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syndrome west

James Weger-Lucarelli, Claudia Rückert, Nunya Chotiwan, Chilinh Nguyen, Selene M Garcia Luna, Joseph R Fauver, Brian D Foy, Rushika Perera, William C Black, Rebekah C Kading, Gregory D Ebel
In 2015, Zika virus (ZIKV; Flaviviridae; Flavivirus) emerged in the Americas, causing millions of infections in dozens of countries. The rapid spread of the virus and the association with disease outcomes such as Guillain-Barré syndrome and microcephaly make understanding transmission dynamics essential. Currently, there are no reports of vector competence (VC) of American mosquitoes for ZIKV isolates from the Americas. Further, it is not clear whether ZIKV strains from other genetic lineages can be transmitted by American Aedes aegypti populations, and whether the scope of the current epidemic is in part facilitated by viral factors such as enhanced replicative fitness or increased vector competence...
October 2016: PLoS Neglected Tropical Diseases
Ingo Helbig, Abou Ahmad N Tayoun
Epileptic encephalopathies are severe often intractable seizure disorders where epileptiform abnormalities contribute to a progressive disturbance in brain function. Often, epileptic encephalopathies start in childhood and are accompanied by developmental delay and various neurological and non-neurological comorbidities. In recent years, this concept has become virtually synonymous with a group of severe childhood epilepsies including West syndrome, Lennox-Gastaut syndrome, Dravet syndrome, and several other severe childhood epilepsies for which genetic factors are increasingly recognized...
September 2016: Molecular Syndromology
Manuella Gautier, Michèle Gueneret, Corinne Plavonil, Eugénie Jolivet, Bruno Schaub
OBJECTIVE: To establish the normal reference range of fetal nasal bone length (NBL) during the second trimester in an Afro-Caribbean population and the likelihood ratio (LR) for fetal trisomy 21. METHODS: Prenatal records of euploid, non-malformed singleton fetuses who underwent second-trimester ultrasonographic scans at 20-24 weeks of gestation were retrospectively analyzed for NBL and gestational age (GA). Only Afro-Caribbean couples were selected. The relationship between fetal NBL and GA was determined...
October 21, 2016: Fetal Diagnosis and Therapy
Amara Krag, Gregory L Holmes
Infantile spasms, one of the catastrophic epilepsies, can be a diagnostic challenge since the clinical manifestations may be subtle and may mimic benign conditions. Because of the rarity of the condition, primary care physicians and pediatricians may never see a case of infantile spasms during their career and may be unfamiliar with the seizure semiology. This is a serious issue since there is evidence that early diagnosis and treatment may improve outcome. Patients and families are increasingly using the internet more than their physician as a source of medical information about epilepsy...
October 17, 2016: Epilepsy & Behavior: E&B
Sanath Allampati, Kevin D Mullen
Hepatic Encephalopathy (HE) is a complex neuropsychiatric syndrome associated with decompensated liver disease. The spectrum of disease ranges from trivial abnormalities in complex decision making and prolonged reaction time to coma in its most severe form1. The very initial stages, recently termed covert Hepatic Encephalopathy (CHE), can only be diagnosed with the help of neuropsychiatric testing while the later and more severe forms, termed overt Hepatic Encephalopathy (OHE), can be diagnosed clinically. Severity of HE is graded based on West Haven Criteria and please refer to table 1 for more details2...
October 20, 2016: Minerva Gastroenterologica e Dietologica
Alex J Elliot, Helen E Hughes, John Astbury, Grainne Nixon, Kate Brierley, Roberto Vivancos, Thomas Inns, Valerie Decraene, Katherine Platt, Iain Lake, Sarah J O'Brien, Gillian E Smith
During August 2015, a boil water notice (BWN) was issued across parts of North West England following the detection of Cryptosporidium oocysts in the public water supply. Using prospective syndromic surveillance, we detected statistically significant increases in the presentation of cases of gastroenteritis and diarrhoea to general practitioner services and related calls to the national health telephone advice service in those areas affected by the BWN. In the affected areas, average in-hours general practitioner consultations for gastroenteritis increased by 24...
October 13, 2016: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
Karan Seegobin, Kamille Abdool, Kanterpersad Ramcharan, Haramnauth Dyaanand, Fidel Rampersad
We describe a case of Parry Romberg syndrome/en coupe de sabre in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainfully employed. The features of generalized tonic-clonic seizures, autoimmune abnormalities, ocular abnormalities, morphea en coup de sabre and brain imaging abnormalities were present. Areas of parietal lobe cerebral calcification were encountered on the computed tomographic scan and bilateral periventricular white matter changes on the magnetic resonance imaging with frontal, temporal and parietal lobe brain atrophy ipsilateral to the facial hemiatrophy...
