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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 ...
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 ...
Knopp C C Institute of Human Genetics, RWTH Aachen University, Pauwelsstr. 30, Aachen, 52074, - - 2014
Clinical overlap makes the diagnosis of overgrowth syndromes challenging. Clinical overlap exists between Simpson-Golabi-Behmel syndrome (SGBS) and Beckwith-Wiedemann syndrome (BWS) which share pre- and postnatal overgrowth, macroglossia, umbilical hernia, organomegaly, ear lobe creases, and occurrence of embryonal tumors as characteristic features. Based on the clinical history of a patient, who ...
Kraai Erik P EP University of New Mexico Health Sciences Center, MSC09 5080, 1 University of New Mexico, Albuquerque, NM, 87131-0001, - - 2014
Citalopram is a selective serotonin reuptake inhibitor (SSRI) with cardiac and neurologic toxicities as well as the potential for serotonin syndrome. In most instances, patients recover fully from toxic ingestions of SSRIs. We describe a fatal case of a citalopram overdose. A 35-year-old woman presented to the emergency department after ...
Ruiz Marina M Neurology Department, Hospital Clínico Universitario, Valladolid, - - 2014
We analyzed characteristics of hypnic headache (HH), migraine and the relationship between both headaches in 23 patients. HH is an uncommon primary headache characterized by exclusively sleep-related attacks. Concurrence of other headaches, mainly migraine, has been reported, but relationship between both syndromes has rarely been considered. We prospectively collected data ...
Weinberg Laurence L Laurence Weinberg, Irene Kearsey, Clarissa Tjoakarfa, Larry McNicol, Peter McCall, Department of Anaesthesia, Austin Hospital, Melbourne, Victoria 3084, - - 2014
Redo-sternotomy and aortic valve replacement in patients with advanced liver disease is rare and associated with a prohibitive morbidity and mortality. Refractory coagulopathy is common and a consequence of intense activation of the coagulation system that can be triggered by contact of blood with the cardiopulmonary bypass circuitry, bypass-induced fibrinolysis, ...
Drivdal Monica M 1 Centre for Rare Disorders, Oslo University Hospital , Oslo, Norway - - 2014
Abstract Background: The characterizations of primary lymphedemas in different hereditary diseases are often published as case reports. In this study, 17 out of 20 Norweigian adult patients with lymphedema cholestasis syndrome 1 (LCS1)/Aagenaes syndrome were examined. The patients exhibited lymphedema and sporadic cholestasis. Individual clinical variations are described. Methods and ...
Raol Nikhila N Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, - - 2014
This study aimed to describe syndromes where preoperative imaging may be essential in determining the course of the carotid arteries before velopharyngeal insufficiency (VPI) surgery and therefore affect surgical planning. Records of children who underwent surgery for VPI between May 1, 2012, and October 30, 2013, at a tertiary care ...
Hare Michelle M Emergency Department, Sheffield Children's Hospital, Sheffield, - - 2014
Supraventricular tachycardia (SVT) is the most common symptomatic tachyarrhythmia in childhood. Treatment involves vagal manoeuvres, pharmacological agents or electrical cardioversion. We report a child with recurrent SVT secondary to Wolff-Parkinson-White syndrome who is consistently able to revert her SVT by doing a handstand. This case highlights a simple non-invasive technique ...
Dugernier Jonathan J Department of Intensive Care Medicine, Cliniques Universitaires Saint-Luc, - - 2014
We report a case of Guillain-Barré syndrome complicated by respiratory failure requiring mechanical ventilation. Neurally adjusted ventilatory assist (NAVA) allowed proper patient-ventilator synchronization by pressure support proportional to the electrical activity of the diaphragm (Edi). Prolonged ventilation with NAVA seems feasible in patients with neuromuscular impairment, but the weaning process ...
Giannouli Stavroula S 2nd Department of Internal Medicine, Medical School, National University of Athens, Athens, - - 2014
Primary myelodysplastic syndromes (MDS) are heterogeneous clonal hemopoietic disorders clinically presented with a varying degree of peripheral cytopenias and an increased probability of leukemic evolution. A distinct subset of MDS patients manifests overt autoimmune-inflammatory manifestations, the underlying pathogenesis and prognostic significance of which still remain controversial. In this review we ...
