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multiple cystic lung disease

Benjamin T Kopp, Juan Antonio Ortega-García, S Christy Sadreameli, Jack Wellmerling, Estelle Cormet-Boyaka, Rohan Thompson, Sharon McGrath-Morrow, Judith A Groner
Secondhand smoke exposure (SHSe) has multiple adverse effects on lung function and growth, nutrition, and immune function in children; it is increasingly being recognized as an important modifier of disease severity for children with chronic diseases such as cystic fibrosis (CF). This review examines what is known regarding the prevalence of SHSe in CF, with the majority of reviewed studies utilizing parental-reporting of SHSe without an objective biomarker of exposure. A wide range of SHSe is reported in children with CF, but under-reporting is common in studies involving both reported and measured SHSe...
October 12, 2016: International Journal of Environmental Research and Public Health
Patricia Moran Losada, Philippe Chouvarine, Marie Dorda, Silke Hedtfeld, Samira Mielke, Angela Schulz, Lutz Wiehlmann, Burkhard Tümmler
Chronic airway infections determine most morbidity in people with cystic fibrosis (CF). Herein, we present unbiased quantitative data about the frequency and abundance of DNA viruses, archaea, bacteria, moulds and fungi in CF lower airways. Induced sputa were collected on several occasions from children, adolescents and adults with CF. Deep sputum metagenome sequencing identified, on average, approximately 10 DNA viruses or fungi and several hundred bacterial taxa. The metagenome of a CF patient was typically found to be made up of an individual signature of multiple, lowly abundant species superimposed by few disease-associated pathogens, such as Pseudomonas aeruginosa and Staphylococcus aureus, as major components...
April 2016: ERJ Open Research
Valerie Waters, Sanja Stanojevic, Felix Ratjen
Introduction Cystic fibrosis (CF) is a disease characterized by recurrent flares of respiratory symptoms, known as pulmonary exacerbations (PExs), which have a cumulative, detrimental effect on lung function decline and overall mortality. Although much research has been done on the effects of PExs in adults with CF, considerably less is known about these events in young children with CF. Areas covered This review describes the typical presentation of PExs in children and their impact on long-term clinical outcomes...
October 8, 2016: Expert Review of Respiratory Medicine
Xiao-Xing Wang, Wenfang Liu, Tian Zheng, Jeong M Park, David E Smith, Meihua R Feng
1. Cystic fibrosis (CF) is a disease affecting multiple organs that may reduce the systemic exposure of some drugs. The objective of this work was to characterize and compare the population pharmacokinetics (PK) of the immunosuppressant mycophenolic acid (MPA), and its glucuronide metabolite (MPAG) in adult lung transplant recipients with and without CF (NCF) following repeated oral administration of the prodrug mycophenolate mofetil (MMF). 2. A population PK model was developed, with simultaneous modeling of MPA and MPAG, using nonlinear mixed effects modeling...
August 10, 2016: Xenobiotica; the Fate of Foreign Compounds in Biological Systems
Dalila Mil-Homens, Sandra N Pinto, Rute G Matos, Cecília Arraiano, Arsenio M Fialho
Chronic lung disease caused by persistent bacterial infections is a major cause of morbidity and mortality in patients with cystic fibrosis (CF). CF pathogens acquire antibiotic resistance, overcame host defenses and impose uncontrolled inflammation that ultimately may cause permanent damage of lungs' airways. Among the multiple CF-associated pathogens, Burkholderia cenocepacia and other B. cepacia complex (Bcc) bacteria have become prominent contributors of disease progression. Here, we demonstrate that BcaA, a trimeric autotransporter adhesin (TAA) from the epidemic strain B...
September 29, 2016: Cellular Microbiology
Michal Gur, Karin Yaacoby-Bianu, Anat Ilivitzki, Ronen Bar-Yoseph, Vered Nir, Fahed Hakim, Yazeed Toukan, Lea Bentur
INTRODUCTION: Bronchiolitis obliterans (BO) is a chronic airway disease following an insult to the lower respiratory tract. Lung clearance index (LCI) measures ventilation inhomogeneity and has been studied in cystic fibrosis (CF). We aimed to evaluate LCI in BO and to compare it to LCI in CF patients. METHODS: LCI was measured in BO patients, compared to CF patients, and correlated with spirometry and CT findings. RESULTS: Twenty BO patients and 26 CF patients (with similar mean age and BMI) underwent evaluation...
