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Dennys syndrome

Usha E A Beijnen, Reid A Maclellan, Jeremy A Goss, Javier A Couto, Dennis J Konczyk, Arin K Greene
Beckwith-Wiedemann syndrome is the most common genetic overgrowth syndrome. Patients with Beckwith-Wiedemann syndrome may have hemihypertrophy, but their lymphatic vasculature is intact. We present a child with Beckwith-Wiedemann syndrome and lower extremity enlargement thought to be due to hemihypertrophy that was instead diagnosed with primary lymphedema. There are many causes of leg overgrowth in the pediatric population and misdiagnosis is common. While extremity enlargement secondary to hemihypertrophy may occur in 15% of patients with Beckwith-Wiedemann syndrome, progression and pitting edema only occur in primary lymphedema...
October 25, 2016: Pediatric Dermatology
Damian Hoy, Salanieta T Saketa, Roy Roger Maraka, Alison Sio, Ian Wanyeki, Pascal Frison, Divi Ogaoga, Dennie Iniakawala, Cynthia Joshua, Sala Duituturaga, Christelle Lepers, Adam Roth, Paul White, Yvan Souares
Mass gatherings pose public health challenges to host countries, as they can cause or exacerbate disease outbreaks within the host location or elsewhere. In July 2012, the 11th Festival of Pacific Arts (FOPA), a mass gathering event involving 22 Pacific island states and territories, was hosted by Solomon Islands. An enhanced syndromic surveillance (ESS) system was implemented for the event. Throughout the capital city, Honiara, 15 sentinel sites were established and successfully took part in the ESS system, which commenced one week before the FOPA (25 June) and concluded eight days after the event (22 July)...
July 2016: Western Pacific Surveillance and Response Journal: WPSAR
Suman Pradhan, Christine Pellino, Kayleigh MacMaster, Dennis Coyle, Alison A Weiss
Seizures and neurologic involvement have been reported in patients infected with Shiga toxin (Stx) producing E. coli, and hemolytic uremic syndrome (HUS) with neurologic involvement is associated with more severe outcome. We investigated the extent of renal and neurologic damage in mice following injection of the highly potent form of Stx, Stx2a, and less potent Stx1. As observed in previous studies, Stx2a brought about moderate to acute tubular necrosis of proximal and distal tubules in the kidneys. Brain sections stained with hematoxylin and eosin (H&E) appeared normal, although some red blood cell congestion was observed...
2016: Frontiers in Cellular and Infection Microbiology
Benjamin Johnstone, Dennis Velakoulis, Cheng Yi Yuan, Anthony Ang, Chris Steward, Patricia Desmond, Terence J O'Brien
OBJECTIVE: Exposure to early life childhood trauma has been implicated as resulting in a vulnerability to epileptic and psychogenic nonepileptic seizures (PNES), hippocampal atrophy, and psychiatric disorders. This study aimed to explore the relationships between childhood trauma, epilepsy, PNES, and hippocampal volume in patients admitted to a video-electroencephalogram monitoring (VEM) unit. METHODS: One hundred thirty-one patients were recruited from the Royal Melbourne Hospital VEM unit...
October 12, 2016: Epilepsy & Behavior: E&B
Veerasathpurush Allareddy, Nicholas Ching, Eric A Macklin, Lauren Voelz, Gil Weintraub, Emily Davidson, Lisa Albers Prock, Dennis Rosen, Richard Brunn, Brian G Skotko
OBJECTIVE: The objective of the present study is to examine the craniofacial development of patients with Down syndrome (DS) and compare them with a neurotypical population. METHODS: This study is a cross-sectional analysis of lateral cephalometric radiographs of participants with DS. The study population consisted of children and young adults with DS aged 3-25 years. Cephalometric data were summarized by age and sex. Raw and normalized z-scores were computed. One-sample t tests were used to test whether mean z-scores differed from zero...
