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Zivković Krešimir K Department of Gynecology and Obstetrics, "Sveti Duh" University Hospital, University of Zagreb, School of Medicine, Sveti Duh 64, 10000, Zagreb, Croatia, - - 2014
The acronym for obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) was created to describe patients with an obstructed hemivagina and ipsilateral renal anomaly and enables inclusion of other uterine anomalies except uterus didelphys. The main goal of this article is to present a rare case of OHVIRA syndrome with intrapartal ...
Ostadkarampour Mahyar M Respiratory Medicine Unit, Department of Medicine Solna and CMM, Karolinska Institutet and Karolinska University Hospital, Stockholm, - - 2014
Sarcoidosis is a granulomatous disorder of unknown etiology. The presence of M. tuberculosis catalase-peroxidase (mKatG) in sarcoidosis tissue has been reported. T helper 1 responses against mKatG have previously been observed. However, little is known about IL-17 and Th17 responses in sarcoidosis. Here, we investigated the levels of IL-17 and ...
Baris Safa S Division of Pediatric Allergy and Immunology, Marmara Medical Faculty, Istanbul, Turkey, - - 2014
IPEX (Immunodysregulation, Polyendocrinopathy, Enteropathy, X-linked) is a rare X-linked recessive life-threatening disorder characterized by autoimmunity and early death. Pulmonary complication related with IPEX has not been elucidated exactly. Here, we report 4 IPEX patients, 3 of which died from severe pulmonary disease. Clinical data and laboratory findings including autoantibodies, immunoglobulin ...
Chung Jae Ho JH 1Division of Pulmonology, Department of Internal Medicine, International St. Mary's Hospital, Kwandong University College of Medicine , Incheon , - - 2014
Abstract Aim: It is not clear whether the restrictive or obstructive pattern of spirometry is associated with metabolic syndrome. We investigated the associations between restrictive and obstructive spirometric patterns and metabolic risk factors using data from the Korea National Health and Nutrition Examination Survey (KNHANES). Additionally, we investigated whether sarcopenia ...
Fu Juan-Juan JJ Respiratory Group, Department of Integrated Traditional Chinese and Western Medicine, West China Hospital, Sichuan University, Chengdu, China. ; Priority Research Centre for Asthma and Respiratory Diseases, University of Newcastle; Hunter Medical Research Institute, Newcastle, NSW, Australia. ; Department of Respiratory and Sleep Medicine, John Hunter Hospital, New Lambton, NSW, - - 2014
The role of systemic inflammation on asthma-COPD overlap syndrome is unknown. This study aimed to examine systemic inflammation in asthma-COPD overlap syndrome, and to identify associations between clinical characteristics and inflammatory mediators in asthma-COPD overlap syndrome. In 108 adults older than 55 years comprising healthy controls (n=29), asthma (n=16), COPD ...
Reiterer Friedrich F Division of Neonatology, Department of Paediatrics, Medical University of Graz, Austria. Electronic address: - - 2014
Congenital pulmonary lymphangiectasis (CPL) is a rare vascular malformation causing dilated lymph vessels and disturbed drainage of lymph fluid. Based on the pathogenesis and clinical phenotype it can be classified as primary or secondary CPL. Associated genetic syndromes with or without lymphedema, familial occurrence and gene mutations have been described. ...
Martínez-Quintana Efrén E 1Cardiology Service,Insular-Materno Infantil University Hospital,Las Palmas de Gran - - 2014
Klippel-Feil syndrome is a skeletal disorder characterised by low hairline and a short neck due to abnormal fusion of two or more cervical vertebrae. Although congenital heart and lung defects are infrequent, some abnormalities such as cor triatriatum, coarctation of the aorta, total anomalous pulmonary venous connection, or lung agenesis ...
Pillai S S Divisions of Respiratory and Sleep Medicine - - 2014
Shrinking lung syndrome (SLS) is a rare pulmonary complication of an underlying autoimmune disorder and is reported in association with systemic lupus erythematosus (SLE). We describe the favorable outcome of SLS in an 18-year-old Hispanic male who presented with SLS as the initial pulmonary manifestation of SLE.
Chao Nelson N Bone Marrow Transplantation Program, Duke University Medical Center, Durham, NC, United States - - 2014
Sinusoidal obstruction syndrome (SOS), previously called veno-occlusive disease (VOD) can be a difficult problem following hematopoietic cell transplantation (HCT). The overall incidence has changed since the early days of allogeneic HCT. Prophylaxis and treatment remains an important component of supportive care. As the indication and the comorbidities for HCT continues ...