August 31, 2016: Lung
Farah Adel, José Manuel Ramia, Luis Gijón, Roberto de la Plaza-Llamas, Vladimir Arteaga-Peralta, Carmen Ramiro-Perez
BACKGROUND: Cystic echinococcosis is a zoonosis caused by larvae of the parasite Echinococcus that is endemic in many countries of the Mediterranean area. It can affect any organ, with the most common sites being liver (70%) and lung (20%). Splenic hydatid disease, despite being rare, is the third most common location. Other locations such as bone, skin, or kidney are exceptional. OBJECTIVE: To present our experience in extrahepatic and extrapulmonary hydatidosis...
September 5, 2016: Cirugia y Cirujanos
B B Singh, R Sharma, J K Sharma, V Mahajan, J P S Gill
Cystic echinococcosis due to Echinococcus granulosus is a serious public health and economic concern in India. The disease is endemic in most of the food producing animals such as cattle, buffalo, sheep, goat and pigs in the country. In this study, tissues comprising of pieces of liver and lungs were collected in 10 % formal buffered saline. The formalin fixed tissues (liver and lungs) from 10 cattle, buffalo, sheep, goat and pigs each were selected and further processed by acetone benzene method for histopathological examinations...
September 2016: Journal of Parasitic Diseases: Official Organ of the Indian Society for Parasitology
Richard W Bohannon, Rebecca Crouch
RATIONALE, AIMS, AND OBJECTIVES: The 6-minute walk test (6MWT) is widely used as a test of functional exercise capacity. Several studies have reported the minimal clinically important difference (MCID) for the 6MWT; however, the findings of the studies have not been examined in the context of one another. In this review, we aimed to summarize available information on the MCID for the 6MWT performed by patients with pathology. METHODS: Relevant literature was identified by searches of 3 electronic databases (PubMed, Scopus, and Cumulative Index of Nursing and Allied Health), examination of article reference lists, and consultation with an expert...
September 4, 2016: Journal of Evaluation in Clinical Practice
Lyle L Pritchard
Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction...
September 2016: FP Essentials
Mirjam Stahl, Mark O Wielpütz, Simon Y Graeber, Cornelia Joachim, Olaf Sommerburg, Hans-Ulrich Kauczor, Michael Puderbach, Monika Eichinger, Marcus A Mall
RATIONALE: Early onset and progression of lung disease in children with cystic fibrosis (CF) indicates that sensitive noninvasive outcome measures are needed for diagnostic monitoring and early intervention clinical trials. The lung clearance index (LCI) and chest magnetic resonance imaging (MRI) were shown to detect early lung disease in CF, however, the relationship between the two measures remains unknown. OBJECTIVES: To correlate the LCI with abnormalities detected by MRI and compare the sensitivity of the two techniques to detect responses to therapy for pulmonary exacerbations in children with CF...
August 30, 2016: American Journal of Respiratory and Critical Care Medicine
Lacrecia J Britton, Gabriela R Oates, Robert A Oster, Staci T Self, Robert B Troxler, Wynton C Hoover, Hector H Gutierrez, William T Harris
OBJECTIVE: The clinical benefit of newborn screening (NBS) for cystic fibrosis (CF) has been primarily nutritional, with less overt respiratory impact. Identification of risk factors for infant CF lung disease could facilitate targeted interventions to improve pulmonary outcomes. METHODS: This retrospective study evaluated socioeconomic information, clinical data, and results from routine infant pulmonary function testing (iPFT) of infants diagnosed with CF through NBS (N = 43) at a single CF center over a 4-year period (2008-2012)...
November 2016: Pediatric Pulmonology
Angela T Nguyen, Jace W Jones, Miguel Cámara, Paul Williams, Maureen A Kane, Amanda G Oglesby-Sherrouse
Cystic fibrosis (CF) is a hereditary disease that predisposes individuals to pulmonary dysfunction and chronic infections. Early infection of the CF lung with Staphylococcus aureus is common, while Pseudomonas aeruginosa becomes dominant as disease progresses. Emergence of P. aeruginosa likely depends on the action of multiple 2-alkyl-4-(1H)-quinolones (AQ) secreted by this organism. We recently showed that antimicrobial activity against S. aureus is enhanced by iron depletion and is dependent upon multiple AQ metabolites...
