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https://www.readbyqxmd.com/read/28214088/a-case-of-seropositive-autoimmune-autonomic-ganglionopathy-with-diffuse-esophageal-spasm
#1
Nobutoshi Morimoto, Sakuma Takahashi, Tomoki Inaba, Motonori Takamiya, Yasuhiko Kageyama, Mizuki Morimoto, Yoshiaki Takahashi, Hirotake Nishimura, Shunya Nakane, Koji Abe
Autoimmune autonomic ganglionopathy (AAG) is an immune-mediated disorder that leads to various autonomic failures associated with anti-ganglionic acetylcholine receptor antibodies (anti-gAChR-Abs). Diffuse esophageal spasm (DES) is an uncommon esophageal motility disorder. We herein report the case of a 68-year-old woman with DES as a partial symptom of AAG. She presented with chronic esophageal transit failure, constipation, and numbness of the hands and feet, Adie's pupil, thermal hypoalgesia, and decreased deep tendon reflexes...
February 14, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28209600/cell-therapy-for-gi-motility-disorders-comparison-of-cell-sources-and-proposed-steps-for-treating-hirschsprung-disease
#2
Lincon A Stamp
Cell therapeutic approaches to treat a range of congenital and degenerative neuropathies are under intense investigation. There have been recent significant advancements in the development of cell therapy to treat disorders of the enteric nervous system, enteric neuropathies. These advances include the efficient generation of enteric neural progenitors from pluripotent stem cells and the rescue of a Hirschsprung Disease model mouse following their transplantation into the bowel. Further, a recent study provides evidence of functional innervation of the bowel muscle by neurons derived from transplanted ENS-derived neural progenitors...
February 16, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28207499/laparoscopic-heller-myotomy-is-not-superior-to-pneumatic-dilation-in-the-management-of-primary-achalasia-conclusions-of-a-systematic-review-and-meta-analysis-of-randomized-controlled-trials
#3
Ji-Wei Cheng, Yin Li, Wen-Qun Xing, Hong-Wei Lv, Hao-Ran Wang
BACKGROUND: Achalasia is an esophageal motility disorder, of unknown cause, which results in increased lower esophageal sphincter tone and symptoms of difficulty swallowing. Current major therapeutic options include laparoscopic Heller myotomy (LHM) and pneumatic dilation (PD). We undertake a systematic review comparing the efficacy and safety of these 2 treatments in the treatment of esophageal achalasia. METHODS: PubMed, Embase, and Cochrane Central Register of Controlled Trials were searched for randomized controlled trial investigating LHM versus PD in the treatment of primary achalasia...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28207290/fatty-acid-and-mineral-receptors-as-drug-targets-for-gastrointestinal-disorders
#4
Pawin Pongkorpsakol, Aekkacha Moonwiriyakit, Chatchai Muanprasat
Nutrient-sensing receptors, including fatty acid receptors (FFA1-FFA4), Ca(2+)-sensing receptors and Zn(2+)-sensing receptors, are involved in several biological processes. These receptors are abundantly expressed in the GI tract, where they have been shown to play crucial roles in regulating GI function. This review provides an overview of the GI functions of fatty acid and mineral receptors, including the regulation of gastric and enteroendocrine functions, GI motility, ion transport and cell growth. Recently, several lines of evidence have implicated these receptors as promising therapeutic targets for the treatment of GI disorders, for example, inflammatory bowel disease, colorectal cancer, metabolic syndrome and diarrheal diseases...
February 16, 2017: Future Medicinal Chemistry
https://www.readbyqxmd.com/read/28191707/mechanisms-underlying-reflux-symptoms-and-dysphagia-in-patients-with-joint-hypermobility-syndrome-with-and-without-postural-tachycardia-syndrome
#5
A Fikree, Q Aziz, D Sifrim
BACKGROUND: The joint hypermobility syndrome (JHS) is a common non-inflammatory connective tissue disorder which frequently co-exists with postural tachycardia syndrome (PoTS), a form of orthostatic intolerance. Gastrointestinal symptoms and dysmotility have been reported in PoTS. Dysphagia and reflux are common symptoms in JHS, yet no studies have examined the physiological mechanism for these, subdivided by PoTS status. METHODS: Thirty patients (28 female, ages: 18-62) with JHS and symptoms of reflux (n=28) ± dysphagia (n=25), underwent high-resolution manometry and 24 hour pH-impedance monitoring after questionnaire-based symptom assessment...
