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Cystic fibrosis anesthesia

Martin Lenihan, Darren Mullane, Donal Buggy, Georgina Flood, Michael Griffin
Cystic fibrosis (CF) is an autosomal recessive disorder affecting approximately 1 in 2,500 live births worldwide, with double this estimated frequency in Ireland. CF is characterized by a genetic defect of the CF transmembrane regulator protein, causing impairment of chloride ion transportation. This has multisystem consequences, particularly in the lungs, where it results in intensely mucoid secretions, which increases susceptibility to infection. Lung transplantation is indicated in CF when there is progressive decline in a patient's functional reserve...
November 23, 2017: Journal of Cardiothoracic and Vascular Anesthesia
Bei-Bei Zhang, Chao Yan, Fan Fang, Ying Du, Rui Ma, Xiang-Yang Li, Qian Yu, Di Meng, Ren-Xian Tang, Kui-Yang Zheng
Previous studies showed that CD4+T cells responses might be involved in the process of biliary fibrosis. However, the underlying mechanism resulting in biliary fibrosis caused by Clonorchis sinensis remains not yet fully elucidated. The objectives of the present study were to investigate the different profiles of hepatic CD4+T cell subsets (Th1, Th2, Th17 and Treg cells) and their possible roles in the biliary fibrosis of different strains of mice (C57BL/6, BALB/c and FVB mice) induced by C. sinensis infection...
2017: PloS One
M Piazza, G Martucci, A Arcadipane
Cystic fibrosis (CF) is the most common life-limiting genetic disease in Caucasians. Declining lung function is the principal cause of death, but liver involvement can lead to the need for liver transplantation. General anesthesia has detrimental effects on pulmonary function, increasing perioperative morbidity and mortality in CF patients. Regional anesthetic techniques improve outcomes by reducing anesthetic drugs and administration of opioids, and hastening extubation, awakening, and restarting respiratory of physiotherapy...
October 2016: Saudi Journal of Anaesthesia
Victoria Peer, Ramzia Abu Hamad, Sylvia Berman, Shai Efrati
BACKGROUND: Massive DNA destruction/accumulation of cell-free DNA debris is a sensitive biomarker of progressive organ/tissue damage. Deleterious consequences of DNA debris accumulation are evident in cardiac ischemia, thrombosis, auto-inflammatory diseases, SLE-induced lupus nephritis and cystic fibrosis. In case of renal pathologies, degradation and elimination of DNA debris are suppressed, due to downregulated DNAse-I activity within the diseased kidneys. The aim of the current study was to evaluate whether exogenous DNAse-I administration might exert renoprotective effects in the setting of acute kidney injury (AKI or acute renal failure)...
2016: American Journal of Nephrology
Nathan C Dean, Don H Van Boerum, Theodore G Liou
BACKGROUND: Rib fractures associated with osteoporosis have been reported to occur ten times more frequently in adults with cystic fibrosis. Fractures cause chest pain, and interfere with cough and sputum clearance leading to worsened lung function and acute exacerbations which are the two main contributors to early mortality in cystic fibrosis. Usual treatment involves analgesics and time for healing; however considerable pain and disability result due to constant re-injury from chronic repetitive cough...
October 1, 2014: BMC Research Notes
Stephen Kirkby, Don Hayes, Margaret Ginn-Pease, John Gatz, Clayton Ellis Wisely, Meredith Lind, Charles Elmaraghy, Nancy Ryan-Wenger, Shahid I Sheikh
BACKGROUND: Flexible fiberoptic bronchoscopy was performed prior to functional endoscopic sinus surgery (FESS) while under general anesthesia to collect bronchoalveolar lavage fluid (BALF) for lower respiratory tract cultures in patients with cystic fibrosis (CF). METHODS: A retrospective chart review was performed on all CF patients who underwent combined FESS and bronchoscopy between January 2009 and October 2010. Along with demographic data, bacterial, fungal, and acid fast bacillus culture data from BALF was collected and compared to oropharyngeal swab and sputum cultures obtained over the year prior to FESS and bronchoscopy...
