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https://www.readbyqxmd.com/read/28163843/outcome-of-guillain-barr%C3%A3-syndrome-in-children-a-prospective-cohort-study-in-a-tertiary-hospital-in-upper-egypt
#1
Abdelrahim Abdrabou Sadek, Ashraf Abou-Taleb, Wafaa Ahmed Ali
INTRODUCTION: Guillain-Barré syndrome is the most common cause of acute flaccid paralysis in children, and defined as an acute inflammatory polyneuropathy. The objective of this study was to assess the clinico-laboratory profile, and outcome of Guillain-Barré syndrome in children at Sohag University Hospital. METHODS: This prospective cohort observational study was conducted in 2014-2015. The included children were subjected to through medical history and detailed systemic and neurological examination...
December 2016: Electronic Physician
https://www.readbyqxmd.com/read/28149085/complications-in-mechanically-ventilated-patients-of-guillain-barre-syndrome-and-their-prognostic-value
#2
Archana Becket Netto, Arun B Taly, Girish B Kulkarni, G S Uma Maheshwara Rao, Shivaji Rao
INTRODUCTION: The spectrum of various complications in critically ill Guillain-Barre syndrome (GBS) and its effect on the prognosis is lacking in literature. This study aimed at enumerating the complications in such a cohort and their significance in the prognosis and mortality. MATERIALS AND METHODS: Retrospective case record analysis of all consecutive mechanically ventilated patients of GBS in neurology Intensive Care Unit (ICU) of a tertiary care institute for 10 years was done...
January 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28148674/computer-hexapod-assisted-orthopaedic-surgery-provides-a-predictable-and-safe-method-of-femoral-deformity-correction
#3
A Hughes, N Heidari, S Mitchell, J Livingstone, M Jackson, R Atkins, F Monsell
AIMS: Computer hexapod assisted orthopaedic surgery (CHAOS), is a method to achieve the intra-operative correction of long bone deformities using a hexapod external fixator before definitive internal fixation with minimally invasive stabilisation techniques. The aims of this study were to determine the reliability of this method in a consecutive case series of patients undergoing femoral deformity correction, with a minimum six-month follow-up, to assess the complications and to define the ideal group of patients for whom this treatment is appropriate...
February 2017: Bone & Joint Journal
https://www.readbyqxmd.com/read/28137504/etiology-and-treatment-of-cough-in-idiopathic-pulmonary-fibrosis
#4
REVIEW
Christine L Vigeland, Andrew H Hughes, Maureen R Horton
Idiopathic pulmonary fibrosis (IPF) is a progressive disease of dysregulated wound healing leading to unremitting scarring and loss of lung function. The predominant symptoms are dyspnea on exertion and a persistent dry cough. For patients with IPF, cough is more than just bothersome; it has a significant negative impact on quality of life and is a marker of disease severity and progression. The etiology of cough in IPF is unclear but may be due to architectural distortion of the lungs, increased sensitivity of the cough reflex, airway inflammation, or changes in mucus production and clearance...
February 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28130593/functional-outcome-following-rectal-surgery-predisposing-factors-for-low-anterior-resection-syndrome
#5
Daniel Ll Hughes, Julie Cornish, Chris Morris
PURPOSE: Developments in surgical techniques and neoadjuvant treatment have enabled an increasing proportion of patients with rectal cancer to undergo sphincter-sparing resections. The avoidance of a permanent stoma can come at the cost of poor bowel function which can significantly impact patients' quality of life. The objective of this study was to identify the incidence and risk factors for the development of bowel dysfunction following rectal cancer surgery. METHODS: Patients undergoing anterior resection for rectal cancer between January 2009 and January 2015 were identified from a rectal cancer database at a single centre...
