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https://www.readbyqxmd.com/read/29143574/investigating-the-preventive-effects-of-baicalin-and-gallocatechin-aginst-glyoxal-induced-cystatin-aggregation
#1
Aamir Sohail, Waseem Feeroze Bhat, Mohammad Furkan, Aaliya Shah, Bilqees Bano
Several mammalian proteins form pathological deposits under nonphysiological conditions that are associated with many degenerative diseases. Protein aggregation is associated with aging, as well as a variety of diseases, including cystic fibrosis, amyotrophic lateral sclerosis (ALS), and hypertrophic cardiomyopathy. There is a lack of any potential anti-amyloidogenic agents and therapeutics till date. Polyphenols have been accredited with myriad biological effects. An analysis of the effects of natural agents like baicalin (BC) and gallocatechin (GC) on aggregation process can open new avenues for the treatment of protein misfolding diseases...
November 16, 2017: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/28828392/thermal-stability-as-a-determinant-of-aav-serotype-identity
#2
Antonette Bennett, Saajan Patel, Mario Mietzsch, Ariana Jose, Bridget Lins-Austin, Jennifer C Yu, Brian Bothner, Robert McKenna, Mavis Agbandje-McKenna
Currently, there are over 150 ongoing clinical trials utilizing adeno-associated viruses (AAVs) to target various genetic diseases, including hemophilia (AAV2 and AAV8), congenital heart failure (AAV1 and AAV6), cystic fibrosis (AAV2), rheumatoid arthritis (AAV2), and Batten disease (AAVrh.10). Prior to patient administration, AAV vectors must have their serotype, concentration, purity, and stability confirmed. Here, we report the application of differential scanning fluorimetry (DSF) as a good manufacturing practice (GMP) capable of determining the melting temperature (Tm) for AAV serotype identification...
September 15, 2017: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/28653300/defining-the-incidence-and-associated-morbidity-and-mortality-of-severe-respiratory-syncytial-virus-infection-among-children-with-chronic-diseases
#3
REVIEW
Paolo Manzoni, Josep Figueras-Aloy, Eric A F Simões, Paul A Checchia, Brigitte Fauroux, Louis Bont, Bosco Paes, Xavier Carbonell-Estrany
INTRODUCTION: REGAL (RSV Evidence-a Geographical Archive of the Literature) has provided a comprehensive review of the published evidence in the field of respiratory syncytial virus (RSV) in Western countries over the last 20 years. This review covers the risk and burden of RSV infection in children with underlying medical conditions or chronic diseases (excluding prematurity and congenital heart disease). METHODS: A systematic review of publications between January 1, 1995 and December 31, 2015 across PubMed, Embase, The Cochrane Library, and Clinicaltrials...
September 2017: Infectious Diseases and Therapy
https://www.readbyqxmd.com/read/28649564/primary-ciliary-dyskinesia-an-update-on-clinical-aspects-genetics-diagnosis-and-future-treatment-strategies
#4
REVIEW
Virginia Mirra, Claudius Werner, Francesca Santamaria
Primary ciliary dyskinesia (PCD) is an orphan disease (MIM 244400), autosomal recessive inherited, characterized by motile ciliary dysfunction. The estimated prevalence of PCD is 1:10,000 to 1:20,000 live-born children, but true prevalence could be even higher. PCD is characterized by chronic upper and lower respiratory tract disease, infertility/ectopic pregnancy, and situs anomalies, that occur in ≈50% of PCD patients (Kartagener syndrome), and these may be associated with congenital heart abnormalities...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28456611/increased-risk-of-ptld-in-lung-transplant-recipients-with-cystic-fibrosis
#5
Erin M Lowery, William Adams, Shellee A Grim, Nina M Clark, Leah Edwards, Jennifer E Layden
BACKGROUND: Post-transplant lymphoproliferative disease (PTLD) is an important cause of morbidity and mortality following lung transplantation. Recipients with cystic fibrosis (CF) may have an increased risk of PTLD although the literature is limited to single center cohorts. Our primary aim is to examine PTLD in an adult lung transplant population by utilizing the International Society for Heart and Lung Transplantation Registry. METHODS: We studied 30,598 adult recipients of lung transplants performed between 1999 and 2011...
November 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28449285/research-review-childhood-chronic-physical-illness-and-adult-emotional-health-a-systematic-review-and-meta-analysis
#6
REVIEW
Ekin Secinti, Ellen J Thompson, Marcus Richards, Darya Gaysina
BACKGROUND: Childhood chronic physical illness is associated with a greater vulnerability for emotional problems (i.e. depression and anxiety) in childhood. However, little is known about life-long effects of childhood chronic physical illness on mental health. The present study aims to systematically review evidence for associations between eight chronic physical illnesses with childhood onset (arthritis, asthma, cancer, chronic renal failure, congenital heart disease, cystic fibrosis, type 1 diabetes, and epilepsy) and adult emotional problems...
