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https://www.readbyqxmd.com/read/29765992/serological-immunoglobulin-free-light-chain-profile-in-myasthenia-gravis-patients
#1
Umberto Basile, Mariapaola Marino, Cecilia Napodano, Krizia Pocino, Paolo Emilio Alboini, Francesca Gulli, Amelia Evoli, Carlo Provenzano, Emanuela Bartoccioni
Background: Serological levels of free immunoglobulin light chains (FLCs), produced in excess of heavy chains during synthesis of immunoglobulins by plasma cells, can be considered a direct marker of B cell activity in different systemic inflammatory-autoimmune conditions and may represent a useful predictor of rituximab (RTX) therapeutic efficacy, as reported for rheumatoid arthritis and systemic lupus erythematosus. Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction with antibodies (abs) targeting the acetylcholine receptor (AChR) or the muscle-specific tyrosine kinase (MuSK), inducing muscle weakness and excessive fatigability...
2018: Journal of Immunology Research
https://www.readbyqxmd.com/read/29758177/the-efficacy-of-antenatal-steroid-therapy-is-dependent-on-the-duration-of-low-concentration-fetal-exposure-evidence-from-a-sheep-model-of-pregnancy
#2
Matthew W Kemp, Masatoshi Saito, Haruo Usuda, Shimpei Watanabe, Shinichi Sato, Takushi Hanita, Yusaku Kumagai, Timothy J Molloy, Michael Clarke, Peter J Eddershaw, Gabrielle C Musk, Augusto Schmidt, Demelza Ireland, Lucy Furfaro, Matthew S Payne, John P Newnham, Alan H Jobe
OBJECTIVE: Antenatal corticosteroids (ANS) are among the most important and widely used interventions to improve outcomes for preterm infants. ANS dosing regimens remain un-optimized and without maternal weight-adjusted dosing. We, and others, have hypothesised that once a low concentration of materno-fetal steroid exposure is achieved and maintained, the duration of the steroid exposure determines treatment efficacy. Using a sheep model of pregnancy, we tested the relationship between steroid dose, and duration of exposure, and treatment efficacy...
May 9, 2018: American Journal of Obstetrics and Gynecology
https://www.readbyqxmd.com/read/29742795/rituximab-in-refractory-myasthenia-gravis-extended-prospective-study-results
#3
Grayson Beecher, Dustin Anderson, Zaeem A Siddiqi
INTRODUCTION: Rituximab appears beneficial in treatment-refractory myasthenia gravis (MG), however, prospective, long-term durability data is lacking. METHODS: In this prospective, open-label study of rituximab in refractory MG, 22 patients (10 AChR, 9 MuSK, 3 seronegative) received rituximab at baseline, with repeat cycles driven by clinical worsening. Manual muscle testing (MMT) scores and CD19/CD20+ B cell counts were serially monitored. RESULTS: At mean follow-up of 28...
May 9, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29736936/hla-and-musk-positive-myasthenia-gravis-a-systemic-review-and-meta-analysis
#4
Y Hong, H-F Li, F Romi, G O Skeie, N E Gilhus
OBJECTIVES: Myasthenia gravis (MG) represents a spectrum of clinical subtypes with differences in disease mechanisms and treatment response. MG with muscle-specific tyrosine kinase (MuSK) antibodies accounts for 1%-10% of all MG patients. We conducted a meta-analysis to evaluate the association between HLA genes and MuSK-MG susceptibility. SUBJECTS AND METHODS: Studies were searched in Pubmed, EMBASE database and other sources between 2001 and 2018. Genotype, allele and haplotype frequencies of HLA loci in MuSK-MG patients and healthy controls were extracted from each included study...
May 7, 2018: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29728025/-explore-compatibility-mechanism-of-xingnaojing-injection-in-treating-cerebral-infarction-based-on-network-pharmacology
#5
Fu-Jiang Song, Bin Fan, Jian Sun
This paper aimed to explore the molecular mechanism of Xingnaojing injection and its scientific connotation of compatibility mechanism from a new perspective on bioinformatics and network biology. Based on the analysis function of intergrative pharmacology platform V1.0, the compatibility mechanism of this prescription was investigated by constructing the herbs-compounds-targets network. Seven hundreds and ninety-five targets from the prescription were screened out, then 302 hub nodes were included in drug targets-disease targets network...
