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Monti Giacomo G Department of Anesthesia and Intensive Care, IRCCS San Raffaele Scientific Institute, Milan, - - 2015
Sepsis is one of the oldest and most elusive syndromes in medicine. With the confirmation of germ theory by Semmelweis, Pasteur, and others, sepsis was considered as a systemic infection by a pathogenic organism. Although the germ is probably the beginning of the syndrome and one of the major enemies ...
Miloslavsky Eli M EM Rheumatology Unit, Massachusetts General Hospital, Yawkey 2, 55 Fruit Street, Boston, MA 02114, - - 2015
The need to distinguish true primary systemic vasculitis from its multiple potential mimickers is one of the most challenging diagnostic conundrums in clinical medicine. This article reviews 9 challenging vasculitis mimickers: fibromuscular dysplasia, calciphylaxis, segmental arterial mediolysis, antiphospholipid syndrome, hypereosinophilic syndrome, lymphomatoid granulomatosis, malignant atrophic papulosis, livedoid vasculopathy, and immunoglobulin ...
Zhao Defeng D Department of Hematology, Navy General Hospital, Beijing 100048, P.R. - - 2014
Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome, is an aggressive hyperinflammatory condition characterized by prolonged fever, cytopenias and hepatosplenomegaly, as well as hemophagocytosis by activated, morphologically benign macrophages. HLH may be characterized into two forms, familial and secondary HLH. Familial HLH usually manifests in children with genetic abnormalities associated ...
Donovan Summer S Pediatric Infectious Diseases Fellow, Virginia Commonwealth University Health System, VCU Sanger Hall 12-041, P.O. Box 980163, Richmond, VA, 23298-0163, USA, - - 2014
Intravenous immunoglobulin (IVIG) has been suggested for the treatment of many ailments due to its ability to modulate the immune system and to provide passive immunity to commonly circulating pathogens. Its use as primary and adjunctive therapy for the treatment of conditions affecting critically ill patients is an attractive option, ...
Hidlay Douglas T DT Department of Neurosurgery, Virginia Commonwealth University, 417 North 11th Street, P.O. Box 980631, Richmond, VA 23298 - - 2014
Thoracic outlet syndrome is an uncommon cause of upper extremity neuropathy resulting from either neurogenic or vascular compromise in the thoracic outlet. Congenital anomalies as the cause of this disease process represent a minority of cases, and the literature regarding their surgical management is still evolving. A 42-year-old female without ...
Vigneri Simone S *Department of Neuroscience/Rehabilitation, Neurophysiology Unit, Arcispedale Sant'Anna, University of Ferrara, Ferrara, Italy; and †Department of Morphology, Surgery and Experimental Medicine, Anaesthesia and Intensive Care Unit, Arcispedale Sant'Anna, University of Ferrara, Ferrara, - - 2014
A 69-year-old white man was admitted because of a clinical history of persistent cough and fever. Chest x-rays showed bilateral lung infiltrates with air bronchograms, whereas the urine antigen test resulted positive for Legionella pneumophila. The next day, he was transferred to the intensive care unit and intubated because of ...
Siddiqui Nauman N 1Department of Medicine, University of Toledo Medical Center, Toledo, OH; and 2Division of Hematology and Oncology, Department of Pathology, University of Toledo Medical Center, Toledo, - - 2014
Evan syndrome (ES) is a rare hematological disorder that involves 2 or more immune cytopenias. It usually includes autoimmune hemolytic anemia and autoimmune thrombocytopenia. Although occasionally associated with immune neutropenia, its association with disseminated intravascular coagulation (DIC) is rare. And, early diagnosis with appropriate intervention is important because mortality from ...
Spiri Daniele - - 2014
ImportanceMigraine is a common disorder and a frequent cause of medical consultation in children. Many childhood episodic syndromes have been described as common precursors of migraine.ObjectiveTo review current knowledge on migraine and childhood episodic syndromes, and to discuss future directions for research and clinical practice.FindingsFor most children it is difficult ...