February 12, 2017: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/28191706/g-protein-coupled-estrogen-receptor-and-estrogen-receptor-ligands-regulate-colonic-motility-and-visceral-pain
#6
M Zielińska, J Fichna, M Bashashati, S Habibi, A Sibaev, J-P Timmermans, M Storr
BACKGROUND: Diarrhea-predominant irritable bowel syndrome (IBS-D) is a functional gastrointestinal (GI) disorder, which occurs more frequently in women than men. The aim of our study was to determine the role of activation of classical estrogen receptors (ER) and novel membrane receptor, G protein-coupled estrogen receptor (GPER) in human and mouse tissue and to assess the possible cross talk between these receptors in the GI tract. METHODS: Immunohistochemistry was used to determine the expression of GPER in human and mouse intestines...
February 12, 2017: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/28188367/current-status-of-achalasia-management-a-review-on-diagnosis-and-treatment
#7
REVIEW
Joshua Tuason, Haruhiro Inoue
BACKGROUND: Achalasia is a rare esophageal motility disorder that is characterized by loss of peristalsis and failure of relaxation of the lower esophageal sphincter (LES), particularly during swallowing. This review focuses on the diagnosis of esophageal motility disorders as defined by the Chicago Classification ver 3.0, and presents management options with regard to per-oral endoscopic myotomy (POEM) as the treatment of choice. METHODS: A concise review of literature was performed for articles related to the management of achalasia, and this was contrasted with our institution's current practice...
February 10, 2017: Journal of Gastroenterology
https://www.readbyqxmd.com/read/28187396/methyl-orvinol-dual-activity-opioid-receptor-ligand-inhibits-gastrointestinal-transit-and-alleviates-abdominal-pain-in-the-mouse-models-mimicking-diarrhea-predominant-irritable-bowel-syndrome
#8
Marta Zielińska, Agata Jarmuż, Andrzej Wasilewski, Gerta Cami-Kobeci, Stephen Husbands, Jakub Fichna
BACKGROUND: Diarrhea-predominant irritable bowel syndrome (IBS-D) is a functional disorder of the gastrointestinal (GI) tract. The major IBS-D symptoms include diarrhea, abdominal pain and discomfort. High density of opioid receptors (ORs) in the GI tract and their participation in the maintenance of GI homeostasis make ORs ligands an attractive option for developing new anti-IBS-D treatments. The aim of this study was to characterize the effect of methyl-orvinol on the GI motility and secretion and in mouse models mimicking symptoms of IBS-D...
December 5, 2016: Pharmacological Reports: PR
https://www.readbyqxmd.com/read/28186368/gastrointestinal-involvement-in-the-ehlers-danlos-syndromes
#9
Asma Fikree, Gisela Chelimsky, Heidi Collins, Katcha Kovacic, Qasim Aziz
Current evidence suggests that an association exists between non-inflammatory hereditary disorders of connective tissue such as the Ehlers-Danlos syndromes (EDS) and gastrointestinal (GI) symptoms. Patients with EDS can present with both structural problems such as hiatus hernias, visceroptosis, rectoceles, and rectal prolapse as well as functional problems such as disordered gut motility. It has recently been demonstrated that patients with hypermobile EDS (hEDS) present with GI symptoms related to the fore and hind-gut and these patients frequently meet the criteria for functional gastrointestinal disorders such as functional dyspepsia and irritable bowel syndrome...
February 10, 2017: American Journal of Medical Genetics. Part C, Seminars in Medical Genetics
https://www.readbyqxmd.com/read/28180937/guidelines-for-the-management-of-postoperative-obstructive-symptoms-in-children-with-hirschsprung-disease
#10
REVIEW
J C Langer, M D Rollins, M Levitt, A Gosain, L de la Torre, R P Kapur, R A Cowles, J Horton, D H Rothstein, A M Goldstein
Although most children with Hirschsprung disease ultimately do well, many experience a variety of ongoing problems after pull-through surgery. The most common include obstructive symptoms, soiling, enterocolitis and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative obstructive symptoms in children with Hirschsprung disease. The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review and expert consensus were then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with obstructive symptoms following pull-through for Hirschsprung disease...