April 2018: Zhongguo Zhong Yao za Zhi, Zhongguo Zhongyao Zazhi, China Journal of Chinese Materia Medica
https://www.readbyqxmd.com/read/29727940/multi-component-determination-of-atmospheric-semi-volatile-organic-compounds-in-soils-and-vegetation-from-tarragona-county-catalonia-spain
#6
Noelia Domínguez-Morueco, Mariana Carvalho, Jordi Sierra, Marta Schuhmacher, José Luis Domingo, Nuno Ratola, Martí Nadal
Tarragona County (Spain) is home to the most important chemical/petrochemical industrial complex in Southern Europe, which raises concerns about the presence and effects of the numerous environmental contaminants. In order to assess the levels and patterns of five classes of semi-volatile organic compounds (SVOCs) - polycyclic aromatic hydrocarbons (PAHs), synthetic musks (SMs), polychlorinated biphenyls (PCBs), brominated flame retardants (BFRs) and one organochlorine pesticide, hexachlorobenzene (HCB), 27 samples of soil and vegetation (Piptatherum L...
August 1, 2018: Science of the Total Environment
https://www.readbyqxmd.com/read/29709294/occurrence-profile-and-spatial-distribution-of-uv-filters-and-musk-fragrances-in-mussels-from-portuguese-coastline
#7
M Castro, J O Fernandes, A Pena, S C Cunha
The increasing production and consumption of Personal Care Products (PCPs), containing UV-filters and musk fragrances, has led to its widespread presence in the aquatic environment which can cause harmful effects to the aquatic organisms due to its intrinsic toxicity. This study aims to evaluate the degree of contamination of wild mussels along the entire Portuguese coastline, continually exposed in their habitat to different contaminants. For this purpose, approximately 1000 mussel specimens were sampled during one year in seven different locations, along the Portuguese coastline...
April 23, 2018: Marine Environmental Research
https://www.readbyqxmd.com/read/29704306/recessive-variants-of-musk-are-associated-with-late-onset-cms-and-predominant-limb-girdle-weakness
#8
David Owen, Ana Töpf, Veeramani Preethish-Kumar, Paolo José Lorenzoni, Bas Vroling, Rosana Herminia Scola, Elza Dias-Tosta, Argemiro Geraldo, Kiran Polavarapu, Saraswati Nashi, Daniel Cox, Teresinha Evangelista, John Dawson, Rachel Thompson, Jan Senderek, Steven Laurie, Sergi Beltran, Marta Gut, Ivo Gut, Atchayaram Nalini, Hanns Lochmüller
Congenital myasthenic syndrome (CMS) is a heterogeneous disorder that causes fatigable muscle weakness. CMS has been associated with variants in the MuSK gene and, to date, 16 patients have been reported. MuSK-CMS patients present a different phenotypic pattern of limb girdle weakness. Here, we describe four additional patients and discuss the phenotypic and clinical relationship with those previously reported. Two novel damaging missense variants are described: c.1742T > A; p.I581N found in homozygosis, and c...
April 28, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29673420/neurologic-complications-associated-with-transdermal-placement-of-intrathecal-catheters-in-sheep
#9
Gabrielle C Musk, Nolan J McDonnell, Mark Newman, Matthew W Kemp
A study using an ovine model of transdermal intrathecal catheterization was planned to investigate the neurotoxicity ofmagnesium sulfate. Nonpregnant Merino cross ewes (n = 8; age, 5 y; weight, 55.0 ± 6.5 kg) were anesthetized for placementof a lumbar intrathecal catheter. The study protocol defined a 5-d recovery period after introduction of the catheter before theadministration of test substances (2 mL of 0.9% saline or 50 or 150 mg MgSO4) followed by euthanasia 1 wk later. Although 3sheep successfully completed the study as planned, one of the remaining 5 sheep was withdrawn when the catheter was accidentally dislodged 2 d after anesthesia; another was withdrawn because of persistent neurologic deficits of the left hindlimb and intense pruritus during the first 24 h after placement of the catheter; and the remaining 3 animals experienced unacceptable complications within the first 4 h of administration of the test substance...
April 19, 2018: Comparative Medicine
https://www.readbyqxmd.com/read/29671605/effects-of-budesonide-and-surfactant-in-preterm-fetal-sheep
#10
T Brett Kothe, Emily Royse, Matthew W Kemp, Augusto F Schmidt, Fabrizio Salomone, Masatoshi Saito, Haruo Usuda, Shimpei Watanabe, Gabrielle C Musk, Alan H Jobe, Noah H Hillman
BACKGROUND: Mechanical ventilation causes lung injury and systemic inflammatory responses in preterm sheep, and is associated with bronchopulmonary dysplasia (BPD) in preterm infants. Budesonide added to surfactant decreased BPD by 20% in infants. OBJECTIVE: Determine the effects of budesonide and surfactant on injury from high tidal volume (VT) ventilation in preterm lambs. METHODS: Ewes at 125 {plus minus} 1 day GA had fetal surgery to expose fetal head and chest with placental circulation intact...
