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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. ...
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 ...
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 ...
Crepaz Roberto - - 2013
Patients with Down's syndrome and Eisenmenger's syndrome (ES) are at high risk of developing pulmonary arterial hypertension (PAH) earlier than patients without Down's syndrome. However, data on the efficacy of PAH-specific therapy in patients with Down's syndrome are limited. The aim of this retrospective analysis was to determine the long-term ...
Sen Hadice Selimoglu - - 2013
Swyer-James-MacLeod Syndrome (SJMS) is a rare constrictive bronchiolitis with airflow obstruction and decreased number and diameter of ipsilateral peripheral pulmonary vessels . This syndrome is characterized by unilateral hyperlucency on chest radiography. Computerized tomography provides useful additional information. The diagnosis is usually made in childhood, but sometimes occur in adulthood. ...
Oka Hiroaki - - 2013
Sjogren's syndrome is characterized by lymphocytic infiltration of the exocrine glands, together with polyclonal B-cell activation, and lung diseases are well-known complications of the disease. Therefore, in most cases associated with Sjogren's syndrome, infiltrating lymphocytes in the lung specimen exhibit the features of B-cells. We herein report an atypical case ...
Ross Mindy K - - 2013
We describe an infant prenatally diagnosed with hydrops fetalis ultimately found to have Noonan syndrome (NS). Prior to genetic confirmation of diagnosis, lung biopsy was performed which revealed widespread pulmonary interstitial glycogenosis (PIG), abnormal alveolarization, and mild inflammation. Although genetic alterations have been identified in NS, the mutations are heterogeneous ...
Simonds Anita K - - 2013
While obstructive sleep apnoea syndrome dominates discussion of the prevalence of sleep disordered breathing, nocturnal hypoventilation remains extremely prevalent in those with chronic ventilatory disorders and in the natural history of these conditions pre-dates the development of daytime ventilatory failure. In this review the clinical management of chronic hypoventilation in ...
Morino Masaaki - - 2013
The combination of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis represents a rare congenital anomaly called Herlyn-Werner-Wunderlich syndrome (HWWS) or obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. Several anomalies have recently been reported to be associated with this syndrome. The present patient with HHWS had multiple anomalies: intestinal ...
Mukherjee Bipasha - - 2013
ABSTRACT Purpose: Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is an autosomal dominant disorder characterized by bilateral ptosis with poor levator function, epicanthus inversus and shortened horizontal palpebral fissures. Method: Eighteen -month -old twin sisters presented with history of watering from their left eyes since birth. Examination revealed features of BPES with left ...
Kashif Waqar - - 2013
Anti-glomerular basement membrane (anti-GBM) disease usually presents as rapidly progressive glomerulonephritis, and, when accompanied with pulmonary hemorrhage, it is called Goodpasture's syndrome. Anti-neutrophilic cytoplasmic antibodies (ANCA) may co-exist with anti-GBM antibodies. In most of these "double positive" cases, ANCA is specific for myeloperoxidase (p-ANCA). We report a rare case of ...
Inaty Hanine - - 2013
Pulmonary amyloidosis in the setting of Sjogren syndrome is rare. It most commonly presents in form of multinodular disease with or without cysts formation. Amyloid plaques may also deposit in the airway submucosa, causing airway narrowing; the condition referred as "tracheobronchial amyloidosis" (TBA). Patients with this condition most commonly present ...
Saeed Rebeen R RR Department of Medicine, General University Teaching Hospital of Sulaimania, Kurdistan, - - 2013
Yellow nail syndrome is a rare entity of unknown etiology. Classically, it consists of a triad of slow-growing yellow nails of fingers and toes, lymphedema, and pulmonary manifestations mainly pleural effusion. Other pulmonary manifestations also have been described in the literature such as bronchiectasis, recurrent pneumonias, bronchitis, and sinusitis. This ...
Stojan George - - 2013
Sjögren's syndrome (SS) is primarily defined by its impact on the oral and ocular system resulting in xerostomia and xerophthalmia. However, SS can also manifest throughout the respiratory system. Subclinical pulmonary involvement is common. Clinically significant involvement can result in a 4-fold increased risk of death. Thus, recognizing the many ...
Cottin Vincent - - 2013
Several groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and ...
Ballew Bari J - - 2013
Dyskeratosis congenita (DC) is a cancer-prone inherited bone marrow failure syndrome caused by aberrant telomere biology. The mucocutaneous triad of nail dysplasia, abnormal skin pigmentation and oral leukoplakia is diagnostic, but is not always present; DC can also be diagnosed by the presence of very short leukocyte telomeres. Patients with ...
Kang Lung-Yun - - 2013
Mucosa-associated lymphoid tissue (MALT) lymphoma of the thymus is rare. We reported a case of a 37-year-old Chinese female with Sjögren's syndrome and hyperglobulinemia. She suffered from chronic cough for 3 weeks. Chest computed tomography (CT) demonstrated a multiloculated cystic mass in mediastinum prevascular space and multiple lung cysts. Laboratory ...
Hayes Don D - - 2013
INTRODUCTION: Antisynthetase Syndrome is associated with interstitial lung disease in adult patients, but this has not been described in children. MATERIALS AND METHODS: A 13-year-old with interstitial lung disease due to Antisynthetase Syndrome and pulmonary arterial hypertension underwent emergent bilateral lung transplantation after a rapid clinical decline. CONCLUSION: We present ...
Brioude Geoffrey - - 2013
Bilateral lung transplantation was performed in a 52-year old man with end-stage Kartagener's syndrome. A postimplantation right lower lobectomy was required for volume reduction and dextrocardia. A bronchial fistula developed with an intractable colonized residual pleural cavity. Closure was obtained successfully with multiple-stage procedures including decortication, muscle flap and an ...
Sullivan T - - 2013
Cryptosporidium rarely affects the lungs, and is not typically associated with the immune reconstitution inflammatory syndrome (IRIS). We describe the first published case of pulmonary IRIS following the initiation of antiretroviral therapy in a patient with AIDS and pulmonary cryptosporidiosis, and discuss its implications for HIV patient care.
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