February 8, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28176794/x-linked-primary-ciliary-dyskinesia-due-to-mutations-in-the-cytoplasmic-axonemal-dynein-assembly-factor-pih1d3
#11
Chiara Olcese, Mitali P Patel, Amelia Shoemark, Santeri Kiviluoto, Marie Legendre, Hywel J Williams, Cara K Vaughan, Jane Hayward, Alice Goldenberg, Richard D Emes, Mustafa M Munye, Laura Dyer, Thomas Cahill, Jeremy Bevillard, Corinne Gehrig, Michel Guipponi, Sandra Chantot, Philippe Duquesnoy, Lucie Thomas, Ludovic Jeanson, Bruno Copin, Aline Tamalet, Christel Thauvin-Robinet, Jean-François Papon, Antoine Garin, Isabelle Pin, Gabriella Vera, Paul Aurora, Mahmoud R Fassad, Lucy Jenkins, Christopher Boustred, Thomas Cullup, Mellisa Dixon, Alexandros Onoufriadis, Andrew Bush, Eddie M K Chung, Stylianos E Antonarakis, Michael R Loebinger, Robert Wilson, Miguel Armengot, Estelle Escudier, Claire Hogg, Serge Amselem, Zhaoxia Sun, Lucia Bartoloni, Jean-Louis Blouin, Hannah M Mitchison
By moving essential body fluids and molecules, motile cilia and flagella govern respiratory mucociliary clearance, laterality determination and the transport of gametes and cerebrospinal fluid. Primary ciliary dyskinesia (PCD) is an autosomal recessive disorder frequently caused by non-assembly of dynein arm motors into cilia and flagella axonemes. Before their import into cilia and flagella, multi-subunit axonemal dynein arms are thought to be stabilized and pre-assembled in the cytoplasm through a DNAAF2-DNAAF4-HSP90 complex akin to the HSP90 co-chaperone R2TP complex...
February 8, 2017: Nature Communications
https://www.readbyqxmd.com/read/28167615/the-familial-dysautonomia-disease-gene-ikbkap-elp1-is-required-in-the-developing-and-adult-central-nervous-system
#12
Marta Chaverra, Lynn George, Marc Mergy, Hannah Waller, Katharine Kujawa, Connor Murnion, Ezekiel Sharples, Julian Thorne, Nathaniel Podgajny, Andrea Grindeland, Yumi Ueki, Steven Eiger, Cassie Cusick, A Michael Babcock, George A Carlson, Frances Lefcort
Hereditary sensory and autonomic neuropathies (HSANs) are a genetically and clinically diverse group of disorders defined by peripheral nervous system (PNS) dysfunction. HSAN Type III, Familial Dysautonomia (FD), results from a single base mutation in the gene IKBKAP that encodes a scaffolding unit for a multi-subunit complex Elongator. Since mutations in other Elongator subunits (ELP2-4) are associated with central nervous system (CNS) disorders, the goal of this study was to investigate a potential CNS requirement for Ikbkap/Elp1 The sensory and autonomic pathophysiology of FD is fatal, with the majority of patients dying by age 40...
February 6, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28162951/dynein-binding-of-competitive-regulators-dynactin-and-nude-involves-novel-interplay-between-phosphorylation-site-and-disordered-spliced-linkers
#13
Jing Jie, Frank Löhr, Elisar Barbar
Dynactin and NudE/Nudel are prominent regulators of cytoplasmic dynein motility and cargo-binding activities. Both interact with the intrinsically disordered N-terminal domain of dynein intermediate chain (IC), which also contains phosphorylation sites that apparently regulate these interactions. Nuclear magnetic resonance and isothermal calorimetry studies demonstrate that the Ser84 phosphorylation site identified in cells is in a disordered linker distant from the N-terminal helix that contains both the dynactin- and the Nudel-binding interfaces...
January 31, 2017: Structure
https://www.readbyqxmd.com/read/28161834/cannabinoid-receptors-in-regulating-the-gi-tract-experimental-evidence-and-therapeutic-relevance
#14
Ulrike Taschler, Carina Hasenoehrl, Martin Storr, Rudolf Schicho
Cannabinoid receptors are fundamentally involved in all aspects of intestinal physiology, such as motility, secretion, and epithelial barrier function. They are part of a broader entity, the so-called endocannabinoid system which also includes their endocannabinoid ligands and the ligands' synthesizing/degrading enzymes. The system has a strong impact on the pathophysiology of the gastrointestinal tract and is believed to maintain homeostasis in the gut by controlling hypercontractility and by promoting regeneration after injury...
