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Reye Syndrom

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https://www.readbyqxmd.com/read/28100057/wilkie%C3%A2-s-syndrome
#1
Carlos Oliva-Fonte, Cristina Fernández Rey, Javier Pereda Rodríguez, Ana María González-Fernández
We report the case of a 14 year-old woman, who presents epigastric abdominal pain, abundant vomits and an important abdominal distension. Previously, she presented similar episodes. She has not any significant prior medical history. CT is performed and the imaging findings are consistent with Wilkie´s syndrome. Wilkie´s syndrome is a rare condition, that results from a intestinal obstruction caused by third portion duodenal compression between the abdominal aorta and the superior mesenteric artery. When the angle between both structures diminishes and the distance between the SMA and the aorta decreases (the most diagnostic value), duodenal obstruction occurs...
January 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28049394/coexistence-of-amyloidosis-and-primary-sj%C3%A3-gren-s-syndrome-an-overview-amyloidosis-and-sj%C3%A3-gren-s-syndrome
#2
Gabriela Hernandez-Molina, David Faz-Muñoz, Miguel Astudillo-Angel, Alejandra Iturralde-Chávez, Edgardo Reyes
BACKGROUND: The association of primary Sjögren´s syndrome (SS) and amyloidosis has been recognized but scarcely assessed. OBJECTIVE: Herein we analyzed the literature regarding this association in order to describe a SS phenotype prone to both conditions. METHODS: PubMed and CINAHL databases were searched from inception until April 2016 for the key words: amyloidosis, amyloid, Sjögren's syndrome and their combinations. RESULTS: Most of the cases corresponded to localized amyloidosis mainly at skin and lung, whereas only three cases were systemic...
January 3, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28039031/worsening-of-memory-deficit-induced-by-energy-dense-diet-in-a-rat-model-of-early-alzheimer-s-disease-is-associated-to-neurotoxic-a%C3%AE-species-and-independent-of-neuroinflammation
#3
Pamela V Martino Adami, Pablo Galeano, Marina L Wallinger, Celia Quijano, Alejandro Rabossi, Eleonora S Pagano, Natividad Olivar, Carlos Reyes Toso, Daniel Cardinali, Luis I Brusco, Sonia Do Carmo, Rafael Radi, Goar Gevorkian, Eduardo M Castaño, A Claudio Cuello, Laura Morelli
Diet is a modifiable risk factor for Alzheimer's disease (AD), but the mechanisms linking alterations in peripheral metabolism and cognition remain unclear. Since it is especially difficult to study long-term effects of high-energy diet in individuals at risk for AD, we addressed this question by using the McGill-R-Thy1-APP transgenic rat model (Tg(+/-)) that mimics presymptomatic AD. Wild-type and Tg(+/-) rats were exposed during 6months to a standard diet or a Western diet (WD), high in saturated fat and sugar...
December 27, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28027808/-splenic-rupture-secondary-to-abscess-rare-cause-of-pneumoperitoneum-case-report
#4
Jorge Enrique Barrón-Reyes, Julio César Chávez-Galván, Jesús Alejandro Martínez-Peralta, Julio César López-Valdés
BACKGROUND: Splenic abscesses are rare entities; reports are commonly described in immunocompromised patients (72%) as: hematologic diseases, diabetes, endocarditis, acquired immunodeficiency syndrome, transplant patients and subjects who had abdominal trauma or splenic infarction. The main and most serious complication is the abscess rupture into the peritoneal cavity or adjacent organs (stomach or colon), which determines hemodynamic instability or septic state. CLINICAL CASE: Fifty-year-old man, who was admitted at Emergency Room due eight days' progressive, oppressive, and current pain; intensity 4/10, irradiated at hemi-back, which was higher intensity during the standing and decreased at supine position...
