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Background: We aimed to investigate the relationship between sleep quality, mood and health-related quality of life (HRQOL) in children with CF and controls., Methods: Children (7-12years) and adolescents (13-18years) with CF and controls completed sleep evaluation: overnight oximetry and 14days of actigraphy. Age-appropriate questionnaires assessed mood (Children's Depression Inventory; CDI or Beck's Depression Inventory), HRQOL (CF Questionnaire-Revised; CFQ-R or PedsQL), and sleepiness (Pediatric Daytime Sleepiness Scale)., Results: 87 CF and 55 controls recruited. Children with CF had poorer sleep quality, more sleepiness and lower mood than controls, with a negative correlation between mood score and sleep efficiency. Sleepiness score was predictive of mood score and multiple CFQ-R domains. Adolescents with CF also demonstrated poorer sleep and more sleepiness than controls, but no difference in mood. Reduced sleep quality predicted lower CFQ-R scores. No correlation between sleep, mood or HRQOL in controls., Conclusions: In children and adolescents with CF, impaired sleep quality is associated with lower mood and HRQOL in an age-specific manner. Future research will aid understanding of effective strategies for prevention and treatment of mood disorders and sleep disturbance in children with CF., (Crown Copyright © 2017. Published by Elsevier B.V. All rights reserved.)

Background: Sleep disturbance is common in children with cystic fibrosis (CF) however there are limited studies investigating the causes for poor sleep quality. In a cross sectional observational study we aimed to evaluate the clinical correlates of sleep disturbance in this population., Methods: Children with CF (7-18years) free from pulmonary exacerbation completed medical review, overnight oximetry, the OSA-18 and 14days of actigraphy recordings with a sleep diary., Results: In addition to FEV 1 <80% and low baseline SpO 2 , CF-related diabetes, PEG feeding and co-morbid behaviour disorder were associated with lower objective sleep quantity. Paternal smoking and a family member with a mood disorder were also associated with sleep disturbance. The use of electronic devices before bedtime was associated with lower sleep quantity and quality. FEV 1 , nocturnal cough, age and a behaviour disorder predicted sleep duration. FEV 1 , nocturnal cough, SpO 2 nadir and asthma predicted sleep efficiency. Conversely, sleep efficiency independently predicted FEV 1 ., Conclusions: Reduced sleep quality in children with CF is related to lung health and co-morbidities. However, family characteristics and poor sleep hygiene in the child were also associated with sleep disturbance. Optimal management of CF would seem to be the primary intervention to alleviate children's sleep disturbance, however our data raises additional targets for attempts to improve sleep., (Copyright © 2017 European Cystic Fibrosis Society. All rights reserved.)

Objective: To measure sleep patterns and quality, objectively and subjectively, in clinically stable children with cystic fibrosis (CF) and healthy control children, and to examine the relationship between sleep quality and disease severity., Study Design: Clinically stable children with CF and healthy control children (7-18 years of age) were recruited. Sleep patterns and quality were measured at home with actigraphy (14 days). Overnight peripheral capillary oxygen saturation was measured via the use of pulse oximetry. Daytime sleepiness was evaluated by the Pediatric Daytime Sleepiness Scale (PDSS) and subjective sleep quality by the Sleep Disturbance Scale for Children and Obstructive Sleep Apnea-18., Results: A total of 87 children with CF and 55 control children were recruited with no differences in age or sex. Children with CF had significantly lower total sleep time and sleep efficiency than control children due to frequent awakenings and more wake after sleep onset. In children with CF, forced expiratory volume in 1 second and overnight peripheral capillary oxygen saturation nadir correlated positively with total sleep time and sleep efficiency and negatively with frequency of awakenings and wake after sleep onset. Patients with CF had significantly greater Sleep Disturbance Scale for Children (45 vs 35; P < .001), Obstructive Sleep Apnea-18 (35 vs 24; P < .001), and PDSS scores (14 vs 11; P < .001). There was a negative correlation between PDSS and forced expiratory volume in 1 second (r = -0.23; P < .05)., Conclusions: Even in periods of clinical stability, children with CF get less sleep than their peers due to more time in wakefulness during the night rather than less time spent in bed. Objective measures of sleep disturbance and subjective daytime sleepiness were related to disease severity. In contrast, parents of children with CF report high levels of sleep disturbance unrelated to disease severity., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Allergic bronchopulmonary aspergillosis (ABPA) results from a hypersensitivity response to Aspergillus fumigatus. It is seen in a number of chronic respiratory conditions, particularly in association with cystic fibrosis (CF). Oral corticosteroids and itraconazole represent the mainstay of treatment. There is evidence for the use of pulse methylprednisolone in other respiratory conditions as well as a number of inflammatory conditions. This is the first reported use of pulse intravenous methylprednisolone in the treatment of ABPA in CF. We present the clinical course of four children with CF and severe ABPA, in whom pulse methyprednisolone was used to manage the disease because of relapses or marked side effects on high-dose oral corticosteroids. Methylprednisolone pulses achieved disease control in 3 of the 4 children. However, troublesome side effects were experienced, in some cases necessitating discontinuation of therapy. Pulse methylprednisolone may represent a treatment option for children with CF and ABPA, where ABPA fails to respond adequately to routine therapy., ((c) 2005 Wiley-Liss, Inc.)