February 2015: Pediatric Pulmonology
Emre Entok, Mehmet Cengiz Ustuner, Cansu Ozbayer, Neslihan Tekin, Fahrettin Akyuz, Berat Yangi, Hulyam Kurt, Irfan Degirmenci, Hasan Veysi Gunes
Inflammation has an important role in many diseases such as cystic fibrosis, allergies and cancer. The free radicals produced during inflammation, can induce gene mutations and posttranslational modifications of cancer related proteins. Nigella sativa L. (N. sativa) is herbaceous plant and commonly used as a natural food. It has many pharmacological effects including antibacterial, antifungal, antitumor, analgesic, antipyretic activity. The aim of this study was to investigate the anti-inflammatuar and anti-oxidant activity of N...
May 2014: Molecular Biology Reports
M Deighan, S Ash, R McMorrow
Cystic fibrosis affects 1 in 1600-2500 live births and is inherited in an autosomal recessive manner. It primarily involves the respiratory, gastrointestinal and reproductive tracts, with impaired clearance of, and obstruction by, increasingly viscous secretions. Severe respiratory disease, diabetes and gastro-oesophageal reflux may result. Improvements in medical management and survival of cystic fibrosis patients means more are committing to pregnancies. Although guidance for anaesthesia in this patient group is available, management and outcome data associated with more severe cases are sparse...
February 2014: International Journal of Obstetric Anesthesia
Barbara Adinolfi, Nicoletta Gava
Background Hypnosis is defined as "as an interaction in which the hypnotist uses suggested scenarios ("suggestions") to encourage a person's focus of attention to shift towards inner experiences". Aim of the work The focus of this review is to summarize the findings of controlled outcome studies investigating the potential of clinical hypnosis in pediatric populations. We will examine the following themes: anesthesia, acute and chronic pain, chemotherapy-related distress, along with other specific medical issues...
August 2013: Acta Bio-medica: Atenei Parmensis
Joanna J Moser, Pamela M Veale, Debbie L McAllister, David P Archer
BACKGROUND: Concern has been expressed that infants and children exposed to uneventful surgery and anesthesia may incur neurological injury that becomes manifest in poor scholastic performance or future learning difficulties. A recent meta-analysis of seven clinical studies examined the relationship between learning or behavior difficulties and pediatric exposure to anesthesia/surgery and reported an odds ratio of 1.4; however, the level of association and causal factors remain unclear...
November 2013: Paediatric Anaesthesia
Chetan Pandit, Roumel Valentin, Jonathan De Lima, Paul Robinson, Dominic Fitzgerald, Peter van Asperen, David Baines, Peter Cooper, Hiran Selvadurai
INTRODUCTION: Children with cystic fibrosis (CF) receive general anesthesia (GA) for a variety of different procedures. Historical studies assessing risk of GA report a high risk of morbidity. There is a paucity of data evaluating the risk of currently available anesthetic agents. The aim of this study was to assess the effect of GA on clinical status and lung function on children with CF. METHODS: Children with CF aged 8-18 years admitted for IV antibiotic treatment for a pulmonary exacerbation were invited to participate...
February 2014: Paediatric Anaesthesia
Lauren S Mott, Karla G Graniel, Judy Park, Nicholas H de Klerk, Peter D Sly, Conor P Murray, Harm A W M Tiddens, M Stick Stephen
OBJECTIVE: The aim of this study was to determine whether assessment of early CT scan-detected bronchiectasis in young children with cystic fibrosis (CF) depends on lung volume. METHODS: This study, approved by the hospital ethics committee, included 40 young children with CF from a newborn screened population contributing paired volume-controlled inspiratory and expiratory volumetric chest CT scans acquired under general anesthesia while clinically stable. Bronchiectasis was assessed with a semiquantitative CT scan score in inspiration and expiration, and the sensitivity of the expiratory CT scan to detect bronchiectasis was compared with the inspiratory CT scan by sensitivity and intraclass correlation coefficient analysis and Bland-Altman plots...
October 2013: Chest
Stefan W Ryter, Augustine M K Choi
Carbon monoxide (CO), a low molecular weight gas, is a ubiquitous environmental product of organic combustion, which is also produced endogenously in the body, as the byproduct of heme metabolism. CO binds to hemoglobin, resulting in decreased oxygen delivery to bodily tissues at toxicological concentrations. At physiological concentrations, CO may have endogenous roles as a potential signaling mediator in vascular function and cellular homeostasis. Exhaled CO (eCO), similar to exhaled nitric oxide (eNO), has been evaluated as a candidate breath biomarker of pathophysiological states, including smoking status, and inflammatory diseases of the lung and other organs...