February 5, 2017: Handbook of Experimental Pharmacology
https://www.readbyqxmd.com/read/28161707/pathogenesis-of-double-dose-proton-pump-inhibitor-resistant-non-erosive-reflux-disease-and-mechanism-of-reflux-symptoms-and-gastric-acid-secretion-suppressive-effect-in-the-presence-or-absence-of-helicobacter-pylori-infection
#15
Noriyuki Kawami, Nana Takenouchi, Mariko Umezawa, Shintaro Hoshino, Yuriko Hanada, Yoshimasa Hoshikawa, Hirohito Sano, Yoshio Hoshihara, Tsutomu Nomura, Eiji Uchida, Katsuhiko Iwakiri
BACKGROUND: Various mechanisms have been suggested to be responsible for contributing to the occurrence of proton pump inhibitor (PPI)-resistant non-erosive reflux disease (NERD). The aims of this study were to clarify the pathogenesis of PPI-resistant NERD. METHODS: Fifty-three patients with NERD, who had persistent reflux symptoms despite taking double-dose PPI, were included in this study. After excluding eosinophilic esophagitis (EoE) and primary esophageal motility disorder, esophageal impedance-pH monitoring was carried out...
February 4, 2017: Digestion
https://www.readbyqxmd.com/read/28155572/exploring-the-role-of-microglia-in-cortical-spreading-depression-in-neurological-disease
#16
Mamoru Shibata, Norihiro Suzuki
Microglia play a pivotal role in innate immunity in the brain. During development, they mature from myeloerythroid progenitor cells in the yolk sac and colonize the brain to establish a resident population of tissue macrophages. In the postnatal brain, they exert phagocytosis and induce inflammatory response against invading pathogens. Microglia also act as guardians of brain homeostasis by surveying the microenvironment using motile processes. Cortical spreading depression (CSD) is a slowly propagating (2-5 mm/min) wave of rapid, near-complete depolarization of neurons and astrocytes followed by a period of electrical suppression of a distinct population of cortical neurons...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28141904/catheter-based-high-frequency-intraluminal-ultrasound-imaging-is-a-powerful-tool-to-study-esophageal-dysmotility-patients
#17
Cecilio Santander, Elena Perea, María Caldas, Pere Clave
High-resolution manometry (HRM) is currently the most important diagnostic test for esophageal motility disorders, providing information on the contraction pattern of the circular muscle layer, which helps classify these esophageal motor diseases. However, with the increasing development of ultrasound, other techniques, such as high-frequency intraluminal ultrasound (HFIUS), have gained importance. This technique uses a flexible shaft with a central wire integrated into a standard endoscope, which facilitates real-time sonography...
January 31, 2017: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/28139656/the-chicago-classification-3-0-results-in-more-normal-findings-and-fewer-hypotensive-findings-with-no-difference-in-other-diagnoses
#18
H Monrroy, D Cisternas, C Bilder, A Ditaranto, J Remes-Troche, A Meixueiro, M A Zavala, J Serra, I Marín, A Ruiz de León, J Pérez de la Serna, A Hani, A Leguizamo, L Abrahao, R Coello, M A Valdovinos
OBJECTIVES: High-resolution manometry (HRM) is the preferred method for the evaluation of motility disorders. Recently, an update of the diagnostic criteria (Chicago 3.0) has been published. The aim of this study was to compare the performance criteria of Chicago version 2.0 (CC2.0) vs. 3.0 (CC3.0) in a cohort of healthy volunteers and symptomatic patients. METHODS: HRM studies of asymptomatic and symptomatic individuals from several centers of Spain and Latin America were analyzed using both CC2...
January 31, 2017: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/28133854/waist-to-hip-ratio-is-a-better-predictor-of-esophageal-acid-exposure-than-body-mass-index
#19
C Ringhofer, J Lenglinger, M Riegler, I Kristo, A Kainz, S Schoppmann
BACKGROUND: Obesity and gastroesophageal reflux disease (GERD) are major health problems showing an inconstant relationship in the literature. Therefore, anthropometric parameters which are predictive and can simply be assessed at first patient presentation may lead to a better patient selection for ambulatory reflux monitoring. We aimed to examine the association of body mass index (BMI) and waist to hip ratio (WHR) with gastroesophageal reflux activity during 24 hour-pH-impedance monitoring...
January 29, 2017: Neurogastroenterology and Motility: the Official Journal of the European Gastrointestinal Motility Society
https://www.readbyqxmd.com/read/28129846/modeling-intestinal-disorders-using-zebrafish
#20
X Zhao, M Pack
Although the zebrafish was initially developed as a model system to study embryonic development, it has gained increasing attention as an advantageous system to investigate human diseases, including intestinal disorders. Zebrafish embryos develop rapidly, and their digestive system is fully functional and visible by 5days post fertilization. There is a large degree of homology between the intestine of zebrafish and higher vertebrate organisms in terms of its cellular composition and function as both a digestive and immune organ...
2017: Methods in Cell Biology
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