Kintu Brett B Department of Paediatrics, Norfolk and Norwich University - - 2014
Pseudo-Bartter syndrome (PBS) describes an uncommon but well recognised complication of cystic fibrosis leading to hypochloraemic, hypokalaemic metabolic alkalosis. Pseudo-Bartter syndrome is usually seen at initial presentation or within the first two years of life in children with cystic fibrosis. Risk factors for development of PBS include warm weather conditions, ...
Kouga Takeshi T From the Department of General Medicine, Kanagawa Children's Medical Center, Yokohama, - - 2014
Syndromic craniosynostosis is associated with a high rate of respiratory difficulty, due mainly to midfacial hypoplasia. Nasopharyngeal airway establishment has been reported as the first-line approach to airway obstruction and may obviate the need for a highly invasive tracheotomy. No previous studies have compared airway obstruction status in syndromic craniosynostosis ...
Burns Natalie S NS Department of Radiology, University of Washington Medical Center, Seattle, WA, - - 2014
Systemic lupus erythematosis (SLE) can affect the lungs and pleura, usually manifesting with pleural effusions or diffuse parenchymal disease. A rare manifestation of SLE is shrinking lung syndrome, a severe restrictive respiratory disorder. While pleuropulmonary complications of pediatric SLE are common, shrinking lung syndrome is exceedingly rare in children. We ...
Ferreira Pedro G PG Pulmonology Department, Coimbra Hospital and University Centre, Coimbra, Portugal. Electronic address: - - 2014
Case report of a male patient with a five-decade follow-up history in a tertiary care hospital distinguished for malabsorption syndrome, failure-to-thrive, meningitis and recurrent bacterial, fungal and mycobacterial pulmonary infections. Additionally, he developed epidermodysplasia verruciformis, several in situ spinocellular carcinomas and an uncharacteristic parenchymal lung disease. Surgical lung biopsy suggested ...
Saygi Murat M Department of Pediatric Cardiology, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Halkali, Istanbul, - - 2014
In approximately 3% to 6% of the patients with tetralogy of Fallot (TOF), the pulmonary valve leaflets are absent or only a rudimentary ridge of tissue is present. Some infants with the severe form of this syndrome die early during the newborn period due to severe respiratory distress, feeding intolerance, ...
Garge Saurabh S Department of Pediatric Surgery, Safdarjung Hospital, New Delhi, - - 2014
We report a rare case of uterovaginal duplication in a prepubertal female. The patient also had a permeable ureter (ureter with urine passing through it) subtending a poorly functioning kidney with ectopic insertion in the obstructed hemivagina.
Flores Padilla Guillermo G Servicio de Medicina Interna, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, IMSS, México DF, - - 2014
A 59 year-old female with a history of injection of an oily material in the buttocks 11 years ago. She developed symmetric aditive polyarthritis as well as superior and inferior airways involvement. There was no evidence of granulomatosis with polyangiitis (Wegener). She had several serum autoantibodies and a skin biopsy ...
Ellenburg Joseph T JT Department of Pediatric Pulmonology, University of Mississippi Medical Center, Jackson, - - 2014
Toxic epidermal necrolysis (TEN) is a rare and potentially fatal mucocutaneous condition that may affect both children and adults. TEN mortality rates vary greatly between both patient populations, but multisystem involvement is common. Management presents many challenges as there is no specific therapy for TEN, and patients often require the ...
Sauter Jennifer L JL Department of Pathology, The University of Vermont, Department of Pathology and Laboratory Medicine, Fletcher Allen Health - - 2014
There are few reports of the pulmonary histopathologic features of anti-synthetase (AS). We report a case of AS syndrome with acute fibrinous and organising pneumonia (AFOP), a pattern of interstitial lung disease (ILD) not previously reported in AS syndrome. AS syndrome is an idiopathic inflammatory myopathy characterised by autoantibodies against ...
Bachh Arshad Altaf AA Department of Pulmonary Medicine, Mamata Medical College and Hospital, Khammam, Andra Pradesh, - - 2014
Failure of development of the primitive lung bud leads to an extremely rare congenital anomaly with a prevalence of 34 per 10 lac live births termed pulmonary aplasia. In half of such cases, associated congenital malformations of the cardiovascular, skeletal, gastrointestinal, or genitourinary systems are present. The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome ...
