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https://www.readbyqxmd.com/read/27900347/isolated-dysphagia-as-initial-sign-of-anti-iglon5-syndrome
#1
Jens Burchard Schröder, Nico Melzer, Tobias Ruck, Anna Heidbreder, Ilka Kleffner, Ralf Dittrich, Paul Muhle, Tobias Warnecke, Rainer Dziewas
OBJECTIVE: To report on dysphagia as initial sign in a case of anti-IgLON5 syndrome and provide an overview of the current literature. METHODS: The diagnostic workup included cerebral MRI, fiber optic endoscopic evaluation of swallowing (FEES) with the FEES tensilon test, a videofluoroscopic swallowing study, evoked potentials and peripheral nerve conduction studies, polysomnography, lumbar puncture, and screening for neural autoantibodies. A systematic review of all published cases of IgLON5 syndrome is provided...
January 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/27900201/the-diagnosis-and-treatment-of-rapunzel-syndrome
#2
Zhe Wang, Feng Cao, Diangang Liu, Yu Fang, Fei Li
Trichobezoars are hairballs or hair-like fibers formed by chewing and swallowing hair or any other indigestible materials. Trichobezoars usually form in the gastric body and are thus prepyloric. However, trichobezoars may rarely pass through the pylorus into the duodenum, jejunum, ileum, and even the colon, in a condition referred to as Rapunzel syndrome. Here, we present a case of a 13-year-old girl with this rare syndrome and discuss the diagnosis and treatment of the disease.
November 2016: Acta Radiologica Open
https://www.readbyqxmd.com/read/27894447/differentiating-familial-neuropathies-from-guillain-barr%C3%A3-syndrome
#3
REVIEW
Brett J Bordini, Priya Monrad
Differentiating Guillain-Barré syndrome (GBS) from inherited neuropathies and other acquired peripheral neuropathies requires understanding the atypical presentations of GBS and its variant forms, as well as historical and physical features suggestive of inherited neuropathies. GBS is typically characterized by the acute onset of ascending flaccid paralysis, areflexia, and dysesthesia secondary to peripheral nerve fiber demyelination. The disorder usually arises following a benign gastrointestinal or respiratory illness, is monophasic, reaches a nadir with several weeks, and responds to immunomodulatory therapy...
February 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27881407/prophylactic-and-therapeutic-treatment-with-flavonone-sakuranetin-ameliorates-lps-induced-acute-lung-injury
#4
Márcia Isabel Bittencourt-Mernak, Nathália Montouro Pinheiro, Fernanda P Roncon Santana, Marina P Guerreiro, Beatriz Mangueira Saraiva-Romanholo, Simone S Grecco, Luciana C Caperuto, Raphael J F Felizardo, Niels Olsen Saraiva Câmara, Iolanda F L Calvo Tibério, Mílton A Martins, João Henrique G Lago, Carla Máximo Prado
BACKGROUND AND PURPOSE: Sakuranetin is the main isolate flavonoid from Baccharis retusa (Asteraceae) leaves and exhibits anti-inflammatory and anti-oxidative activities. Acute respiratory distress syndrome is an acute failure of the respiratory system for which effective treatment is urgently necessary. This study investigated the preventive and therapeutic effects of sakuranetin on lipopolysaccharide (LPS)-induced acute lung injury (ALI) in mice. EXPERIMENTAL APPROACH: Animals were treated with intranasal sakuranetin 30 minutes before or 6 hours after instillation of LPS...
November 23, 2016: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/27876783/genetic-characterization-of-novel-fowl-aviadenovirus-4-isolates-from-outbreaks-of-hepatitis-hydropericardium-syndrome-in-broiler-chickens-in-china
#5
Yanke Liu, Wenyan Wan, Dongsheng Gao, Yongtao Li, Xia Yang, Hongying Liu, Huixia Yao, Lu Chen, Chuanqing Wang, Jun Zhao
Since May 2015, severe outbreaks of hepatitis-hydropericardium syndrome (HHS) associated with infections of fowl aviadenovirus (FAdV) have emerged in broiler chickens in several Chinese provinces. To identify the genotype and gain a better understanding of the genetic properties of the FAdV strains responsible for the recent HHS outbreaks in China, the complete genome sequences of five isolates from outbreaks of HHS in broiler chickens in five provinces were determined. The results demonstrated that a novel fowl aviadenovirus 4 (FAdV-4) genotype was epidemic in China...