O'Neill S S School of Pharmacy and Pharmaceutical Sciences, Trinity Biomedical Sciences Institute, Trinity College Dublin, Dublin, - - 2014
Obesity is reaching epidemic proportions with recent worldwide figures estimated at 1.4 billion and rising year-on-year. Obesity affects all socioeconomic backgrounds and ethnicities and is a pre-requisite for metabolic syndrome. Metabolic syndrome is a clustering of risk factors, such as central obesity, insulin resistance, dyslipidaemia and hypertension that together culminate ...
Halcox Julian J 1 University of Swansea , Singleton Park, Swansea, United Kingdom - - 2014
Abstract Individuals with mixed atherogenic dyslipidemia, type 2 diabetes mellitus (T2DM), and metabolic syndrome are at high risk of developing cardiovascular disease (CVD) and can often benefit greatly from preventive lifestyle and medical interventions. These conditions typically co-exist in an individual, and the lipid profiles associated with them have several ...
Hanna Elias B EB Department of Medicine, Cardiovascular Section, Louisiana State University, New Orleans, - - 2014
Congestion and acute renal dysfunction are at the center of acute heart failure (HF) syndromes. Acute cardiorenal syndrome, which refers to worsening of renal function in a patient with acute HF syndrome, is partly related to venous congestion and high renal afterload. Aggressive decongestion improves renal and myocardial flow and ...
Costa Luisa L Rheumatology Unit, Department of Clinical Medicine and Surgery, University Federico II, via S. Pansini 5, 80131, Naples, Italy, - - 2014
The aim of the study was to evaluate the influence of metabolic syndrome (MetS) on achieving minimal disease activity (MDA) in psoriatic arthritis (PsA) patients treated with anti-tumor necrosis factor (TNF)-α with a follow-up period of 24 months. A cohort of PsA patients was assessed at the University Federico II of ...
Ganzetti G G Dermatological Clinic, Department of Clinical and Molecular Medicine, Polytechnic MarcheUniversity, Ancona, Italy - - - 2014
Toxic epidermal necrolysis (TEN), also known as Lyell syndrome, is a potential life--threatening muco--cutaneous disease with important systemic implications. It affects the skin and mucous membranes, with involvement of more than 30% of body surface and it is mostly caused by drugs. Although the pathogenesis is not fully elucidated, it ...
Jabrane M M Nephrology and Kidney Transplantation, Medical University Hospital, University Caddi Ayad, Marrakech, Morocco, - - 2014
Posterior reversible encephalopathy syndrome (PRES) is primarily a radiological diagnosis. The syndrome is characterized by headache, altered mental status, seizures, and bilateral posterior white matter edema in a nonvascular distribution on neuroimaging with resolution of findings usually in 7-14 days (Casey et al. in AJNR Am J Neuroradiol 21:1199-1206, 2000). In ...
Mulliez S M N SM Coagulation Laboratory, Department of Clinical Chemistry, Microbiology and Immunology, Ghent University Hospital, Ghent, - - 2014
Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare acquired disorder caused by prothrombin antibodies. The disease is most common in the pediatric age group (<16 years), and more prevalent in women. There are well-established clinical diseases associated with LA-HPS, most notably systemic lupus erythematosus (SLE) and viral infections. The clinical manifestation ...
Ayyappan Sujith S Department of Neurology, Royal Melbourne Hospital, Victoria, Australia, - - 2014
Introduction: Paraneoplastic neuropathies are well recognized as a remote effect of cancer, and subacute sensory neuronopathy is a recognized syndrome. Demyelinating neuropathies are relatively rare. Distal acquired demyelinating symmetric (DADS) neuropathy associated with lymphoproliferative disease has been previously reported. We present the association of DADS neuropathy with solid tumor. Methods: ...
Apley Michael M Kansas State University,111B Mosier - - 2014
The clinical syndrome of bovine respiratory disease (BRD) continues to be a major challenge in bovine production systems. We are challenged by our ability to predict morbidity in groups of cattle, our ability to accurately diagnose and provide a prognosis for individual cases, and our ability to evaluate the results ...
