Prospective evaluation of respiratory exacerbations in children with cystic fibrosis from newborn screening to 5 years of age

Background Newborn screening allows novel treatments for cystic fibrosis (CF) to be trialled in early childhood before irreversible lung injury occurs. As respiratory exacerbations are a potential trial outcome variable, we determined their rate, duration and clinical features in preschool children with CF; and whether they were associated with growth, lung structure and function at age 5 years. Methods: Respiratory exacerbations were recorded prospectively in Australasian CF Bronchoalveolar Lavage trial subjects from enrolment after newborn screening to age 5 years, when all participants underwent clinical assessment, chest CT scans and spirometry. Results 168 children (88 boys) experienced 2080 exacerbations, at an average rate of 3.66 exacerbations per person-year; 80.1% were community managed and 19.9% required hospital admission. There was an average increase in exacerbation rate of 9% (95% CI 4% to 14%; p<0.001) per year of age. Exacerbation rate differed by site (p<0.001) and was 26% lower (95% CI 12% to 38%) in children receiving 12 months of prophylactic antibiotics. The rate of exacerbations in the first 2 years was associated with reduced forced expiratory volume in 1 s z scores. Ever having a hospitalmanaged exacerbation was associated with bronchiectasis (OR 2.67, 95% CI 1.13 to 6.31) in chest CT scans, and lower weight z scores at 5 years of age (coefficient -0.39, 95% CI -0.74 to -0.05). Conclusions Respiratory exacerbations in young children are markers for progressive CF lung disease and are potential trial outcome measures for novel treatments in this age group.

Can simple mobile phone applications provide reliable counts of respiratory rates in sick infants and children? An initial evaluation of three new applications

BACKGROUND: Respiratory rate is an important sign that is commonly either not recorded or recorded incorrectly. Mobile phone ownership is increasing even in resource-poor settings. Phone applications may improve the accuracy and ease of counting of respiratory rates. OBJECTIVES: The study assessed the reliability and initial users' impressions of four mobile phone respiratory timer approaches, compared to a 60-second count by the same participants. METHODS: Three mobile applications (applying four different counting approaches plus a standard 60-second count) were created using the Java Mobile Edition and tested on Nokia C1-01 phones. Apart from the 60-second timer application, the others included a counter based on the time for ten breaths, and three based on the time interval between breaths ('Once-per-Breath', in which the user presses for each breath and the application calculates the rate after 10 or 20 breaths, or after 60s). Nursing and physiotherapy students used the applications to count respiratory rates in a set of brief video recordings of children with different respiratory illnesses. Limits of agreement (compared to the same participant's standard 60-second count), intra-class correlation coefficients and standard errors of measurement were calculated to compare the reliability of the four approaches, and a usability questionnaire was completed by the participants. RESULTS: There was considerable variation in the counts, with large components of the variation related to the participants and the videos, as well as the methods. None of the methods was entirely reliable, with no limits of agreement better than -10 to +9 breaths/min. Some of the methods were superior to the others, with ICCs from 0.24 to 0.92. By ICC the Once-per-Breath 60-second count and the Once-per-Breath 20-breath count were the most consistent, better even than the 60-second count by the participants. The 10-breath approaches performed least well. Users' initial impressions were positive, with little difference between the applications found. CONCLUSIONS: This study provides evidence that applications running on simple phones can be used to count respiratory rates in children. The Once-per-Breath methods are the most reliable, outperforming the 60-second count. For children with raised respiratory rates the 20-breath version of the Once-per-Breath method is faster, so it is a more suitable option where health workers are under time pressure.

