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The heart in cystic fibrosis

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https://www.readbyqxmd.com/read/29619223/respiratory-exacerbation-in-a-young-adult-with-cystic-fibrosis-and-tricuspid-atresia
#1
Jamie Wood, Abbey Sawyer, Siobhain Mulrennan, Andrew Bullock
Tricuspid atresia (TAt) is a complex congenital heart defect (CHD) characterized by the absence of the tricuspid valve and right ventricular hypoplasia requiring surgery in childhood, the Fontan procedure. We present a case of a 21-year-old male with TAt and cystic fibrosis (CF), who underwent a Fontan procedure in childhood, presenting to an adult CF clinic with severe deterioration in his respiratory status and multi-organ dysfunction associated with CF. This report describes problems associated with the management of a CF respiratory exacerbation and extrapulmonary manifestations of CF in the unique situation of a Fontan circulation, a circulation with absence of a subpulmonary ventricle and pulsatile pulmonary arterial blood flow where maintenance of systemic cardiac output is totally dependent on good respiratory function and low pulmonary artery pressures...
July 2018: Respirology Case Reports
https://www.readbyqxmd.com/read/29597050/did-fr%C3%A3-d%C3%A3-ric-chopin-die-from-heart-failure
#2
A Perciaccante, P Charlier, C Negri, A Coralli, O Appenzeller, R Bianucci
On October 17, 1849, Poland's greatest composer, Frédéric Chopin (1810-1849) died aged 39. His cause of death remains unknown. An investigation of the documental sources was performed to reconstrcuct the medical history of the artist. Since his earliest years, his life had been dominated by poor health. Recurrent episodes of cough, fever, headaches, lymphadenopathy- a series of symptoms that may be attributed to viral respiratory infections- manifested in his teens. Later in life, he had chest pain, hemoptysis, hematemesis, neuralgia, and arthralgia...
March 26, 2018: Journal of Cardiac Failure
https://www.readbyqxmd.com/read/29557624/massive-hemorrhage-a-late-complication-of-replacement-percutaneous-endoscopic-gastrostomy-case-report
#3
Tamer Sekmenli, Metin Gündüz, Hikmet Akbulut, H Haldun Emiroglu, Mustafa Koplay, Ilhan Ciftci
Percutaneous endoscopic gastrostomy (PEG) is used as an alternative to enteral/nasoenteral feeding in situations where long-term oral feeding is ineffective or not tolerated. It is mostly preferred in patients with neurological conditions and also to support nutrition in patients with congenital heart diseases, cystic fibrosis, inflammatory bowel disease, and various oropharyngeal diseases. Although it is easily applicable compared to many invasive procedures, it has complications ranging from wound infection to death...
April 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29556698/-specific-characteristics-of-chest-x%C3%A2-ray-in-childhood-basics-for-radiologists
#4
K Schneider
The radiographic technique of pediatric chest X‑rays is substantially different from that in adults. In nearly all cases ap/pa X‑rays are sufficient and lateral radiographs are rarely needed. In the first years of life the thymus may overshadow the heart, the great vessels and the lung hila. The most important anatomical structures essential for diagnosing pathological findings of the lungs and mediastinum are the trachea with the bifurcation and the main bronchi with the adjacent great vessels. For the assessment of distended lungs and intrathoracic consolidations, fundamental knowledge of the anatomy in childhood and malformations which can involve the airways, the lungs, the heart, as well as systemic and pulmonary vessels are indispensable...
March 19, 2018: Der Radiologe
https://www.readbyqxmd.com/read/29444099/airway-ciliary-dysfunction-and-respiratory-symptoms-in-patients-with-transposition-of-the-great-arteries
#5
Maliha Zahid, Abha Bais, Xin Tian, William Devine, Dong Ming Lee, Cyrus Yau, Daniel Sonnenberg, Lee Beerman, Omar Khalifa, Cecilia W Lo
BACKGROUND: Our prior work on congenital heart disease (CHD) with heterotaxy, a birth defect involving randomized left-right patterning, has shown an association of a high prevalence of airway ciliary dysfunction (CD; 18/43 or 42%) with increased respiratory symptoms. Furthermore, heterotaxy patients with ciliary dysfunction were shown to have more postsurgical pulmonary morbidities. These findings are likely a reflection of the common role of motile cilia in both airway clearance and left-right patterning...