September 30, 2016: Neurology International
Nathan D Grubaugh, Kristian G Andersen
The epidemics of Ebola virus in West Africa and Zika virus in America highlight how viruses can explosively emerge into new territories. These epidemics also exposed how unprepared we are to handle infectious disease emergencies. This is also true when we consider hypothesized new clinical features of infection, such as the associations between Zika virus infection and severe neurological disease, including microcephaly and Guillain-Barré syndrome. On the surface, these pathologies appear to be new features of Zika virus infection, however, causal relationships have not yet been established...
2016: F1000Research
Sean Ekins, John Liebler, Bruno J Neves, Warren G Lewis, Megan Coffee, Rachelle Bienstock, Christopher Southan, Carolina H Andrade
The Zika virus (ZIKV) is a flavivirus of the family Flaviviridae, which is similar to dengue virus, yellow fever and West Nile virus. Recent outbreaks in South America, Latin America, the Caribbean and in particular Brazil have led to concern for the spread of the disease and potential to cause Guillain-Barré syndrome and microcephaly. Although ZIKV has been known of for over 60 years there is very little in the way of knowledge of the virus with few publications and no crystal structures. No antivirals have been tested against it either in vitro or in vivo...
2016: F1000Research
P Gouda, J Chua, D Langan, T Hannon, A Scott, A O'Regan
BACKGROUND: While domiciliary non-invasive ventilation (NIV) was initially used in the treatment of respiratory failure resulting from chest wall deformity, the main indication is now chronic obstructive pulmonary disease (COPD) with recurrent exacerbations associated with type 2 respiratory failure. A longitudinal study of domiciliary NIV provides insights into the evolution of this treatment in the west of Ireland. METHODS: The cohort of patients receiving new prescriptions for domiciliary NIV from Galway University Hospital from 2000 to 2012 was reviewed using study coordinator chart reviews and telephone follow-ups...
October 15, 2016: Irish Journal of Medical Science
X Qi, G Han, X Guo, V De Stefano, K Xu, Z Lu, H Xu, A Mancuso, W Zhang, X Han, D C Valla, D Fan
BACKGROUND: China may have the largest number of Budd-Chiari syndrome (BCS) cases in the world (at least 1914 original papers were published, and at least 20 191 BCS patients were reported). Considering the discrepancy in the clinical profiles and preferred treatment selection of primary BCS between the West and China, understanding its aetiology in these two different regions is very important. AIM: To review the data from large cohort studies and meta-analyses to illustrate the epidemiology of risk factors for BCS in the West and China...
October 13, 2016: Alimentary Pharmacology & Therapeutics
Satyendra Khichar, Shyama Choudhary, Veer Bahadur Singh, Priyanka Tater, Arvind Rv
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Abdelrahman Ibrahim Abushouk, Ahmed Negida, Hussien Ahmed
The current outbreak of Zika virus (ZIKV) in South America is one of the most serious public health emergencies since the Ebola outbreak of West Africa [2014]. ZIKV belongs to the flaviviridae family and has two lineages (Asian and African). The virus was first discovered in Uganda [1947] and the first human infection was identified in Nigeria [1952]. The current epidemic is the third of its type after that of Yap Island, Micronesia [2007] and French Polynesia [2013]. Phylogenetic studies revealed that the current strain shares about 99...
October 3, 2016: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
Rupsa Basu, Ebenezer Tumban
Zika virus (ZIKV) is a mosquito-borne flavivirus that is transmitted through the bite of Aedes spp mosquitoes and less predominantly, through sexual intercourse. Prior to 2007, ZIKV was associated with only sporadic human infections with minimal or no clinical manifestations. Recently the virus has caused disease outbreaks from the Pacific Islands, the Americas, and off the coast of West Africa with approximately 1.62 million people suspected to be infected in more than 60 countries around the globe. The recent ZIKV outbreaks have been associated with guillain-barré syndrome, congenital syndrome (microcephaly, congenital central nervous system anomalies), miscarriages, and even death...
October 6, 2016: Virology Journal
R Hofmann-Lehmann, N Wagmann, M L Meli, B Riond, M Novacco, D Joekel, F Gentilini, F Marsilio, M G Pennisi, A Lloret, T Carrapiço, F S Boretti
'Candidatus Neoehrlichia mikurensis' is an emerging tick-borne zoonotic agent that primarily affects immunocompromised human patients. Dogs and foxes are frequently exposed to ticks, and both species are in close proximity to humans. This is the first study to systematically investigate the occurrence of 'Candidatus Neoehrlichia mikurensis' in Canidae in Europa. We analyzed 1'739 blood samples from dogs in Switzerland, Italy, Spain and Portugal and 162 blood samples from free-ranging red foxes (Vulpes vulpes) in Switzerland...
