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fiber syndrome

Audrey M Bernstein, Robert Ritch, J Mario Wolosin
Exfoliation syndrome (XFS) is an age-related disease involving the deposition of aggregated fibrillar material (XFM) at extracellular matrices in tissues that synthesize elastic fibers. Its main morbidity is in the eye, where XFM accumulations form on the surface of the ciliary body, iris and lens. Exfoliation glaucoma (XFG) occurs in a high proportion of persons with XFS and can be a rapidly progressing disease. Worldwide, XFG accounts for about 25% of open-angle glaucoma cases. XFS and XFG show a sharp age-dependence, similarly to the many age-related diseases classified as aggregopathies...
March 15, 2018: Journal of Glaucoma
M A Akulov, O R Orlova, T V Tabashnikova, V V Karnaukhov, A S Orlova
Surgical treatment of posterior cranial fossa and cerebellopontine angle tumors is associated with a risk of facial nerve dysfunction. The causes for facial muscle paresis include nerve compression by the tumor, destruction of the nerve structure by the tumor growing from nerve fibers, nerve injury during surgical removal of the tumor, etc. The first 3 months after facial nerve injury are a potential therapeutic window for the use of botulinum toxin type A (BTA). During this period, the drug is introduced both in the healthy side to improve the facial symmetry at rest and during mimetic movements and in the affected side to induce drug-induced ptosis...
2018: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
Cordell M Baker, Joshua D Burks, Robert G Briggs, Adam D Smitherman, Chad A Glenn, Andrew K Conner, Dee H Wu, Michael E Sughrue
Introduction: Supplementary motor area (SMA) syndrome is a constellation of temporary symptoms that may occur following tumors of the frontal lobe. Affected patients develop akinesia and mutism but often recover within weeks to months. With our own case examples and with correlations to fiber tracking validated by gross anatomical dissection as ground truth, we describe a white matter pathway through which recovery may occur. Methods: Diffusion spectrum imaging from the Human Connectome Project was used for tractography analysis...
March 2018: Brain and Behavior
Takuya Furuta, Hiroaki Miyoshi, Satoru Komaki, Fumiko Arakawa, Motohiro Morioka, Koichi Ohshima, Mitsutoshi Nakada, Yasuo Sugita
Epithelioid glioblastoma (eGBM) is a rare variant of GBM which was adopted in the 2016 WHO classification. eGBM and pleomorphic xanthoastrocytoma (PXA) sometimes show overlapping features histologically and genetically, such as epithelioid pattern and a highly frequent V600E mutation in the gene for vRAF murine sarcoma viral oncogene homolog B1 (BRAF), respectively. Accurate diagnosis of these rare tumors is challenging according to the new criteria in the revised 2016 WHO classification. It is an urgent task to elucidate the biological properties of the tumors and to select appropriate treatment...
March 13, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Sedigheh Asgary, Ali Rastqar, Mahtab Keshvari
Cardiovascular disease (CVD) is now the leading cause of death globally and is a growing health concern. Lifestyle factors, including nutrition, play an important role in the etiology and treatment of CVD. Functional foods based on their basic nutritional functions can decrease the risk of many chronic diseases and have some physiological benefits. They contain physiologically active components either from plant or animal sources, marketed with the claim of their ability to reduce heart disease risk, focusing primarily on established risk factors, which are hyperlipidemia, diabetes, metabolic syndrome, obesity/overweight, elevated lipoprotein A level, small dense low-density lipoprotein cholesterol (LDL-C), and elevated inflammatory marker levels...
March 12, 2018: Journal of the American College of Nutrition
Kazuchika Suzuki, Hiroaki Sakai, Kenji Takahashi
We herein report anesthetic management during aortic valve replacement for aortic valve regurgitation in a patient with adult mucopolysaccharidosis type II (MPS type 2) (Hunter syndrome). This disorder is rare and related to the accumulation of a mucopolysaccharide in lysosomes. It affects various organs, including the airways, heart, and central nerves. In children with MPS type 2, the risk of airway obstruction during anesthesia/sedation is high, and the degree of difficulty increases with aging. The patient described herein was a 33-year-old male without mental retardation...
2018: JA Clin Rep
Andrew G Lee, Thomas H Mader, C Robert Gibson, Tyson J Brunstetter, William J Tarver
Interesting novel and somewhat perplexing physiologic and pathologic neuro-ocular findings have been documented in astronauts during and after long duration space flight (LDSF). These findings collectively have been termed the "space flight-associated neuro-ocular syndrome" (SANS). The National Aeronautics and Space Administration (NASA) in the United States has meticulously and prospectively documented the clinical, ultrasound, optical coherence tomography imaging, and radiographic findings of SANS including unilateral and bilateral optic disc edema, globe flattening, choroidal and retinal folds, hyperopic refractive error shifts, and nerve fiber layer infarcts (i...