Tun Nay T NT aDepartment of Internal Medicine bDivision of Nephrology, Department of Internal Medicine, Easton Hospital, Drexel University, School of Medicine, Easton, Pennsylvania, - - 2014
Antiphospholipid syndrome (APS) and heparin-induced thrombocytopenia (HIT) are thrombotic disorders due to specific autoimmune-mediated antibodies. Catastrophic APS (CAPS), also known as Asherman's syndrome, is a life-threatening severe form of APS. Diagnostic criteria for CAPS include the development of a thrombotic event of three or more organs in less than a ...
Goh K G KG Department of Medicine, University Malaya Medical Centre, Kuala Lumpur, - - 2014
Patients with autoimmune connective tissue disease may manifest as overlap syndrome with features of systemic lupus erythematosus (SLE), systemic sclerosis, rheumatoid arthritis and myositis. Those presenting with active SLE can present with immune thrombocytopenia (IT) and may be complicated with subdural hematoma which, though rare, is potentially life-threatening. We report ...
Reidler Jay S JS Department of Orthopaedic Surgery, Johns Hopkins Hospital, Baltimore, MD; Hand and Upper Extremity Service, Hospital for Special Surgery, New York, NY; Department of Vascular Surgery, New York-Presbyterian Hospital, New York, - - 2014
We present 2 cases of combined arterial and neurogenic thoracic outlet syndrome triggered by trauma in patients with congenital synostoses of the first and second ribs. These patients were successfully treated with supraclavicular resection of the first and second ribs and scalenectomy. We review these cases and the associated literature ...
Jayarangaiah Apoorva - - 2014
BackgroundNeuromyelitis optica (NMO) is a rare relapsing auto-immune disease of the central nervous system which is sometimes found in association with other autoimmune disorders including Sjogren¿s syndrome. We present the case of a middle aged female with Sjogren¿s syndrome (SS) and Neuromyelitis optica spectrum disorders (NMOSD) who had a rapidly ...
Cantarini Luca L Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy. Electronic address: - - 2014
Autoimmunity and autoinflammation are generally considered as mutually exclusive mechanisms of diseases but may concur to specific syndromes. Idiopathic recurrent acute pericarditis (IRAP) is defined as the recurrence of pericardial symptoms at any point following the prior cessation of acute pericarditis, and the latency is generally 6 weeks. Manifestations of ...
Pechánová O O Institute of Normal and Pathological Physiology and Centre of Excellence for Regulatory Role of Nitric Oxide in Civilization Diseases, Slovak Academy of Sciences, Bratislava, Slovak Republic; Faculty of Natural Sciences, Comenius University, Bratislava, Slovak - - 2014
It is well documented that metabolic syndrome (i.e. a group of risk factors, such as abdominal obesity, elevated blood pressure, elevated fasting plasma glucose, high serum triglycerides, and low cholesterol level in high-density lipoprotein ), which raises the risk for heart disease and diabetes, is associated with increased reactive oxygen ...
Sriratanaviriyakul Narin - - 2014
Drug reaction with eosinophilia and systemic symptoms syndrome is a potentially life-threatening cutaneous hypersensitivity reaction characterized by extensive mucocutaneous eruption, fever, hematologic abnormalities including eosinophilia and/or atypical lymphocytosis, and extensive organ involvement. The drugs most often responsible for causing drug reaction with eosinophilia and systemic symptoms syndrome are anticonvulsants, antimicrobial ...
Tatsumoto Muneto M Department of Neurology, Dokkyo Medical University, Tochigi, - - 2014
Introduction: Dr. C. Miller Fisher described the appearance of unilateral facial palsy after the resolution of ataxia in a patient with the eponymic Miller Fisher syndrome (MFS). However, there have been very few reports of delayed appearance of facial weakness in Guillain-Barré syndrome (GBS) and MFS, when the other neurological ...
Wu Teddy Y TY Department of Neurology, Auckland City Hospital, Grafton Road, Auckland, New Zealand. Electronic address: - - 2014
We present a patient with newly diagnosed Cowden syndrome and congestive myeloradiculopathy secondary to spinal dural arteriovenous fistula (SDAVF). This patient illustrates the difficulties that can be encountered in diagnosing SDAVF and emphasises the need to pursue the diagnosis in the appropriate clinical setting, as treatment can lead to significant ...
