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Paediatric Respiratory Reviews

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https://www.readbyqxmd.com/read/30413352/clinical-pulmonary-function-testing-in-duchenne-muscular-dystrophy
#1
REVIEW
Oscar Henry Mayer
In Duchenne muscular dystrophy there is a well-established progressive loss of upper and lower extremity muscle function that is evident from the earliest stages of decline and through permanent loss of function. There is not the same visible evidence of decline in respiratory muscle function decline toward respiratory failure; therefore, comprehensive pulmonary function testing provides a critical component of a comprehensive longitudinal respiratory assessment. There are multiple ways of assessing separate inspiratory and expiratory muscle function and also a summative output of each to provide broad information to help identify disease status and guide intervention as appropriate...
October 4, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30366651/a-shared-vision-of-quality-of-life-partnering-in-decision-making-to-understand-families-realities
#2
REVIEW
Andreea Gorgos, Shuvo Ghosh, Antoine Payot
Quality of life (QOL) measures are increasingly used when important prognostication and treatment decisions need to be made in the care of a critically ill child. Unfortunately, health-care professionals and families experience difficulties when attempting to accurately predict and estimate QOL for a patient. Aspects such as subjectivity, complexity and adaptation to illness play an important role in how QOL is ultimately experienced. This often leads to inaccurate estimates of QOL, when performed by individuals other than the patient, such as clinicians or family members...
September 20, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30201135/bronchopulmonary-dysplasia-pathophysiology-and-potential-anti-inflammatory-therapies
#3
REVIEW
Paris C Papagianis, J J Pillow, Timothy J Moss
Inflammation of the preterm lungs is key to the pathogenesis of bronchopulmonary dysplasia (BPD), whether it arises as a consequence of intrauterine inflammation or postnatal respiratory management. This review explores steroidal and non-steroidal therapies for reducing neonatal pulmonary inflammation, aimed at treating or preventing BPD.
July 29, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30170958/diagnosis-management-and-pathophysiology-of-central-sleep-apnea-in-children
#4
REVIEW
Anya T McLaren, Saadoun Bin-Hasan, Indra Narang
Central sleep apnea (CSA) is thought to occur in about 1-5% of healthy children. CSA occurs more commonly in children with underlying disease and the presence of CSA may influence the course of their disease. CSA can be classified based on the presence or absence of hypercapnia as well as the underlying condition it is associated with. The management of CSA needs to be tailored to the patient and may include medication, non-invasive ventilation, and surgical intervention. Screening children at high risk will allow for earlier diagnosis and timely therapeutic interventions for this population...
July 25, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30396824/the-role-of-sleep-diagnostics-and-non-invasive-ventilation-in-children-with-spinal-muscular-atrophy
#5
REVIEW
Ruth Grychtol, Francois Abel, Dominic A Fitzgerald
Spinal muscular atrophy (SMA) is a degenerative motor neurone disorder causing progressive muscular weakness. Without assisted ventilation or novel therapies, most children with SMA type 1 die before the second year of life due to respiratory failure as the respiratory muscles and bulbar function are severely affected. Active respiratory treatment (mechanically assisted cough, invasive or non-invasive ventilation) has improved survival significantly in recent decades, but often at the cost of becoming ventilator dependent...
July 24, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30414814/when-is-actigraphy-useful-for-the-diagnosis-and-treatment-of-sleep-problems
#6
REVIEW
Lisa J Meltzer
No abstract text is available yet for this article.
July 18, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30153979/sleep-disordered-breathing-in-duchenne-muscular-dystrophy
#7
REVIEW
Hemant Sawnani
Symptoms of sleep disordered breathing (SDB) in younger boys with DMD are often poorly perceived and/or articulated by the patients or their families. As a result it is the watchful eye of the care-provider that determines the need for early polysomnographic (PSG) assessments. The use of polysomnography without capnometry should be considered completely inadequate when it comes to diagnosis and management of SDB in these patients. The stabilization of gas exchange with non-invasive ventilation may be achieved by the use of pressure or volume support ventilation...
July 18, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30414816/spinal-muscular-atrophy-a-modifiable-disease-emerges
#8
EDITORIAL
Dominic A Fitzgerald, Francois Abel, Kristi J Jones, Michelle A Farrar
No abstract text is available yet for this article.
July 12, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30414815/changing-respiratory-expectations-with-the-new-disease-trajectory-of-nusinersen-treated-spinal-muscular-atrophy-sma-type-1
#9
REVIEW
Dominic A Fitzgerald, Michael Doumit, Francois Abel
Spinal muscular atrophy [SMA] is the most common genetic cause of childhood mortality, primarily from the most severe form SMA type 1. It is a severe, progressive motor neurone disease, affecting the lower brainstem nuclei and the spinal cord. There is a graded level of severity with SMA children from a practical viewpoint described as "Non-sitters", "Sitters" and less commonly, "Ambulant" correlating with SMA Type 0/Type 1, Type 2 and Type 3 respectively. Children with SMA Type 0 have a severe neonatal form whilst those with SMA Type 1 develop hypoventilation, pulmonary aspiration, recurrent lower respiratory tract infections, dysphagia and failure to thrive before usually succumbing to respiratory failure and death before the age of 2 years...
July 12, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30145147/a-systematic-cochrane-review-of-autogenic-drainage-ad-for-airway-clearance-in-cystic-fibrosis
#10
EDITORIAL
P McCormack, P Burnham, K W Southern
No abstract text is available yet for this article.