Hansen Aksel A Department of Psychiatry,Psychiatrische Dienste - - 2014
In 1765 Giovanni Morgagni described a syndrome consisting of hyperostosis frontalis interna (HFI), obesity and hirsutism. In 1928 Stewart and in 1930 Morel added neuropsychiatric symptoms, e.g. depression and dementia, which led to the definition of the Morgagni-Stewart-Morel Syndrome (MSM). Although mostly women were characterized in literature no gender specifity ...
Frisbee Jefferson C JC Department of Physiology and Pharmacology, West Virginia University Health Sciences Center, Morgantown, WV, USA Center for Cardiovascular and Respiratory Sciences, West Virginia University Health Sciences Center, Morgantown, WV, USA - - 2014
A major challenge facing public health is the increased incidence and prevalence of the metabolic syndrome, a clinical condition characterized by excess adiposity, impaired glycemic control, dyslipidemia and moderate hypertension. The greatest concern for this syndrome is the profound increase in risk for development of peripheral vascular disease (PVD) in ...
Kronbichler Andreas A Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, Innsbruck, - - 2014
Treatment of nephrotic syndrome due to minimal change disease and focal segmental glomerulosclerosis remains a challenge since steroid dependence, steroid resistance and a relapsing disease course exhibits a high cumulative steroid dosage. The necessity of using alternative steroid-sparing immunosuppressive agents with potential toxic side effects also restricts their long-term use. ...
Marti Brigitte G BG Service de Rééducation, Hôpital Saint Antoine, Paris, - - 2014
Overactive bladder (OAB) is a syndrome characterized by symptoms of urgency with or without incontinence, frequency, and nocturia. Pathophysiology of OAB is incompletely characterized. Therefore, there is great variability surrounding OAB evaluation. In some cases, urodynamics is normal and the challenge is to propose the least invasive treatment. Behavioral therapies ...
Sharma Abhishek A Division of Cardiovascular Medicine, State University of New York Downstate Medical Center, New York, N.Y., - - 2014
Despite an early invasive strategy and the use of dual antiplatelet therapy, patients with acute coronary syndrome (ACS) continue to be at substantial risk for recurrent ischemic events. It is believed that this risk is, at least in part, due to an intrinsic coagulation pathway that remains activated for a ...
Stater Brian J BJ Division of Pediatric Otolaryngology, Department of Otolaryngology-Head and Neck Surgery, Weill Cornell Medical College, New York, New - - 2014
We report 2 new cases of tracheal cartilaginous sleeve and perform a systematic literature review of all documented cases of this condition. We aim to increase awareness of this anomaly and provide recommendations for both intraoperative and postoperative management of patients with tracheal cartilaginous sleeve undergoing tracheostomy. We studied the ...
Wada Hideo H Department of Molecular and Laboratory Medicine, Mie University Graduate School of Medicine, Mie, - - 2014
The differential diagnosis of thrombotic microangiopathy (TMA) has become clearer following the establishment of the relationships between (1) diarrhea-associated hemolytic uremic syndrome (HUS) and Shiga toxin-producing Escherichia coli-HUS (STEC-HUS), (2) a markedly reduced ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) level and typical thrombotic ...
Gulleroglu Kaan K Kaan Gulleroglu, Esra Baskin, Department of Pediatric Nephrology, Baskent University, 06540 Ankara, - - 2014
The nutcracker phenomenon [left renal vein (LRV) entrapment syndrome] refers to compression of the LRV most commonly between abdominal aorta and superior mesenteric artery. Term of nutcracker syndrome (NCS) is used for patients with clinical symptoms associated with nutcracker anatomy. LRV entrapment divided into 2 types: anterior and posterior. Posterior ...