December 24, 2016: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/28027792/-clinical-practice-guidelines-irritable-bowel-syndrome-with-constipation-and-functional-constipation-in-adults-concept-diagnosis-and-healthcare-continuity-part-1-of-2
#5
F Mearin, C Ciriza, M Mínguez, E Rey, J J Mascort, E Peña, P Cañones, J Júdez
In this Clinical practice guide, an analysis is made of the diagnosis and treatment of adult patients with constipation and abdominal discomfort, under the spectrum of irritable bowel syndrome and functional constipation. These have an important personal, health and social impact, affecting the quality of life of these patients. In irritable bowel syndrome with a predominance of constipation, this is the predominant change in bowel movements, with recurrent abdominal pain, bloating and frequent abdominal distension...
December 24, 2016: Atencion Primaria
https://www.readbyqxmd.com/read/28025360/patterns-of-cerebral-blood-flow-modulation-during-painful-stimulation-in-fibromyalgia-a-transcranial-doppler-sonography-study
#6
Casandra I Montoro, Stefan Duschek, Cristina Muñóz Ladrón de Guevara, Gustavo A Reyes Del Paso
OBJECTIVE: This study analyzed the temporal dynamics of cerebral blood flow (CBF) modulations, during painful stimulation in fibromyalgia syndrome (FMS), using functional transcranial Doppler sonography. METHOD: Blood flow velocities were recorded bilaterally in the anterior (ACA) and middle (MCA) cerebral arteries of 24 FMS patients and 20 healthy individuals during exposure to painful pressure stimulation. Participants were presented with two stimulation blocks: a) fixed pressure (2...
December 2016: Pain Medicine: the Official Journal of the American Academy of Pain Medicine
https://www.readbyqxmd.com/read/28002403/xrcc1-mutation-is-associated-with-parp1-hyperactivation-and-cerebellar-ataxia
#7
Nicolas C Hoch, Hana Hanzlikova, Stuart L Rulten, Martine Tétreault, Emilia Komulainen, Limei Ju, Peter Hornyak, Zhihong Zeng, William Gittens, Stephanie A Rey, Kevin Staras, Grazia M S Mancini, Peter J McKinnon, Zhao-Qi Wang, Justin D Wagner, Grace Yoon, Keith W Caldecott
XRCC1 is a molecular scaffold protein that assembles multi-protein complexes involved in DNA single-strand break repair. Here we show that biallelic mutations in the human XRCC1 gene are associated with ocular motor apraxia, axonal neuropathy, and progressive cerebellar ataxia. Cells from a patient with mutations in XRCC1 exhibited not only reduced rates of single-strand break repair but also elevated levels of protein ADP-ribosylation. This latter phenotype is recapitulated in a related syndrome caused by mutations in the XRCC1 partner protein PNKP and implicates hyperactivation of poly(ADP-ribose) polymerase/s as a cause of cerebellar ataxia...
January 5, 2017: Nature
https://www.readbyqxmd.com/read/27956295/intraoperative-complications-of-phacoemulsification-in-pseudoexfoliation-%C3%A2-metaanalysis
#8
REVIEW
Pedro Vazquez-Ferreiro, Francisco J Carrera-Hueso, Jaime E Poquet Jornet, Narjis Fikri-Benbrahim, Marta Diaz-Rey, Rafael Sanjuan-Cerveró
: This review analyzed the effect of pseudoexfoliation (PXF) syndrome on the risk for surgical complications during phacoemulsification. Peer-reviewed literature in Medline, Embase, Lilacs, Web of Science, and Cochrane databases was systematically searched, along with gray literature in the Teseo and National Institutes of Health clinical trials database. Clinical trial, cohort, case-control, and cross-sectional studies comparing phacoemulsification complications in patients with and without PXF were identified, and 22 of 30 studies were selected for analysis...
