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Sepsis remains a major cause of morbidity and mortality among children worldwide. Furthermore, refractory septic shock and multiple organ dysfunction syndrome are the most critical groups which account for a high mortality rate in pediatric sepsis, and their clinical course often deteriorates rapidly. Resuscitation based on hemodynamics can provide objective values for identifying the severity of sepsis and monitoring the treatment response. Hemodynamics in sepsis can be divided into two groups: basic and advanced hemodynamic parameters. Previous therapeutic guidance of early-goal directed therapy (EGDT), which resuscitated based on the basic hemodynamics (central venous pressure and central venous oxygen saturation (ScvO2)) has lost its advantage compared with "usual care". Optimization of advanced hemodynamics, such as cardiac output and systemic vascular resistance, has now been endorsed as better therapeutic guidance for sepsis. Despite this, there are still some important hemodynamics associated with prognosis. In this article, we summarize the common techniques for hemodynamic monitoring, list important hemodynamic parameters related to outcomes, and update evidence-based therapeutic recommendations for optimizing resuscitation in pediatric septic shock.

Background Optic nerve sheath diameter (ONSD) ultrasound is a noninvasive and repeatable tool to dynamically evaluate intracranial pressure with high diagnostic accuracy; however, data in neonates are scarce. The aim of this study was to determine the reference value of ONSD and potential influencing factors in healthy term neonates. Methods We retrospectively reviewed 250 full-term neonates who underwent cranial ultrasound as part of selective newborn screening over a 2-year period. Neonates with any of the following conditions were excluded: using mechanical ventilation, sedatives and/or vasopressors, or signs of infection which needed cerebrospinal fluid analysis and/or intracranial pathologies. Data on sex, gestational age, birth body weight, birth body height, birth head circumference, Apgar score and types of delivery were collected. The neurodevelopmental outcomes were reviewed. Results A total of 234 neonates (123 girls and 111 boys) were included. The mean ONSD value was 3.30 ± 0.27 mm in the right eye and 3.30 ± 0.23 mm in the left eye, with no significant difference between both eyes (p = 0.797). Male neonates had a larger ONSD than female neonates (3.34 ± 0.22 mm versus 3.26 ± 0.20 mm, p = 0.007), and ONSD was correlated with birth weight in the males. Otherwise, there were no statistically significant associations between ONSD and other birth characteristics in both sexes. Most (63%) cases were followed for at least 12 months, and 98% had normal neurodevelopment. Conclusion The reference value reported in this study may be used to evaluate the ONSD in healthy term neonates. Sex differences should be considered in this age group.

Background Tracheobronchomalacia (TBM) contributes to the increased morbidity and mortality observed in preterm infants. Effective strategies for the prevention of TBM are necessary to achieve better outcomes. We sought to identify risk factors associated with the development of TBM in preterm infants. Optimal cut-off values for each risk factor were also determined. Methods A total of 80 infants who were born at 36 week's gestation or earlier and underwent flexible bronchoscopy were included in our study sample. A comparison of demographic and clinical risk factors between those with TBM (n = 35, 44%) and those without TBM (n = 45, 56%) was conducted using multivariate logistic regression analysis. Receiver operating characteristic curve analysis was performed to determine the appropriate cut-off values for predicting the development of TBM. Results In the multivariate analysis, only peak inspiratory pressure (PIP) and the number of intubation days remained significantly different between infants with and without TBM. Preterm infants with TBM received higher PIP (odds ratio: [OR], 1.067; 95% confidence interval [CI], 1.010-1.128; p = .020) and were intubated for longer (odds ratio [OR], 1.019; 95% CI, 1.003-1.035; p = .016) than those without TBM. Infants who received PIP > 19.5 cmH2 O or were intubated for >79.5 days were associated with a significantly higher risk of presence of TBM. Conclusion High PIP and prolonged intubation were major risk factors for the development of TBM in premature infants. Those who require PIP > 19.5 cmH2 O or intubation >79.5 days warrant bronchoscopy examination for early diagnosis and management of TBM.