January 27, 2017: International Journal of Colorectal Disease
https://www.readbyqxmd.com/read/28117080/genomic-and-clinical-profiling-of-a-national-nephrotic-syndrome-cohort-advocates-a-precision-medicine-approach-to-disease-management
#6
Agnieszka Bierzynska, Hugh J McCarthy, Katrina Soderquest, Ethan S Sen, Elizabeth Colby, Wen Y Ding, Marwa M Nabhan, Larissa Kerecuk, Shivram Hegde, David Hughes, Stephen Marks, Sally Feather, Caroline Jones, Nicholas J A Webb, Milos Ognjanovic, Martin Christian, Rodney D Gilbert, Manish D Sinha, Graham M Lord, Michael Simpson, Ania B Koziell, Gavin I Welsh, Moin A Saleem
Steroid Resistant Nephrotic Syndrome (SRNS) in children and young adults has differing etiologies with monogenic disease accounting for 2.9-30% in selected series. Using whole exome sequencing we sought to stratify a national population of children with SRNS into monogenic and non-monogenic forms, and further define those groups by detailed phenotypic analysis. Pediatric patients with SRNS were identified via a national United Kingdom Renal Registry. Whole exome sequencing was performed on 187 patients, of which 12% have a positive family history with a focus on the 53 genes currently known to be associated with nephrotic syndrome...
January 20, 2017: Kidney International
https://www.readbyqxmd.com/read/28116566/an-integrated-soft-computing-approach-to-hughes-syndrome-risk-assessment
#7
João Vilhena, M Rosário Martins, Henrique Vicente, José M Grañeda, Filomena Caldeira, Rodrigo Gusmão, João Neves, José Neves
The AntiPhospholipid Syndrome (APS) is an acquired autoimmune disorder induced by high levels of antiphospholipid antibodies that cause arterial and veins thrombosis, as well as pregnancy-related complications and morbidity, as clinical manifestations. This autoimmune hypercoagulable state, usually known as Hughes syndrome, has severe consequences for the patients, being one of the main causes of thrombotic disorders and death. Therefore, it is required to be preventive; being aware of how probable is to have that kind of syndrome...
March 2017: Journal of Medical Systems
https://www.readbyqxmd.com/read/28109652/epilepsy-in-patients-with-grin2a-alterations-genetics-neurodevelopment-epileptic-phenotype-and-response-to-anticonvulsive-drugs
#8
C von Stülpnagel, M Ensslen, R S Møller, D K Pal, S Masnada, P Veggiotti, E Piazza, M Dreesmann, T Hartlieb, T Herberhold, E Hughes, M Koch, C Kutzer, K Hoertnagel, J Nitanda, M Pohl, K Rostásy, T B Haack, K Stöhr, G Kluger, I Borggraefe
OBJECTIVE: To delineate the genetic, neurodevelopmental and epileptic spectrum associated with GRIN2A alterations with emphasis on epilepsy treatment. METHODS: Retrospective study of 19 patients (7 females; age: 1-38 years; mean 10.1 years) with epilepsy and GRIN2A alteration. Genetic variants were classified according to the guidelines and recommendations of the American College of Medical Genetics (ACMG). Clinical findings including epilepsy classification, treatment, EEG findings, early childhood development and neurodevelopmental outcome were collected with an electronic questionnaire...
January 14, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28098766/single-electrode-deep-brain-stimulation-with-dual-targeting-at-dual-frequency-for-the-treatment-of-chronic-pain-a-case-series-and-review-of-the-literature
#9
REVIEW
Milo Hollingworth, Hugh P Sims-Williams, Anthony E Pickering, Neil Barua, Nikunj K Patel
Deep Brain Stimulation (DBS) has been used to target many deep brain structures for the treatment of chronic pain. The periaqueductal grey and periventricular grey (PAG/PVG) is an effective target but results are variable, sometimes short-lived or subject to tolerance. The centromedian intra-laminar parafascicular complex (CMPf) modulates medial pain pathways and CMPf DBS may address the affective aspects of pain perception. Stimulation of multiple deep brain targets may offer a strategy to optimize management of patients with complex pain symptomatology...
January 13, 2017: Brain Sciences
https://www.readbyqxmd.com/read/28086838/clinical-characteristics-of-genital-chlamydia-infection-in-pelvic-inflammatory-disease
#10
Sung Taek Park, Suk Woo Lee, Min Jeong Kim, Young Mo Kang, Hye Min Moon, Chae Chun Rhim
BACKGROUND: Chlamydia infection in acute pelvic inflammatory disease (PID) is associated with serious complications including ectopic pregnancy, tubal infertility, Fitz-Hugh-Curtis syndrome and tubo-ovarian abscess (TOA). This study compared clinical and laboratory data between PID with and without chlamydia infection. METHODS: The medical records of 497 women who were admitted with PID between 2002 and 2011 were reviewed. The patients were divided into two groups (PID with and without chlamydia infection), which were compared in terms of the patients' characteristics, clinical presentation, and laboratory findings, including inflammatory markers...