March 12, 2018: Eye
Andrei D Sdrulla, Yun Guan, Srinivasa N Raja
The field of spinal cord stimulation (SCS) owes its inception to the concept of gate control theory (GCT), put forth by Wall and Melzack in their landmark 1965 paper, which proposed that "control of pain may be achieved by selectively activating the large, rapidly conducting fibers". The first reported clinical application of dorsal column stimulation came 2 years later, and the field has gradually expanded ever since. Today, an estimated 50,000 spinal cord neurostimulators are implanted annually...
March 11, 2018: Pain Practice: the Official Journal of World Institute of Pain
Khazar Ahmadi, Alessio Fracasso, Jelle A van Dijk, Charlotte Kruijt, Maria van Genderen, Serge O Dumoulin, Michael B Hoffmann
A fundamental scheme in the organization of the early visual cortex is the retinotopic representation of the contralateral visual hemifield on each hemisphere. We determined the cortical organization in a novel congenital visual pathway disorder, FHONDA-syndrome, where the axons from the temporal retina abnormally cross to the contralateral hemisphere. Using ultra-high field fMRI at 7 T, the population receptive field (pRF) properties of the primary visual cortex were modeled for two affected individuals and two controls...
March 7, 2018: NeuroImage
Ping Wang, Jianjun Zhang, Weikang Wang, Tuofan Li, Guangchen Liang, Hongxia Shao, Wei Gao, Aijian Qin, Jianqiang Ye
A recent outbreak of hepatitis-hydropericardium syndrome caused by serotype 4 fowl adenovirus (FAdV-4) has resulted in significant economic losses to the poultry industry worldwide. However, little is known about the molecular pathogenesis of FAdV-4. In this study, a novel monoclonal antibody (mAb) targeting the fiber-2 protein of FAdV-4 was generated, mAb 3C2. Indirect immunofluorescence assay showed that mAb 3C2 neither reacted with serotype 8 fowl adenovirus (FAdV-8) nor reacted with the fiber-1 protein of FAdV-4; it specifically reacted with the fiber-2 protein of FAdV-4...
March 9, 2018: Veterinary Research
Senol Tonyali, Deniz Ates, Filiz Akbiyik, Duygu Kankaya, Dilek Baydar, Ali Ergen
BACKGROUND: Interstitial cystitis (IC)/bladder pain syndrome (BPS) is a challenging disease, affecting thousands of people all around the world, especially women. Although there have been numerous theories regarding IC/BPS etiology, the physiopathology of the disease still remains unclear and there is a lack of certain treatment. OBJECTIVES: The aim of the study was to assess the role of nerve fibers and nerve growth factor (NGF) in the etiopathogenesis of IC/BPS symptoms by demonstrating if there is a correlation between urine NGF levels, amount of peripheral nerves in bladder mucosa and symptom severity...
February 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Ilaria Biasato, Renato Zanatta, Lorella Maniscalco, Rocchina Evangelista, Bryan Iotti, Selina Iussich
The unexpected demise of a 12-year-old male neutered English bulldog solicited a gross examination, which revealed a blood-filled space occurring in the proximal left subclavian artery (LSA). It originated about 1 cm from the branching point of the vessel and progressively dilated for 3 cm distal to this origin. Histopathological investigation showed that the tunica media of the LSA was more than 50% split, with the blood-filled space dissecting through the arterial wall. In the tunica media of the LSA, severe multifocal fragmentation and/or loss of the elastic fibers was observed...
March 5, 2018: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
Hongbin Zhong, Tingjun Wang, Guili Lian, Changsheng Xu, Huajun Wang, Liangdi Xie
Sinoatrial node fibrosis is involved in the pathogenesis of sinus sick syndrome (SSS). Transient receptor potential (TRP) subfamily M member 7 (TRPM7) is implicated in cardiac fibrosis. However, the mechanisms underlying the regulation of sinoatrial node (SAN) fibrosis in SSS by TRPM7 remain unknown. The aim of this study was to investigate the role of angiotensin II (Ang II)/TRPM7/Smad pathway in the SAN fibrosis in rats with SSS. The rat SSS model was established with sodium hydroxide pinpoint pressing permeation...
