부산대학교 도서관

Background & Objective: We present 11-year data of patients with neuromuscular disease (NMD) that were treated in the pediatric intensive care unit of a tertiary children's hospital. Methods: The data of all cases followed-up in the pediatric intensive care unit (PICU) were retrospectively analyzed. Patients were evaluated in terms of age, gender, diagnosis, PRISM, neuroanatomical localization, hospitalization time-age, cause of admission to pediatric intensive care unit, clinical course, complications and clinical discharge status. Results: A NMD was detected in 43 of the 1,411 patients admitted within the study period and accounted for approximately 3% of pediatric intensive care unit admissions. NMD consisted of genetic (n= 35, 74.8%), acquired (n=6, 13.8%) and metabolic (n=2, 4.6%) causes. The diagnoses included spinal muscular atrophy type 1 (n=12, 27.9%), Duchenne and Becker muscular dystrophy (n=7, 16.2%), congenital myopathy (n=6, 13.9%), congenital muscular dystrophy (n=5, 11.6%), Guillain-Barre syndrome and its variants (n=2, 4.6%), spinal muscular atrophy associated with respiratory distress type 1 (n=2, 2.3%), critical illness neuropathy (n=2, 4.6%), acute flaccid myelitis (n=2, 4.6%), congenital myasthenic syndrome (n=1, 2.3%), peripheral neuropathy associated with disorder of riboflavin transporter (n=1, 2.3%), juvenile amyotrophic lateral sclerosis (n=1, 2.3%), stress-induced childhood-onset neurodegeneration with ataxia and variable seizures (n=1, 2.3%), and metabolic myopathy (n=1, 2.3%). Respiratory complications (n=31, 72%) were the most common reasons of admission to the pediatric intensive care unit. Seven (16.2%) patients have the NMD diagnosis confirmed during their first admission in PICU. These included both genetic cause of NMDs (n=4) and acquired NMDs (n=3). Mortality rate was 6.9% (n=3). Forty-three patients diagnosed with NMD had 75 times PICU admissions. The disease with the highest rate of re-admission to the PICU was SMA type 1, and the most common reason for re-admission was respiratory reasons. Conclusion: Accurate diagnosis of NMD and knowledge of causes of admission to PICU is crucial for increasing awareness, sensitivity and effectiveness when treating these diseases. [ABSTRACT FROM AUTHOR]