January 13, 2017: BMC Women's Health
https://www.readbyqxmd.com/read/28074391/etiologic-spectrum-and-functional-outcome-of-the-acute-inflammatory-myelitis
#11
Edgar Carnero Contentti, Javier Pablo Hryb, Ana Diego, José Luis Di Pace, Mónica Perassolo
Clinical, neuroimaging, and laboratory features are not specific enough to establish the etiological diagnosis of the acute inflammatory myelitis (AIM). Longitudinally extensive transverse myelitis (LETM) seen on magnetic resonance imaging (MRI) has been associated with a poor functional prognosis. The aim of this study was to assess the functional outcomes of a first AIM event comparing patients with LETM vs. no LETM on MRI and to report the differential diagnosis. Clinical, radiological, biochemical aspects were collected, and Winner-Hughes Functional Disability Scale (WHFDS) was performed after 3 and 6 months...
January 10, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28062188/a-comprehensive-analysis-and-immunobiology-of-autoimmune-neurological-syndromes-during-the-zika-virus-outbreak-in-c%C3%A3%C2%BAcuta-colombia
#12
Juan-Manuel Anaya, Yhojan Rodríguez, Diana M Monsalve, Daniel Vega, Ernesto Ojeda, Diana González-Bravo, Mónica Rodríguez-Jiménez, Carlos A Pinto-Díaz, Pablo Chaparro, María L Gunturiz, Aftab A Ansari, M Eric Gershwin, Nicolás Molano-González, Carolina Ramírez-Santana, Yeny Acosta-Ampudia
We have focused on the epidemiology and immunobiology of Zika virus (ZIKV) infection and factors associated with the development of Guillain-Barré syndrome (GBS) and other neurological syndromes in Cúcuta, the capital of North Santander department, Colombia. Data of patients with ZIKV disease reported to the national population-based surveillance system were used to calculate the basic reproduction number (R0) and the attack rates (ARs) as well as to develop epidemiological maps. Patients with neurological syndromes were contacted and their diagnoses were confirmed...
February 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28057084/chlamydia-trachomatis-induced-fitz-hugh-curtis-syndrome-a-case-report
#13
Cyril Jabea Ekabe, Jules Kehbila, Tsi Njim, Benjamin Momo Kadia, Celestine Ntemlefack Tendonge, Gottlieb Lobe Monekosso
BACKGROUND: Fitz-Hugh-Curtis syndrome is defined as perihepatitis associated with pelvic inflammatory disease. Chlamydia trachomatis is one of its most common aetiologies. This syndrome usually presents with right upper quadrant abdominal pain mimicking other hepatobiliary and gastrointestinal pathologies, hence, posing a diagnostic dilemma in settings with limited diagnostic tools. CASE REPORT: A 32 year old African female presented with acute right upper quadrant abdominal pain and vaginal discharge, for which she had previously received treatment in another health center with no improvement...
January 3, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28042737/thyroid-hormone-level-is-associated-with-the-frequency-and-severity-of-guillain-barr%C3%A3-syndrome
#14
Yuanyuan Huang, Zhaojian Ying, Zhibo Chen, Weiwei Xiang, Zhongqian Su, Weiwei Quan, Yiyun Weng, Xu Zhang
OBJECTIVE: Guillain-Barré syndrome (GBS) is a neurodegenerative and inflammatory demyelination disorder, and oxidative stress is concerned with the pathogenesis of the disease. Also, we found that thyroid hormone level is correlated to the oxidative and antioxidant status in previous studies. Our study was aimed to find the possible relationship between the frequency and severity of GBS and thyroid hormone levels. MATERIALS AND METHODS: We measured serum levels of thyroid-stimulating hormone (TSH), free thyroxine (FT4) and free triiodothyronine (FT3) in 238 individuals, including 90 GBS, 44 multiple sclerosis and 104 healthy controls...