November 23, 2016: Emerging Microbes & Infections
https://www.readbyqxmd.com/read/27867498/bone-muscle-crosstalk-targets-muscle-regeneration-pathway-regulated-by-core-circadian-transcriptional-repressors-dec1-and-dec2
#6
REVIEW
Jeffrey P Gorski, Jeffrey L Price
Deletion of proprotein convertase Mbtps1 in bone osteocytes leads to a significant postnatal increase in skeletal muscle size and contractile function, while causing only a 25% increase in stiffness in long bones. Concerns about leakiness in skeletal muscle were discounted since Cre recombinase expression does not account for our findings, and, Mbtps1 protein and mRNA is not deleted. Interestingly, the response of normal skeletal muscle to exercise and the regenerative response of skeletal muscle to the deletion of Mbtps1 in bone share some key regulatory features including a preference for slow twitch muscle fibers...
2016: BoneKEy Reports
https://www.readbyqxmd.com/read/27866728/what-is-new-in-2015-in-dysimmune-neuropathies
#7
REVIEW
T Kuntzer
This review discusses and summarizes the concept of nodopathies, the diagnostic features, investigations, pathophysiology, and treatment options of chronic inflammatory demyelinating polyradiculoneuropathy, and gives updates on other inflammatory and dysimmune neuropathies such as Guillain-Barré syndrome, sensory neuronopathies, small-fiber-predominant ganglionitis, POEMS syndrome, neuropathies associated with IgM monoclonal gammopathy and multifocal motor neuropathy. This field of research has contributed to the antigenic characterization of the peripheral motor and sensory functional systems, as well as helping to define immune neuropathic syndromes with widely different clinical presentation, prognosis and response to therapy...
November 17, 2016: Revue Neurologique
https://www.readbyqxmd.com/read/27862026/unusual-presentation-of-sj%C3%A3-gren-associated-neuropathy-with-plasma-cell-rich-infiltrate
#8
Elie Naddaf, Sarah E Berini, P James B Dyck, Ruple S Laughlin
INTRODUCTION: Sjögren syndrome is thought to be a lymphocyte-driven process. Peripheral nervous system involvement occurs in about 20-25% of patients. A sensory predominant, large fiber peripheral neuropathy is most common, and it is usually associated with a subacute to chronic presentation. METHODS: We report a rare case of an acute Sjögren-associated, sensory predominant, length-dependent peripheral neuropathy mimicking Guillain-Barré syndrome. The patient presented with sensory ataxia preceded by fever and polyarthralgia...
November 16, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27854132/changes-of-retinal-nerve-fiber-layer-thickness-in-obstructive-sleep-apnea-syndrome-a-systematic-review-and-meta-analysis
#9
Wei Wang, Miao He, Wenyong Huang
PURPOSE: Many studies have assessed the changes of retinal nerve fiber layer (RNFL) thickness in patients with obstructive sleep apnea syndrome (OSAS), but the results were inconsistent. Therefore, a meta-analysis was performed to evaluate the RNFL changes in OSAS measured in vivo. METHODS: Pertinent studies were identified by a comprehensive search of PubMed, Embase, Web of science, Cochrane library, Scopus, and Chinese biomedical disc databases from inception to August 2016...
November 17, 2016: Current Eye Research
https://www.readbyqxmd.com/read/27853523/recent-advances-in-understanding-provoked-vestibulodynia
#10
REVIEW
Ahinoam Lev-Sagie, Steven S Witkin
Vulvodynia refers to pain in the vulva of at least 3 months' duration in the absence of a recognized underlying cause. Provoked, localized vestibulodynia is the term used to describe superficial pain confined to the vulvar vestibule, provoked by touch. This review will focus on provoked vestibulodynia with regard to its suggested causative factors and will discuss the role of inflammation, vulvovaginal infections, mucosal nerve fiber proliferation, hormonal associations, central pain mechanisms, pelvic floor muscle dysfunction, and genetic factors...