Background: Patients with epilepsy have a higher mortality rate than the general population. Up-to-date estimates of epilepsy incidence, prevalence, and medication use are critical to assist policymaking. Methods: Using the National Taiwan Insurance Research Database, the standardized incidence and prevalence of epilepsy were estimated in each calendar year from 2007 to 2015. We used the incident cases of epilepsy to analyze the change in prescribing patterns from 2007 to 2015. Joinpoint regression was used to estimate secular trends. Results: From 2007 to 2015, the age- and sex-standardized incidence decreased from 0.72 (95% confidence interval [CI] 0.70–0.73) to 0.54 (95% CI 0.53–0.55) per 1,000 person-years, giving an annual percentage change (APC) of −2.73 (p < 0.05). Among patients younger than 20 years, the incidence did not change significantly. The age- and sex-standardized prevalence decreased from 6.94 (95% CI 6.90–6.98) to 6.86 (95% CI, 6.82–6.89) per 1,000 people, giving an APC of −0.31 (p < 0.05). However, the prevalence increased in the 35- to 49- and 50- to 64-year age-groups. The most common first-line anticonvulsant was phenytoin in 2007 and valproate in 2015. The use of levetiracetam, clobazam, and valproate increased during the study period, with APCs of 25.48% (95% CI 19.97–31.24), 6.41 (3.09–9.85), and 2.83 (1.51–4.16), respectively. The use of carbamazepine, phenytoin, and topiramate decreased; the APCs were −23.86% (95% CI −25.25 to −22.44), −6.61 (−8.40 to −4.79), and −4.29% (−7.87 to −0.57), respectively. Conclusions: The overall prevalence and incidence of epilepsy decreased slightly from 2007 to 2015. The prescribed first-line anticonvulsant also changed over time.

Background The aim of this study was to examine the predictive value of amplitude-integrated electroencephalography (aEEG) on 12-month seizure outcomes of infants with neonatal hypoxic-ischemic encephalopathy (HIE) treated with therapeutic hypothermia. Methods We conducted this retrospective cohort study in a tertiary neonatal intensive care unit between May 2012 and September 2017. Neonates with HIE who received both therapeutic hypothermia (TH) and aEEG were enrolled. Results A total of 23 infants (14 boys, nine girls) with a mean gestational age of 38.9 weeks were enrolled. Fifteen (65%) infants had moderate HIE and eight (35%) had severe HIE according to modified Sarnat staging. The mean aEEG recording time was 107.5 h. Twenty (86.9%) infants had seizure activity during the first 24 h after cooling and 14 (60.8%) had seizure activity during the first 24 h after rewarming. At 12 months, five (21.7%) infants had poor seizure outcomes. Repetitive seizures or status epilepticus pattern during the first 24 h after rewarming, but not the first 24 h after cooling, were associated with the presence of epilepsy at 12 months (p = 0.037). Conclusions We identified a high incidence of electrographic seizures in infants with neonatal HIE treated with therapeutic hypothermia, and post-neonatal epilepsy in the children who survived after HIE. Repetitive seizures or status epilepticus pattern during the first 24 h after rewarming, but not in the first 24 h after cooling, were associated with the presence of epilepsy at 12 months.

Acute encephalopathy is a life-threatening disease involving acute brain dysfunction, and it is one of the most important causes of mortality and severe neurological sequelae in infants and children. Approximately 30% of cases of acute encephalopathy result in some degree of neurological sequelae. Although many strategies have been proposed, effective therapies to ameliorate the outcomes of acute encephalopathy have not yet been established. Target temperature management (TTM), previously termed therapeutic hypothermia, has been shown to be effective for various brain injuries due to multiple neuroprotective mechanisms, and it may be considered to be the cornerstone of neuroprotective strategies. Consequently, TTM is currently used in the neurocritical care of adult patients with cardiac arrest with shockable rhythm and perinatal asphyxia. In addition, increasing evidence also indicates that TTM could be useful in other acute encephalopathies, including status epilepticus, acute encephalitis/encephalopathy and traumatic brain injury. In this review, we discuss the recent practical aspects of TTM as a potential intervention for various acute encephalopathies in children.