July 2017: Journal of Child Psychology and Psychiatry, and Allied Disciplines
https://www.readbyqxmd.com/read/28443234/plastic-bronchitis-an-unusual-complication-of-acute-chest-syndrome-in-adult
#7
Sarah Feray, Pierre Mora, Maxens Decavele, Tài Pham, El Mahdi Hafiani, Muriel Fartoukh
Plastic bronchitis is used to designate endobronchial plugs of rubber-like consistency that form into bronchial trees. It has been described in several diseases like asthma, cystic fibrosis, pulmonary infection, cyanotic congenital heart disease and in few young children with homozygous sickle cell disease. We report the first sickle cell adult case of plastic bronchitis during acute chest syndrome. He developed severe acute respiratory distress syndrome. This unusual presentation related to obstruction by voluminous casts may alert physicians to focus more on the bronchi in sickle cell patients...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28387447/withdrawn-interventions-for-fatigue-and-weight-loss-in-adults-with-advanced-progressive-illness
#8
REVIEW
Cathy Payne, Philip J Wiffen, Suzanne Martin
BACKGROUND: Fatigue and unintentional weight loss are two of the commonest symptoms experienced by people with advanced progressive illness. Appropriate interventions may bring considerable improvements in function and quality of life to seriously ill people and their families, reducing physical, psychological and spiritual distress. OBJECTIVES: To conduct an overview of the evidence available on the efficacy of interventions used in the management of fatigue and/or unintentional weight loss in adults with advanced progressive illness by reviewing the evidence contained within Cochrane reviews...
April 7, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28364441/survival-after-lung-transplantation-for-cystic-fibrosis-in-sweden
#9
MULTICENTER STUDY
Marita Gilljam, Ulla Nyström, Göran Dellgren, Ingrid Skog, Lennart Hansson
Objectives: In Sweden, lung transplantation has been performed in patients with end-stage lung disease since 1990. We assessed survival after lung transplantation for cystic fibrosis (CF) with focus on early mortality and outcome for patients infected with certain multiresistant bacteria, considered a relative contraindication for lung transplantation. Methods: Review of CF and transplant databases and patient charts. The Kaplan-Meier method and log-rank test were used for survival analysis and group comparison...
March 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28337699/lung-ultrasound-is-comparable-with-chest-roentgenogram-for-diagnosis-of-community-acquired-pneumonia-in-hospitalised-children
#10
Krishna Kumar Yadav, Shally Awasthi, Anit Parihar
OBJECTIVE: To evaluate the accuracy of lung ultrasound (LUS) in comparison to chest roentgenogram (CXR) in hospitalised children with community-acquired pneumonia (CAP). METHODS: This study was a hospital based prospective observational study, conducted between January 2014 and December 2014. Hospitalised children aged 2 to 59 mo with community-acquired pneumonia were included in the study. The informed written consent was taken from parents (or legal guardian) before recruitment...
July 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28314540/technological-advances-shed-light-on-left-ventricular-cardiac-disturbances-in-cystic-fibrosis
#11
REVIEW
Zahra N Sayyid, Zachary M Sellers
Cystic fibrosis (CF), the most common autosomal recessive lethal disease in Caucasians, causes chronic pulmonary disease and can lead to cor pulmonale with right ventricular dysfunction. The presence of the cystic fibrosis transmembrane conductance regulator (CFTR) in cardiac myocardia has prompted debate regarding possible defective ion channel-induced cardiomyopathy. Clinical heart disease in CF is considered rare and is restricted to case reports. It has been unclear if this is due to the lack of physiological importance of CFTR in the heart, the relatively short lifespan of those with CF, or a technical inability to detect subclinical disease...
July 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28247443/acute-kidney-injury-in-patients-treated-with-iv-beta-lactam-beta-lactamase-inhibitor-combinations
#12
W Cliff Rutter, David S Burgess
STUDY OBJECTIVE: Increased acute kidney injury (AKI) incidence has been reported in patients receiving piperacillin-tazobactam (PTZ) therapy compared with other β-lactams. The authors sought to determine if the addition of β-lactamase inhibitors impacts AKI incidence by comparing patients treated with PTZ or ampicillin-sulbactam (SAM). DESIGN: Retrospective cohort study. SETTING: Large academic tertiary care hospital. PATIENTS: Overall, 2448 patients received PTZ (n=1836) or SAM (n=612) for at least 48 hours between September 1, 2007, and September 30, 2015...