Cystic fibrosis in children of the eastern Arabian peninsula : a clinical, spatial and genetic study

Aim: The aim of this thesis is to describe the process by which the inherited disease, cystic fibrosis, (CF) was recognised as an important clinical entity in the United Arab Emirates (UAE) and the Sultanate of Oman (Oman). It examines the clinical presentation of the first patients and assesses their degree of severity. Further, it describes the first studies carried out to determine the underlying CF mutations associated with the disease in the UAE and Oman. An estimate is offered of the birth frequency of the condition. Overall, the cultural, geographical and historical aspect of the societies in which the disease occurs is stressed. Methods: An initial literature search was carried out using Medline of any literature pertaining to the Arab World and CF. this was read and classified into the relevance to Arabs in general, the Middle East and then specifically the Arab (Persian) Gulf societies. Thereafter, a clinic was established at Tawam Hospital, Al Ain, UAE, for children presenting With chronic respiratory disease that could serve as a national referral centre. It was run by the Author as a service of the Paediatric Department of the UAE University Medical School. I sent a letter to every Paediatrician working in the UAE informing them of our clinic and offering our services for the diagnosis and management of chronic respiratory disease in children. This was based on the author's experience as a respiratory paediatrician in Australia and New Zealand and as the Professor of Paediatrics in the UAE. No such service then existed in the UAE. Funding was sought to establish a research programme and develop a molecular genetics laboratory in the UAE Medical School. A series of successful research applications provided the grants to commence the investigations. Once a small number of children had been identified as having CF from those referred to the respiratory clinic, the initial project was to assess and report their clinical presentation. Following this an early start was made on the identification of the mutations responsible. Once these were established an attempt was made to estimate the frequency of the condition at birth. Additional clinical studies revolved around assessing the severity of the condition that was associated with the main mutations that were identified. A clinical comparison was made with those with the mutation AF508 and the other main mutation, despite the obvious limitation of small numbers then available. Radiological assessment was made to evaluate the progression of the disease. The final aspect of the study was to assess patients from Oman and compare their findings and mutations with the neighbouring UAE. Based on information gained hypotheses are proposed regarding the spread of the gene mutation by population drift. Thesis outline: A literature review is presented in the form of a critique on the disease and a resume of the relevant aspects of the genetics of CF. Additionally, facts about the two countries' geography and history are presented. Finally, knowledge about CF mutations and population origins from other areas is presented. The second main section deals with the clinical features of the disorder as it presents in the UAE. Molecular findings are then presented and details of the common mutation found in Bedouin Arabs. Hypotheses are then presented based on the information gathered. Results: CF is not a rare disease in the Arab children of the UAE and Oman. These findings refute previous reports of CF being a rare or non-existent disease in Arabs. The condition presents with a severe clinical picture, with early colonisation of the respiratory tract with staphylococcus, haemophilus and pseudomonas organisms, even with conventional CF management practices in place. The CF mutation S549R is prevalent in Arabs of Bedouin stock, while AF508 is found in those of Baluch origin. The former may be descendants of Arabs who left southern Arabia and travelled to the Trucial Coast at the time of the destruction of the great dam at Marib. The origins of this mutation may lie in the area that corresponds to the modern Republic of Yemen. The latter groups are descendants of those who came originally from Baluchistan. It is hypothesised also that the ancestral home of the AF508 mutation may be in the geographical area now known as Baluchistan, that spans three separate modern political territories. The evidence presented supports the concept that the S549R mutation may be associated with a severe, if not the severest, clinical pattern recognised. It equates with that seen with the homozygous AF508 genotype. The absence of an additional mutation in the promoter region accounts for the different clinical pattern seen in previously described patients. Conclusions: There needs to be a major awareness of the presence of CF as a severe clinical disease in the children of the Gulf States. The clinical presentation and findings support the concept of under recognition of the disease. Climatic conditions put the children at special risk of hyponatraemia and electrolyte imbalance. The absence of surviving adults with the disease suggests premature deaths have occurred, but the high fertility rates have maintained the gene pool for this recessive disorder.

