Prevalence of tracheobronchomalacia is higher than previously reported in children with cystic fibrosis.

BACKGROUND: Tracheobronchomalacia (TBM) is estimated to be present in 1 in 2100 children. Previous reports suggest the prevalence is higher in children with cystic fibrosis (CF). This has clinical implications with potential to influence airway clearance and lung health. AIM: To determine the prevalence and clinical associations of TBM in Western Australian children with CF. METHODS: Children with CF born between 2001 and 2016 were included. Operation reports from bronchoscopies performed until the age of 4 were retrospectively reviewed. Data were collected on the presence, persistence defined as a repeat diagnosis, and severity of TBM. Data on genotype, pancreatic status, and symptoms at CF diagnosis were extracted from the medical record. Associations between categorical variables were compared using χ2 and Fisher's exact test. RESULTS: Of 167 children (79 male), 68 (41%) were diagnosed with TBM at least once, with TBM persistent in 37 (22%) and severe in 31 (19%). TBM was significantly associated with pancreatic insufficiency (χ2 = 7.874, p < 0.05, odds ratio [OR] 3.4), delta F508 gene mutation (χ2 = 6.489, p < 0.05, OR 2.3), and a presentation of meconium ileus (χ2 = 8.615, p < 0.05, OR 5.0). Severe malacia was less likley in females (χ2 = 4.523, p < 0.05, OR 0.42) . No significant relationship was found with respiratory symptoms at the time of CF diagnosis (χ2 = 0.742, p = 0.39). CONCLUSIONS: TBM was common in this group of children under the age of 4 with CF. A high index of suspicion for airway malacia should be considered in children with CF, particularly those who present with meconium ileus and have gastrointestinal symptoms at diagnosis.

Factors influencing participation in home, school, and community settings by children and adolescents with neuromuscular disorders: A qualitative descriptive study.

AIM: This study explored how children and adolescents with a neuromuscular disorder (NMD) and their parents experienced barriers and enablers to the child's participation. METHOD: This was a qualitative descriptive design. Fourteen semi-structured interviews were conducted (n = 13 mothers, n = 4 fathers, n = 8 children and adolescents) including one to three family members for each interview according to their preference. Data were analysed by content analysis, using the family of Participation-Related Constructs (fPRC), to characterize the components of participation. RESULTS: Meaningful participation was illustrated in the personal categories of the fPRC including the child's sense of self, preferences, and competence to perform activities. Enablers and barriers related to adaptive equipment and activity modification, social relationships, inclusion, accessibility to venues, social attitudes, and policies. INTERPRETATION: Personal motivators are critical to understanding what participation is meaningful to children and adolescents with NMDs. Social and physical supports within the child's immediate environment as well as accessibility and advocacy more widely in the community enable participation. The fPRC is a useful tool for understanding participation in these children; it informs how to support participation and suggests domains for evaluation in future intervention studies. Advocacy for participation should consider targets in the immediate and broader environments. WHAT THIS PAPER ADDS: The family of Participation-Related Constructs classified the components of participation for children and adolescents with neuromuscular disorders. Meaningful participation involved a complex interaction between personal and environmental factors. Barriers to participation included poor accessibility, lack of equipment, and social exclusion.

Comparison of home ambulatory type 2 polysomnography with a portable monitoring device and in-laboratory type 1 polysomnography for the diagnosis of obstructive sleep apnea in children.