Karlin Joshua G JG Department of Orthopaedics (V.P. and D.C.), College of Medicine (J.G.K.), University of Texas Health Science Center at San Antonio (UTHSCSA), 7703 Floyd Curl Drive, San Antonio, TX - - 2014
Jarcho-Levin syndrome represents a spectrum of clinical and radiographic irregularities including abnormal vertebral segmentation or formation defects, rib deformities, and short-trunk dwarfism. These abnormalities cause reduced thoracic capacity for lung development, resulting in thoracic insufficiency syndrome. In the present study, we reviewed outcome measures related to scoliotic curve correction, thoracic ...
Ong David S Y DS 1Department of Medical Microbiology, University Medical Center Utrecht, Utrecht, The Netherlands. 2Department of Intensive Care Medicine, University Medical Center Utrecht, Utrecht, The Netherlands. 3Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, The Netherlands. 4Department of Mathematics, Utrecht University, Utrecht, The Netherlands. 5Department of Intensive Care, Academic Medical Center, University of Amsterdam, Amsterdam, The - - 2014
Cytomegalovirus reactivation may complicate critical illness in latent carriers of the virus, even in patients who were previously immunocompetent. Patients with acute respiratory distress syndrome are considered to be prone for reactivation. Prophylactic antiviral therapy in immunocompetent cytomegalovirus seropositive patients admitted to the ICU with acute respiratory distress syndrome has ...
Smith Saxon D SD Department of Dermatology, Royal North Shore Hospital, Sydney, Australia; Gosford Dermatology, Gosford, New South Wales, - - 2014
Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are severe mucocutaneous eruptions. There is currently no defined optimal approach to wound care. The objective of this study was to evaluate silver dressings in the wound-care management of TEN and SJS/TEN syndrome overlap with a retrospective case review of nine patients ...
Sheehan Rory R Mental Health Sciences Unit, University College London, London, UK; Camden Learning Disabilities Service, London, - - 2014
Dementia is a common clinical presentation among older adults with Down syndrome. The presentation of dementia in Down syndrome differs compared with typical Alzheimer's disease. The performance of manualised dementia criteria in the International Classification of Diseases (ICD)-10 and Diagnostic and Statistical Manual of Mental Disorders-IV-Text Revision (DSM-IV-TR) is uncertain ...
Tsai Richard M RM Department of Neurology, University of California San Francisco, 675 Nelson Rising Lane, Suite 190, San Francisco, CA, 94115, USA, - - 2014
Frontotemporal dementia (FTD) encompasses a spectrum of neurodegenerative diseases with heterogeneous clinical presentations and two predominant types of underlying neuropathology. FTD typically comprises three distinct clinical syndromes: behavioral variant frontotemporal dementia (bvFTD), semantic variant primary progressive aphasia (svPPA), and nonfluent variant primary progressive aphasia (nfvPPA). FTD also frequently overlaps both ...
Linderholm Angela L AL Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of California, Davis, 4150 V Street, Suite 3100, Davis, CA, - - 2014
Asthma is a complex syndrome that affects an estimated 26 million people in the United States but gaps exist in the recognition and management of asthmatic subgroups. This article proposes alternative approaches for future treatments of adult obese asthmatics who do not respond to standard controller therapies, drawing parallels between ...
Qing Cui C Department of Orthopaedics, Cangzhou Hospital of Integrated Traditional Chinese and Western Medicine, Hebei Medical University, Cangzhou, Hebei 061001, P.R. - - 2014
The aim of the present study was to investigate a new clinical classification of cubital tunnel syndrome that provides an improved basis for the clinical diagnosis and treatment of the disease. Retrospective analysis was performed on 341 patients with cubital tunnel syndrome. Based on the etiology, signs and symptoms, neurophysiological ...
Hu Er-Wei EW Department of General Surgery, Tianjin Medical University General Hospital, Heping, Tianjin 300052, P.R. - - 2014
Pendred syndrome is a rare genetic disease that causes a disturbance in thyroid hormone synthesis, which results in thyroid dysfunction and the development of goiter and sensorineural deafness. The present report describes the case of a young euthyroid male, who developed a large goiter and hearing impairment, despite no family ...