The cost of seasonal respiratory illnesses in Australian children: the dominance of patient and family costs and implications for vaccine use

Respiratory viral infections are one of the next group of diseases likely to be targeted for prevention in childhood by the use of vaccines. To begin collecting necessary epidemiology and cost information about the illnesses caused by these viruses, we conducted a prospective cohort study in 118 Melbourne children between 12 and 71 months of age during winter and spring 2001. We were interested in calculating an average cost per episode of community-managed acute respiratory disease, in identifying the key cost drivers of such illness, and to identify the proportion of costs borne by the patient and family. There were 202 community-managed influenza-like illnesses identified between July and December 2001, generating 89 general practitioner visits, and 42 antibiotic prescriptions. The average cost of community-managed episodes (without hospitalisation) was $241 (95% CI $191 to $291), with the key cost drivers being carer time away from usual activities caring for the ill child (70% of costs), use of non-prescription medications (5.4%), and general practice visits (5.0%). The patient and family met 87per cent of total costs. The lowest average cost occurred in households from the highest income bracket. Acute respiratory illness managed in the community is common, with the responsibility for meeting the cost of episodes predominantly borne by the patient and family in the form of lost productivity. These findings have implications for preventive strategies in children, such as the individual use of, or implementation of public programs using, currently available vaccines against influenza and vaccines under development against other viral respiratory pathogens.

The cost of community-managed viral respiratory illnesses in a cohort of healthy preschool-aged children

Background : Acute respiratory illnesses (ARIs) during childhood are often caused by respiratory viruses, result in significant morbidity, and have associated costs for families and society. Despite their ubiquity, there is a lack of interdisciplinary epidemiologic and economic research that has collected primary impact data, particularly associated with indirect costs, from families during ARIs in children.
Methods : We conducted a 12-month cohort study in 234 preschool children with impact diary recording and PCR testing of nose-throat swabs for viruses during an ARI. We used applied values to estimate a virus-specific mean cost of ARIs.
Results : Impact diaries were available for 72% (523/725) of community-managed illnesses between January 2003 and January 2004. The mean cost of ARIs was AU$309 (95% confidence interval $263 to $354). Influenza illnesses had a mean cost of $904, compared with RSV, $304, the next most expensive single-virus illness, although confidence intervals overlapped. Mean carer time away from usual activity per day was two hours for influenza ARIs and between 30 and 45 minutes for all other ARI categories.
Conclusion : From a societal perspective, community-managed ARIs are a significant cost burden on families and society. The point estimate of the mean cost of community-managed influenza illnesses in healthy preschool aged children is three times greater than those illnesses caused by RSV and other respiratory viruses. Indirect costs, particularly carer time away from usual activity, are the key cost drivers for ARIs in children. The use of parent-collected specimens may enhance ARI surveillance and reduce any potential Hawthorne effect caused by compliance with study procedures. These findings reinforce the need for further integrated epidemiologic and economic research of ARIs in children to allow for comprehensive cost-effectiveness assessments of preventive and therapeutic options.

Fundamental movement skills in children and adolescents : review of associated health benefits

The mastery of fundamental movement skills (FMS) has been purported as contributing to children's physical, cognitive and social development and is thought to provide the foundation for an active lifestyle. Commonly developed in childhood and subsequently refined into context- and sport-specific skills, they include locomotor (e.g. running and hopping), manipulative or object control (e.g. catching and throwing) and stability (e.g. balancing and twisting) skills. The rationale for promoting the development of FMS in childhood relies on the existence of evidence on the current or future benefits associated with the acquisition of FMS proficiency. The objective of this systematic review was to examine the relationship between FMS competency and potential health benefits in children and adolescents. Benefits were defined in terms of psychological, physiological and behavioural outcomes that can impact public health. A systematic search of six electronic databases (EMBASE, OVID MEDLINE, PsycINFO, PubMed, Scopus and SportDiscus) was conducted on 22 June 2009. Included studies were cross-sectional, longitudinal or experimental studies involving healthy children or adolescents (aged 3–18 years) that quantitatively analysed the relationship between FMS competency and potential benefits. The search identified 21 articles examining the relationship between FMS competency and eight potential benefits (i.e. global self-concept, perceived physical competence, cardio-respiratory fitness [CRF], muscular fitness, weight status, flexibility, physical activity and reduced sedentary behaviour). We found strong evidence for a positive association between FMS competency and physical activity in children and adolescents. There was also a positive relationship between FMS competency and CRF and an inverse association between FMS competency and weight status. Due to an inadequate number of studies, the relationship between FMS competency and the remaining benefits was classified as uncertain. More longitudinal and intervention research examining the relationship between FMS competency and potential psychological, physiological and behavioural outcomes in children and adolescents is recommended.