January 16, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/28027331/is-serum-albumin-associated-with-guillain-barr%C3%A3-syndrome-outcomes
#15
Richard A C Hughes
No abstract text is available yet for this article.
February 1, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28003317/rare-small-intestinal-volvulus-from-entrapment-in-hepato-diaphragmatic-adhesions-in-a-45-year-old-lady
#16
Iyiade Olatunde Olaoye, Micheal Dapo Adesina
Small intestinal volvulus is rare in adults and rarely caused by string adhesions between the liver and the diaphragm. Similar adhesions were described in Fitz-Hugh-Curtis syndrome. We report a 45-year-old lady with small intestinal volvulus from entrapment of a loop in string adhesions between the liver and the diaphragm. Her plain radiographs showed a significant shadow of the trapped loop.
December 20, 2016: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/27943236/the-treatment-of-hidradenitis-suppurativa-with-the-glucagon-like-peptide-1-agonist-liraglutide
#17
L Jennings, L Nestor, O Molloy, R Hughes, B Moriarty, B Kirby
Hidradenitis suppurativa (HS) is a cutaneous disease associated with systemic inflammation, obesity and metabolic syndrome. Effective treatment options are limited. The anti-diabetic agents, incretins, have been used successfully to treat psoriasis - a disease also associated with metabolic syndrome. We report the use of liraglutide, a glucagon-like peptide -1 agonist, in a patient with HS, leading to subsequent weight loss and improvement in disease control. To our knowledge, this is the first report of liraglutide used in the treatment of HS...
December 11, 2016: British Journal of Dermatology
https://www.readbyqxmd.com/read/27932480/magi2-mutations-cause-congenital-nephrotic-syndrome
#18
Agnieszka Bierzynska, Katrina Soderquest, Philip Dean, Elizabeth Colby, Ruth Rollason, Caroline Jones, Carol D Inward, Hugh J McCarthy, Michael A Simpson, Graham M Lord, Maggie Williams, Gavin I Welsh, Ania B Koziell, Moin A Saleem
Steroid-resistant nephrotic syndrome (SRNS), a heterogeneous disorder of the renal glomerular filtration barrier, results in impairment of glomerular permselectivity. Inheritance of genetic SRNS may be autosomal dominant or recessive, with a subset of autosomal recessive SRNS presenting as congenital nephrotic syndrome (CNS). Mutations in 53 genes are associated with human SRNS, but these mutations explain ≤30% of patients with hereditary cases and only 20% of patients with sporadic cases. The proteins encoded by these genes are expressed in podocytes, and malfunction of these proteins leads to a universal end point of podocyte injury, glomerular filtration barrier disruption, and SRNS...
December 8, 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27921062/long-qt-syndrome-and-duodenal-ampullary-adenoma-a-new-association
#19
F N U Asad-Ur-Rahman, Laura Hughes, Muhammad Talha Khan, Muhammad Khalid Hasan, Irteza Inayat
KCNQ1 gene mutation has a well-known association with long QT syndrome (LQTS). However, recent studies suggest that it may be implicated in intestinal neoplasia. We present a 27-year-old Hispanic man with a known history of LQTS secondary to KCNQ1 mutation, who presented with painless jaundice. Endoscopic retrograde pancreatic cholangiography revealed a prominent ampulla, with histology consistent with ampullary adenoma with high-grade dysplasia. Further endoscopic studies did not suggest familial adenomatous polyposis...
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/27903855/fitz-hugh-curtis-syndrome-consequent-to-a-wound-infection-following-removal-of-a-peritoneal-dialysis-catheter
#20
Tal Zilberman-Daniels, Keren Cohen-Hagai, Yael Einbinder, Alexandra Osadchy, Sydney Benchetrit, Ze'ev Korzets
Fitz-Hugh-Curtis syndrome (FHCS) is a condition characterized by inflammation of the liver capsule (perihepatitis) and adjacent peritoneal surfaces. We report a case of FHCS developing in a peritoneal dialysis (PD) patient in whom catheter removal due to recurrent peritonitis was complicated by post-operative wound infection. To the best of our knowledge, this is the first case description of FHCS in the context of PD.
November 2016: Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis
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