Clinical characteristics of 13 children with spinal muscular atrophy combined with pneumonia / 中华实用儿科临床杂志

ABSTRACT

Objective:To investigate the clinical manifestations of hospitalized patients with spinal muscular atrophy (SMA) combined with pneumonia and compare outcomes of different plans based on the manifestations, so as to improve the diagnosis and treatment of this disease.Methods:The clinical data of 13 SMA children with pneumonia hospitalized in the Department of Respiratory in Children′s Hospital Affiliated to Capital Institute of Pediatrics from January 2015 to June 2019 were retrospectively analyzed.General information including the age of patients, the classification of their diseases, the type of respiratory failure and complications was collected.For the pneumonia, the pathogens, complications, related respiratory failure type, and the treatments for SMA children during hospitalization were documented.The pathogen of pulmonary infection and treatment were described.The mode, duration, and outcome of mechanical ventilation for the SMA children during hospitalization and after discharge from the hospital were also analyzed.Results:There were 8 SMA patients with type 1 suffering from more severe conditions, most of whom had type Ⅱ respiratory failure and pneumonia complications.The average duration of hospitalization for type 1 patients [(39.8±30.3) d] was longer than that of type 2 patients [(7.8±2.2) d]( t=2.318, P=0.041). Six SMA children underwent tracheal intubation and tracheotomy, who had multiple drug-resistant bacterial infections and needed long-term treatments with rotated multiple antibiotics.The average duration of hospitalization of these 6 children [(51.3±25.3) d] was longer than that of other 4 children with non-invasive ventilation [(7.5±2.4) d]( t=3.391, P=0.009). In contrast, 3 patients who underwent tracheal intubation achieved a stable condition (normal body temperature; percutaneous oxygen saturation condition >94% under air condition; chest radiography without inflammation; sputum suction frequency less than 4 hours) after active application of airway clearance technique.They were successfully extubated and shifted to non-invasive ventilation.Three children who got rid of mechanical ventilation in daytime still took long-term application of nighttime non-invasive ventilation after discharge since their polysomnography indicated sleeping disorders.None of those 3 patients were hospitalized again due to severe pneumonia. Conclusions:In order to avoid infection of multi-resistant bacteria, SMA children with pneumonia receiving tracheal intubation should be extubated and shift to non-invasive ventilation as soon as their condition is stable.Children who get rid of non-invasive ventilation should take polysomnography before being discharged from hospital.Long-term application of nighttime non-invasive ventilation should be taken at home if the polysomnography suggests sleeping disorders, so as to reduce the risk of re-hospitalization due to recurrent pneumonia.