STUDY OBJECTIVES: To compare type 2 polysomnography (T2PSG) to the gold standard type 1 in-laboratory polysomnography (T1PSG) for diagnosing obstructive sleep apnea (OSA) in children; validate home T2PSG in children with suspected OSA. METHODS: Eighty-one participants (ages 6-18) with suspected OSA had simultaneous T1PSG and T2PSG in the sleep laboratory, 47 participants (ages 5-16) had T1PSG in the sleep laboratory and T2PSG performed at home. Sleep scientists staged and scored polysomnography data, and pediatric sleep physicians assigned a diagnosis of normal or OSA. Participant demographics, polysomnography variables, and diagnoses were compared using chi-square and Fisher's exact tests for nominal variables, t test for continuous variables and Cohen's kappa to assess concordance. RESULTS: Acceptable recordings were obtained for every home T2PSG. When T1PSG and T2PSG were simultaneous, correlation between the number of arousals, respiratory disturbance index, and sleep stages was excellent. T2PSG at home demonstrated less stage 2 sleep, more rapid eye movement sleep, and higher sleep efficiency. Comparison of home T2PSG to T1PSG for diagnosing OSA showed a false-positive rate of 6.6% and false-negative rate of 3% for those performed at home. CONCLUSIONS: T2PSG in the home is feasible with excellent concordance with T1PSG for the purposes of diagnosing OSA in children aged 5-18 years. Home T2PSG may be more representative of a "normal" night for children and could benefit those suspected of having OSA by reducing waiting times for laboratory PSG, improving access to PSG and possibly reducing costs of investigating and treating OSA. CITATION: Withers A, Maul J, Rosenheim E, O'Donnell A, Wilson A, Stick S. Comparison of home ambulatory type 2 polysomnography with a portable monitoring device and in-laboratory type 1 polysomnography for the diagnosis of obstructive sleep apnea in children. J Clin Sleep Med. 2022;18(2):393-402.

Posaconazole-induced hypertension in children with cystic fibrosis.

Posaconazole is a triazole antifungal with a broad spectrum of activity against moulds including Aspergillus spp. Emerging data suggest posaconazole may be effective in the treatment of allergic bronchopulmonary aspergillosis (ABPA) complicating cystic fibrosis (CF). Rarely, posaconazole can cause pseudohyperaldosteronism, manifesting as hypertension and electrolyte abnormalities, with a number of cases recently reported in individuals without CF. We describe two cases of children with CF who developed hypertension, likely due to pseudohyperaldosteronism, following the initiation of posaconazole for the treatment of ABPA.

Highlights from the Respiratory Failure and Mechanical Ventilation 2020 Conference.

The Respiratory Intensive Care Assembly of the European Respiratory Society organised the first Respiratory Failure and Mechanical Ventilation Conference in Berlin in February 2020. The conference covered acute and chronic respiratory failure in both adults and children. During this 3-day conference, patient selection, diagnostic strategies and treatment options were discussed by international experts. Lectures delivered during the event have been summarised by Early Career Members of the Assembly and take-home messages highlighted.

Transition for Adolescents and Young Adults With Asthma.

Asthma is a complex, heterogenous medical condition which is very common in children and adults. The transition process from pediatric to adult health care services can be a challenge for young people with chronic medical conditions. The significant changes in physical and mental health during this time, as well as the many unique developmental and psychosocial challenges that occur during adolescence can complicate and impede transition if not adequately addressed and managed. The transition period can also be a challenging time for health professionals to assess readiness for transition and manage some of the complications which are particularly common during this time, including poor adherence to therapy, smoking, drug use, and emerging mental health conditions. The natural history, presentation, symptoms, and management of asthma is often significantly different when comparing pediatric and adult practice. In addition, management in infants, toddlers, school aged children, and adolescents differs significantly, offering an additional challenge to pediatric physicians managing asthmatic children and young people. Despite these challenges, if the transition process for young people with asthma is planned and performed in a formalized manner, many of these issues can be addressed, allowing the transition to occur smoothly despite changes that may occur in medical and psychosocial domains.

Management issues for adolescents with cystic fibrosis.

The healthy adolescent will encounter major changes in biological and psychosocial domains. The adolescent period can be greatly affected by a chronic illness. Cystic fibrosis is a terminal illness that can significantly affect an adolescent's biological, mental and psychosocial health. This paper discusses general issues to consider when managing an adolescent with a chronic medical condition, and specifically how cystic fibrosis may impact upon puberty, body image, risk-taking behaviours, mental health, independence, nonadherence, reproductive health, transition, lung transplantation, and end of life care.

Air travel and the risks of hypoxia in children.

In infants and children with chronic respiratory disease, hypoxia is a potential risk of aircraft travel. Although guidelines have been published to assist clinicians in assessing an individual's fitness to fly, they are not wholly evidence based. In addition, most evidence relates to adults with chronic obstructive pulmonary disease and thus cannot be extrapolated to children and infants. This review summarises the current literature as it applies to infants and children potentially at risk during air travel. Current evidence suggests that the gold standard for assessing fitness to fly, the hypoxia flight simulation test, may not be accurate in predicting in flight hypoxia in infants and children with respiratory disease. Further research is needed to determine the best methods of assessing safety of flight in infants and children.