Papaiwannou Antonis A 1 Pulmonary Department-Oncology Unit, "G. Papanikolaou" General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece ; 2 Cardiology Department, "Saint Luke" Private Clinic, Thessaloniki, Panorama, Greece ; 3 Surgery Department (NHS), University General Hospital of Alexandroupolis, Democritus University of Thrace, Alexandroupolis, Greece ; 4 Internal Medicine Department, "Theageneio" Cancer Hospital, Thessaloniki, Greece ; 5 Department of Cardiac Surgery, University of Ioannina, School of Medicine, Greece ; 6 Anesthisiology Department, 7 Oncology Department, 8 Cardiology Department, "Saint Luke" Private Clinic, Thessaloniki, Panorama, - - 2014
Asthma and chronic obstructive pulmonary disease (COPD) are chronic diseases, very common in general population. These obstructive airway illnesses are manifested with chronic inflammation affecting the whole respiratory tract. Obstruction is usually intermittent and reversible in asthma, but is progressive and irreversible in COPD. Asthma and COPD may overlap and ...
Piatti G G Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Italy. Electronic address: - - 2014
Usher syndrome is a ciliopathy involving photoreceptors and cochlear hair cells (sensory cilia): since sensory and motor ciliopathies can overlap, we analysed the respiratory cilia (motile) in 17 patients affected by Usher syndrome and 18 healthy control subject. We studied the mucociliary transport time with the saccharine test, ciliary motility ...
Pathak Vikas - - 2014
Bronchus-associated lymphoid tissue is a normal component of the lung's immune system and may be analogous to gut-associated lymphoid tissue, a form of mucosa-associated lymphoid tissue. Bronchial-associated lymphoid tissue lymphoma is a distinct subgroup of low-grade B-cell extranodal non-Hodgkin lymphoma, classified as marginal-zone lymphoma. It is a rare disorder and ...
Clement Jan J Belgian National Reference Laboratory for Hantavirus Infections, Clinical Virology Laboratory, University Hospital Gasthuisberg, Catholic University of Leuven, Herestraat, 49, B-3000 Leuven, Belgium. Electronic address: - - 2014
Since the first clinical description in 1994 of the so-called "Hantavirus Pulmonary Syndrome" (HPS) as a "newly recognized disease", hantavirus infections have always been characterized as presenting in two distinct syndromes, the so-called "Hemorrhagic Fever with Renal Syndrome" (HFRS) in the Old World, with the kidney as main target organ, ...
Todd Betsy B Betsy Todd is an infection prevention consultant. Contact author: betsytoddrn@gmail.com. The author has disclosed no potential conflicts of interest, financial or - - 2014
Epidemiology on the ground identifies and tracks a new respiratory virus.
Lugones Ignacio I Fundación Favaloro University Hospital, Buenos Aires, Argentina. Electronic address: - - 2014
A wide variety of surgical strategies has been described to treat scimitar syndrome. However, the incidence of pathway obstruction remains high. An alternative approach is described, in which the pulmonary venous return is widely connected to the left atrium through a tunnel constructed with the in-situ pericardium.
Gupta Pankaj P Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, - - 2014
Mounier-Kuhn syndrome, also referred to as tracheobronchomegaly, is a rare idiopathic clinical and radiologic disorder characterized by significant tracheobronchial dilation. It results in recurrent lower respiratory tract infections and bronchiectasis. In severe cases, patients may present with acute respiratory distress requiring hospital admission and ventilatory support. Clinical examination and chest ...
Takhar Rajendra R Department of Pulmonary Medicine, Peoples College of Medical Sciences and Research Centre, Bhopal, Madhya Pradesh, - - 2014
A 50-year-old man presented with dyspnoea while sitting, standing and walking but resolved completely in supine position. On cardiorespiratory examinations, fine crackles were noted over bibasal area. Chest X-ray showed bilateral reticulonodular shadows, restrictive pattern on spirometry, elevated alveolar arterial O2 gradient on arterial blood gas. High-resolution CT of the ...
Ciçek Sertaç S Anadolu Medical Center Hospital, Center for Heart and Vascular Care, Section of Cardiovascular Surgery and Cardiac Anesthesia, Kocaeli, Turkey. Electronic address: - - 2014
Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies characterized by an anomalous pulmonary vein (scimitar vein) that drains into the inferior vena cava, a hypogenetic right lung, and dextroposition of the heart. It has been reported in 3% to 6% of patients with partial anomalous pulmonary venous connection. ...