Werdich Xiang Q XQ Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School , Boston, Massachusetts , - - 2014
Abstract Inherited retinal degeneration (IRD) may occur in isolation or as part of a multi-systemic condition. Ocular manifestations may be the presenting symptom of a syndromic disease and can include retinitis pigmentosa, cone-rod dystrophy, or maculopathy. Alternatively, patients affected with syndromic disease may already have other systemic manifestations at the ...
Goodkin Michael B MB From the Cardiology Consultants of Philadelphia in Media, Pennsylvania (Dr Goodkin), and from private practice in Claymont, Delaware, and the Department of Osteopathic Manipulative Medicine at the Philadelphia College of Osteopathic Medicine in Pennsylvania (Dr Bellew) - - 2014
Postural orthostatic tachycardia syndrome (POTS) is associated with many symptoms including orthostatic intolerance, fatigue, palpitations, and cognitive dysfunction. Treatment, which typically consists of exercise, increased dietary sodium and fluids, compression garments, and medications for orthostatic intolerance, frequently produces unsatisfactory results. The authors report the case of a 26-year-old woman who ...
Patterson Karen C - - 2014
Both inherited and acquired immunodeficiency and chronic pulmonary disease predispose to the development of a variety of pulmonary syndromes in response to Aspergillus, a fungus that is ubiquitous in the environment. These syndromes include invasive aspergillosis, which is now recognized to occur in patients with critical illness without neutropenia and ...
Ross Whitney Trotter WT From the Department of Obstetrics and Gynecology, East Tennessee State University, Johnson - - 2014
Individuals with Down syndrome have an increased risk for many conditions, including cardiovascular disease, cancer, infections, and osteoporosis, and endocrine, neurological, orthopedic, auditory, and ophthalmic disorders. They also are at increased risk for abuse and human rights violations and receive fewer screenings and interventions than the population without Down syndrome. ...
Bingol Ugur Anil UA From the Department of Plastic, Reconstructive and Aesthetic Surgery, Yeditepe Medical School, Yeditepe - - 2014
Pachydermoperiostosis is a rare syndrome that hinders patients' quality of life thru its aesthetics manifestations and functional obstacles. Many techniques for addressing and correcting aesthetic defects associated with pachydermoperiostosis have been introduced, including facelift surgery. This case presentation includes treatment of facial pachydermoperiostosis and restoration of facial aesthetics via treatment ...
Ferreira do Amaral Cristhiane Olivia CO From the Departments of *Special Care Dentistry, †Pediatric Dentistry, and ‡Prosthodontics and Geriatic Dentistry, Dental School, University of Western Sao Paulo, Presidente Prudente, Sao Paulo, - - 2014
The purpose of this study is to present a clinical report of a patient with Bardet-Biedl syndrome, aiming to help the dentist to identify the general aspects, systemic changes, alterations. Bardet-Biedl syndrome is defined as a genetic disorder of autosomal recessive condition; this case is a male patient, 20 years ...
Villavicencio Edgar E Servicio de Urología. Hospital Arnau de Vilanova. Lleida. - - 2014
To review the etiology, diagnosis and treatment of spontaneous retroperitoneal hemorrhage. We report the cases of two men 59 and 79 years old, presenting acute abdominal pain secondary to retroperitoneal bleeding (Wünderlich's syndrome) due to spontaneous renal rupture caused by urolithiasis and angiomyolipoma respectively. Diagnosis of acute abdomen due to ...
Tanni Suzana E SE 1 Pulmonology Division of Botucatu Medical School-UNESP , Botucatu, São Paulo, Brazil - - 2014
Abstract The aim of this study was to evaluate the risk of mortality according to the presence of metabolic syndrome in chronic obstructive pulmonary disease (COPD) patients who were followed for 5 years. We did not establish the influence of metabolic syndrome on mortality rate. However, an increase of 100 mg ...
