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Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that is probably due to an autoimmune mechanism with an acute presentation and a monophasic course. The management of patients with ADEM is based on supportive therapy, corticosteroids, and intravenous immunoglobulin, and in selected cases, with therapeutic plasma exchange (TPE). The aim of our study is to evaluate the efficacy of TPE, as adjuvant therapy in pediatric patients with ADEM. We retrospectively reviewed the medical records of children with the diagnosis of ADEM between 2009 and 2011 to which TPE was indicated and were admitted in the ICU of Hospital Sant Joan de Deu (Spain). The diagnosis of ADEM was made by clinical and laboratory criteria and by the presence of compatible lesions on cranio-spinal Magnetic Resonance Imaging (MRI). For signaling TPE, we followed the guidelines established by the American Association of Apheresis (ASFA) in 2010. Five cases were identified. The predominant neurological symptoms in our patients were: altered level of consciousness, seizures, motor deficits, cranial nerve disorders, and aphasia. Most important demyelinating lesions were located in cortical and subcortical white matter of the brain and highlighted brainstream. Patients performed between 4 and 5 sessions, with no reported side effects. Progressive clinical improvement was evident in all patients, with good neurosensory response to stimulation, cessation of seizures, and recovery of limb mobility. Nowadays, one patient's right paresis persists and another suffers epileptic seizures. None of the cases in our series presented new episodes of demyelination. Due to the suggested immune-mediated pathogenesis of ADEM, treatment is based on immunomodulatory agents, being glucocorticoids the most important ones. The treatment can be complemented with intravenous immunoglobulin and plasmapheresis. Available data suggests that plasma exchange is beneficial in children with ADEM who fail these treatments. The good tolerance of the procedure without adverse reactions and the progressive neurological improvement detected in the reviewed cases suggest that the use of TPE in pediatric patients is a good therapeutic option when performed in an experienced center.

Objective To describe and analyze the characteristics and the early risk factors for mortality of noninvasive ventilation (NIV) in critically ill children. Study design A multicenter, prospective, observational 2‐year study carried out with critically ill patients (1 month – 18 years of age) who needed NIV. Clinical data and NIV parameters during the first 12 h of admission were collected. A multilevel mixed‐effects logistic regression was performed to identify mortality risk factors. Results A total of 781 patients (44.2 ± 57.7 months) were studied (57.8% male). Of them, 53.7% had an underlying condition, and 47.1% needed NIV for lower airway respiratory pathologies. Bi‐level NIV was the initial support in 78.2% of the patients. Continuous positive airway pressure (CPAP) was used more in younger patients (33.7%) than in older ones (9.7%; p < .001). About 16.7% had to be intubated and 6.2% died. The risk factors for mortality were immunodeficiency (odds ratio [OR] = 11.79; 95% confidence interval [CI] = 2.95–47.13); cerebral palsy (OR = 5.86; 95% CI = 1.94–17.65); presence of apneas on admission (OR = 5.57; 95% CI = 2.13–14.58); tachypnea 6 h after NIV onset (OR = 2.59; 95% CI = 1.30–6.94); and NIV failure (OR = 6.54; 95% CI = 2.79–15.34). Conclusion NIV is used with great variability in types of support. Younger children receive CPAP more frequently than older children. Immunodeficiency, cerebral palsy, apneas on admission, tachypnea 6 h after NIV onset, and NIV failure are the early factors associated with mortality.

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