2016: F1000Research
https://www.readbyqxmd.com/read/27837354/congenital-cranial-dysinnervation-disorders
#11
REVIEW
Anupam Singh, P K Pandey, Ajai Agrawal, Sanjeev Kumar Mittal, Kartik Maheshbhai Rana, Chirag Bahuguna
The European Neuromuscular Centre (ENMC) derived the term Congenital Cranial Dysinnervation Disorders in 2002 at an international workshop for a group of congenital neuromuscular diseases. CCDDs are congenital, non-progressive ophthalmoplegia with restriction of globe movement in one or more fields of gaze. This group of sporadic and familial strabismus syndromes was initially referred to as the 'congenital fibrosis syndromes' because it was assumed that the primary pathologic process starts in the muscles of eye motility...
November 11, 2016: International Ophthalmology
https://www.readbyqxmd.com/read/27834920/dietary-strategies-implicated-in-the-prevention-and-treatment-of-metabolic-syndrome
#12
REVIEW
Rocio de la Iglesia, Viviana Loria-Kohen, Maria Angeles Zulet, Jose Alfredo Martinez, Guillermo Reglero, Ana Ramirez de Molina
Metabolic syndrome (MetS) is established as the combination of central obesity and different metabolic disturbances, such as insulin resistance, hypertension and dyslipidemia. This cluster of factors affects approximately 10%-50% of adults worldwide and the prevalence has been increasing in epidemic proportions over the last years. Thus, dietary strategies to treat this heterogenic disease are under continuous study. In this sense, diets based on negative-energy-balance, the Mediterranean dietary pattern, n-3 fatty acids, total antioxidant capacity and meal frequency have been suggested as effective approaches to treat MetS...
November 10, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27830331/posterior-fossa-syndrome-with-delayed-mr-evidence-of-unilateral-superior-cerebellar-peduncle-scp-damage
#13
Kevin Carr, Pegah Ghamasaee, Achint Singh, Izabela Tarasiewicz
Posterior fossa syndrome (PFS) is a well-known sequela of midline posterior fossa tumor resection. Patients typically exhibit transient behavioral, motor, and oculomotor disturbances that resolve within a few weeks to several months after surgery. The underlying pathophysiology of PFS is not completely understood, but contemporary literature has implicated injury to the dentate nucleus and/or exiting dentatothalamocortical fiber bundles as a causative factor. The authors present a case of a young male who developed a delayed variant of PFS typified by motor deficits and demonstrated diffusion restriction in the ipsilateral superior cerebellar peduncle...
November 9, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27830211/clues-from-crouzon-insights-into-the-potential-role-of-growth-factors-in-the-pathogenesis-of-myelinated-retinal-nerve-fibers
#14
Giancarlo A Garcia, Jack J Tian, Supanut Apinyawasisuk, Sarah Kim, Handan Akil, Alfredo A Sadun
PURPOSE: We present a case of bilateral extensive peripapillary myelinated retinal nerve fibers (MRNF) in an individual with Crouzon syndrome, an inherited form of craniosynostosis caused by overactivation of fibroblast growth factor receptor 2. As a secondary aim, we examine the utility of optical coherence tomography (OCT) angiography for visualization of peripapillary vasculature obscured by myelination on other imaging modalities. METHODS: A 24-year-old woman with Crouzon syndrome was evaluated for suspected optic neuritis in the right eye...
December 2016: Journal of Current Ophthalmology
https://www.readbyqxmd.com/read/27829582/postprandial-hyperlipidemia-and-remnant-lipoproteins
#15
Daisaku Masuda, Shizuya Yamashita
Fasting hypertriglyceridemia is positively associated with the morbidity of coronary heart disease (CHD), and postprandial (non-fasting) hypertriglyceridemia is also correlated with the risk status for CHD, which is related to the increase in chylomicron (CM) remnant lipoproteins produced from the intestine. CM remnant particles, as well as oxidized low density lipoprotein (LDL) or very low density lipoprotein (VLDL) remnants, are highly atherogenic and act by enhancing systemic inflammation, platelet activation, coagulation, thrombus formation, and macrophage foam cell formation...