December 2016: Progress in Orthodontics
Stephanie R Harrison, Dennis McGonagle, Sharmin Nizam, Stephen Jarrett, Jeroen van der Hilst, Michael F McDermott, Sinisa Savic
BACKGROUND: Some adult patients presenting with unexplained pyrexia, serositis, skin rashes, arthralgia, myalgia, and other symptoms commonly found in autoinflammatory disorders may not fit a specific diagnosis, either because their clinical phenotype is nondiagnostic or genetic tests are negative. We used the term undifferentiated systemic autoinflammatory disorder (uSAID) to describe such cases. Given that well-defined autoinflammatory diseases show responses to IL-1 blockade, we evaluated whether anakinra was useful for both diagnosing and treating uSAID patients...
May 5, 2016: JCI Insight
Sean D McEvoy, Amy Lee, Andrew Poliakov, Seth Friedman, Dennis Shaw, Samuel R Browd, Richard G Ellenbogen, Jeffrey G Ojemann, Christine L Mac Donald
Posterior fossa syndrome is a severe transient loss of language that frequently complicates resection of tumors of the cerebellum. The associated pathophysiology and relevant anatomy to this language deficit remains controversial. We performed a retrospective analysis of all cerebellar tumor resections at Seattle Children's Hospital from 2010 to 2015. Diffusion tensor imaging was performed on each of the patients as part of their clinical scan. Patients included in the study were divided into groups based on language functioning following resection: intact (N = 19), mild deficit (N = 19), and posterior fossa syndrome (N = 9)...
2016: NeuroImage: Clinical
Sergio Leonardi, Enrico Frigoli, Martina Rothenbühler, Eliano Navarese, Paolo Calabró, Paolo Bellotti, Carlo Briguori, Marco Ferlini, Bernardo Cortese, Alessandro Lupi, Salvatore Lerna, Dennis Zavallonito-Parenti, Giovanni Esposito, Simone Tresoldi, Antonio Zingarelli, Stefano Rigattieri, Cataldo Palmieri, Armando Liso, Fabio Abate, Marco Zimarino, Marco Comeglio, Gabriele Gabrielli, Alaide Chieffo, Salvatore Brugaletta, Ciro Mauro, Nicolas M Van Mieghem, Dik Heg, Peter Jüni, Stephan Windecker, Marco Valgimigli
OBJECTIVE:  To test the optimal antithrombotic regimen in patients with acute coronary syndrome. DESIGN:  Randomised controlled trial. SETTING:  Patients with acute coronary syndrome with and without ST segment elevation in 78 centres in Italy, the Netherlands, Spain, and Sweden. PARTICIPANTS:  7213 patients with acute coronary syndrome and planned percutaneous coronary intervention: 4010 with ST segment elevation and 3203 without ST segment elevation...
September 27, 2016: BMJ: British Medical Journal
Kaushik Parvathaneni, Sanjay Belani, Dennis Leung, Christopher J L Newth, Robinder G Khemani
OBJECTIVE: The Pediatric Acute Lung Injury Consensus Conference has developed a pediatric-specific definition of acute respiratory distress syndrome, which is a significant departure from both the Berlin and American European Consensus Conference definitions. We sought to test the external validity and potential impact of the Pediatric Acute Lung Injury Consensus Conference definition by comparing the number of cases of acute respiratory distress syndrome and mortality rates among children admitted to a multidisciplinary PICU when classified by Pediatric Acute Lung Injury Consensus Conference, Berlin, and American European Consensus Conference criteria...
September 23, 2016: Pediatric Critical Care Medicine
Susan L Rossell, Paul S Francis, Cherrie Galletly, Anthony Harris, Dan Siskind, Michael Berk, Kiymet Bozaoglu, Frances Dark, Olivia Dean, Dennis Liu, Denny Meyer, Erica Neill, Andrea Phillipou, Jerome Sarris, David J Castle
BACKGROUND: Clozapine is an effective treatment for a proportion of people with schizophrenia (SZ) who are resistant to the beneficial effects of other antipsychotic drugs. However, anything from 40-60 % of people on clozapine experience residual symptoms even on adequate doses of the medication, and thus could be considered 'clozapine resistant'. Agents that could work alongside clozapine to improve efficacy whilst not increasing the adverse effect burden are both desired and necessary to improve the lives of individuals with clozapine-resistant SZ...
