Dysfunctional Breathing in Children and Adults With Asthma

Asthma occurs across the life course. Its optimal treatment includes the use of personalized management plans that recognize the importance of co-morbidities including so-called “dysfunctional breathing.” Such symptoms can arise as a result of induced laryngeal obstruction (ILO) or alterations in the mechanics of normal breathing called breathing pattern disorders. Whilst these two types of breathing abnormalities might be related, studies tend to focus on only one of them and do not consider their relationship. Evidence for these problems amongst childhood asthmatics is largely anecdotal. They seem rare in early childhood. Both types are more frequently recognized in the second decade of life and girls are affected more often. These observations tantalizingly parallel epidemiological studies characterizing the increasing prevalence and severity of asthma that also occurs amongst females after puberty. Exercise ILO is more common amongst adolescents and young adults. It should be properly delineated as it might be causally related to specific treatable factors. More severe ILO occurring at rest and breathing pattern disorders are more likely to be occurring within a psychological paradigm. Dysfunctional breathing is associated with asthma morbidity through a number of potential mechanisms. These include anxiety induced breathing pattern disorders and the enhanced perception of subsequent symptoms, cooling and drying of the airways from hyperventilation induced hyperresponsiveness and a direct effect of emotional stimuli on airways constriction via cholinergic pathways. Hyperventilation is the most common breathing pattern disorder amongst adults. Although not validated for use in asthma, the Nijmegen questionnaire has been used to characterize this problem. Studies show higher scores amongst women, those with poorly controlled asthma and those with psychiatric problems. Evidence that treatment with breathing retraining techniques is effective in a primary care population including all types of asthmatics suggests the problem might be more ubiquitous than just these high-risk groups. Future challenges include the need for studies characterizing all types of dysfunctional breathing in pediatric and adult patient cohorts and clearly defined, age appropriate, interventional studies. Clinicians caring for asthmatics in all age groups need to be aware of these co-morbidities and routinely ask about symptoms that suggest these problems

Monitoring of Collapsed Built-Up Areas with High Resolution SAR Images

A 16-year-old male with cystic fibrosis (CF) was admitted to hospital with a severe infective exacerbation. Despite standard management, including conventionally selected intravenous antibiotics for Pseudomonas aeruginosa chest physiotherapy, and institution of noninvasive ventilation (NIV) for progressive hypercapneic respiratory failure, he continued to deteriorate. Direct sputum sensitivity testing (DSST) revealed a novel combination of antibiotics that resulted in a rapid and remarkable clinical improvement. DSST is a form of "whole" sputum sensitivity testing that provides information on antibiotic synergy, and may more accurately reflect in vivo antibiotic sensitivity patterns in cystic fibrosis.</p

A public health emergency among young people.

While some countries have banned the use of e-cigarettes or vaping products altogether (eg, India), and others have strongly advised against their use (eg, Australia), in the UK, Public Health England (PHE) appears to be a lone voice in stating that vaping is 95% safer than smoking tobacco. Here we consider whether vaping can be considered safe; whether vaping is a means of smoking cessation or at least harm reduction; and the correct response to the spiralling epidemic of vaping in young people (<18 years)

Studying bacteria in respiratory specimens by using conventional and molecular microbiological approaches

<p>Abstract</p> <p>Background</p> <p>Drawing from previous studies, the traditional routine diagnostic microbiology evaluation of samples from chronic respiratory conditions may provide an incomplete picture of the bacteria present in airways disease. Here, the aim was to determine the extent to which routine diagnostic microbiology gave a different assessment of the species present in sputa when analysed by using culture-independent assessment.</p> <p>Methods</p> <p>Six different media used in routine diagnostic microbiology were inoculated with sputum from twelve patients. Bacterial growth on these plates was harvested and both RNA and DNA extracted. DNA and RNA were also extracted directly from the same sample of sputum. All nucleic acids served as templates for PCR and reverse transcriptase-PCR amplification of "broad range" bacterial 16S rRNA gene regions. The regions amplified were separated by Terminal Restriction Fragment Length Polymorphism (T-RFLP) profiling and compared to assess the degree of overlap between approaches.</p> <p>Results</p> <p>A mean of 16.3 (SD 10.0) separate T-RF band lengths in the profiles from each sputum sample by Direct Molecular Analysis, with a mean of 8.8 (SD 5.8) resolved by DNA profiling and 13.3 (SD 8.0) resolved by RNA profiling. In comparison, 8.8 (SD 4.4) T-RF bands were resolved in profiles generated by Culture-derived Molecular Analysis. There were a total of 184 instances of T-RF bands detected in the direct sputum profiles but not in the corresponding culture-derived profiles, representing 83 different T-RF band lengths. Amongst these were fifteen instances where the T-RF band represented more than 10% of the total band volume (with a mean value of 23.6%). Eight different T-RF band lengths were resolved as the dominant band in profiles generated directly from sputum. Of these, only three were detected in profiles generated from the corresponding set of cultures.</p> <p>Conclusion</p> <p>Due to their focus on isolation of a small group of recognised pathogens, the use of culture-dependent methods to analyse samples from chronic respiratory infections can provide a restricted understanding of the bacterial species present. The use of a culture-independent molecular approach here identifies that there are many bacterial species in samples from CF and COPD patients that may be clinically relevant.</p

Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis

Cystic fibrosis (CF) has entered the era of variant-specific therapy, tailored to the genetic variants in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. CFTR modulators, the first variant-specific therapy available, have transformed the management of CF.The latest standards of care from the European CF Society (2018) did not include guidance on variant-specific therapy, as CFTR modulators were becoming established as a novel therapy. We have produced interim standards to guide healthcare professionals in the provision of variant-specific therapy for people with CF.Here we provide evidence-based guidance covering the spectrum of care, established using evidence from systematic reviews and expert opinion. Statements were reviewed by key stakeholders using Delphi methodology, with agreement (≥80%) achieved for all statements after one round of consultation. Issues around accessibility are discussed and there is clear consensus that all eligible people with CF should have access to variant-specific therapy

Cystic fibrosis nutrition - Chewing the fat

Survival data for successive birth cohorts of cystic fibrosis infants born in the twentieth century have shown consistent improvements. More recent UK and US data suggest a plateau in improvements for clinically relevant outcomes. Better treatment of malnutrition has arguably been the most important advance in CF care, but despite this nearly a half of the UK CF population has a sub-optimal BMI. Nutritional decline typically occurs in late childhood and early adult life. Addressing poor adherence and more targeted multi-disciplinary interventions to prevent or reverse this pattern are key to achieving better outcomes for CF patients in the future.</p

Nutritional outcomes in cystic fibrosis - are we doing enough?

Although outcome data for individuals with cystic fibrosis (CF) have shown consistent improvements throughout the twentieth century, more recent national registry data suggests that outcomes have reached a plateau. Median values for nutritional outcomes in CF currently cluster around the fiftieth centile for the normal population. These data suggest that up to half of CF patients have sub-optimal body mass index (BMI) which might have a significant adverse impact on their respiratory status. BMI might be underestimating the extent to which more important lean body mass might also be reduced. Nutritional decline is a particular problem during adolescence and commonly persists into early adult life. Current treatment strategies to optimize nutrition include the use of high energy diets, proton pump inhibitors and optimal use of enzyme preparations including higher strength preparations to decrease pill burden. Whilst these are all of potential benefit, poor adherence to nutritional care recommendations is probably the greatest impediment to future health improvement. More effective strategies to impact on treatment adherence are needed.</p

Routine use of daily oral vitamin K to treat infants with cystic fibrosis

Vitamin K is routinely administered after birth in the UK to prevent haemorrhagic disease of the newborn. Despite this, vitamin K-deficient coagulopathy still occurs in infants with high morbidity and mortality. Up to 50% of late onset bleeding presents with intracranial haemorrhage. The risk of developing vitamin K coagulopathy is higher in infants with cystic fibrosis (CF) and those that are exclusively breast fed due to low vitamin K levels in breast milk and intestinal changes in bacterial flora.Oral vitamin K supplementation is a simple addition to routine CF treatment during infancy to prevent complications from significant coagulopathy.</p

Diffuse microcystic pancreatic enlargement in a cystic fibrosis patient causing severe gastrointestinal symptoms and successfully treated by total pancreatectomy

ExcerptThe typical morphologic changes within the pancreas of patients with cystic fibrosis are inter- and intralobular fibrosis and destruction of the pancreatic parenchyma. The small ducts become obstructed by secretions and cellular debris, leading to formation of microcysts. Eventually, the pancreas becomes shrunken and fibrosed, with fatty infiltration of the parenchyma(1-3). On rare occasions, it contains epithelia-lined macrocysts and calcification (4). The pancreatic changes in cystic fibrosis rarely cause abdominal pain except in small numbers of patients who suffer acute pancreatitis. In most of these cases, patients have sufficient pancreatic function(5,6). We report a child with pancreatic insufficiency with cystic fibrosis, in whom severe gastrointestinal symptoms developed, associated with a previously undescribed complication of the pancreas