July 7, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30158080/cftr-modulator-therapy-in-patients-with-cystic-fibrosis-and-an-organ-transplant
#11
REVIEW
Ruth M Mitchell, Andrew M Jones, Peter J Barry
CFTR modulators are a class of drugs which directly target the defective CFTR protein in cystic fibrosis (CF), improving its function with resultant clinical improvements. Currently these drugs are confined to people with a limited selection of genetic mutations. New modulators are in development which will lead to the majority of patients with CF becoming eligible for treatment. CFTR modulators are currently considered contraindicated in patients with a solid organ transplant. This excludes many patients who may benefit from the multisystem effects of CFTR modulator treatment...
June 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30158079/exercise-testing-in-cystic-fibrosis-who-and-why
#12
REVIEW
D S Urquhart, Z L Saynor
Annual review exercise testing is recommended by the Cystic Fibrosis (CF) Trust. Testing to date has focused on evaluating aerobic fitness, a key prognostic indicator. Tests available range from simple field tests, to comprehensive evaluations of aerobic exercise (dys)function - cardiopulmonary exercise testing (CPET). 'Field tests', although easy to perform are limited in the information they provide,whereas CPET, the 'gold standard' measure of aerobic fitness, is recommended as the first-choice exercise test by the European CF Society Exercise Working Group...
June 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/30153896/urinary-tract-stones-in-cystic-fibrosis
#13
REVIEW
J Graham Young
Urinary tract stones are a common problem in a general population but increasingly so in cystic fibrosis (CF) patients as survival improves. Mechanisms of stone formation are discussed, particularly those unique to CF patients. Modalities of treatment and the decision making process in this choice is outlined as well as possible future preventative strategies.
June 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/29937141/sleep-frequently-asked-questions-question-1-what-abnormalities-do-babies-with-cleft-lip-and-or-palate-have-on-polysomnography
#14
REVIEW
Joanna E MacLean
No abstract text is available yet for this article.
June 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/29933897/assessment-of-liver-disease-in-cystic-fibrosis
#15
REVIEW
Suzanne Davison
Liver disease in cystic fibrosis has many causes, with biliary fibrosis due to abnormal CFTR protein predominating. Assessment requires aetiology to be defined. Biliary fibrosis may progress to cirrhosis and portal hypertension, which although initially asymptomatic, may cause varices and splenomegaly. Monitoring progression includes clinical and ultrasound assessment with endoscopic assessment of varices for those at risk. Extrapolated primarily from longitudinal assessment of viral hepatitis in adults, non-invasive elastography has a potential role...
June 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/29914748/ready-steady-go-achieving-successful-transition-in-cystic-fibrosis
#16
REVIEW
Gary James Connett, Arvind Nagra
Successful transfer to adult services is the paediatric team's anticipated endpoint for the care they provide to their patients. The preceding transition process needs to be well planned and designed to address young peoples' psycho-social, educational and vocational as well as their medical needs. Ready, Steady, Go is a generic programme that has been successfully implemented to make the transition process an integral part of the routine care of young people with cystic fibrosis. Used in combination with other initiatives, the programme helps achieve the more seamless transfer of young people better prepared to meet their ongoing needs...
June 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/29914747/royal-society-of-medicine-cystic-fibrosis-symposium-2017
#17
EDITORIAL
Andrew M Jones
No abstract text is available yet for this article.
June 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/29914746/multi-resistant-pseudomonas-aeruginosa-st235-in-cystic-fibrosis
#18
REVIEW
Annabelle C Lee, Andrew L Jones
Chronic Pseudomonas aeruginosa infection is associated with a decline in lung function and overall poorer prognosis in the cystic fibrosis population. Molecular typing of P. aeruginosa has identified multiple clonal strains with increased virulence and transmissibility. P. aeruginosa ST235 is an emerging clonal strain with multi-drug resistance and is associated with more virulent infections. Novel cephalosporins, which have recently been introduced to clinical practice, may have higher efficacy against multi-drug resistant bacteria...
June 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/29914743/the-use-of-lumacaftor-ivacaftor-to-treat-acute-deterioration-in-paediatric-cystic-fibrosis
#19
REVIEW
James A Hammond, Gary J Connett
Lumacaftor/ivacaftor is a precision medicine targeting the defective cystic fibrosis transmembrane regulator (CFTR) protein in cystic fibrosis (CF) patients homozygous for Phe508del genotype. Whilst there is evidence for efficacy in children aged 6-11 years who are stable with good lung function, there are little data about the use of this medication for children with acute deterioration in this age group. We describe the use of this drug to treat a child with an unusually severe exacerbation of CF lung disease and review the potential of lumacaftor/ivacaftor as a rescue therapy in the paediatric CF population...
June 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/29703693/hypersensitivity-reactions-to-intravenous-antibiotics-in-cystic-fibrosis
#20
REVIEW
Marie F A Wright, Andrew Bush, Siobhan B Carr
Hypersensitivity reactions to intravenous antibiotics are common in cystic fibrosis (CF). As well as causing immediate morbidity, the need for future avoidance of the causative antibiotic can have a long-term negative impact on CF management. This paper reviews the epidemiology and clinical presentation of hypersensitivity reactions in CF patients, and using an illustrative case describes a rare but severe form of delayed drug reaction for which a high index of suspicion is required.
June 2018: Paediatric Respiratory Reviews
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