September 15, 2016: BMC Psychiatry
A K Burnett, N H Russell, R K Hills, J Kell, O J Nielsen, M Dennis, P Cahalin, C Pocock, S Ali, S Burns, S Freeman, D Milligan, R E Clark
The study was designed to compare clofarabine plus daunorubicin versus daunorubicin/ara-C in older patients with acute myeloid leukaemia (AML) or high-risk myelodysplastic syndrome (MDS). Eight hundred and six untreated patients in the UK NCRI AML16 trial with AML/high-risk MDS (median age, 67yrs; range 56-84) and normal serum creatinine were randomised to two courses of induction chemotherapy with either daunorubicin/ara-C (DA) or daunorubicin/clofarabine (DClo). Patients were also included in additional randomisations; +/- one dose of gemtuzumab ozogamicin in course 1; 2v3 courses and +/- azacitidine maintenance...
September 2, 2016: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Neil Thomas, John Farhall, Fiona Foley, Susan L Rossell, David Castle, Emma Ladd, Denny Meyer, Cathrine Mihalopoulos, Nuwan Leitan, Cassy Nunan, Rosalie Frankish, Tara Smark, Sue Farnan, Bronte McLeod, Leon Sterling, Greg Murray, Ellie Fossey, Lisa Brophy, Michael Kyrios
BACKGROUND: Psychosocial interventions have an important role in promoting recovery in people with persisting psychotic disorders such as schizophrenia. Readily available, digital technology provides a means of developing therapeutic resources for use together by practitioners and mental health service users. As part of the Self-Management and Recovery Technology (SMART) research program, we have developed an online resource providing materials on illness self-management and personal recovery based on the Connectedness-Hope-Identity-Meaning-Empowerment (CHIME) framework...
2016: BMC Psychiatry
Marjan Islam, Dennis Karter, Jerry Altshuler, Diana Altshuler, David Schwartz, Gianluca Torregrossa
Infections from Streptococcus dysgalactiae ssp. equisimilis (SDSE) can cause a wide variety of infections, ranging from mild cellulitis to invasive disease, such as endocarditis and streptococcal toxic shock-like syndrome (TSLS). Despite prompt and appropriate antibiotics, mortality rates associated with shock have remained exceedingly high, prompting the need for adjunctive therapy. IVIG has been proposed as a possible adjunct, given its ability to neutralize a wide variety of superantigens and modulate a dysregulated inflammatory response...
2016: Case Reports in Infectious Diseases
Dennis K Ledford, Sally Wenzel
No abstract text is available yet for this article.
September 2016: Journal of Allergy and Clinical Immunology in Practice
Stephen Bruehl, Richard Ohrbach, Sonia Sharma, Eva Widerstrom-Noga, Robert H Dworkin, Roger B Fillingim, Dennis C Turk
UNLABELLED: The Analgesic, Anesthetic, and Addiction Clinical Trial Translations, Innovations, Opportunities, and Networks-American Pain Society Pain Taxonomy (AAPT) is designed to be an evidence-based multidimensional chronic pain classification system that will facilitate more comprehensive and consistent chronic pain diagnoses, and thereby enhance research, clinical communication, and ultimately patient care. Core diagnostic criteria (dimension 1) for individual chronic pain conditions included in the initial version of AAPT will be the focus of subsequent empirical research to evaluate and provide evidence for their reliability and validity...