Controversy exists over whether the lower airway inflammation that characterizes cystic fibrosis (CF) is initiated primarily by the genetic defect. To determine if inflammation precedes infection, we examined bronchoalveolar lavage (BAL) fluid cytology, cytokines (interleukin (IL)-1beta, IL-4, IL-5, IL-6, IL-8, IL-10, and tumor necrosis factor-alpha), and free neutrophil elastase activity from 70 CF (aged 1.5-71 months) children detected by newborn screening and 19 (aged 2.0-48 months) controls with chronic stridor. CF subjects were selected and categorized as pristine (13 aged /= 10(5) colony-forming units/ml of pathogenic bacteria in BAL), and uninfected (15 aged > 6 months, asymptomatic, not taking antibiotics at bronchoscopy, and free of pathogens in their BAL). To further resolve if inflammation develops without infection, inflammatory mediators in paired annual BAL samples from 38 CF subjects were measured, and results were grouped according to whether BAL showed persistence (n = 6), acquisition (n = 8), clearance (n = 13), or absence (n = 11) of infection. While pristine, uninfected, and control subjects had similar BAL profiles, infected patients showed elevated inflammatory indices, including increased IL-10 (P < 0.001). Pristine subjects had the fewest signs of inflammation. Analysis of BAL pairs found differences between the four infection groups for changes in neutrophil percentages, IL-8 (P < 0.001), and free neutrophil elastase (P = 0.009). Infection was associated with elevated inflammatory mediators in BAL fluid. In contrast, minimal or reduced signs of inflammation accompanied absence of eradication of infection from BAL fluid. We conclude that in CF, infection initiates and sustains airway inflammation., ((c) 2005 Wiley-Liss, Inc.)

Fifty five teenage girls with cystic fibrosis and their mothers were interviewed to assess the provision of sexual health information. Parents were the most common source of information for adolescents. The cystic fibrosis doctor was identified as the key resource for parents. Yet few parents had spoken to their doctor about these issues, and 96% requested more information. This information was wanted before puberty by mothers, and from puberty onwards by girls.

Cross-infection by Pseudomonas aeruginosa between unrelated patients with cystic fibrosis (CF) is believed to be uncommon. After detecting a genotypically identical strain of P. aeruginosa in five unrelated children with CF dying from severe lung disease, we determined its prevalence within a large CF clinic using pulsed-field gel electrophoresis and random amplified polymorphic DNA assays. The clinical status of P. aeruginosa-infected patients was also determined. Between September and December 1999, 152 patients, aged 3.9-20.7 years, provided sputum for culture. P. aeruginosa was detected in 118 children of mean (SD) age 13.5 (3.8) years. The genotyping techniques were concordant, showing that 65 (55%) infected patients carried an indistinguishable or closely related strain. No distinctive antibiogram or environmental reservoir was found. Patients with the clonal strain were more likely than those with unrelated isolates to have been hospitalized in the preceding 12 months for respiratory exacerbations. This study demonstrates extensive spread of a single, clonal strain of P. aeruginosa in a large pediatric CF clinic. Whether this strain is also more virulent than sporadic isolates remains to be determined. As transmissible strains could emerge elsewhere, other CF clinics may also need to consider molecular methods of surveillance for cross-infection.

