Use of Positive Expiratory Pressure physiotherapy in Australian children with cystic fibrosis under five years of age

Wilson, Chris; Wright, Sheila Leddington; Wainwright, C.

doi:10.1016/s1569-1993(10)60279-2

Cited by 1 publication

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“…More broadly in Australia, it has been reported that TBM was found to be present in 12% of children with CF under the age of 5 years. 14 This was an incidental finding where the study methodology was not aimed as specifically identifying presence of TBM and may have resulted in underreporting of prevalence.…”

Section: Discussionmentioning

confidence: 99%

“…[7][8][9][10] The prevalence of TBM in children with cystic fibrosis (CF) has been reported as higher than the general paediatric population (6%-17%). [11][12][13][14] Understanding the prevalence of TBM in children with CF is important as it may impact approaches to diagnosis and management including airway clearance strategies. Perth Children's Hospital (PCH) CF center provides care for all children born with CF in Western Australia.…”

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confidence: 99%

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

Depiazzi

Bourke

Stick

et al. 2023

Pediatric Pulmonology

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BackgroundTracheobronchomalacia (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.AimTo determine the prevalence and clinical associations of TBM in Western Australian children with CF.MethodsChildren 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.ResultsOf 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).ConclusionsTBM 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.

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Section: Discussionmentioning

confidence: 99%