September 2016: Journal of Pain: Official Journal of the American Pain Society
Zeljka Calic, Cecilia Cappelen-Smith, Craig S Anderson, Wei Xuan, Dennis J Cordato
BACKGROUND AND PURPOSE: The diagnosis of cerebellar infarction (CBI) is often challenging due to non-specific or subtle presenting symptoms and signs. We aimed to determine whether a common syndromic cluster of symptoms, signs or vascular risk factors were associated with delayed presentation or misdiagnosis to an Emergency Department (ED). The degree of misdiagnosis between ED and neurology physicians and the influence of delayed presentation or misdiagnosis on outcome were also investigated...
August 27, 2016: Cerebrovascular Diseases
Michele Pohlen, Nils H Thoennissen, Jan Braess, Johannes Thudium, Christoph Schmid, Matthias Kochanek, Karl-Anton Kreuzer, Pia Lebiedz, Dennis Görlich, Hans U Gerth, Christian Rohde, Torsten Kessler, Carsten Müller-Tidow, Matthias Stelljes, Thomas Büchner, Günter Schlimok, Michael Hallek, Johannes Waltenberger, Wolfgang Hiddemann, Wolfgang E Berdel, Bernhard Heilmeier, Utz Krug
BACKGROUND: This retrospective, multicenter study aimed to reveal risk predictors for mortality in the intensive care unit (ICU) as well as survival after ICU discharge in patients with acute myeloid leukemia (AML) requiring treatment in the ICU. METHODS AND RESULTS: Multivariate analysis of data for 187 adults with AML treated in the ICU in one institution revealed the following as independent prognostic factors for death in the ICU: arterial oxygen partial pressure below 72 mmHg, active AML and systemic inflammatory response syndrome upon ICU admission, and need for hemodialysis and mechanical ventilation in the ICU...
2016: PloS One
Sampat Sindhar, Michael Lugo, Mark D Levin, Joshua R Danback, Benjamin D Brink, Eric Yu, Dennis J Dietzen, Amy L Clark, Carolyn A Purgert, Jessica L Waxler, Robert W Elder, Barbara R Pober, Beth A Kozel
OBJECTIVE: To evaluate the timing, trajectory, and implications of hypercalcemia in Williams-Beuren syndrome (WBS) through a multicenter retrospective study. STUDY DESIGN: Data on plasma calcium levels from 232 subjects with WBS aged 0-67.1 years were compared with that in controls and also with available normative data. Association testing was used to identify relevant comorbidities. RESULTS: On average, individuals with WBS had higher plasma calcium levels than controls, but 86...
August 26, 2016: Journal of Pediatrics
Lucia Tomas-Aragones, Silla M Consoli, Sylvie G Consoli, Francoise Poot, Klaus-Michael Taube, M Dennis Linder, Gregor B E Jemec, Jacek C Szepietowski, John de Korte, Andrey N Lvov, Uwe Gieler
The classification of self-inflicted skin lesions proposed by the European Society for Dermatology and Psychiatry (ESDaP) group generated questions with regard to specific treatments that could be recommended for such cases. The therapeutic guidelines in the current paper integrate new psychotherapies and psychotropic drugs without forgetting the most important relational characteristics required for dealing with people with these disorders. The management of self-inflicted skin lesions necessitates empathy and a doctor-patient relationship based on trust and confidence...
August 26, 2016: Acta Dermato-venereologica
Shi-Jiao He, Cynthia Chan, Zhi-Dong Xie, Dian Shi, Xiao-Bin Hu, Hai-Yan Li, Dennis Wang, Min-Zhen Wang, Ya-Na Bai
The aim of the study was to explore the association between serum uric acid (SUA) and metabolic syndrome (MetS) in premenopausal and postmenopausal women in the Jinchang Cohort. We studied 3808 female Jinchuan Nonferrous Metals Corporation workers aged 40-60 years. Cohort data from epidemiological surveys and medical exams were used. MetS was defined using the 2009 Joint Interim Society criteria. The relationship between SUA and MetS was evaluated using multiple logistic regression after adjusting for potential confounders...
August 25, 2016: Gynecological Endocrinology
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