Isik Ahmet Turan AT Department of Geriatric Medicine, Faculty of Medicine, Dokuz Eylul University, Izmir, Turkey - - 2014
In this case report, neuroleptic malignant syndrome (NMS) in an elderly patient with normal pressure hydrocephalus overlapping corticobasal degeneration was reported. The case highlights the need for clinicians to be cautious when using dopaminergic medication in the elderly patients, since these agents have risks for NMS which is a life-threatening ...
Cummings A A Clinical Fellow, University College London Hospitals Foundation NHS Trust, London WC1N - - 2014
Multiple sclerosis, always challenging, hands down a particular gauntlet with the concept of the radiologically isolated syndrome. This article discusses what it is, recent developments in the field and how these patients should be managed.
Bonaca Marc P MP Cardiovascular Division, Brigham and Women's Hospital, 75 Francis Street, Boston, MA, 02115, USA, - - 2014
Acute aortic syndromes constitute a spectrum of conditions characterized by disruptions in the integrity of the aortic wall that may lead to potentially catastrophic outcomes. They include classic aortic dissection, intramural hematoma, and penetrating aortic ulcer. Although imaging studies are sensitive and specific, timely diagnosis can be delayed because of ...
Cicero Arrigo F G AF aMedicine and Surgery Department, University of Bologna, Bologna bInternal Medicine and Therapeutics Department, University of Pavia, Pavia, - - 2014
Metabolic syndrome is an increasingly incident complex metabolic disorder, affecting around 30% of adults in the USA as well as in Europe. In a meta-analysis that evaluated cardiovascular risk associated with the third National Cholesterol Education Program definition of metabolic syndrome in 951 083 patients, metabolic syndrome was associated with a ...
Wilson Sg S Leeds Teaching Hospitals NHS Trust, - - 2014
Complications from HELLP (Haemolysis, Elevated Liver enzymes and Low Platelet) syndrome may present as an emergency to any surgeon. We review the ten-year experience of a tertiary hepatobiliary centre managing HELLP patients. Three selected cases are described to highlight our management strategy and a systematic review of the recent literature ...
Radtke Andrew C AC Division of Pediatric Urology, Department of Urology, University of Wisconsin School of Medicine and Public Health, Madison, - - 2014
Diagnosis, decision making, and counseling for patients with disorders of sexual development pose challenges for physicians and families. Accurate antenatal evaluation combined with effective communication between the family and multidisciplinary team is important to provide the best patient outcome. We reviewed 2 cases from our institution that illustrate the complexity ...
Ritch Robert R Einhorn Clinical Research Center, New York Eye and Ear Infirmary of Mount Sinai, New York, - - 2014
Exfoliation syndrome is an age-related disease characterized by the production and progressive accumulation of a fibrillar extracellular material in many ocular tissues. It leads to the most common identifiable cause of open-angle glaucoma worldwide, comprising the majority of glaucoma in some countries. The material in the eye appears as white ...
Hudson Christopher C C CC Department of Anesthesiology, Division of Cardiac Anesthesiology and Critical Care Medicine, University of Ottawa Heart Institute, Ottawa, Ontario, - - 2014
We present a case of severe tracheobronchial compression from a complex aorto-subclavian aneurysm in a patient with Turner's syndrome undergoing open surgical repair. Significant airway compression is a challenging situation and requires careful preoperative preparation, maintenance of spontaneous breathing when possible, and consideration of having an alternative source of oxygenation ...
Dunn John C JC Department of Orthopaedic Surgery and Rehabilitation, William Beaumont Army Medical Center, 5005 North Piedras Street, El Paso, TX 79920-5001, - - 2014
Chronic exertional compartment syndrome causes leg pain during exercise and resolves at rest. Paresthesia, especially in the distribution of the superficial peroneal nerve, is common. Nonoperative management may not lead to long-term clinical success. This condition is more difficult to treat in the military setting. Operative treatment is reserved for ...