April 19, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29670572/myasthenia-gravis-with-thymoma-manifesting-as-achr-ab-positive-distinct-bulbar-palsy-accompanied-by-dysgeusia-a-case-series-and-review-of-literature
#11
Kai Zhu, Jiaxin Chen, Jingjing Li, Haiyan Wang, Xin Huang, Yan Li, Huiyu Feng
In this review, we summarized three cases of myasthenia gravis (MG) with taste disorder and describe their clinical features in detail. Three MG patients presented with significant bulbar palsy symptoms, high AChR-Ab titers, and negative MuSK-Ab, were diagnosed with thymoma. Furthermore, we observed that dysgeusia could manifest earlier than the occurrence of typical MG symptoms, even predict a MG relapse or a myasthenic crisis in the course of MG. We believe that dysgeusia is a non-motor symptom of MG, which especially exists in MG patients with thymoma and serious bulbar palsy...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29663639/a-novel-ecel1-mutation-expands-the-phenotype-of-distal-arthrogryposis-multiplex-congenita-type-5d-to-include-pretibial-vertical-skin-creases
#12
Eva-Lena Stattin, Josefin Johansson, Sanna Gudmundsson, Adam Ameur, Staffan Lundberg, Marie-Louise Bondeson, Maria Wilbe
Arthrogryposis multiplex congenita (AMC) is a heterogeneous disorder characterized by multiple joint contractures often in association with other congenital abnormalities. Pretibial linear vertical creases are a rare finding associated with arthrogryposis, and the etiology of the specific condition is unknown. We aimed to genetically and clinically characterize a boy from a consanguineous family, presenting with AMC and pretibial vertical linear creases on the shins. Whole exome sequencing and variant analysis revealed homozygous novel missense variants of ECEL1 (c...
April 16, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29656754/malignant-cells-from-pleural-fluids-in-malignant-mesothelioma-patients-reveal-novel-mutations
#13
Sophie Sneddon, Ian Dick, Y C Gary Lee, A W Bill Musk, Ann-Marie Patch, John V Pearson, Nicola Waddell, Richard J N Allcock, Robert A Holt, Bruce W S Robinson, Jenette Creaney
OBJECTIVES: Malignant mesothelioma (MM) is an asbestos related tumour affecting cells of serosal cavities. More than 70% of MM patients develop pleural effusions which contain tumour cells, representing a readily accessible source of malignant cells for genetic analysis. Although common somatic mutations and losses have been identified in solid MM tumours, the characterization of tumour cells within pleural effusions could provide novel insights but is little studied. MATERIALS AND METHODS: DNA and RNA were extracted from cells from short term cultures of 27 human MM pleural effusion samples...
May 2018: Lung Cancer: Journal of the International Association for the Study of Lung Cancer
https://www.readbyqxmd.com/read/29655451/muscle-specific-tyrosine-kinase-and-myasthenia-gravis-owing-to-other-antibodies
#14
REVIEW
Michael H Rivner, Mamatha Pasnoor, Mazen M Dimachkie, Richard J Barohn, Lin Mei
Around 20% of patients with myasthenia gravis are acetylcholine receptor antibody negative; muscle-specific tyrosine kinase antibodies (MuSK) were identified as the cause of myasthenia gravis in 30% to 40% of these cases. Anti MuSK myasthenia gravis is associated with specific clinical phenotypes. One is a bulbar form with fewer ocular symptoms. Others show an isolated head drop or symptoms indistinguishable from acetylcholine receptor-positive myasthenia gravis. These patients usually respond well to immunosuppressive therapy, but not as well to cholinesterase inhibitors...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655446/practical-anatomy-of-the-neuromuscular-junction-in-health-and-disease
#15
REVIEW
Hiroshi Nishimune, Kazuhiro Shigemoto
Neuromuscular junctions (NMJs) form between nerve terminals of spinal cord motor neurons and skeletal muscles, and perisynaptic Schwann cells and kranocytes cap NMJs. One muscle fiber has one NMJ, which is innervated by one motor nerve terminal. NMJs are excitatory synapses that use P/Q-type voltage-gated calcium channels to release the neurotransmitter acetylcholine. Acetylcholine receptors accumulate at the postsynaptic specialization called the end plate on the muscle fiber membrane, the sarcolemma. Proteins essential for the organization of end plates include agrin secreted from nerve terminals, Lrp4 and MuSK receptors for agrin, and Dok-7 and rapsyn cytosolic proteins in the muscle...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29652910/elderly-dendritic-cells-respond-to-lps-ifn-%C3%AE-and-cd40l-stimulation-despite-incomplete-maturation
#16
Joanne K Gardner, Scott M J Cornwall, Arthur W Musk, John Alvarez, Cyril D S Mamotte, Connie Jackaman, Anna K Nowak, Delia J Nelson
There is evidence that dendritic cells (DCs) undergo age-related changes that modulate their function with their key role being priming antigen-specific effector T cells. This occurs once DCs develop into antigen-presenting cells in response to stimuli/danger signals. However, the effects of aging on DC responses to bacterial lipopolysaccharide (LPS), the pro-inflammatory cytokine interferon (IFN)-γ and CD40 ligand (CD40L) have not yet been systematically evaluated. We examined responses of blood myeloid (m)DC1s, mDC2s, plasmacytoid (p)DCs, and monocyte-derived DCs (MoDCs) from young (21-40 years) and elderly (60-84 years) healthy human volunteers to LPS/IFN-γ or CD40L stimulation...
