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Paediatric Respiratory Reviews

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...
May 19, 2018: Paediatric Respiratory Reviews
Greta Di Mattia, Ambra Nicolai, Antonella Frassanito, Laura Petrarca, Raffaella Nenna, Fabio Midulla
In the last twenty years, despite high vaccination coverage, epidemics of pertussis are occurring in both developing and developed countries. Many reasons could explain the pertussis resurgence: the increasing awareness of the disease, the availability of new diagnostic tests with higher sensitivity, the emergence of new Bordetella pertussis (B. pertussis) strains different from those contained in the current vaccines, the asymptomatic transmission of B. pertussis in adolescents and adults and the shorter duration of protection given by the acellular pertussis (aP) vaccine...
May 19, 2018: Paediatric Respiratory Reviews
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...
May 19, 2018: Paediatric Respiratory Reviews
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...
May 18, 2018: Paediatric Respiratory Reviews
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...
May 18, 2018: Paediatric Respiratory Reviews
Andrew M Jones
No abstract text is available yet for this article.
May 18, 2018: Paediatric Respiratory Reviews
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...
May 18, 2018: Paediatric Respiratory Reviews
Joanna E MacLean
No abstract text is available yet for this article.
May 17, 2018: Paediatric Respiratory Reviews
Michael D Davis, Stephen J Fowler, Alison J Montpetit
Exhaled breath is a robust matrix of biomarkers divided between three fractions - gaseous breath, volatile breath, and breath condensate. Breath is collected non-invasively through bags (for gaseous breath), cold condensation chambers (breath condensate), and adsorbent traps (volatile breath). Due to the incredibly dilute nature of breath matrices, breath biomarker analysis requires precise analytical techniques, highly sensitive technology and often challenges the limit of detection of even the most advanced assays...
May 17, 2018: Paediatric Respiratory Reviews
Osayame A Ekhaguere, Amsa B Mairami, Haresh Kirpalani
Over 80% of the global burden of childhood deaths occur in Low- and Middle-Income Countries (LMIC). Of the leading causes of death, respiratory failure is common to the top three. Bubble Continuous Positive Airway Pressure (bCPAP) is a standard therapy considered safe and cost effective in high resource settings. Although high-quality trials from LMIC are few, pooled available trial data considered alongside studies from high-income countries suggest that bCPAP: (i) reduces mortality; (ii) reduces the need for mechanical ventilation; and (iii) prevents extubation failure...
May 4, 2018: Paediatric Respiratory Reviews
Sherie Smith, Christopher T Edwards
No abstract text is available yet for this article.
April 22, 2018: Paediatric Respiratory Reviews
Matthew J Weiss, Wendy Sherry, Laura Hornby
Pediatric donation after circulatory death (pDCD) is an established pathway for organ donation. It remains, however, a relatively rare event worldwide, and most clinicians outside of the pediatric intensive care unit (PICU) are unfamiliar with it. The goal of this review is to introduce the processes and concepts of pDCD. While most children die in circumstances that would not allow pDCD, many children that die after withdrawal of life sustaining therapy (WLST) may be eligible for donation of some organs. The potential benefits of this practice to patients on the wait list are well known, but donation can also be an opportunity to honor a patient's or family's desire to altruistically improve the lives of others...
April 14, 2018: Paediatric Respiratory Reviews
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...
April 13, 2018: Paediatric Respiratory Reviews
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...
April 11, 2018: Paediatric Respiratory Reviews
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.
April 5, 2018: Paediatric Respiratory Reviews
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...
April 5, 2018: Paediatric Respiratory Reviews
Katharine C Pike, Melika Akhbari, Dylan Kneale, Katherine M Harris
No abstract text is available yet for this article.
April 4, 2018: Paediatric Respiratory Reviews
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...
March 17, 2018: Paediatric Respiratory Reviews
Iolo Doull
The number of published articles on Cystic Fibrosis (CF) continues to increase year on year. The evidence base for small molecule therapies in CF has continued to expand, with evidence for lumacaftor/ivacaftor in younger patients and longer-term evidence in adults, and pivotal studies on tezacaftor/ivacaftor. There were reports on emerging CFTR mutation agnostic therapies, and new evidence for long standing therapies.
March 15, 2018: Paediatric Respiratory Reviews
Rebecca Normansell, Rachel Knightly, Stephen J Milan, Jennifer A Knopp-Sihota, Brian H Rowe, Colin Powell
No abstract text is available yet for this article.
March 2018: Paediatric Respiratory Reviews
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