March 6, 2018: Heart and Vessels
Melissa G Y Lee, Johnny Millar, Elizabeth Rose, Aleesha Jones, Dora Wood, Taryn L Luitingh, Diana Zannino, Johann Brink, Igor E Konstantinov, Christian P Brizard, Yves d'Udekem
OBJECTIVES: To determine the incidence of vocal cord paresis (VCP) after neonatal aortic arch repair/Norwood-type procedure, and the effectiveness of noninvasive laryngeal ultrasound in detecting VCP compared with gold standard invasive nasoendoscopy. METHODS: Fifty-two patients who underwent an arch repair (39 of 52; 75%) or Norwood-type procedure (13 of 52; 25%) via sternotomy between April 1, 2015, and April 30, 2017 underwent laryngeal ultrasound (50 of 52; 96%) and/or flexible fiber optic nasoendoscopy (39 of 52; 75%) at 48 to 72 hours after endotracheal extubation...
February 9, 2018: Journal of Thoracic and Cardiovascular Surgery
Maria Laura Duque Lasio, Beth A Kozel
Elastic fibers provide recoil to tissues that undergo repeated deformation, such as blood vessels, lungs and skin. Composed of elastin and its accessory proteins, the fibers are produced within a restricted developmental window and are stable for decades. Their eventual breakdown is associated with a loss of tissue resiliency and aging. Rare alteration of the elastin (ELN) gene produces disease by impacting protein dosage (supravalvar aortic stenosis, Williams Beuren syndrome and Williams Beuren region duplication syndrome) and protein function (autosomal dominant cutis laxa)...
February 28, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
Paula Casas, Francisco J Ascaso, Eugenio Vicente, Gloria Tejero-Garcés, María I Adiego, José A Cristóbal
BACKGROUND: To assess the retinal sensitivity in obstructive sleep apnea hypopnea syndrome (OSAHS) patients evaluated with standard automated perimetry (SAP). And to correlate the functional SAP results with structural parameters obtained with optical coherence tomography (OCT). METHODS: This prospective, observational, case-control study consisted of 63 eyes of 63 OSAHS patients (mean age 51.7 ± 12.7 years, best corrected visual acuity ≥20/25, refractive error less than three spherical or two cylindrical diopters, and intraocular pressure < 21 mmHg) who were enrolled and compared with 38 eyes of 38 age-matched controls...
March 2, 2018: BMC Ophthalmology
Gisah Amaral Carvalho, Gilberto Paz-Filho, Cleo Otaviano Mesa Junior, Hans Graf
Hypothyroidism is one of the most common hormone deficiencies in adults. Most of the cases, particularly those of overt hypothyroidism, are easily diagnosed and managed, with excellent outcomes if treated adequately. However, minor alterations of thyroid function determine nonspecific manifestations. Primary hypothyroidism due to chronic autoimmune thyroiditis is largely the most common cause of thyroid hormone deficiency. Central hypothyroidism is a rare and heterogeneous disorder characterized by decreased thyroid hormone secretion by an otherwise normal thyroid gland, due to lack of TSH...
February 28, 2018: European Journal of Endocrinology
María E Soto, Verónica Guarner-Lans, Karla Y Herrera-Morales, Israel Pérez-Torres
Marfan syndrome (MFS) is a pleiotropic genetic disease involving the cardiovascular system where a fibrillin-1 mutation is present. This mutation is associated with accelerated activation of transforming growth factor β (TGFβ1) which contributes to the formation of aneurysms in the root of the aorta. There is an imbalance in the synthesis of thromboxane A2 (TXA2 ) and prostacyclin, that is a consequence of a differential protein expression of the isoforms of cyclooxygenases (COXs), suggesting an alteration of arachidonic acid (AA) metabolism...
2018: Frontiers in Physiology
Jaime Gallo-Villegas, Juan Carlos Aristizabal, Mauricio Estrada, Luis H Valbuena, Raul Narvaez-Sanchez, Jorge Osorio, Daniel C Aguirre-Acevedo, Juan C Calderón
BACKGROUND: Evidence of the efficacy of high-intensity, low-volume interval training (HIIT-low volume) in treating insulin resistance (IR) in patients with metabolic disorders is contradictory. In addition, it is unknown whether this effect is mediated through muscle endocrine function, which in turn depends on muscle mass and fiber type composition. Our aims were to assess the efficacy of HIIT-low volume compared to continuous aerobic exercise (CAE) in treating IR in adults with metabolic syndrome (MS) and to establish whether musclin, apelin, muscle mass and muscle composition are mediators of the effect...
February 27, 2018: Trials
Mohammad G Saklayen
Metabolic syndrome, variously known also as syndrome X, insulin resistance, etc., is defined by WHO as a pathologic condition characterized by abdominal obesity, insulin resistance, hypertension, and hyperlipidemia. Though there is some variation in the definition by other health care organization, the differences are minor. With the successful conquest of communicable infectious diseases in most of the world, this new non-communicable disease (NCD) has become the major health hazard of modern world. Though it started in the Western world, with the spread of the Western lifestyle across the globe, it has become now a truly global problem...
February 26, 2018: Current Hypertension Reports
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