, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28101344/evaluation-of-pharmacological-relaxation-effect-of-the-natural-product-naringin-on-in-vitro-cultured-airway-smooth-muscle-cells-and-in-vivo-ovalbumin-induced-asthma-balb-c-mice
#3
Yue Wang, Yun Lu, Mingzhi Luo, Xiaohao Shi, Yan Pan, Huilong Zeng, Linhong Deng
Asthma has become a common chronic respiratory disease worldwide and its prevalence is predicted to continue increasing in the next decade, particularly in developing countries. A key component in asthma therapy is to alleviate the excessive bronchial airway narrowing ultimately due to airway smooth muscle contraction, which is often facilitated by a smooth muscle relaxant, such as the β2-adrenergic agonists. Recently, bitter taste receptor (TAS2R) agonists, including saccharin and chloroquine, have been found to potently relax the airway smooth muscle cells (ASMCs) via intracellular Ca(2+) signaling...
December 2016: Biomedical Reports
https://www.readbyqxmd.com/read/28099113/rage-deficiency-predisposes-mice-to-virus-induced-paucigranulocytic-asthma
#4
Jaisy Arikkatt, Md Ashik Ullah, Kirsty Renfree Short, Vivan Zhang, Wan Jun Gan, Zhixuan Loh, Rhiannon B Werder, Jennifer Simpson, Peter D Sly, Stuart B Mazzone, Kirsten M Spann, Manuel Ar Ferreira, John W Upham, Maria B Sukkar, Simon Phipps
Asthma is a chronic inflammatory disease. Although many patients with asthma develop type-2 dominated eosinophilic inflammation, a number of individuals develop paucigranulocytic asthma, which occurs in the absence of eosinophilia or neutrophilia. The aetiology of paucigranulocytic asthma is unknown. However, both respiratory syncytial virus (RSV) infection and mutations in the receptor for advanced glycation endproducts (RAGE) are risk factors for asthma development. Here, we show that RAGE deficiency impairs anti-viral immunity during an early-life infection with pneumonia virus of mice (PVM; a murine analogue of RSV)...
January 18, 2017: ELife
https://www.readbyqxmd.com/read/28099079/lymphangioleiomyomatosis-a-monogenic-model-of-malignancy
#5
Vera P Krymskaya, Francis X McCormack
Lymphangioleiomyomatosis (LAM) is a rare, low-grade, metastasizing neoplasm that arises from an unknown source, spreads via the lymphatics, and targets the lungs. All pulmonary structures become infiltrated with benign-appearing spindle and epithelioid cells (LAM cells) that express smooth-muscle and melanocyte-lineage markers, harbor mTOR-activating mutations in tuberous sclerosis complex (TSC) genes, and recruit abundant stromal cells. Elaboration of lymphangiogenic growth factors and matrix remodeling enzymes by LAM cells enables their access to lymphatic channels and likely drives the cystic lung remodeling that often culminates in respiratory failure...
January 14, 2017: Annual Review of Medicine
https://www.readbyqxmd.com/read/28096431/activation-of-respiratory-muscles-does-not-occur-during-cold-submergence-in-bullfrogs-lithobates-catesbeianus
#6
Joseph M Santin, Lynn K Hartzler
Semiaquatic frogs may not breathe air for several months because they overwinter in ice-covered ponds. In contrast to many vertebrates that experience decreased motor performance after inactivity, respiratory motor function in bullfrogs, Lithobates catesbeianus, remains functional following cold-submergence. Unlike mammalian hibernators with unloaded limb muscles and inactive locomotor systems, respiratory mechanics of frogs counterintuitively allow for ventilatory maneuvers when submerged. Thus we hypothesized that bullfrogs generate respiratory motor patterns during cold-submergence to avoid disuse and preserve motor performance...
