No abstract

Purpose: To determine whether regional changes in lung ventilation in a group of pediatric cystic fibrosis (CF) patients following a course of chest physiotherapy could be detected with 3 He MRI. Materials and Methods:The reproducibility of lung ventilation volume measurements obtained with 3 He lung magnetic resonance imaging (MRI) was established in a group of five children with CF age 6-15 years. The same methodology was then used to evaluate whether standard chest physiotherapy (percussion and drainage) had any immediate effect on regional ventilated lung volumes in a further group of nine age-matched CF children (5-15 years).Results: Global lung ventilation volumes remained the same within the limits of sensitivity derived from the reproducibility study; however, regional lung ventilation was observed to change in most patients after therapy. Conclusion:3 He MRI can be successfully used in children with CF, and has the sensitivity to detect regional quantitative changes in lung ventilation following chest physiotherapy.

Dysfunctional breathing (DB) is an overarching term describing deviations in the normal biomechanical patterns of breathing which have a significant impact on quality of life, performance and functioning. Whilst it occurs in both children and adults, this article focuses specifically on children. DB can be viewed as having two components; breathing pattern disorder (BPD) and inducible laryngeal obstruction (ILO). They can be considered in isolation, however, are intricately related and often co-exist. When both are suspected, we propose both BPD and ILO be investigated within an all-encompassing multidisciplinary dysfunctional breathing clinic. The MDT clinic can diagnose DB through expert history taking and a choice of appropriate tests/examinations which may include spirometry, breathing pattern analysis, exercise testing and laryngoscopic examination. Use of the proposed algorithm presented in this article will aid decision making regarding choosing the most appropriate tests and understanding the diagnostic implications of these tests. The most common symptoms of DB are shortness of breath and chest discomfort, often during exercise. Patients with DB typically present with normal spirometry and an altered breathing pattern at rest which is amplified during exercise. In pediatric ILO, abnormalities of the upper airway such as cobblestoning are commonly seen followed by abnormal activity of the upper airway structures provoked by exercise. This may be associated with a varying degree of stridor. The symptoms, however, are often misdiagnosed as asthma and the picture can be further complicated by the common co-presentation of DB and asthma. Associated conditions such as asthma, extra-esophageal reflux, rhinitis, and allergy must be treated appropriately and well controlled before any directed therapy for DB can be started if therapy is to be successful. DB in pediatrics is commonly treated with a course of non-pharmaceutical therapy. The therapy is provided by an experienced physiotherapist, speech and language therapist or psychologist depending on the dominant features of the DB presentation (i.e., BPD or ILO in combination or in isolation) and some patients will benefit from input from more than one of these disciplines. An individualized treatment program based on expert assessment and personalized goals will result in a return to normal function with reoccurrence being rare.

No abstract

No abstract

Historically, thoracic kyphosis has been reported to be common amongst patients with cystic fibrosis (CF). The mechanisms leading to the development of this abnormality of the chest wall are not fully understood. In order to explore the prevalence of the condition amongst children with CF in the early twenty-first century and to explore factors that might be contributing to its development, a retrospective cross sectional study was undertaken in a regional CF unit. Data were obtained from 74 children with CF aged 8–16 years attending for their annual review. Thoracic kyphosis was measured from lateral chest X-ray using an alternative Cobb method. Lung function, disease severity, and nutritional status were also recorded. Correlations between measures were explored using a multiple linear regression model. The range of Cobb angles measured was 5.4–44.3° with thoracic kyphosis identified in only two subjects. There was no correlation between age and thoracic kyphosis, however, there was a significant correlation between lung function and thoracic kyphosis (p = 0.004). Regression coefficient (b) was −0.26 (95% CI: −0.44, −0.08). The prevalence of thoracic kyphosis is significantly less amongst children with CF than previously reported. This appears likely to be associated with the overall improvements in pulmonary status. Studies of older populations may bring further understanding of increasing thoracic kyphosis in people with CF.

Tidal breathing measurements can be used to identify changes in respiratory status. Structured light plethysmography (SLP) is a non-contact tidal breathing measurement technique. Lack of reference equations for SLP parameters makes clinical decision-making difficult. We have developed a set of growth adjusted reference equations for seven clinically pertinent parameters of respiratory rate (RR), inspiratory time (Ti), expiratory time (Te), duty cycle (Ti/Total-breath-time), phase (thoraco-abdominal asynchrony or TAA), relative thoracic contribution (RTC) and IE50 (tidal inspiratory/expiratory flow at 50% volume).Reference equations were developed based on a cohort of 198 seated healthy subjects (age 2–75 years, height 82 cm–194 cm, 108 Male). We adopted the same methodological approach as the global lung function initiative (GLI) report on spirometric reference equations [1]. Five minutes of tidal breathing was recorded per subject. Parameters were summarised with their medians. The online supplement provided is an integral part of this work and a reference range calculator is also provided therein.We found predicted RR to decrease with age and height rapidly in the first 20 years and slowly thereafter. Expected Ti, Te and RTC followed the opposite trend. RTC was 6.7% higher in females. Duty cycle increased with age, peaked at 13 and decreased after. TAA was high and variable in early life and declined rapidly with age. Predicted IE50 was constant as it did not correlate with growth.These reference ranges for seven key measures ensure clinicians and researchers can identify tidal breathing patterns in disease and better understand and interpret SLP and tidal breathing data.

ObjectivesTo provide up-to-date information on the use of long-term ventilation (LTV) in the UK paediatric population and to compare the results with data collected 10 and 20 years previously.DesignA single timepoint census completed by LTV centres in the UK, carried out via an online survey.Setting and patientsAll patients attending paediatric LTV services in the UK.ResultsData were collected from 25 LTV centres in the UK. The total study population was 2383 children and young people, representing a 2.5-fold increase in the last 10 years. The median age was 9 years (range 0–20 years). Notable changes since 2008 were an increase in the proportion of children with central hypoventilation syndrome using mask ventilation, an increase in overall numbers of children with spinal muscular atrophy (SMA) type 1, chronic lung disease of prematurity and cerebral palsy being ventilated, and a 4.2-fold increase in children using LTV for airway obstruction. The use of 24-hour ventilation, negative pressure ventilation and tracheostomy as an interface had declined. 115 children had received a disease-modifying drug. The use of ataluren and Myozyme did not influence the decision to treat with LTV, but in 35% of the children with SMA type 1 treated with nusinersin, the clinician stated that the use of this drug had or may have influenced their decision to initiate LTV.ConclusionThe results support the need for national database for children and young people using LTV at home to inform future recommendations and assist in resource allocation planning.