Objectively measured sedentary behaviour and health and development in children and adolescents: systematic review and meta-analysis

Sedentary behaviour has emerged as a unique determinant of health in adults. Studies in children and adolescents have been less consistent. We reviewed the evidence to determine if the total volume and patterns (i.e. breaks and bouts) of objectively measured sedentary behaviour were associated with adverse health outcomes in young people, independent of moderate-intensity to vigorous-intensity physical activity. Four electronic databases (EMBASE MEDLINE, Ovid EMBASE, PubMed and Scopus) were searched (up to 12 November 2015) to retrieve studies among 2- to 18-year-olds, which used cross-sectional, longitudinal or experimental designs, and examined associations with health outcomes (adiposity, cardio-metabolic, fitness, respiratory, bone/musculoskeletal, psychosocial, cognition/academic achievement, gross motor development and other outcomes). Based on 88 eligible observational studies, level of evidence grading and quantitative meta-analyses indicated that there is limited available evidence that the total volume or patterns of sedentary behaviour are associated with health in children and adolescents when accounting for moderate-intensity to vigorous-intensity physical activity or focusing on studies with low risk of bias. Quality evidence from studies with robust designs and methods, objective measures of sitting, examining associations for various health outcomes, is needed to better understand if the overall volume or patterns of sedentary behaviour are independent determinants of health in children and adolescents.

Feasibility, design and conduct of a pragmatic randomized controlled trial to reduce overweight and obesity in children: the electronic games to aid motivation to exercise (eGAME) study

BACKGROUND: Childhood obesity has reached epidemic proportions in developed countries. Sedentary screen-based activities such as video gaming are thought to displace active behaviors and are independently associated with obesity. Active video games, where players physically interact with images onscreen, may have utility as a novel intervention to increase physical activity and improve body composition in children. The aim of the Electronic Games to Aid Motivation to Exercise (eGAME) study is to determine the effects of an active video game intervention over 6 months on: body mass index (BMI), percent body fat, waist circumference, cardio-respiratory fitness, and physical activity levels in overweight children.

METHODS/DESIGN: Three hundred and thirty participants aged 10-14 years will be randomized to receive either an active video game upgrade package or to a control group (no intervention).

DISCUSSION: An overview of the eGAME study is presented, providing an example of a large, pragmatic randomized controlled trial in a community setting. Reflection is offered on key issues encountered during the course of the study. In particular, investigation into the feasibility of the proposed intervention, as well as robust testing of proposed study procedures is a critical step prior to implementation of a large-scale trial.

Individual differences in children's suggestibility : a comparison between intellectually disabled and mainstream samples