October 2016: Schweizer Archiv Für Tierheilkunde
Nuri Kodaman, Melinda C Aldrich, Rafal Sobota, Folkert W Asselbergs, Nancy J Brown, Jason H Moore, Scott M Williams
BACKGROUND: Metabolic syndrome (MetS) is diagnosed by the presence of at least 3 of the following: obesity, hypertension, hyperglycemia, hypertriglyceridemia, and low high-density lipoprotein. Individuals with MetS also typically have elevated plasma levels of the antifibrinolytic factor, plasminogen activator inhibitor-1 (PAI-1), but the relationships between PAI-1 and MetS diagnostic criteria are not clear. Understanding these relationships can elucidate the relevance of MetS to cardiovascular disease risk, because PAI-1 is associated with ischemic events and directly involved in thrombosis...
October 3, 2016: Journal of the American Heart Association
E Jolivet, C Plavonil, M Sainte-Rose, B Schaub
OBJECTIVES: The aim of this study is to evaluate the screening for trisomy 21 (T21) between 2011 and 2013 on Martinique French West Indies after the decree of 23 June 2009. MATERIALS AND METHODS: Were used the prenatal data provided by accredited laboratories and the data from the Registry of Congenital French West Indies (REMALAN). RESULTS: A total of 85.9 % of patients underwent screening: 60.5 % on a combined calculation of risk (CRC), 14...
September 29, 2016: Journal de Gynécologie, Obstétrique et Biologie de la Reproduction
James Rush Priest, Charles Gawad, Kristopher M Kahlig, Joseph K Yu, Thomas O'Hara, Patrick M Boyle, Sridharan Rajamani, Michael J Clark, Sarah T K Garcia, Scott Ceresnak, Jason Harris, Sean Boyle, Frederick E Dewey, Lindsey Malloy-Walton, Kyla Dunn, Megan Grove, Marco V Perez, Norma F Neff, Richard Chen, Katsuhide Maeda, Anne Dubin, Luiz Belardinelli, John West, Christian Antolik, Daniela Macaya, Thomas Quertermous, Natalia A Trayanova, Stephen R Quake, Euan A Ashley
Somatic mosaicism, the occurrence and propagation of genetic variation in cell lineages after fertilization, is increasingly recognized to play a causal role in a variety of human diseases. We investigated the case of life-threatening arrhythmia in a 10-day-old infant with long QT syndrome (LQTS). Rapid genome sequencing suggested a variant in the sodium channel NaV1.5 encoded by SCN5A, NM_000335:c.5284G > T predicting p.(V1762L), but read depth was insufficient to be diagnostic. Exome sequencing of the trio confirmed read ratios inconsistent with Mendelian inheritance only in the proband...
October 11, 2016: Proceedings of the National Academy of Sciences of the United States of America
Takayuki Hatori, Yohei Sugiyama, Shinichiro Yamashita, Yuka Hirakubo, Kazuhito Nonaka, Ko Ichihashi
In a patient with cardiofaciocutaneous syndrome complicated by intractable infantile spasms (West syndrome), cardiac hypertrophy developed during adrenocorticotropic hormone treatment. Various types of antiepileptic drugs, intravenous immunoglobulin, thyrotropin releasing hormone, and a ketogenic diet were ineffective in this case. However, vigabatrin both decreased clinical seizures and improved electroencephalogram findings. Although vigabatrin has not been approved for use in Japan, the results in the present case suggest that this drug should be considered as an alternative therapy for cases of infantile spasms associated with syndromes involving cardiomyopathy or its potential risk factors, such as cardiofaciocutaneous syndrome...
2016: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
Karen Sousa, Natalia Decker, Thienne Rocha Pires, Débora Kuck Mausolff Papke, Vanessa Rodrigues Coelho, Pricila Pflüger, Patrícia Pereira, Jaqueline Nascimento Picada
RATIONALE: Vigabatrin (VGB) is a drug indicated mostly for the treatment of spasms in childhood and West's syndrome patients. This drug inhibits irreversibly the enzyme GABA-transaminase (GABA-T), increasing GABA concentrations and enhancing GABAergic neurotransmission in the brain, which is known to induce behavioral changes. OBJECTIVES: The aims of this study were to evaluate the effects of VGB in the short-term memory (STM), long-term memory (LTM), motivation, locomotion, and exploratory behavior tests and to detect deleterious or protective effects on DNA in target tissues of the drug...
September 27, 2016: Psychopharmacology
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