Klingelhoefer Lisa L National Parkinson Foundation International Centre of Excellence, King's College Hospital and King's College, London, UK and resident, Department of Neurology, Technical University Dresden, Dresden, Germany - - 2014
Restless legs syndrome (RLS), recently renamed Willis-Ekbom disease (WED), is a common movement disorder. It is characterised by the need to move mainly the legs due to uncomfortable, sometimes painful sensations in the legs, which have a diurnal variation and a release with movement. Management is complex. First, centres should ...
Terzi Eirini E 1 Internal Medicine Department-Unit of Infectious Diseases, "AHEPA" Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece ; 2 Pulmonary Department, "G. Papanikolaou" General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece ; 3 Surgery Department, University General Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis, Greece ; 4 Hematology Department, "Laiko" University General Hospital, Athens, Greece ; 5 Obstetric-Gynecology Department, "Thriassio" General Hospital of Athens, Athens, Greece ; 6 Internal Medicine Department, "Theiageneio" Anticancer Hospital, Thessaloniki, Greece ; 7 Thoracic Surgery Department, "G. Papanikolaou" General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece ; 8 Institute for Pulmonary Diseases of Vojvodina, Clinic for Thoracic Oncology, Faculty of Medicine, University of Novi Sad, - - 2014
Acute respiratory distress syndrome (ARDS) can occur during the treatment of several diseases and in several interventional procedures as a complication. It is a difficult situation to handle and special care should be applied to the patients. Mechanical ventilation is used for these patients and several parameters are changed constantly ...
Levy Robert R North Memorial Family Medicine Residency, University of Minnesota, Minneapolis, MN, - - 2014
A drug used for cancer patients may provide some relief to patients with IBS.
Takahara Mitsuyoshi M Department of Metabolic Medicine, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka, 565-0871, - - 2014
A clustering of metabolic abnormalities such as dyslipidemia, hypertension, and diabetes mellitus, all of which are major risk factors for cardiovascular disease (CVD), occurs more often than by chance. Numerous epidemiological studies, as well as basic researches, have revealed that visceral fat accumulation is closely involved in this risk clustering. ...
Metz David Kieran DK Royal Children's Hospital, Melbourne, Victoria, - - 2014
Childhood nephrotic syndrome is a condition managed by general paediatricians and paediatric nephrologists. Whether treating a first presentation or a relapse, the clinician requires expertise in order to minimise the risk of serious complications and optimise long-term care. Indeed, many children suffer a difficult relapsing course in their disease, warranting ...
Vargel Ibrahim I From the *Department of Plastic Reconstructive and Aesthetic Surgery, Faculty of Medicine, Kirikkale University; †Plastic Reconstructive and Aesthetic Surgery Clinic, Ankara Training and Research Hospital; ‡Department of Plastic Reconstructive and Aesthetic Surgery, Faculty of Medicine, Hacettepe University; and §Orthopedics and Traumatology Clinic, Ankara Güven Hospital, Ankara, - - 2014
Apert syndrome is characterized by short, radially deviated thumbs, leading to difficulties in daily life such as holding a fork or a spoon and buttoning up. The main goal of surgery is to achieve thumb to index finger pinch to overcome these difficulties. Seven patients (14 extremities) followed up with ...
Huntley Catherine C Center for Orthopaedics, The Royal London, Barts and The London Children's Hospitals, Barts Health NHS Trust, Whitechapel Road, Whitechapel, London E1 1BB, - - 2014
Joseph Merrick, the Elephant Man, presented to the Royal London Hospital in 1884 with an obscure condition that puzzled his contemporaries, and fascinates clinicians to this day. Throughout the 1900s, a number of theories were advanced to explain the numerous growths that covered his body: neurofibromatosis, Proteus syndrome, a combination ...
Cahill Joseph A JA PGY3 Neurology, University of Wisconsin Hospitals and Clinics, Madison, - - 2014
Ptosis and anisocoria in a child may be subtle indications of occult pathology, and making the observation acutely in the emergency department (ED) is important in guiding patient management and treatment. Emergency physicians must evaluate patients to exclude serious or life-threatening emergencies and ensure correct disposition of patients. Horner syndrome ...