November 8, 2016: Journal of Atherosclerosis and Thrombosis
https://www.readbyqxmd.com/read/27829577/chronic-early-life-stress-in-rat-pups-alters-basal-corticosterone-intestinal-permeability-and-fecal-microbiota-at-weaning-influence-of-sex
#16
Nabila Moussaoui, Jonathan P Jacobs, Muriel Larauche, Mandy Biraud, Mulugeta Million, Emeran Mayer, Yvette Taché
Background/Aims: Wistar rat dams exposed to limited nesting stress (LNS) from postnatal days (PND) 2 to 10 display erratic maternal behavior and their pups show delayed maturation of the hypothalamic-pituitary-adrenal axis and impaired epithelial barrier at PND10 and a visceral hypersensitivity at adulthood. Little is known about the impact of early life stress on the offspring before adulthood and the influence of sex. We investigated whether male and female rats previously exposed to LNS displays at weaning altered corticosterone, intestinal permeability and microbiota...
November 8, 2016: Journal of Neurogastroenterology and Motility
https://www.readbyqxmd.com/read/27829533/dermatomal-sensory-manifestations-in-opalski-syndrome
#17
Tomoya Kon, Yukihisa Funamizu, Tatsuya Ueno, Rie Haga, Haruo Nishijima, Akira Arai, Chieko Suzuki, Jinichi Nunomura, Masayuki Baba, Masahiko Tomiyama
A 31-year-old Japanese woman presented with sudden-onset unstable gait followed by nuchal pain. A neurological examination revealed right-sided limb weakness and decreased pain and thermal sensation on the left side below the level of the L1 dermatome. A lower lateral medullary infarction with ipsilateral hemiplegia, known as Opalski syndrome, caused by spontaneous vertebral artery dissection was diagnosed by magnetic resonance imaging. The spinothalamic tract in the medulla oblongata has a topographic arrangement of sensory fibers, and the dermatomal sensory deficit in this case can be explained in relation to that...
November 7, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/27826444/efficacy-of-recombinant-human-soluble-thrombomodulin-for-the-treatment-of-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a-single-arm-non-randomized-prospective-clinical-trial
#18
Sho Hayakawa, Yasuo Matsuzawa, Tamako Irie, Hagino Rikitake, Noriaki Okada, Yasuo Suzuki
BACKGROUND: Coagulation abnormalities are involved in the pathogenesis of acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF). The administration of recombinant human soluble thrombomodulin (rhTM), which has both anti-inflammatory and anticoagulant activities, improves outcomes and respiratory function in patients with acute respiratory distress syndrome. Therefore, we conducted a prospective clinical study to examine the effects of rhTM on respiratory function, coagulation markers, and outcomes for patients with AE-IPF...
2016: Multidisciplinary Respiratory Medicine
https://www.readbyqxmd.com/read/27822045/alpha-synuclein-in-cutaneous-small-nerve-fibers
#19
Timo Siepmann, Ben Min-Woo Illigens, Kristian Barlinn
Despite progression in the development of pharmacological therapy, treatment of alpha synucleinopathies, such as Parkinson's disease (PD) and some atypical parkinsonism syndromes, is still challenging. To date, our knowledge of the mechanisms whereby the pathological form of alpha-synuclein causes structural and functional damage to the nervous system is limited and, consequently, there is a lack of specific diagnostic tools to evaluate pathology in these patients and differentiate PD from other neurodegenerative proteinopathies...
2016: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/27816328/tubular-aggregates-in-autoimmune-lambert-eaton-myasthenic-syndrome
#20
Isabell Cordts, Fabian Funk, Jörg B Schulz, Joachim Weis, Kristl G Claeys
Tubular aggregates are accumulations of densely packed tubules in muscle fibers, occurring in distinct hereditary and acquired disorders. We present a patient with tubular aggregates and autoimmune Lambert-Eaton myasthenic syndrome. Initially, he showed mild proximal weakness, borderline decrement on 3 Hz stimulation, and slightly elevated creatine kinase. Muscle biopsy revealed tubular aggregates in type II fibers. Due to a good response to pyridostigmine, a limb-girdle myasthenia with tubular aggregates was suspected, but genetic analyses of GFPT1, DPGAT1, and ALG2 were normal...
September 20, 2016: Neuromuscular Disorders: NMD
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