Abstract

Assessment of and intervention for sleep-disordered breathing and malnutrition are related to the prevention of recurrent respiratory tract infections (RRTIs) and acute respiratory failure (ARF) in children with spinal muscular atrophy (SMA). However, specific standards for sleep-disordered breathing and malnutrition in the prevention of RRTIs and ARF have not been clarified. The study aimed to identify the risk factors and predictive indices for RRTIs and/or ARF in children with SMA. In this retrospective study, the differences in clinical characteristics between patients with and without RRTIs and ARF were compared, and binary logistic regression analysis was carried out. The optimal cutoff points for positive predictors were obtained. SMA type 1 (odds ratio (OR) = 5.21, 95% confidence interval (CI) 1.50-18.17, p = 0.010) and the apnea-hypopnea index (AHI) (OR = 1.12, 95% CI 1.01-1.24, p = 0.026) were risk factors, while the body mass index z score (BMIz) (OR = 0.65, 95% CI 0.46-0.91, p = 0.013) and mean pulse oxygen saturation (MSpO2 ) (OR = 0.72, 95% CI 0.52-1.00, p = 0.049) were protective factors. A standard consisting of (i) MSpO2 < 96% and (ii) AHI > 10 events/h and/or BMIz < -1 predicted the occurrence of RRTIs and/or ARF in the next year with a sensitivity of 0.513 and a specificity of 0.957. SMA type 1, BMIz, AHI and MSpO2 should be used to estimate the risk of RRTI and/or ARF in children with SMA. MSpO2 < 96% combined with AHI > 10 events/h or BMIz < -1 should be used as the intervention standard.

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