May 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28125022/pediatric-exercise-testing-value-and-implications-of-peak-oxygen-uptake
#13
REVIEW
Paolo T Pianosi, Robert I Liem, Robert G McMurray, Frank J Cerny, Bareket Falk, Han C G Kemper
Peak oxygen uptake (peak V ˙ O 2 ) measured by clinical exercise testing is the benchmark for aerobic fitness. Aerobic fitness, estimated from maximal treadmill exercise, is a predictor of mortality in adults. Peak V ˙ O 2 was shown to predict longevity in patients aged 7-35 years with cystic fibrosis over 25 years ago. A surge of exercise studies in young adults with congenital heart disease over the past decade has revealed significant prognostic information. Three years ago, the first clinical trial in children with pulmonary arterial hypertension used peak V ˙ O 2 as an endpoint that likewise delivered clinically relevant data...
January 24, 2017: Children
https://www.readbyqxmd.com/read/27880745/announcement-national-family-history-day-november-24-2016
#14
(no author information available yet)
In 2004, the U.S. Surgeon General declared that Thanksgiving would be National Family History Day, a day designed to encourage American families to learn about and create a written record of their family health history. Family history can identify those persons with a higher-than-average risk for many common diseases, such as heart disease, cancer, and type 2 diabetes. Having at least one first-degree relative with a disease can increase a person's risk twofold or more (1). Family history is also a determinant of less common diseases like sickle cell disease and cystic fibrosis (1)...
November 25, 2016: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/27780555/relation-of-left-atrial-size-to-atrial-fibrillation-in-patients-aged-%C3%A2-22%C3%A2-years
#15
Douglas Y Mah, Divya Shakti, Kimberlee Gauvreau, Steven D Colan, Mark E Alexander, Dominic J Abrams, David W Brown
Left atrial (LA) dilation has been shown to be associated with atrial fibrillation (AF) in the adult population, with some studies indicating that larger LAs are more prone to AF recurrence. The relation of LA size to AF in the pediatric and young adult population has not been investigated. In this study, all pediatric patients (aged ≤22 years) who presented to Boston Children's Hospital from January 2002 to December 2012 with AF were reviewed. Patients with significant congenital heart disease, cardiomyopathies, proven channelopathies, previous cardiac surgery, end-stage renal disease, or severe lung disease/cystic fibrosis were excluded...
January 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/27678488/health-related-quality-of-life-of-young-people-with-long-term-illnesses-before-and-after-transfer-from-child-to-adult-healthcare
#16
A E While, E Heery, A M Sheehan, I Coyne
BACKGROUND: The numbers of children with long-term illnesses surviving into adulthood and transferring from child to adult services has increased dramatically in the last 30 years. This study aimed to examine health-related quality of life pre- and post-transfer from child to adult healthcare for young people with three long-term illnesses. METHODS: A total of 217 young people with cystic fibrosis, congenital heart defects or diabetes attending child and adult hospital services in Dublin, Ireland completed a questionnaire survey...
January 2017: Child: Care, Health and Development
https://www.readbyqxmd.com/read/27578468/heart-involvement-in-cystic-fibrosis-a-specific-cystic-fibrosis-related-myocardial-changes
#17
REVIEW
Fabien Labombarda, Eric Saloux, Jacques Brouard, Emmanuel Bergot, Paul Milliez
Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy...
September 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27551814/ultrashort-echo-time-magnetic-resonance-imaging-is-a-sensitive-method-for-the-evaluation-of-early-cystic-fibrosis-lung-disease
#18
David J Roach, Yannick Crémillieux, Robert J Fleck, Alan S Brody, Suraj D Serai, Rhonda D Szczesniak, Stephanie Kerlakian, John P Clancy, Jason C Woods
RATIONALE: Recent advancements that have been made in magnetic resonance imaging (MRI) improve our ability to assess pulmonary structure and function in patients with cystic fibrosis (CF). A nonionizing imaging modality that can be used as a serial monitoring tool throughout life can positively affect patient care and outcomes. OBJECTIVES: To compare an ultrashort echo-time MRI method with computed tomography (CT) as a biomarker of lung structure abnormalities in young children with early CF lung disease...
November 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27492388/feasibility-tolerability-and-safety-of-pediatric-hyperpolarized-129-xe-magnetic-resonance-imaging-in-healthy-volunteers-and-children-with-cystic-fibrosis
#19
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...
November 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27454104/enzyme-replacement-therapy-for-anderson-fabry-disease
#20
REVIEW
Regina El Dib, Huda Gomaa, Raíssa Pierri Carvalho, Samira E Camargo, Rodrigo Bazan, Pasqual Barretti, Fellype C Barreto
BACKGROUND: Anderson-Fabry disease is an X-linked defect of glycosphingolipid metabolism. Progressive renal insufficiency is a major source of morbidity, additional complications result from cardio- and cerebro-vascular involvement. Survival is reduced among affected males and symptomatic female carriers.This is an update of a Cochrane review first published in 2010, and previously updated in 2013. OBJECTIVES: To evaluate the effectiveness and safety of enzyme replacement therapy compared to other interventions, placebo or no interventions, for treating Anderson-Fabry disease...
July 25, 2016: Cochrane Database of Systematic Reviews
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