2018: PloS One
https://www.readbyqxmd.com/read/29411252/the-fertility-management-experiences-of-australian-women-with-a-non-communicable-chronic-disease-findings-from-the-understanding-fertility-management-in-contemporary-australia-survey
#6
Sara Holton, Aberaami Thananjeyan, Heather Rowe, Maggie Kirkman, Lynne Jordan, Kathleen McNamee, Christine Bayly, John McBain, Vikki Sinnott, Jane Fisher
Introduction Despite the considerable and increasing proportion of women of reproductive age with a chronic non-communicable disease (NCD) and the potential adverse implications of many NCDs for childbearing, little is known about the fertility management experiences of women with an NCD, including their contraceptive use, pregnancy experiences and outcomes, and reproductive health care utilisation. The aim of this study was to investigate the fertility management experiences of women with an NCD and draw comparisons with women without an NCD...
February 6, 2018: Maternal and Child Health Journal
https://www.readbyqxmd.com/read/29143574/investigating-the-preventive-effects-of-baicalin-and-gallocatechin-against-glyoxal-induced-cystatin-aggregation
#7
Aamir Sohail, Waseem Feeroze Bhat, Sheraz Ahmad 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 30, 2017: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/28828392/thermal-stability-as-a-determinant-of-aav-serotype-identity
#8
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
#9
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
#10
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/28540046/evaluation-of-a-novel-technique-in-airway-clearance-therapy-specific-cough-technique-sct-in-cystic-fibrosis-a-pilot-study-of-a-series-of-n-of-1-randomised-controlled-trials
#11
Sandra Gursli, Leiv Sandvik, Egil Bakkeheim, Bjørn Skrede, Britt Stuge
OBJECTIVES: The aim of this pilot study was to evaluate the efficacy, safety and participants' perception of a novel technique in airway clearance therapy - specific cough technique in cystic fibrosis. METHODS: We conducted randomised controlled individual trials (N-of-1 randomised controlled trials) in six adults. Each trial included 8 weeks of treatment with two interventions each week, one with specific cough technique and one with forced expiration technique...
2017: SAGE Open Medicine
https://www.readbyqxmd.com/read/28456611/increased-risk-of-ptld-in-lung-transplant-recipients-with-cystic-fibrosis
#12
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/28452739/-experimental-model-and-currency-of-experiment-of-distant-results-of-lead-exposition
#13
G Pataraia, T Bagashvili, G Andronikashvili, T Gurashvili, K Gogeshvili, M Avalishvili
In order to explore the distant results of exposition of little doses of lead, for the objective of the experiment model we have selected 32 mongral rats, of different age, but aged of both sex. Experimental animals were divided in two groups. During first two month from the beginning of the experiment, together with permissible food, animals were given the water, in which was open Pb(NO3)2 - to first group 1.5 mg on kg/weight and to II group 15 mg on kg/weight during the day and night. Before the beginning of the experiment, in the time of process and after it, observation was conducted, description and collection of photo-video materials about the behavior of animals, physiological parameters, possible change of weight, clear-sighted changes in appearance...
February 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28449285/research-review-childhood-chronic-physical-illness-and-adult-emotional-health-a-systematic-review-and-meta-analysis
#14
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
#15
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
#16
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/28366682/indications-for-lung-resection-surgery-and-lung-transplant-in-south-american-children-with-cystic-fibrosis
#17
REVIEW
Fabíola Villac Adde, Sílvia Vidal Campos, Ricardo Henrique de Oliveira Braga Teixeira, Joaquim Carlos Rodrigues
The current available literature evaluating lung resection surgery and lung transplantation in children with cystic fibrosis (CF) was reviewed through a PubMed search and references from selected studies were additionally included. Pulmonary resections, i.e. lobectomy, segmentectomy, and pneumonectomy, are seldom performed in CF. The main indications, in patients with a forced expiratory volume in 1second (FEV1 ) that is greater than 30% predicted, are localized bronchiectasis/atelectasis, severe hemoptysis, and bronchopleural fistula refractory to medical management...
January 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28364441/survival-after-lung-transplantation-for-cystic-fibrosis-in-sweden
#18
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
#19
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
#20
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
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