This study examined whether age, gender, intelligence, communication ability and shyness predict intellectually disabled children’s susceptibility to an interviewer’s misleading suggestions. Further, the study examined whether the relative influence of these factors differs between intellectually disabled and mainstream samples. Participants included 75 children with mild and borderline intellectual disabilities (aged 77–158 months) and 83 mainstream children (aged 68–152 months). All children were individually administered the Yield and Shift subscales of the Gudjonsson Suggestibility Scale (Form 2) as well as standardised measures of IQ, shyness and communication ability. For the intellectually disabled children, multiple regression analyses revealed that age, IQ and communication inversely predicted Yield suggestibility, however, none of the factors predicted Shift suggestibility. For the mainstream children, age made a significant independent contribution to both Yield and Shift suggestibility, while IQ was a significant predictor of Shift suggestibility. When comparing the relative impact of these factors across the samples, age had a significantly greater impact on mainstream (compared with intellectually disabled) children’s Shift suggestibility, while IQ had a significantly greater influence on intellectually disabled (compared with mainstream) children’s Yield scores. These findings highlight the limited generalisability of previous findings involving mainstream children’s suggestibility to intellectually disabled samples.

Subjective quality of life and self-esteem in children: the role of primary and secondary control in coping with everyday stress

Two major predictors of subjective quality oflife (SQOL) in adults are known to beself-esteem and a sense of primary control.Moreover, secondary control is known to be animportant defence strategy when primary controlfails. This study aimed to determine whetherthese relationships also apply to children. Asample of 66 children aged from 5 to 12 yearswere compared on their use of primary andsecondary control and on their ratings of SQOLand self-esteem. SQOL was measured using theComprehensive Quality of Life Scale,self-esteem by using the CoopersmithSelf-Esteem Inventory, and primary andsecondary control were measured by codingchildren's responses to three short video clipsof children in stressful situations. It wasfound that younger children use more primarycontrol and less secondary control than olderchildren. However, five year olds were foundcapable of producing secondary controlstrategies. Contrary to expectation, primaryand secondary control did not predict eitherself-esteem or SQOL. However, self-esteempredicted SQOL as expected and no sexdifferences were found. These findingsemphasise important differences from the adultliterature and the reasons for this arediscussed.

Trends in children`s physical activity and weight status in high and low socio-economic status areas of Melbourne, Victoria, 1985-2001

Objective:
To examine trends in active transport to and from school, in school sport and physical education (PE), and in weight status among children from high and low socio-economic status (SES) areas in Melbourne, Victoria, between 1985 and 2001.

Methods:
Cross-sectional survey data and measured height and weight from 1985 (n=557) and 2001 (n=926) were compared for children aged between 9–13 years within high and low SES areas.

Results:
From 1985 to 2001, the frequency of walking to or from school declined (4.38±4.3 vs. 3.61 ± 3.8 trips/wk, p<0.001), cycling to or from school also declined (1.22±2.9 vs. 0.36±1.5 trips/wk, p<0.001), and the frequency of PE lessons declined (1.64±1.1 vs. 1.18±0.9 lessons/wk, p<0.001). However, the frequency of school sport increased (0.9±1.22 vs. 1.24±0.8 sessions/wk, p<0.001). In 1985, 11.7% of children were overweight or obese compared with 28.7% in 2001 (p<0.001). Apart from walking to school and school sport, there were greater relative declines in cycling to school and PE, and increases in overweight and obesity among children attending schools in low SES areas compared with those attending schools in high SES areas.

Conclusions:
Declines in active school transport and PE have occurred at the same time as increases in overweight and obesity among Australian children.

Implications:
Promoting active school transport and maintaining school sport and PE should be important public health priorities in Australia. Current inequities in school sport and PE and in prevalence of overweight and obesity by area-level SES also need to be addressed.

A longitudinal study of body image and strategies to lose weight and increase muscles in children

A longitudinal study was used to examine age differences in the role of body mass index (BMI) and sociocultural pressures in predicting changes in body image and strategies to both lose weight and increase muscles among 443 children aged between 8 and 12 years (207 boys, 236 girls) over a 16-month period. The strongest predictors of body image and these strategies were BMI, the media and mothers, and to a lesser extent fathers and best friends. Girls were focused on losing weight, whereas boys were focused on both increasing muscle and losing weight. Surprisingly, there was a reduction in strategies both to lose weight and increase muscles as children approached adolescence. The implications of these findings for preventative educational programs for boys and girls are discussed.