January 17, 2017: Journal of Experimental Biology
https://www.readbyqxmd.com/read/28093429/paediatric-ed-bipap-continuous-quality-improvement-programme-with-patient-analysis-2005-2013
#7
Thomas Abramo, Abby Williams, Samaiya Mushtaq, Mark Meredith, Rawle Sepaule, Kristen Crossman, Cheryl Burney Jones, Suzanne Godbold, Zhuopei Hu, Todd Nick
OBJECTIVE: In paediatric moderate-to-severe asthmatics, there is significant bronchospasm, airway obstruction, air trapping causing severe hyperinflation with more positive intraplural pressure preventing passive air movement. These effects cause an increased respiratory rate (RR), less airflow and shortened inspiratory breath time. In certain asthmatics, aerosols are ineffective due to their inadequate ventilation. Bilevel positive airway pressure (BiPAP) in acute paediatric asthmatics can be an effective treatment...
January 16, 2017: BMJ Open
https://www.readbyqxmd.com/read/28093112/clinical-indicators-of-blood-gas-disturbances-elevated-l-lactate-concentration-and-other-abnormal-blood-parameters-in-newborn-beef-calves
#8
E R Homerosky, N A Caulkett, E Timsit, E A Pajor, J P Kastelic, M C Windeyer
Blood gas disturbances, commonly resulting from dystocia, are associated with failed transfer of passive immunity, morbidity and mortality in newborn calves. Modified APGAR scores intended to identify compromised calves are not widely adopted due to lack of practicality and inconsistent associations with blood parameters. The objective of this study was to determine clinical indicators of acidemia in newborn beef calves. Blood parameters at 10 min and 24 h after birth were compared to at-birth clinical examination parameters and calving characteristics in 77 commercial beef calves...
January 2017: Veterinary Journal
https://www.readbyqxmd.com/read/28091836/assessment-of-edema-using-stir-via-3d-cardiovascular-magnetic-resonance-imaging-in-patients-with-suspected-myocarditis
#9
Michael Jeserich, Bela Merkely, Pascal Schlosser, Simone Kimmel, Gabor Pavlik, Stephan Achenbach
OBJECTIVE: To evaluate three-dimensional T2-weighted fast spin echo triple inversion recovery sequences (STIR+) for the diagnosis of myocardial edema in patients with suspected early myocarditis after respiratory or gastrointestinal tract viral infection and at follow-up. MATERIALS AND METHODS: We prospectively examined 28 patients with suspected myocarditis and 37 controls matched for gender and age. An ECG-triggered STIR+ was used to cover the entire left ventricle in short-axis images with 10-mm slice thickness and no interslice gap...
January 16, 2017: Magma
https://www.readbyqxmd.com/read/28087734/increased-mitophagy-in-the-skeletal-muscle-of-spinal-and-bulbar-muscular-atrophy-patients
#10
Doriana Borgia, Adriana Malena, Marco Spinazzi, Maria Andrea Desbats, Leonardo Salviati, Aaron P Russell, Giovanni Miotto, Laura Tosatto, Elena Pegoraro, Gianni Sorarù, Maria Pennuto, Lodovica Vergani
Spinal and bulbar muscular atrophy (SBMA) is a neuromuscular disorder caused by polyglutamine expansion in the androgen receptor (AR) and characterized by the loss of lower motor neurons. Here we investigated pathological processes occurring in muscle biopsy specimens derived from SBMA patients and, as controls, age-matched healthy subjects and patients suffering from amyotrophic lateral sclerosis (ALS) and neurogenic atrophy. We detected atrophic fibers in the muscle of SBMA, ALS and neurogenic atrophy patients...
January 13, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28087340/electrophysiological-alterations-in-diaphragm-muscle-caused-by-abdominal-ischemia-reperfusion
#11
Seckin Tuncer, Nizamettin Dalkilic, Ilksen Burat
Ischemia-reperfusion injury is the major complication of abdominal aortic surgery, and it mainly affects the lower extremities and remote organs. In the present study, the electrophysiological alterations in diaphragm that underlie the post-operative respiratory dysfunction were investigated. Wistar Albino rats were randomly divided into two groups: SHAM (only laparotomy was performed) and IR (abdominal aorta was clamped for 30min and reperfused for 2h). Following the operational period diaphragm muscles were isolated and electrophysiological experiments were carried out in-vitro...