November 2016: Journal of Cataract and Refractive Surgery
https://www.readbyqxmd.com/read/27911138/phase-1-dose-escalation-study-of-oral-abexinostat-for-the-treatment-of-patients-with-relapsed-refractory-higher-risk-myelodysplastic-syndromes-acute-myeloid-leukemia-or-acute-lymphoblastic-leukemia
#9
Norbert Vey, Thomas Prebet, Claire Thalamas, Aude Charbonnier, Jerome Rey, Ioana Kloos, Emily Liu, Ying Luan, Remus Vezan, Thorsten Graef, Christian Recher
Histone deacetylase (HDAC) inhibitor abexinostat is under investigation for the treatment of various cancers. Epigenetic changes including aberrant HDAC activity are associated with cancers, including myelodysplastic syndromes (MDS), acute myeloid leukemia (AML), and acute lymphoblastic leukemia (ALL). In this phase 1 dose-escalation study, 17 patients with relapsed/refractory higher-risk MDS, AML, or ALL received oral abexinostat (60, 80 [starting dose], 100, or 120 mg) twice daily (bid) on Days 1-14 of 21-day cycles...
December 2, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/27900261/targeting-the-endocannabinoid-cb1-receptor-system-for-treating-obesity-in-prader-willi-syndrome
#10
Ibrahim Knani, Brian J Earley, Shiran Udi, Alina Nemirovski, Rivka Hadar, Asaad Gammal, Resat Cinar, Harry J Hirsch, Yehuda Pollak, Itai Gross, Talia Eldar-Geva, Daniela P Reyes-Capo, Joan C Han, Andrea M Haqq, Varda Gross-Tsur, Rachel Wevrick, Joseph Tam
OBJECTIVE: Extreme obesity is a core phenotypic feature of Prader-Willi syndrome (PWS). Among numerous metabolic regulators, the endocannabinoid (eCB) system is critically involved in controlling feeding, body weight, and energy metabolism, and a globally acting cannabinoid-1 receptor (CB1R) blockade reverses obesity both in animals and humans. The first-in-class CB1R antagonist rimonabant proved effective in inducing weight loss in adults with PWS. However, it is no longer available for clinical use because of its centrally mediated, neuropsychiatric, adverse effects...
December 2016: Molecular Metabolism
https://www.readbyqxmd.com/read/27888862/functional-and-motor-gastrointestinal-disorders
#11
Fermín Mearin, Enrique Rey, Agustín Balboa
This article discusses the most interesting presentations at Digestive Disease Week, held in San Diego, in the field of functional and motor gastrointestinal disorders. One of the most important contributions was undoubtedly the presentation of the new Rome IV diagnostic criteria for functional gastrointestinal disorders. We therefore devote some space in this article to explaining these new criteria in the most common functional disorders. In fact, there has already been discussion of data comparing Rome IV and Rome III criteria in the diagnosis of irritable bowel syndrome, confirming that the new criteria are somewhat more restrictive...
September 2016: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/27885891/wiskott-aldrich-syndrome-in-a-child-presenting-with-macrothrombocytopenia
#12
Jose Maria Bastida, Monica Del Rey, Nuria Revilla, Rocio Benito, Martin Perez-Andrés, Berta González, Susana Riesco, Kamila Janusz, Jose Padilla, Ana Hortal Benito-Sendin, David Bueno, Elena Blanco, Maria Hernández-Rivas, Vicente Vicente, Jose Rivera, Ramon González-Porras, Maria Luisa Lozano
Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive disease resulting from variants in the WAS gene, characterized by a triad of immunodeficiency, eczema, and thrombocytopenia. Despite the fact that WAS is traditionally differentiated from immune thrombocytopenia (ITP) by small size of WAS platelets, in practice, microthrombocytopenia may occasionally not be present, and in certain cases, WAS patients exhibit some parallelism to ITP patients. We characterized one patient presenting with the classic form of the disease but increased mean platelet volume...
