Exhaled Breath Testing – A Tool for the Clinician and Researcher
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 r equires precise analytical techniques, highly sensitive technology and often challenges the limit of detection of even the most advanced assays. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 17, 2018 Category: Respiratory Medicine Authors: Michael D. Davis, Stephen J. Fowler, Alison J. Montpetit Source Type: research

Sleep Frequently Asked Questions: Question 1: What abnormalities do babies with cleft lip and/or palate have on polysomnography?
Cleft lip and/or palate (CL/P) is one of the most common congenital malformations with an estimate birth prevalence world wide of 12.44 (95% CI 9.72-15.93) per 10, 000 births for naturally conceived singleton births; prevalence is higher in infants conceived using assisted reproductive technologies.[1] CL/P can occur in isolation or with other anomalies or chromosomal variations and are classified as non-syndromic and syndromic CL/P based on the absence or presence of other congenital anomalies;[2] approximately 2/3rd of CL/P occurs in isolation or as non-syndromic CL/P and 1/3rd as syndromic. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 17, 2018 Category: Respiratory Medicine Authors: Joanna E. MacLean Source Type: research

“To trach or not to trach, that is the question”
Progressive neuromuscular disease requires increasing degrees of respiratory support to sustain life. Each step from intermittent to continuous —and noninvasive to invasive—ventilation requires thoughtful consideration based on the goals of the patient and family, and the inherent benefits and burdens of the treatment. Tracheostomy, in particular, should not be viewed as an inevitable next step when less permanent or invasive methods pr ove insufficient. Like other modes of respiratory support, tracheostomy may represent a bridge to recovery of pulmonary function, or a stabilizing action in the hope that novel therapie...
Source: Paediatric Respiratory Reviews - May 17, 2018 Category: Respiratory Medicine Authors: Robert Macauley Tags: Mini-symposium: Ethics and Palliative Care Source Type: research

Ready, Steady, Go – Achieving successful transition in Cystic Fibrosis
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 suc cessfully 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 t...
Source: Paediatric Respiratory Reviews - May 17, 2018 Category: Respiratory Medicine Authors: Gary James Connett, Arvind Nagra Tags: The 2017 Royal Society Of Medicine Cystic Fibrosis Symposium Source Type: research

Multi-resistant Pseudomonas aeruginosa ST235 in cystic fibrosis
Chronic Pseudomonas aeruginosa infection is associated with a decline in lung function and overall poorer prognosis in the cystic fibrosis population. Molecular typing of Pseudomonas aeruginosa has identified multiple clonal strains with increased virulence and transmissibility. Pseudomonas 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. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 17, 2018 Category: Respiratory Medicine Authors: Annabelle Lee, Andrew L. Jones Tags: The 2017 Royal Society Of Medicine Cystic Fibrosis Symposium Source Type: research

Assessment of liver disease in cystic fibrosis
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. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 17, 2018 Category: Respiratory Medicine Authors: Suzanne Davison Tags: The 2017 Royal Society Of Medicine Cystic Fibrosis Symposium Source Type: research

What are the effects of inhaled corticosteroids on growth in children?
This review summarizes the current evidence regarding the effects of inhaled corticosteroids (ICS) on growth in children with asthma. The evidence from randomized trials showed a mean reduction of -0.48 cm/year (95% CI -0.65 to -0.30) in linear growth velocity and of -0.61 cm (95% CI -0.83 to -0.38) in height during a one-year treatment with ICS. Some first-generation drugs had a slightly larger suppressive effect on growth than newer drugs, with a mean reduction in linear growth velocity of -0.91, -0.59, -0.08 and -0.39 cm/year for beclomethasone, budesonide, ciclesonide and fluticasone, respectively. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 17, 2018 Category: Respiratory Medicine Authors: Paulo Camargos, Linjie Zhang, Laura Lasmar, Paul Brand Tags: Asthma Frequently Asked Questions Source Type: research

Exercise Testing in Cystic Fibrosis: Who and Why?
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. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 17, 2018 Category: Respiratory Medicine Authors: D.S. Urquhart, Z.L. Saynor Tags: The 2017 Royal Society Of Medicine Cystic Fibrosis Symposium Source Type: research

Risk and Benefits of Bubble Continuous Positive Airway Pressure for Neonatal and Childhood Respiratory Diseases in Low- and Middle-Income Countries
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 ar e few, pooled available trial data considered alongside studies from high─income countries suggests that bCPAP: (i) reduces mortality; (ii) reduces the need for mechanical ventilation; and (iii) prevents extubation failure. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - May 4, 2018 Category: Respiratory Medicine Authors: Osayame A. Ekhaguere, Amsa B. Mairami, Haresh Kirpalani Source Type: research

CFTR modulator therapy in patients with cystic fibrosis and an organ transplant
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. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - April 24, 2018 Category: Respiratory Medicine Authors: Ruth M. Mitchell, Andrew M. Jones, Peter J. Barry Tags: The 2017 Royal Society Of Medicine Cystic Fibrosis Symposium Source Type: research

Long-acting inhaled bronchodilators for cystic fibrosis
Cystic fibrosis (CF) is the most common life-limiting autosomal recessive inherited disease in white populations; it affects approximately 70,000 children and adults worldwide[1], with a rate of one in 2500 newborns in the UK[2]. It was once considered a childhood disease, but with improvements in diagnosis and treatments, many people with CF are living well into adulthood. In 2016, data from the UK Cystic Fibrosis National Registry reported a median predicted survival of 47 years[3]. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - April 20, 2018 Category: Respiratory Medicine Authors: Sherie Smith, Christopher T. Edwards Source Type: research

Pediatric donation after circulatory determination of death (pDCD): A narrative review
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. (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - April 13, 2018 Category: Respiratory Medicine Authors: Matthew J. Weiss, Wendy Sherry, Laura Hornby Tags: Mini-symposium: Ethics and Palliative Care Source Type: research

Persistent and progressive long-term lung disease in survivors of preterm birth
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 (Source: Paediatric Respiratory Reviews)
Source: Paediatric Respiratory Reviews - April 13, 2018 Category: Respiratory Medicine Authors: Rhea Urs, Sailesh Kotecha, Graham L. Hall, Shannon J Simpson Tags: Review Source Type: research