Aims: To determine the relation between lower airway infection and inflammation, respiratory symptoms, and lung function in infants and young children with cystic fibrosis (CF)., Methods: A prospective study of children with CF aged younger than 3 years, diagnosed by a newborn screening programme. All were clinically stable and had testing as outpatients. Subjects underwent bronchial lavage (BL) and lung function testing by the raised volume rapid thoracoabdominal compression technique under general anaesthesia. BL fluid was cultured and analysed for neutrophil count, interleukin 8, and neutrophil elastase. Lung function was assessed by forced expiratory volume in 0.5, 0.75, and 1 second., Results: Thirty six children with CF were tested on 54 occasions. Lower airway infection shown by BL was associated with a 10% reduction in FEV(0.5) compared with subjects without infection. No relation was identified between airway inflammation and lung function. Daily moist cough within the week before testing was reported on 20/54 occasions, but in only seven (35%) was infection detected. Independent of either infection status or airway inflammation, those with daily cough had lower lung function than those without respiratory symptoms at the time of BL (mean adjusted FEV(0.5) 195 ml and 236 ml respectively)., Conclusions: In young children with CF, both respiratory symptoms and airway infection have independent, additive effects on lung function, unrelated to airway inflammation. Further studies are needed to understand the mechanisms of airway obstruction in these young patients.

Objectives: Urinary incontinence is common in healthy women and occurs with increased frequency in adult women with chronic lung disease, including cystic fibrosis (CF). This study aimed to determine the prevalence, severity, and impact on daily life of urinary incontinence in female adolescents with CF., Methods: An interviewer-administered questionnaire was conducted with females who were aged 12 years and older and attend the CF Clinic at the Royal Children's Hospital in Melbourne, Australia., Results: Fifty-five (96%) of 57 eligible adolescents (age: 12-19 years) participated. Twenty-six (47%) reported ever having been incontinent of urine; 12 (22%) reported daytime incontinence twice a month or more. Median age of onset of incontinence was 13 years (range: 7-16 years). No relationship was seen with age, lung function, body mass index, or menarchal status. Coughing and laughing were the most commonly reported precipitants (affecting 84% and 68%, respectively). Of those with incontinence once a year or more, 42% reported that it sometimes prevented them from doing effective physiotherapy. Social life was affected by incontinence in approximately one third of respondents. Nearly half (42%) of affected adolescents had told no one, and only 2 had discussed the problem with their physician., Conclusions: Urinary incontinence is common in female adolescents with CF and is not related to illness severity. Urinary incontinence has a negative impact on the performance of chest physiotherapy. Given the lack of adolescent report and knowledge of treatment availability, inquiry about symptoms of urinary incontinence should be part of the routine assessment of female adolescents with CF.

Objective: To determine the clinical consequences of acquiring Pseudomonas aeruginosa infection during early childhood in children with cystic fibrosis (CF)., Design: Prospective, observational cohort study of 56 children with CF identified by newborn screening during 1990-92. Each child underwent an annual bronchial lavage during the first 2 to 3 years of life. Clinical outcome was determined at 7 years of age., Results: P aeruginosa infection was diagnosed in 24 (43%) cohort subjects. Four children died before 7 years of age, all of whom had been infected with a multi-resistant, mucoid strain of P aeruginosa (P =.04). In survivors, P aeruginosa infection was associated with significantly increased morbidity as measured by lower National Institutes of Health scores, greater variability in lung function, increased time in the hospital, and higher rates of recombinant human deoxyribonuclease therapy (P <.01). In this young CF cohort, best forced expiratory volume in 1 second was an insensitive measure of increased morbidity., Conclusions: Acquisition of P aeruginosa was common by 7 years of age in this CF birth cohort and was associated with increased morbidity and mortality. An improved disease severity score would improve the evaluation and study of early CF lung disease.