November 25, 2016: Platelets
https://www.readbyqxmd.com/read/27871769/effect-of-mechanical-vibration-on-transcutaneous-oxygen-levels-in-the-feet-of-type-2-diabetes-mellitus-patients
#13
Gerardo Rodríguez Reyes, Lidia Núñez Carrera, Aldo Alessi Montero, Adriana Solís Vivanco, Ivett Quiñones Uriostegui, Alberto Isaac Pérez Sanpablo
BACKGROUND AND OBJECTIVE: Foot conditions in patients with diabetes mellitus (DM) are major causes of morbidity and disability. Whole body vibration may promote blood circulation in the lower limbs, hence facilitating perfusion and promoting the supply of nutrients and oxygen to comprised tissues. Transcutaneous oxygen levels (TcPO2)>40mmHg in cases of diabetic foot syndrome are associated with a good prognosis in the resolution of ulcers. The objective of this study was to determine whether whole body vibration favors some parameters of interest related to complications associated with the diabetic foot syndrome...
January 6, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/27856258/aspirin-increases-mitochondrial-fatty-acid-oxidation
#14
Radha Uppala, Brianne Dudiak, Megan E Beck, Sivakama S Bharathi, Yuxun Zhang, Donna B Stolz, Eric S Goetzman
The metabolic effects of salicylates are poorly understood. This study investigated the effects of aspirin on fatty acid oxidation. Aspirin increased mitochondrial long-chain fatty acid oxidation, but inhibited peroxisomal fatty acid oxidation, in two different cell lines. Aspirin increased mitochondrial protein acetylation and was found to be a stronger acetylating agent in vitro than acetyl-CoA. However, aspirin-induced acetylation did not alter the activity of fatty acid oxidation proteins, and knocking out the mitochondrial deacetylase SIRT3 did not affect the induction of long-chain fatty acid oxidation by aspirin...
January 8, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/27810257/-clinical-practice-guidelines-irritable-bowel-syndrome-with-constipation-and-functional-constipation-in-adults-concept-diagnosis-and-healthcare-continuity-part-1-of-2
#15
F Mearin, C Ciriza, M Mínguez, E Rey, J J Mascort, E Peña, P Cañones, J Júdez
In this Clinical practice guide, an analysis is made of the diagnosis and treatment of adult patients with constipation and abdominal discomfort, under the spectrum of irritable bowel syndrome and functional constipation. These have an important personal, health and social impact, affecting the quality of life of these patients. In irritable bowel syndrome with a predominance of constipation, this is the predominant change in bowel movements, with recurrent abdominal pain, bloating and frequent abdominal distension...
January 2017: Semergen
https://www.readbyqxmd.com/read/27799946/anti-m%C3%A3-llerian-hormone-as-a-marker-of-steroid-and-gonadotropin-action-in-the-testis-of-children-and-adolescents-with-disorders-of-the-gonadal-axis
#16
REVIEW
Nadia Y Edelsztein, Romina P Grinspon, Helena F Schteingart, Rodolfo A Rey
In pediatric patients, basal testosterone and gonadotropin levels may be uninformative in the assessment of testicular function. Measurement of serum anti-Müllerian hormone (AMH) has become increasingly widespread since it provides information about the activity of the male gonad without the need for dynamic tests, and also reflects the action of FSH and androgens within the testis. AMH is secreted in high amounts by Sertoli cells from fetal life until the onset of puberty. Basal AMH expression is not dependent on gonadotropins or sex steroids; however, FSH further increases and testosterone inhibits AMH production...
2016: International Journal of Pediatric Endocrinology
https://www.readbyqxmd.com/read/27792799/characterization-of-burning-mouth-syndrome-in-patients-with-parkinson-s-disease
#17
David Bonenfant, Pierre H Rompré, Nathalie Rei, Nicolas Jodoin, Valerie Lynn Soland, Veronica Rey, Christine Brefel-Courbon, Fabienne Ory-Magne, Olivier Rascol, Pierre J Blanchet
AIMS: To determine the prevalence and characteristics of burning mouth syndrome (BMS) in a Parkinson's disease (PD) population through a self-administered, custom-made survey. METHODS: A total of 218 surveys were collected during regular outpatient visits at two Movement Disorders Clinics in Montreal (Canada) and Toulouse (France) to gather information about pain experience, PD-related symptoms, and oral and general health. A neurologist confirmed the diagnosis of PD, drug treatment, Hoehn-Yahr stage, and Schwab & England Activity of Daily Living score...