January 10, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28087067/-successful-use-of-sugammadex-for-caesarean-section-in-a-patient-with-myasthenia-gravis
#12
Lokman Soyoral, Ugur Goktas, Muhammed Bilal Cegin, Volkan Baydi
Myasthenia gravis is an autoimmune disorder that is characterized by muscle weakness that fluctuates, worsening with exertion, and improving with rest. Diagnosis of myasthenia gravis is made following clinical and physical examination and is confirmed by serum immunoassays to measure autoantibody levels. Myasthenia gravis especially when associated with pregnancy is a high-risk disease, and its course is unpredictable. We described the second report about use of sugammadex after rocuronium for a caesarean delivery with myasthenia gravis, but, unlike our case that formerly was diagnosed with myasthenia gravis, the patient was extubated on postoperative successfully and we did not encounter any respiratory problems...
January 10, 2017: Revista Brasileira de Anestesiologia
https://www.readbyqxmd.com/read/28087053/six-minute-walk-test-results-predict-risk-of-hospitalization-for-youths-with-cystic-fibrosis-a-5-year-follow-up-study
#13
Márcio V F Donadio, João P Heinzmann-Filho, Fernanda M Vendrusculo, Patrícia X H Frasson, Paulo J C Marostica
OBJECTIVE: To evaluate the association of 6-minute walk test (6MWT) and other variables (anthropometry, chronic Pseudomonas aeroginosa colonization, pulmonary function, and respiratory muscle strength) with the risk of hospitalization for pulmonary exacerbation in children and adolescents with cystic fibrosis (CF). STUDY DESIGN: Cohort study that included patients with CF aged 6-18 years. All participants underwent spirometry, manovacuometry, and 6MWT during the 5-year follow-up...
January 10, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28079665/anesthetic-management-of-a-patient-with-antimuscle-specific-kinase-antibody-positive-myasthenia-gravis-undergoing-an-open-cholecystectomy-a-case-report
#14
Masahiko Akatsu, Yukihiro Ikegami, Choichiro Tase, Koichi Nishikawa
Myasthenia gravis (MG) is an autoimmune disease characterized by the production of antibodies against the acetylcholine receptor, muscle-specific kinase (MuSK), or other proteins at the neuromuscular junction. MG with antibodies against MuSK (MuSK-MG) has been described recently. Here, we report the first case of anesthetic management of a patient with MuSK-MG undergoing an open cholecystectomy. In our case, propofol and remifentanil-based anesthesia were used for successful management without using muscle relaxants...
January 11, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28079615/neuromuscular-weakness-in-chronic-obstructive-pulmonary-disease-chest-wall-diaphragm-and-peripheral-muscle-contributions
#15
Adam Alter, Loutfi S Aboussouan, Eduardo Mireles-Cabodevila
PURPOSE OF REVIEW: Chronic obstructive lung disease affects the lung parenchyma and airways leading to well described effects in respiratory function. This review describes the current knowledge and advances regarding neuromuscular function and chest wall mechanics, which are affected in chronic obstructive pulmonary disease (COPD). RECENT FINDINGS: In COPD, progressive lung hyperinflation becomes constrained by a chest wall with decreasing capacity to expand, resulting in respiratory muscle inefficiency...
January 11, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28078253/effects-of-eicosapentaenoic-acid-and-docosahexaenoic-acid-on-mitochondrial-dna-replication-and-pgc-1%C3%AE-gene-expression-in-c2c12-muscle-cells
#16
Mak-Soon Lee, Yoonjin Shin, Sohee Moon, Seunghae Kim, Yangha Kim
Mitochondrial biogenesis is a complex process requiring coordinated expression of nuclear and mitochondrial genomes. The peroxisome proliferator-activated receptor gamma co-activator 1-alpha (PGC-1α) is a key regulator of mitochondrial biogenesis, and it controls mitochondrial DNA (mtDNA) replication within diverse tissues, including muscle tissue. The aim of this study was to investigate the effects of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) on mtDNA copy number and PGC-1α promoter activity in C2C12 muscle cells...
