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Paediatric Respiratory Reviews

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https://read.qxmd.com/read/30745153/bronchopulmonary-dysplasia-rationale-for-a-pathophysiological-rather-than-treatment-based-approach-to-diagnosis
#1
REVIEW
Benjamin Stoecklin, Shannon J Simpson, J Jane Pillow
Bronchopulmonary dysplasia (BPD), also known as Chronic Lung Disease (CLD), is a chronic respiratory condition of prematurity with potential life-long consequences for respiratory well-being. BPD was first described by Northway in 1967, when the mean gestation of preterm infants with BPD was 34 weeks' postmenstrual age (PMA). Survival of preterm infants at lower gestational ages has increased steadily since 1967 associated with marked improvements in respiratory management of respiratory distress syndrome...
December 19, 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/30553655/cough-and-airway-clearance-in-duchenne-muscular-dystrophy
#2
REVIEW
F Camela, M Gallucci, G Ricci
People with Duchenne muscular dystrophy (DMD), develop a respiratory muscle weakness that results in weakened cough, airway clearance impairment and over time respiratory failure and death. Assessment of cough effectiveness through vital capacity, peak cough flow and maximal inspiratory and expiratory pressures has been used to identify the optimal timing of cough augmentation techniques initiation. The choice of therapies depends on physician knowledge, and patient/care giver abilities. The purpose of this review is to clarify mechanisms of action, benefits and disadvantages of available techniques, such as manual cough-assisting manoeuvres, glossopharyngeal breathing, air stacking by resuscitator bag or by volume-cycle ventilator, and mechanical insufflator-exsufflator...
November 24, 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/30584049/an-approach-to-the-child-with-a-wet-cough
#3
REVIEW
F J Gilchrist
When children have a wet cough, it suggests the presence of secretions in their airways. This often has an infectious aetiology which is usually a self-limiting viral infection requiring no investigation or treatment. In those with acute wet cough it is, however, important to identify features suggestive of community acquired pneumonia or an inhaled foreign body as these causes require specific management. When there is chronic wet cough, the most common diagnoses are protracted bacterial bronchitis (PBB) and bronchiectasis...
November 23, 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/30528365/question-3-can-we-diagnose-asthma-in-children-under-the-age-of-5-years
#4
REVIEW
C L Yang, J M Gaffin, D Radhakrishnan
The diagnosis of asthma in children under five years has been controversial due to changing concepts of what true asthma is in this age group. Previous diagnostic algorithms that used clinical indices to predict the persistence of asthma symptoms or phenotypes based on asthma triggers do not predict which children will benefit from asthma medication. A pragmatic approach to asthma diagnosis in this age group is based on identifying signs and symptoms of reversible airflow obstruction and documenting their response to asthma medication...
October 24, 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/30552058/early-onset-children-s-interstitial-lung-diseases-discrete-entities-or-manifestations-of-pulmonary-dysmaturity
#5
REVIEW
Andrew Bush, Matthias Griese, Elias Seidl, Eitan Kerem, Simone Reu, Andrew G Nicholson
Interstitial lung diseases in children (chILD) are rare and diverse. The current classifications include a group of early onset chILD specific to infancy, namely neuro-endocrine cell hyperplasia of infancy (NEHI), pulmonary interstitial glycogenosis (PIG) and the alveolar capillary-congenital acinar dysplasia (ACD-CAD) spectrum, as well as alveolar growth disorders. NEHI and PIG cells are seen in the normal developing foetal lung. We hypothesise that these conditions are in fact overlapping manifestations of pulmonary dysmaturity, respectively of airway, mesenchymal and vascular elements, rather than discrete clinical conditions in their own right...
October 9, 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/30413352/clinical-pulmonary-function-testing-in-duchenne-muscular-dystrophy
#6
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://read.qxmd.com/read/30366651/a-shared-vision-of-quality-of-life-partnering-in-decision-making-to-understand-families-realities
#7
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://read.qxmd.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.
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.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...
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/30414814/question-2-when-is-actigraphy-useful-for-the-diagnosis-and-treatment-of-sleep-problems
#10
REVIEW
Lisa J Meltzer
No abstract text is available yet for this article.
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/30396824/the-role-of-sleep-diagnostics-and-non-invasive-ventilation-in-children-with-spinal-muscular-atrophy
#11
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...