Breyer Marie-Kathrin MK Department of Respiratory Medicine, Maastricht University Medical Center + (MUMC+), Maastricht, the Netherlands; Program Development Center (CIRO), Center of expertise for chronic organ failure, Horn, the Netherlands; Department of Respiratory and Critical Care Medicine and Ludwig Boltzmann Institute for COPD and Pulmonary Epidemiology, Otto Wagner Hospital, Vienna, - - 2014
The prevalence of metabolic syndrome in COPD patients and its impact on patient related outcomes has been little studied. We evaluated the prevalence of metabolic syndrome and clinical and functional characteristics in patients with COPD and healthy subjects. 228 COPD patients and 156 healthy subjects were included. Metabolic syndrome was ...
Nowotny N N Viral Zoonoses, Emerging and Vector-Borne Infections Group, Institute of Virology, University of Veterinary Medicine Vienna, Vienna, - - 2014
A countrywide survey in Oman revealed Middle Eastrespiratory syndrome coronavirus (MERS-CoV) nucleicacid in five of 76 dromedary camels. Camel-derivedMERS-CoV sequences (3,754 nucleotides assembled from partial sequences of the open reading frame (ORF)1a, spike, and ORF4b genes) from Oman and Qatar were slightly different from each other, but closely related to ...
Savage Sharon A SA Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, - - 2014
Telomeres consist of long nucleotide repeats and a protein complex at chromosome ends essential for chromosome stability. Telomeres shorten with each cell division and thus are markers of cellular age. Dyskeratosis congenita (DC) is a cancer-prone inherited bone marrow failure syndrome caused by germ-line mutations in key telomere biology genes ...
Gungor Ugurlucan Funda F Istanbul University School of Medicine, Department of Obstetrics and Gynecology, Istanbul, - - 2013
Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) or Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital urogenital anomaly. A 13-year-old female presented with acute abdominal pain and dysmenorrhea. Ultrasonography and magnetic resonance imaging showed uterus didelphys, hematometrocolpos, obstructed hemivagina, and right renal agenesis. Hemivaginal septal resection and anastomosis ...
Muthialu Nagarajan N Department of Cardiothoracic Surgery, Great Ormond Street Hospital NHS Trust, London, UK - - 2013
Postpneumonectomy syndrome is a rare complication following pneumonectomy with its related change in mediastinal configuration. Symptoms range from airway obstruction to esophageal symptoms, leading at times to a persistent requirement for respiratory support. Surgery is often beneficial, which is in the form of placement of a prosthesis, with variable results. ...
Kapnadak Siddhartha G SG These authors contributed equally to this - - 2013
Malignant melanoma is the most aggressive form of skin cancer and carries a predisposition for metastasis to many different organs. Pulmonary dissemination is common, most often presenting as multiple discrete pulmonary nodules. While a variety of other intrathoracic patterns can occur, diffuse parenchymal infiltration causing acute respiratory failure is an ...
Padma S - - 2013
Hepatopulmonary syndrome, also known as hepatorenal syndrome, is a triad of liver disease, impaired oxygenation, and intrapulmonary vascular abnormalities. Forty-seven percent of patients with end-stage liver disease may have hepatopulmonary syndrome, an independent predictor of poor prognosis. Gross dilatation of pulmonary precapillary and capillary vessels, as well as an absolute ...
Terças Ana Cláudia Pereira - - 2013
An outbreak of hantavirus pulmonary syndrome occurred in the Sobradinho Indian settlement of the Kayabí ethnic group in northern Mato Grosso during December 2009-January 2010. We conducted a retrospective study to clarify the outbreak's epidemiologic and clinical characteristics. Results suggest a relationship between the outbreak and deforestation and farming expansion ...
Park Soo Kyung - - 2013
The prevalence of metabolic syndrome has been reported to be 20% to 50% in people with chronic obstructive pulmonary disease (COPD). Because such people are sedentary and physically inactive, they are at risk of metabolic syndrome. The extent of this problem, however, is not fully understood. This study examined the ...
Pleasants Roy A - - 2013
Abstract Background: Persons with chronic obstructive pulmonary disease (COPD) and/or asthma have great risk for morbidity. There has been sparse state-specific surveillance data to estimate the impact of COPD or COPD with concomitant asthma (overlap syndrome) on health-related impairment. Methods: The North Carolina (NC) Behavioral Risk Factor Surveillance System (BRFSS) ...