Parisi Pasquale P Pediatric Neurology Outpatient Service, Pediatric Gastroenterology Outpatient Service, Sleep Pediatric Service, Pediatric Pulmonology Service, NESMOS Department, Sapienza University of Rome, Faculty of Medicine & Psychology, c/o "Sant' Andrea" Hospital, Via di Grottarossa, 1035-1039, 00189 Rome, Italy. Electronic address: - - 2014
Autonomic signs and symptoms could be of epileptic or nonepileptic origin, and the differential diagnosis depends on a number of factors which include the nature of the autonomic manifestations themselves, the occurrence of other nonictal autonomic signs/symptoms, and the age of the patient. Here, we describe twelve children (aged from ...
Jung Sungmo S Department of Internal Medicine, Institute of Gastroenterology, College of Medicine, Yonsei University, Seoul, South - - 2014
Evidence indicates that irritable bowel syndrome can occur after gastroenteritis. However, little is known about its incidence after diverticulitis. This study was designed to identify the incidence and risk factors of irritable bowel syndrome after diverticulitis in Korea. A survey regarding irritable bowel syndrome was performed in patients allocated to ...
Janocha-Litwin Justyna J First Department of Infectious Disease, J. Gromkowski Specialist Regional Hospital in Wroclaw, Wroclaw, - - 2014
Ephedrone encephalopathy is referred to as a group of symptoms of manganese deposition within the central nervous system (CNS), resulting from the abuse of ephedrone (methcathinone), obtained in reaction using the excess amount of manganese-containing oxidants. The diagnosis is based on the contrast-enhanced head MRI findings characteristic for this syndrome, ...
Pérez-de José Ana - - 2014
Nefrologia 2014;34(5):XXX. doi10.3265/Nefrologia.pre2014.Jun.12620 Reply to Esteve et al. Comment on Nefrologia 2014;34(4):xx. Nefrologia 2014;34(1):69-75.
Kuo James A JA Cook Children's Medical Center, Department of Cardiology, 1500 Cooper Street, 3rd Floor, Fort Worth, TX, - - 2014
Venovenous collateral vessels are a common cause for desaturation in patients who have undergone a Fontan procedure. We describe a patient with heterotaxy syndrome (leftward pointing apex) and complex single ventricle with Fontan physiology that was desaturated due to a hepatocardiac vein. The vessel was entered via a left transhepatic ...
Barbosa Oséias Vargas OV Faculdade de Medicina, Universidade de Caxias do Sul (FMUCS), Caxias do Sul, Rio Grande do Sul, - - 2014
Li-Fraumeni syndrome is a rare disease with an autosomal dominant inheritance pattern and high penetrance that defines a 50% chance of developing cancer before the age of 30 years, including cases of breast sarcoma. Patients with this syndrome who require radiotherapy have an increased risk of developing secondary malignancies including ...
Yeung Aaron M AM University Hospital Coventry and Warwickshire NHS Trust , Coventry, West Midlands , United - - 2014
Abstract Purpose: Floppy eyelid syndrome is a condition that is difficult to identify and diagnose and with no clear guidelines on its management. We propose a method of reliably grading this syndrome and have proposed a management algorithm based on the grading. Materials and Methods: Retrospective data collection of patients ...
Ahn Annie K AK Department of Otolaryngology, Vanderbilt University Medical Center, Nashville, - - 2014
Moyamoya syndrome is a rare, occlusive cerebrovascular arteriopathy with significant risk for stroke. Populations that frequently undergo otolaryngologic procedures, including patients with Down syndrome and sickle cell disease, are particularly at risk for moyamoya. The initial presentation of moyamoya syndrome as stroke in the perioperative period of an otolaryngologic procedure ...
Rondon-Berrios Helbert H Renal-Electrolyte Division, Department of Medicine, University of Pittsburgh School of Medicine, A915 Scaife Hall, 3550 Terrace Street, Pittsburgh, PA, 15261, USA, - - 2014
Hyponatremia has complex pathophysiology, is frequent and has potentially severe clinical manifestations, and its treatment is associated with high risks. Hyponatremia can be hypertonic, isotonic or hypotonic. Hypotonic hyponatremia has multiple etiologies, but only two general mechanisms of development, defective water excretion, usually because of elevated serum vasopressin levels, or ...
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