Neuromuscular diseases in the pediatric intensive care unit: 11 years of experience from a tertiary children’s hospital

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Sarıkaya Uzan G., Edem P., Besci T., Paketçi C., Evren G., Hız A. S., ...Daha Fazla

NEUROLOGY ASIA, cilt.27, sa.2, ss.327-334, 2022 (SCI-Expanded)

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 27 Sayı: 2
  • Basım Tarihi: 2022
  • Doi Numarası: 10.54029/2022nea
  • Dergi Adı: NEUROLOGY ASIA
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, EMBASE
  • Sayfa Sayıları: ss.327-334
  • Dokuz Eylül Üniversitesi Adresli: Evet

Özet

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.