Ferlito Alfio A University of Udine School of Medicine, Piazzale S. Maria della Misericordia, 33100, Udine, Italy, - - 2014
Paraneoplastic syndromes are associated with a variety of malignant neoplasms and are systemic and non-metastatic manifestations that develop in a minority of cancer patients. This review examines all published cases of paraneoplastic syndromes associated with neuroendocrine carcinomas of the larynx. There are a total of ten patients reported with paraneoplastic ...
Gregory Thomas T From the University of Toronto (TG); Toronto Rehabilitation Institute, The University Centre (HS); and Mount Sinai Hospital and the University Health Network, Department of Medical Imaging, Toronto, Ontario, Canada - - 2014
A case of quadrilateral space syndrome is presented, where a large near-circumferential glenoid labrum tear led to a paralabral cyst that dissected into the quadrilateral space and caused a compressive neuropathy of the axillary nerve. This led to a 6-mo history of left shoulder pain, parasthesias, marked weakness to abduction, ...
Cheungpasitporn Wisit - - 2014
Atypical hemolytic uremic syndrome (aHUS) is a relatively rare disorder described by the triad of hemolytic anemia, thrombocytopenia, and renal failure. Atypical HUS could be genetic, acquired, or idiopathic (without known genetic changes or environmental triggers). Monoclonal protein has uncommonly been reported as a cause of microangiopathic hemolytic anemia (MAHA). ...
Kanariou Maria M Department of Immunology-Histocompatibility, Specialized Center and Referral Center for Primary Immunodeficiencies-Pediatric Immunology, - - 2014
Neonatal onset multisystem inflammatory disease (NOMID)/chronic infantile neurologic cutaneous and articular (CINCA) syndrome is a rare, early-onset autoinflammatory disorder and the most severe form of cryopyrin-associated periodic syndrome, which is associated with overproduction of interleukin (IL)-1β. This is a case report of a 70-day-old boy, who was diagnosed with NOMID/CINCA ...
O'Rafferty C C Department of Haematology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland, - - 2014
Down syndrome (DS), the most common syndromic chromosomal abnormality is associated with a unique susceptibility to develop both acute myeloid (ML) and lymphoblastic leukaemia (ALL). These leukaemias differ from the non-DS-related types of leukaemia and are thought to be distinct biological entities. To perform a retrospective review of our experience ...
Cugno Massimo M Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, - - 2014
We are very thankful to Dr Wehling and Dr Kirschfink for their commentary regarding our paper on monitoring eculizumab regimen in patients with atypical hemolytic uremic syndrome (aHUS) [1] in which they provide an expert overview to our management strategy [2]. However, from a practical point of view, we have two ...
Bloch Yuval Y The Emotion-Cognition Research Center, Shalvata Mental Health Care Center , Hod-Hasharon , - - 2014
Objectives. In a considerable minority of patients who suffer from Tourette syndrome (TS) the disorder persists into adulthood and is associated with severe symptoms and limited therapeutic options. Repetitive transcranial magnetic stimulation (rTMS) to the supplementary motor area (SMA) has shown promising therapeutic results. Deep rTMS is a novel technology ...
Lim Soo S Department of Internal Medicine, Seoul National University College of Medicine and Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, Seongnam-city, 463-707, South Korea, - - 2014
Metabolic syndrome is a disorder based on insulin resistance. Metabolic syndrome is diagnosed by a co-occurrence of three out of five of the following medical conditions: abdominal obesity, elevated blood pressures, elevated glucose, high triglycerides, and low high-density lipoprotein-cholesterol (HDL-C) levels. Clinical implication of metabolic syndrome is that it increases ...
O'Connell K K Department of Neurology, St Vincent's University Hospital, Elm Park, Dublin 4, Ireland, - - 2014
Posterior reversible encephalopathy syndrome (PRES) is a medical emergency but prompt recognition, early institution of supportive care and identifying and removing potential triggers are associated with a good clinical outcome. We report an unusual case of PRES associated with liquorice consumption. A 56-year-old lady presented with thunderclap headache, visual disturbance ...
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