2018: PloS One
https://www.readbyqxmd.com/read/29650477/determination-of-24-personal-care-products-in-fish-bile-using-hybrid-solvent-precipitation-and-dispersive-solid-phase-extraction-cleanup-with-ultrahigh-performance-liquid-chromatography-tandem-mass-spectrometry-and-gas-chromatography-mass-spectrometry
#17
Li Yao, Yin-Zhi Lv, Li-Juan Zhang, Wang-Rong Liu, Jian-Liang Zhao, You-Sheng Liu, Qian-Qian Zhang, Guang-Guo Ying
Personal care products (PCPs) are ubiquitous in aquatic environments owing to the continuous discharge of domestic wastewater from highly urbanized regions. These PCPs can be adsorbed by fish and thereafter usually enter the bile of the fish through biliary excretion. In this study, a sensitive method based on a combination of hybrid solvent precipitation and dispersive solid phase extraction (d-SPE) purification was developed to simultaneously extract and detect 24 PCPs, namely, 16 biocides, 4 synthetic musks, and 4 benzotriazoles, from fish bile...
April 5, 2018: Journal of Chromatography. A
https://www.readbyqxmd.com/read/29605991/deriving-in-vivo-bioconcentration-factors-of-a-mixture-of-fragrance-ingredients-using-a-single-dietary-exposure-and-internal-benchmarking
#18
Chang-Er L Chen, Karin Löfstrand, Margaretha Adolfsson-Erici, Michael S McLachlan, Matthew MacLeod
Chemicals in mixtures that are hydrophobic with logKow > 4 are potentially bioaccumulative. Here, we evaluate an abbreviated and benchmarked in vivo BCF measurement methodology by exposing rainbow trout to a mixture of eight test chemicals found in fragrance substances and three benchmark chemicals (musk xylene (MX), hexachlorobenzene (HCB) and PCB52) via a single contaminated feeding event followed by a 28-day depuration period. Concentrations of HCB and PCB52 in fish did not decline significantly (their apparent depuration rate constants - kT were close to zero), while kT for MX was 0...
March 31, 2018: Environmental Science & Technology
https://www.readbyqxmd.com/read/29604268/mild-aerobic-training-with-blood-flow-restriction-increases-the-hypertrophy-index-and-musk-in-both-slow-and-fast-muscles-of-old-rats-role-of-pgc-1%C3%AE
#19
Mohammad-Ali Bahreini Pour, Siyavash Joukar, Fariborz Hovanloo, Hamid Najafipour, Vida Naderi, Alireza Raji, Saeed Esmaeili-Mahani
AIMS: Existing evidence emphasize the role of mitochondrial dysfunction in sarcopenia which is revealed as loss of skeletal muscle mass and neuromuscular junction remodeling. We assessed the effect of low-intensity aerobic training along with blood flow restriction on muscle hypertrophy index, muscle-specific kinase (MuSK), a pivotal protein of the neuromuscular junction and Peroxisome proliferator-activated receptor gamma co-activator 1-alpha (PGC-1α) in aged male rats. MAIN METHODS: Animals groups were control (CTL), sham (Sh), leg blood flow restriction (BFR), exercise (Ex), sham + exercise (Sh + Ex), and BFR plus exercise (BFR + Ex) groups...
March 28, 2018: Life Sciences
https://www.readbyqxmd.com/read/29602799/drawing-the-line-in-clinical-treatment-of-companion-animals-recommendations-from-an-ethics-working-party
#20
Herwig Grimm, Alessandra Bergadano, Gabrielle C Musk, Klaus Otto, Polly M Taylor, Juliet Clare Duncan
Modern veterinary medicine offers numerous options for treatment and clinicians must decide on the best one to use. Interventions causing short-term harm but ultimately benefitting the animal are often justified as being in the animal's best interest. Highly invasive clinical veterinary procedures with high morbidity and low success rates may not be in the animal's best interest. A working party was set up by the European College of Veterinary Anaesthesia and Analgesia to discuss the ethics of clinical veterinary practice and improve the approach to ethically challenging clinical cases...
March 30, 2018: Veterinary Record
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