Spinal muscular atrophy complicated with pulmonary infection:A clinical analysis of 36 children / 中国实用儿科杂志

ABSTRACT

OBJECTIVE: To summarize the clinical features of spinal muscular atrophy complicated with pulmonary infection in children,and to improve clinicians' understanding of the disease and improve the prognosis. METHODS: The clinical data of 36 children with SMA complicated with pulmonary infection,who were admitted to Yuying Children's Hospital of the Second Affiliated Hospital of Wenzhou Medical University from January 1,2008 to December 31,2017,were retrospectively analyzed. RESULTS: Among of the 36 patients,19 were typeⅠ,9 were typeⅡ,and 8 were typeⅢ. The common clinical manifestations and signs were fever,cough,shortness of breath,laborious breathing,three-concave signs,and crackles in the lungs. Respiratory failure occurred in 11 children,including 7 children(63.6%)with typeⅠSMA,2 children(18.2%)with typeⅡ SMA and 2 children(18.2%)with typeⅢ SMA. Imaging findings showed 5 cases of scoliosis,3 cases being typeⅡ SMA,and 2 being typeⅢ SMA. Pathogenic tests were positive in 18 children10 cases(55.5%)of type Ⅰ SMA,4 cases(22.2%)of typeⅡ SMA,4 cases(22.2%)of typeⅢ SMA;nosocomial mixed infection with conditioned pathogens was common,among which Burkholderia cepacia was the most common. Three patients died in the hospital,22 patients improved and discharged,and the remaining 11 patients gave up treatment. The number of hospitalizations,the incidence of severe pneumonia and respiratory failure was significantly different between the first 5 years(2008-2012)and the last 5 years(2013-2018)(P<0.05). CONCLUSION: SMA is verysusceptible to pulmonary infection. We should be alert to opportunistic pathogenic bacteria infection and use mechanical ventilation in time for respiratory failure patients. Active and effective respiratory care can reduce the incidence of pulmonary infection and improve the prognosis of SMA children.