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/29914745/tracheal-bronchus-diagnosed-in-children-undergoing-flexible-bronchoscopy
#12
REVIEW
Estela Pérez Ruiz, Pilar Caro Aguilera, Ana Isabel Valdivielso, Sonia Sanchís Cárdenas, Yazmina Martínez García, Javier Pérez Frías
This paper describes the clinical features of paediatric patients with tracheal bronchus (TB) identified with flexible bronchoscopy (FB) in a tertiary care hospital. A retrospective review of every FB with diagnosis of TB carried out in our centre since 1990 was performed which considered specifically: age at diagnosis, gender, semiology, somatic anomalies, tracheal bronchus type, other bronchoscopic findings and clinical progress. Out of 1665 FB in 1337 patients, TB was found in 26 (1.9%). The median age was 15 months (age range 1 month-13 years), with no gender differences...
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/29793860/outdoor-air-pollution-and-cystic-fibrosis
#13
REVIEW
Rossa Brugha, Claire Edmondson, Jane C Davies
Outdoor air pollution is increasingly identified as a contributor to respiratory and cardiovascular disease. Pro-inflammatory particles and gases are inhaled deep into the lungs, and are associated with impaired lung growth and exacerbations of chronic respiratory diseases. The magnitude of these effects are of interest to patients and families, and have been assessed in studies specific to CF. Using systematic review methodology, we sought to collate these studies in order to summarise the known effects of air pollution in cystic fibrosis, and to present information on decreasing personal air pollution exposures...
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/29754778/long-acting-inhaled-bronchodilators-for-cystic-fibrosis
#14
Sherie Smith, Christopher T Edwards
No abstract text is available yet for this article.
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/29752125/persistent-and-progressive-long-term-lung-disease-in-survivors-of-preterm-birth
#15
REVIEW
Rhea Urs, Sailesh Kotecha, Graham L Hall, Shannon J Simpson
Preterm birth accounts for approximately 11% of births globally, with rates increasing across many countries. Concurrent advances in neonatal care have led to increased survival of infants of lower gestational age (GA). However, infants born <32 weeks of GA experience adverse respiratory outcomes, manifesting with increased respiratory symptoms, hospitalisation and health care utilisation into early childhood. The development of bronchopulmonary dysplasia (BPD) - the chronic lung disease of prematurity - further increases the risk of poor respiratory outcomes throughout childhood, into adolescence and adulthood...
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/29703692/new-and-developing-therapies-in-spinal-muscular-atrophy
#16
REVIEW
Didu Kariyawasam, Kate A Carey, Kristi J Jones, Michelle A Farrar
Great progress has been made in the clinical translation of several therapeutic strategies for spinal muscular atrophy (SMA), including measures to selectively address Survival Motor Neuron (SMN) protein deficiency with SMN1 gene replacement or modulation of SMN2 encoded protein levels, as well as neuroprotective approaches and supporting muscle strength and function. This review highlights these novel therapies. This is particularly vital with the advent of the first disease modifying therapy, which has brought to the fore an array of questions surrounding who, how and when to treat, and stimulated challenges in resource limited healthcare systems to streamline access for those eligible for drug therapy...
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/29627169/side-effects-of-medications-used-to-treat-childhood-interstitial-lung-disease
#17
REVIEW
Oded Breuer, André Schultz
Interstitial lung disease in children (chILD) comprises a range of different rare diseases. There is limited evidence for the treatment of chILD and no randomised clinical trials of treatment have been undertaken. Most treatments are therefore prescribed off-label based on expert opinion. The off-label nature of prescription of drugs for chILD highlights the importance of a solid understanding of the side effects to facilitate risk-benefit assessment. The European Respiratory Society chILD guidelines recommend the use of systemic glucocorticosteroids, hydroxychloroquine and azithromycin...
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/29361392/preschool-wheeze-genes-and-treatment
#18
REVIEW
Chinedu Nwokoro, Jonathan Grigg
Preschool wheeze is a common but poorly understood cause of respiratory morbidity that is both distinct from and overlaps with infantile bronchiolitis and school age asthma. Attempts at classification by epidemiology, pathophysiology, therapeutic response and clinical phenotype are imperfect and yet fundamental to both treatment choice and research design. The four main therapeutic classes for preschool wheeze, namely beta2 agonists, anticholinergics, corticosteroids and leukotriene modifiers are employed with variable and often scanty evidence base, with evidence for a genetic influence on response variations...
September 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/30553654/duchenne-muscular-dystrophy-longitudinal-pulmonary-assessment-and-management
#19
EDITORIAL
Oscar Henry Mayer
No abstract text is available yet for this article.
August 22, 2018: Paediatric Respiratory Reviews
https://read.qxmd.com/read/30201135/bronchopulmonary-dysplasia-pathophysiology-and-potential-anti-inflammatory-therapies
#20
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
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