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Forced oscillation technique in spinal muscular atrophy
Leanne M Gauld1, Lucy A Keeling2, Claire E Shackleton2
1Queensland Children's Medical Research Institute, The State of Queensland (Queensland Health), Herston, QLD, Australia; Royal Children's Hospital Brisbane, The State of Queensland (Queensland Health), Herston, QLD, Australia.
Insights
The forced oscillation technique (FOT) is feasible for assessing respiratory function in young children with spinal muscular atrophy (SMA). This nonvolitional method shows abnormal lung function that worsens over time, correlating with other respiratory measures.
Area of Science:
- Pediatric Pulmonology
- Neuromuscular Disorders
- Respiratory Physiology
Background:
- Spinal muscular atrophy (SMA) significantly impacts respiratory function in young children, posing assessment challenges.
- The nonvolitional forced oscillation technique (FOT) offers a potential solution for evaluating respiratory compromise in this population.
Purpose of the Study:
- To evaluate the feasibility and usefulness of the forced oscillation technique (FOT) in young children diagnosed with spinal muscular atrophy (SMA).
Main Methods:
- Recruited children with SMA under 10 years old for FOT assessments every 3 months over 12 months.
- Conducted spirometry and peak cough flow (PCF) measurements where feasible; polysomnography for type 2 SMA.
- Utilized regression analysis to correlate FOT parameters (Xrs8, Rrs8) with FVC, PCF, and apnea/hypopnea index (AHI).
Main Results:
- FOT was feasible in 12 young children with SMA; abnormal respiratory reactance (Xrs8) and resistance (Rrs8) were observed.
- Significant linear relationships were found between Xrs8 and FVC, unassisted/assisted PCF, and AHI.
- Over 12 months, both Xrs8z and Rrs8z scores demonstrated a worsening trend, indicating disease progression.
Conclusions:
- The forced oscillation technique (FOT) is a viable method for assessing respiratory status in young children with SMA.
- Abnormal FOT values, particularly Xrs8, correlate with established respiratory monitoring parameters and worsen over time.
- Further research is recommended to explore the clinical utility of FOT in managing SMA patients.
Background:
Spinal muscular atrophy (SMA) causes respiratory compromise that is difficult to assess in young children. The forced oscillation technique (FOT) is commercially available for children as young as 2 years of age and is nonvolitional. The aim of this study was to assess the usefulness of FOT in young children with SMA.
Methods:
Children with SMA aged < 10 years were recruited. FOT was performed every 3 months for 12 months (five visits). Spirometry and assisted and unassisted peak cough flow (PCF) were performed where possible. Polysomnography was performed on children with type 2 SMA. Clinical information included SMA type, chest infections, Cobb angle, medications, and mobility. Regression analysis assessed relationships between FOT and FVC, PCF, and apnea/hypopnea index (AHI). Analysis of variance sought relationships to clinical characteristics.
Results:
Twelve children (seven male) were recruited; mean age was 6.26 (± 2.59) years. Respiratory reactance at 8 Hz (Xrs8) (mean z score, +1.41; SD, 1.90; P < .03) and respiratory resistance at 8 Hz (Rrs8) (mean z score, +0.66; SD, 1.34; P = .12) were abnormal. Four children performed spirometry. Linear relationships to Xrs8 exist: FVC (R2, 0.54), unassisted PCF (R2, 0.33), assisted PCF (R2, 0.43), and AHI (R2, 0.32). Over 12 months, Xrs8z score worsened (rate of change of +1.08, P < .001) and Rrs8z score worsened (rate of change +0.51, P < .001). No relationship (P > .05) was found between clinical characteristics and FOT values.
Conclusions:
FOT is feasible in young children with SMA, with abnormal values of reactance and resistance on grouped data, worsening over 12 months. Xrs8 is related to respiratory tests used to monitor progress in SMA (FVC, PCF, AHI). Further research on the value of FOT in managing individuals is warranted.
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