Yu D - - 2013
To examine the association between lung function and metabolic syndrome/Type 2 diabetes. A total of 1454 adults from rural Victoria, Australia, from randomly selected households included in the Crossroads study, provided spirometric measurements including forced vital capacity, forced expiratory volume in 1 s, predicted percentage value of forced expiratory volume ...
Lastinger Allison - - 2013
Scimitar syndrome is a rare congenital anomaly characterized by partial anomalous pulmonary venous drainage of the right lung to the inferior vena cava (IVC) creating a tubular opacity paralleling the right cardiac border on chest radiography which resembles a curved Turkish sword or scimitar. Associated pulmonary and vascular anomalies have ...
Johannesma P C - - 2013
In this case, we describe a patient with a history of recurrent pneumothorax. Based on CT-thorax and histopathology of the lung tissue, the Birt-Hogg-Dubé syndrome was suspected and confirmed after genetic testing. Recognizing this syndrome by pulmonologists and radiologists is very important, because the risk on developing of renal cell ...
Ichikado Kazuya K Divison of Respiratory Medicine, Saiseikai Kumamoto Hospital, Kumamoto City, Kumamoto, Japan. Electronic address: - - 2014
Diffuse alveolar damage (DAD) is the pathologic feature of rapidly progressive lung diseases, including acute respiratory distress syndrome, acute interstitial pneumonia, and acute exacerbation of idiopathic pulmonary fibrosis. The clinical significance and limitation of high-resolution computed tomography (HRCT) findings in these diseases were reviewed. The HRCT findings correlate well with ...
Rhee Chin Kook - - 2013
Abstract Background: Little information is available regarding medical utilization and cost in patients with overlap syndrome of chronic obstructive pulmonary disease (COPD) and asthma. The purpose of this study is to analyze medical utilization and cost in patients with overlap syndrome and to compare them to COPD patients without asthma. ...
Kline Jeffrey A - - 2013
Excessive radiation exposure remains a concern for patients with symptoms suggesting acute coronary syndrome and pulmonary embolism but must be judged in the perspective of pretest probability and outcomes. We quantify and qualify the pretest probability, outcomes, and radiation exposure of adults with both chest pain and dyspnea. This was ...
Thijs Willemien - - 2013
Several studies have reported a positive relationship between lung function impairment and the metabolic syndrome. This is most usually explained by abdominal adiposity. We hypothesized that the main determinant of the association between lung function impairment and abdominal obesity is the presence of visceral fat. The present study is a ...
Chatterjee Soumya - - 2013
In recent years, antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibodies to aminoacyl-transfer RNA synthetases and is characterized by a constellation of manifestations, including fever, myositis, interstitial lung disease, "mechanic's ...
Copeland S - - 2013
Capsaicin causes direct irritation of the eyes, mucous membranes, and respiratory tract. It is used in self-defense, in crowd control, and as a less lethal weapon in police work. Controlled trials suggest that capsaicin has minimal serious acute effects. Herein, we report a woman who had a 20-minute exposure to ...
Prabhu Varsha A VA Department of Pharmacy Practice, Manipal College of Pharmaceutical Sciences, Karnataka, - - 2013
Steven Johnson syndrome (SJS) is a rare drug induced mucocutaneous reaction. Here, we present an elaborate report of a 28-year-old female patient who developed Phenytoin induced SJS, which was exacerbated by cefepime.
Sybilski Adam J AJ Department of the Prevention of Environmental Hazards and Allergology, Medical University of Warsaw, Warsaw, Poland ; Department of Pediatrics and Neonatology, Central Clinical Hospital of the Ministry of Internal Affairs, Warsaw, - - 2013
Scimitar syndrome is a rare and complex congenital anomaly characterized by partial or complete anomalous pulmonary venous return from the right or left lung into the inferior vena cava, through drainage into the hepatic vein, right atrium or left atrium. The syndrome is commonly associated with hypoplasia of the right ...
Vos R - - 2013
This case report describes the evolution of pulmonary function findings (FVC, FEV1 and TLC) and CT features with pirfenidone treatment for restrictive allograft syndrome following lung transplantation. Furthermore, we herein report hypermetabolic activity on (18) F-FDG PET imaging in this setting, which could indicate active fibroproliferation and pleuroparenchymal remodeling. These ...
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