October 2016: Journal of Oral & Facial Pain and Headache
https://www.readbyqxmd.com/read/27789187/validation-study-of-the-medication-adherence-rating-scale-results-from-the-face-sz-national-dataset
#18
Guillaume Fond, Laurent Boyer, Mohamed Boucekine, Latif A Aden, Franck Schürhoff, Arnaud Tessier, Meja Andrianarisoa, Fabrice Berna, Lore Brunel, Delphine Capdevielle, Isabelle Chereau, Jasmina Mallet, Hélène Denizot, Jean Michel Dorey, Caroline Dubertret, Julien Dubreucq, Catherine Faget, Franck Gabayet, Romain Rey, Raphaelle Richieri, Christine Passerieux, Aurélie Schandrin, Mathieu Urbach, Pierre Vidailhet, Pierre Michel Llorca, David Misdrahi
OBJECTIVE: The Medication Adherence Rating Scale (MARS) is one of the most widely used measurements of adherence in schizophrenia (SZ). However, the data available regarding its psychometric properties are scarce. The aim of this study was to provide new data regarding the psychometric properties of the MARS in a multicenter community-dwelling sample of SZ patients. METHODS: This study was conducted in the French National network of the 10 FondaMental Expert Centers for SZ...
October 24, 2016: Schizophrenia Research
https://www.readbyqxmd.com/read/27783711/mechanistic-insight-into-bunyavirus-induced-membrane-fusion-from-structure-function-analyses-of-the-hantavirus-envelope-glycoprotein-gc
#19
Pablo Guardado-Calvo, Eduardo A Bignon, Eva Stettner, Scott Allen Jeffers, Jimena Pérez-Vargas, Gerard Pehau-Arnaudet, M Alejandra Tortorici, Jean-Luc Jestin, Patrick England, Nicole D Tischler, Félix A Rey
Hantaviruses are zoonotic viruses transmitted to humans by persistently infected rodents, giving rise to serious outbreaks of hemorrhagic fever with renal syndrome (HFRS) or of hantavirus pulmonary syndrome (HPS), depending on the virus, which are associated with high case fatality rates. There is only limited knowledge about the organization of the viral particles and in particular, about the hantavirus membrane fusion glycoprotein Gc, the function of which is essential for virus entry. We describe here the X-ray structures of Gc from Hantaan virus, the type species hantavirus and responsible for HFRS, both in its neutral pH, monomeric pre-fusion conformation, and in its acidic pH, trimeric post-fusion form...
October 2016: PLoS Pathogens
https://www.readbyqxmd.com/read/27780632/-symptomatic-infection-associated-acute-encephalopathy-in-children-with-underlying-neurological-disorders
#20
Yoshimichi Hirayama, Yoshiaki Saito, Yoshihiro Maegaki
BACKGROUND: Development of infection-associated acute encephalopathy (AE) is precipitated by several factors, including viral agents, age, and genetic polymorphisms. In addition, children with prior underlying neurological disorders can also present with AE. METHOD: We reviewed 55 children with AE who were referred to hospitals participating in the Status Epilepticus Study Group from 1988 to 2013. AE was classified into eight subtypes: acute encephalopathy with biphasic seizures and late reduced diffusion (AESD); hemiconvulsion-hemiplegia syndrome (HH); acute necrotizing encephalopathy; hemorrhagic shock and encephalopathy syndrome (HSES); clinically mild encephalitis/encephalopathy with a reversible splenial lesion; acute encephalitis with refractory, repetitive partial seizures; Reye-like syndrome; and unclassified...
October 22, 2016: Brain & Development
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