2016: Frontiers in Microbiology
Per Magnus Gustafsson, Paul David Robinson, Anders Lindblad, Daniel Oberli
Multiple breath inert gas washout (MBW) is ideally suited for early detection and monitoring of serious lung disease, such as cystic fibrosis, in infants and young children. Validated commercial options for the MBW technique are limited, and suitability of Nitrogen (N2) based MBW is of concern given the detrimental effect of exposure to pure O2 on infant breathing pattern. We propose novel methodology using commercially available N2 MBW equipment to facilitate 4% sulfur hexafluoride (SF6) multiple breath inert gas washin and washout suitable for the infant age range...
August 4, 2016: Journal of Applied Physiology
Laura L Walkup, Robert P Thomen, Teckla G Akinyi, Erin Watters, Kai Ruppert, John P Clancy, Jason C Woods, Zackary I Cleveland
BACKGROUND: Hyperpolarized (129)Xe is a promising contrast agent for MRI of pediatric lung function, but its safety and tolerability in children have not been rigorously assessed. OBJECTIVE: To assess the feasibility, safety and tolerability of hyperpolarized (129)Xe gas as an inhaled contrast agent for pediatric pulmonary MRI in healthy control subjects and in children with cystic fibrosis. MATERIALS AND METHODS: Seventeen healthy control subjects (ages 6-15 years, 11 boys) and 11 children with cystic fibrosis (ages 8-16 years, 4 boys) underwent (129)Xe MRI, receiving up to three doses of (129)Xe gas prepared by either a commercially available or a homebuilt (129)Xe polarizer...
August 5, 2016: Pediatric Radiology
M Leigh Anne Daniels, Katherine R Birchard, Jared R Lowe, Michael V Patrone, Peadar G Noone, Michael R Knowles
RATIONALE: Idiopathic bronchiectasis patients are predominantly female with an asthenic body morphotype and have frequent nontuberculous mycobacterial respiratory infections. They also demonstrate phenotypic features (scoliosis, pectus deformity, mitral valve prolapse) that are commonly seen in heritable connective tissue disorders. OBJECTIVES: Determine whether lumbar dural sac size is increased in idiopathic bronchiectasis as compared to controls, and assess whether dural sac size is correlated with phenotypic characteristics seen in heritable connective tissue disorders...
July 13, 2016: Annals of the American Thoracic Society
Brijesh Kumar, Silvia T Cardona
Burkholderia cenocepacia belongs to the Burkholderia cepacia complex (Bcc), a group of at least 18 distinct species that establish chronic infections in the lung of people with the genetic disease cystic fibrosis (CF). The sputum of CF patients is rich in amino acids and was previously shown to increase flagellar gene expression in B. cenocepacia. We examined flagellin expression and flagellar morphology of B. cenocepacia grown in synthetic cystic fibrosis sputum medium (SCFM) compared to minimal medium. We found that CF nutritional conditions induce increased motility and flagellin expression...
2016: Frontiers in Cellular and Infection Microbiology
Ozgur Sogut, Demet Tas
No abstract text is available yet for this article.
2016: Pan African Medical Journal
Rebecca J Theilmann, Chantal Darquenne, Ann R Elliott, Barbara A Bailey, Douglas J Conrad
Translational investigations in cystic fibrosis (CF) have a need for improved quantitative and longitudinal measures of disease status. To establish a non-invasive quantitative MRI technique to monitor lung health in patients with CF and correlate MR metrics with airway physiology as measured by multiple breath washout (MBW). Data were collected in 12 CF patients and 12 healthy controls. Regional (central and peripheral lung) measures of fractional lung water density (FLD: air to 100% fluid) were acquired both at FRC and TLC on a 1...
2016: PloS One
Vinod Kumar Arora, Neha Chopra, Poorva Singh, Vasantha Kumar Venugopal, Salil Narang
Hydatid disease is a zoonotic infestation caused by larval cestode of genus Echinococcus. Cystic form of this infection mostly involves liver and lung. Hydatid disease of the parotid gland is very rare even in endemic areas and is often clinically mistaken for parotid tumors or cysts. The presence of protoscolices, laminated membranes, and isolated hooklets are characteristic cytological features observed on fine-needle aspirate from hydatid cysts. We report unusual cytological features from a hydatid cyst of parotid in a 13-year-old girl...
September 2016: Diagnostic Cytopathology
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