December 2016: Preventive Nutrition and Food Science
https://www.readbyqxmd.com/read/28078065/clinical-features-of-neuromuscular-disorders-in-patients-with-n-type-voltage-gated-calcium-channel-antibodies
#17
Andreas Totzeck, Petra Mummel, Oliver Kastrup, Tim Hagenacker
Neuromuscular junction disorders affect the pre- or postsynaptic nerve to muscle transmission due to autoimmune antibodies. Members of the group like myasthenia gravis and Lambert-Eaton syndrome have pathophysiologically distinct characteristics. However, in practice, distinction may be difficult. We present a series of three patients with a myasthenic syndrome, dropped-head syndrome, bulbar and respiratory muscle weakness and positive testing for anti-N-type voltage-gated calcium channel antibodies. In two cases anti-acetylcholin receptor antibodies were elevated, anti-P/Q-type voltage-gated calcium channel antibodies were negative...
September 15, 2016: European Journal of Translational Myology
https://www.readbyqxmd.com/read/28077823/association-of-health-symptoms-with-low-level-exposure-to-organophosphates-dna-damage-ache-activity-and-occupational-knowledge-and-practice-among-rice-corn-and-double-crop-farmers
#18
Surat Hongsibsong, Nalin Sittitoon, Ratana Sapbamrer
OBJECTIVES: This study aims to determine (1) total dialkylphosphate (ΣDAP) levels, occupational knowledge and practice, DNA damage, AChE activity, and health symptoms in rice, corn, and double-crop farmers; (2) the association of health symptoms with ΣDAP levels, occupational knowledge and practice, DNA damage, and AChE activity in farmers; and (3) the prevalence of health symptoms between farmers and non-farmers. METHODS: A cross-sectional study was conducted by interviewing as well as analyzing urine and blood samples during July to August 2014...
January 11, 2017: Journal of Occupational Health
https://www.readbyqxmd.com/read/28076295/convolutive-blind-source-separation-of-surface-emg-measurements-of-the-respiratory-muscles
#19
Eike Petersen, Herbert Buchner, Marcus Eger, Philipp Rostalski
Electromyography (EMG) has long been used for the assessment of muscle function and activity and has recently been applied to the control of medical ventilation. For this application, the EMG signal is usually recorded invasively by means of electrodes on a nasogastric tube which is placed inside the esophagus in order to minimize noise and crosstalk from other muscles. Replacing these invasive measurements with an EMG signal obtained non-invasively on the body surface is difficult and requires techniques for signal separation in order to reconstruct the contributions of the individual respiratory muscles...
January 11, 2017: Biomedizinische Technik. Biomedical Engineering
https://www.readbyqxmd.com/read/28073937/activity-of-tachykinin1-expressing-pet1-raphe-neurons-modulates-the-drive-to-breathe
#20
Morgan L Hennessy, Andrea Corcoran, Rachael D Brust, Eugene E Nattie, Susan Dymecki
: Homeostatic control of breathing, heart rate, and body temperature relies on circuits within the brainstem modulated by the neurotransmitter serotonin (5-HT). Mounting evidence points to specialized neuronal subtypes within the 5-HT system, which have borne out in functional studies, including the modulation of distinct facets of homeostatic control. These functional differences, read out at the organismal level, are likely subserved by differences among 5-HT neuron subtypes at the cellular and molecular levels, including differences in the capacity to co-express other neurotransmitters such as glutamate, GABA, thyrotropin releasing hormone, and substance P encoded by the Tachykinin-1 (Tac1) gene...
January 10, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
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