March 2013: Journal of Breath Research
Lauren S Mott, Judy Park, Catherine L Gangell, Nicholas H de Klerk, Peter D Sly, Conor P Murray, Stephen M Stick
OBJECTIVE: To examine the distribution of early structural lung changes in clinically stable infants and young children with cystic fibrosis using chest computed tomography (CT). STUDY DESIGN: This cross-sectional study included 62 children aged 1-6 years with volume-controlled volumetric chest CT scans performed under general anesthesia as part of an early surveillance program. Each lobe was scored for presence and extent of bronchiectasis, mucus plugging, and air trapping using a semiquantitative score...
July 2013: Journal of Pediatrics
Brandy Adams, Daniel Chiem, Cynthia Wang, Jacques Neelankavil
No abstract text is available yet for this article.
December 2013: Journal of Cardiothoracic and Vascular Anesthesia
Beatriz Lara, Inocencia Fornet, María Goya, Francisco López, José Ramón De Miguel, María Molina, Pilar Morales, Esther Quintana, Sabina Salicrú, Elena Suárez, Piedad Usetti, Felipe Zurbano
Three percent of rare diseases are pneumopathies. Improvements in survival and quality of life have led to a new situation where patients with rare respiratory diseases want to plan their reproductive lives. The intention of this review is to present the experience accumulated in the field of the reproductive health of these women. In several rare respiratory diseases, a genetic base has been identified. The combination of preimplantation genetic diagnosis, assisted reproduction and molecular biology techniques enable embryos to be studied genetically before being transplanted into the uterus...
October 2012: Archivos de Bronconeumología
Ji-Hyun Chung, Seung-Cheol Cha, Jin-Hwan Hwang, Seong Chang Woo
A 48-year-old woman with cystic fibrosis and a previous left pneumonectomy had surgery planned for single lung transplantation under general anesthesia. Due to progressive dyspnea and recurrent respiratory infection, she could not maintain her normal daily life without lung transplantation. The anesthetic management and surgical procedure was expected to be difficult because of the left mediastinal shift and an asymmetric thorax after the left pneumonectomy, but the single lung transplantation was successfully done under cardiopulmonary bypass...
May 2012: Korean Journal of Anesthesiology
Marie-Louise Felten, Mériem Sinaceur, Michèle Treilhaud, Hadrien Roze, Jean-François Mornex, Julien Pottecher, Didier Journois, Marc Fischler
OBJECTIVES: Primary graft dysfunction (PGD) occurs in 10-25% of cases and remains responsible for significant morbidity and mortality after lung transplantation. Our goal was to explore donor and recipient variables and procedure factors that could be related to early graft failure in cystic fibrosis patients receiving bilateral lung transplantation, the PGD grade being derived from the PaO(2)/FiO(2) ratio measured at the sixth post-operative hour. METHODS: Data from 122 cystic fibrosis patients having undergone lung transplantation in six transplant centres in France were retrospectively analysed...
March 2012: European Journal of Cardio-thoracic Surgery
Frank A J A Bodewes, Marjan Wouthuyzen-Bakker, Marcel J Bijvelds, Rick Havinga, Hugo R de Jonge, Henkjan J Verkade
Cystic fibrosis liver disease (CFLD) is treated with ursodeoxycholate (UDCA). Our aim was to evaluate, in cystic fibrosis transmembrane regulator knockout (Cftr(-/-)) mice and wild-type controls, whether the supposed therapeutic action of UDCA is mediated via choleretic activity or effects on bile salt metabolism. Cftr(-/-) mice and controls, under general anesthesia, were intravenously infused with tauroursodeoxycholate (TUDCA) in increasing dosage or were fed either standard or UDCA-enriched chow (0.5% wt/wt) for 3 wk...
May 1, 2012: American Journal of Physiology. Gastrointestinal and Liver Physiology
Dusica Simić, Neboj Ladjević, Miodrag Milenović, Angelina Bogićević, Veljko Strajina, Radmilo Janković
In patients with respiratory pathology changes in respiratory physiology may lead to clinical problems during the conduct of anesthesia and the perioperative period. An understanding of the disease processes that can affect the lungs and pleura allows the anesthesiologist to account for the potential complications of these conditions and manage the anesthetic accordingly. This article describes the initial evaluation of a patient with respiratory problems. A thorough medical history, physical examination and some functional tests are the keys in decision-making in preparation for anesthesia and surgery...
2011: Acta Chirurgica Iugoslavica
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