Archives of Disease in Childhood最新文献

Background: Research on spacers for paediatric breathing patterns is limited, especially for disposable types, which may be a practical alternative to reusable valved holding chambers (VHC) in certain clinical settings.

Methods: In vitro, the fine particle dose (FPD) of salbutamol from a pressurised metered-dose inhaler (pMDI) was tested using two paperboard spacers-DispozABLE (Diz) and LiteAire (LA)-and three reusable VHCs: AeroChamber Plus Flow-Vu (AC), EasyChamber (EC) and OptiChamber Diamond (OC). The pMDI+VHC setup was connected to a child throat model without a facemask. Salbutamol availability for inhalation was measured using a Next Generation Impactor under three paediatric breathing patterns: calm breathing (6 and 4 year olds) and obstructive breathing.

Results: Median FPD in the respirable range (1-5 µm) was significantly higher for EC compared with LA, Diz and AC. Obstructive breathing increased throat deposition for all spacers, with Diz showing the highest. LA had the lowest throat deposition in calm breathing, and EC in obstructive breathing.

Conclusion: Traditional VHCs, especially EC and OC, outperformed disposable spacers. Among disposables, the valved LA performed better than the valveless Diz and may offer a cost-effective, practical alternative to reusable spacers in specific scenarios.

Objective: To determine the prevalence of invasive bacterial infection (IBI) in a UK cohort of febrile infants aged 90 days and younger with positive urinalysis (PU) results.

Design: A planned secondary analysis of data from the Febrile Infant Diagnostic Assessment and Outcome study, a prospective multicentre observational cohort study.

Setting: 35 paediatric emergency departments and assessment units across the UK and Ireland, between 6 July 2022 and 31 August 2023.

Patients: Febrile infants aged 90 days and younger presenting to emergency care.

Main outcome measures: IBI rates, namely bacterial meningitis or bacteraemia, among febrile infants with PU results were compared with those with negative urinalysis (NU) results.

Results: 1480 of 1821 infants underwent urinalysis testing. 549 infants had PU results and 931 had NU results. 42/549 (7.7%) and 20/931 (2.2%) infants had IBI in the PU and NU groups, respectively. Of the IBI cases within the PU group, 5/549 (0.9%) were bacterial meningitis and 39/549 (7.1%) were bacteraemia, with two concomitant cases. Of the IBI cases in the NU group, there were 4/931 (0.4%) cases of bacterial meningitis and 18/931 (1.9%) cases of bacteraemia, with two concomitant cases. There were no bacterial meningitis cases in infants over 60 days of age or those with confirmed urinary tract infection (UTI).

Conclusions: The prevalence of bacteraemia was high (7.1%) among PU infants, while the prevalence of bacterial meningitis was low (0.9%), with PU or NU. These findings support existing data that older infants with suspected UTI are at low risk of bacterial meningitis.

Objective: This retrospective observational study aimed to examine the associations among genetic mutations, demographic characteristics and hearing outcomes in children diagnosed with primary ciliary dyskinesia (PCD). By identifying potential predictors of adverse auditory outcomes, we hope to inform future approaches to clinical care and intervention.

Design: A total of 84 children, aged 1-17 years with confirmed PCD, underwent audiological assessments, including age-appropriate audiometry and tympanometry. Hearing loss severity scores (HLSS) were calculated (from 1 (worse hearing) to 4 (better hearing)) using hearing threshold data and analysed alongside tympanometry findings, in relation to age, sex, ethnicity and specific genetic variants, to determine factors influencing hearing outcomes.

Results: Children with oligocilia-associated genetic mutations demonstrated significantly worse hearing thresholds (mean HLSS 2.13) compared with the other groups (mean HLSS 3.44) (p<0.001) and had a greater incidence of type B tympanograms (p<0.001). Middle ear effusions were found to improve significantly with increasing age (p<0.001). Male participants showed significantly poorer tympanometry outcomes (p=0.017). Caucasian participants were found to have better hearing thresholds (mean=3.50) versus non-Caucasian children (mean=3.25) (p=0.018).

Implications: These results highlight key clinical considerations for the management of hearing in paediatric PCD. Routine, early audiological evaluation should be standard practice. Tympanostomy tube insertion should be considered carefully, given that some children exhibit age-related improvement. Male children may warrant more intensive monitoring for middle ear pathology. Genetic profiling may offer prognostic value and support a more individualised approach to management.

Objectives: Sleep-disordered breathing is a key childhood complication in children with achondroplasia. This retrospective study aimed to document the prevalence of sleep-disordered breathing in children with achondroplasia assessed by polysomnography.

Design: The prevalence of sleep-disordered breathing assessed by polysomnography among children aged 0-18 years with achondroplasia from 2013 to 2024 at The Royal Children's Hospital, Australia, was retrospectively reviewed.

Results: The cohort included 80 children with achondroplasia (54% females, 95% confirmed molecular diagnosis) with an average number of 3.6 polysomnographies collected per child (n=288). A total of 85% (68/80) had sleep-disordered breathing and 21% reported no prior symptoms. Sleep-disordered breathing subtypes included obstructive sleep apnoea in 81% (55/68), central sleep apnoea in 3% (2/68), mixed sleep apnoea in 7% (5/68) and primary snoring in 9% (6/68). Among those with obstructive and mixed sleep apnoea, 58% (35/60) had moderate or severe obstructive sleep apnoea. In 44 children, a corresponding MRI was evaluated for foramen magnum stenosis using the Achondroplasia Foramen Magnum Score. No correlation was found with sleep-disordered breathing severity (Spearman's coefficient (ρ)=0.03). Among 27 children who received a precision therapy for achondroplasia (vosoritide, n=18, infigratinib, n=8 and recifercept, n=1), the median respiratory disturbance index/hour improved from 2.7 (25th-75th percentile, (0.9-4.8)) to 1.1 (0.3-2.6) after 1 year of treatment compared with baseline.

Conclusions: Sleep-disordered breathing was present in 85% of 80 children with achondroplasia, with 21% being asymptomatic. Respiratory parameters did not correlate with foramen magnum stenosis severity and improved after 1 year of treatment in those treated with a precision therapy.