Background: To compare the clinical characteristics and outcomes of pediatric patients with refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE) who received therapeutic hypothermia (TH) plus anticonvulsants or anticonvulsants alone. Methods: Two-medical referral centers, retrospective cohort study. Pediatric Intensive Care Unit (PICU) at Taoyuan Chang Gung Children's hospital and Kaohsiung Chang Gung Memorial Hospital. We reviewed the medical records of 23 patients with RSE/SRSE who were admitted to PICU from January 2014 to December 2017. Of these, 11 patients received TH (TH group) and 12 patients did not (control group). Results: The selective endpoints were RSE/SRSE duration, length of PICU stay, and Glasgow Outcome Scale (GOS) score. We applied TH using the Artic Sun® temperature management system (target temperature, 34–35 °C; duration, 48–72 h). Of the 11 patients who received TH, 7 had febrile infection-related epilepsy syndrome (FIRSE), one had Dravet syndrome, and three had traumatic brain injury. The TH group had significantly shortern seizure durations than did the control group (hrs; median (IQR) 24(40) vs. 96(90), p

The rapid diagnosis of increased intracranial pressure is urgently needed for therapeutic reasons in neurocritically ill children, however this can rarely be achieved without invasive procedures. Point-of-care ultrasound of the optic nerve sheath diameter has been proposed as a non-invasive and reliable means to detect increased intracranial pressure in adults. Accordingly, clinicians may be able to use this technique to initiate early treatment and monitor the effectiveness of treatment in conjunction with other clinical examination and diagnostic modalities. Two meta-analyses and a systematic review have been published on this topic in adults. However, data on the correlation between optic nerve sheath diameter and intracranial pressure in neurocritically ill children are scarce. The aim of this review was to briefly describe what is being measured with point-of-care ultrasound of the optic nerve sheath diameter, summarize the most recent findings from adult literature, and provide an update of current work in children.

Background: Vasoplegia is vascular hyporesponsiveness to vasopressors and is an important phenomenon in children with refractory septic shock. This study aimed to develop an objective formula correlated with vasoplegia and evaluate the predictive power for mortality in children with refractory septic shock. Materials and Methods: We retrospectively analyzed children with refractory septic shock admitted to a pediatric intensive care unit (PICU) and monitored their hemodynamics via a pulse index continuous cardiac output (PiCCO) system. Serial hemodynamic data including cardiac index (CI), systemic vascular resistant index (SVRI) and vasoactive-inotropic score (VIS) were recorded during the first 72 hours after PICU admission. We defined vascular reactivity index (VRI) as SVRI/VIS and analyzed the effect of VRI in predicting mortality in children with refractory septic shock. Results: Thirty-three children with refractory septic shock were enrolled. The SVRI was lower in the mortality group compared to the survival group ( P < .05). The average area under the receiver operating characteristic curve of VRI within the first 72 hours was 0.8 and the serial values of VRI were significantly lower in the mortality group during the period from 0 to 48 hours ( P < .05). However, there were no significant differences in serial CI values between the survival and mortality groups. Conclusions: Vasoactive-inotropic score may potentially be used to quantify the severity of vasoplegia based on the clinical response of vessels after resuscitation with vasopressors. Lower VRI levels may indicate a higher risk of mortality in children with septic shock.

Aim:To identify the risk factors associated with the development of tracheobronchomalacia (TBM) in preterm infants with bronchopulmonary dysplasia (BPD).Methods:This was a retrospective cohort study using chart reviews of preterm infants born at ≤ 36 week's gestation who underwent flexible fiberoptic bronchoscopy in a tertiary pediatric referral center between January 2015 and January 2020. Indications for the bronchoscopy examination included lobar atelectasis on plain chest film, persistent CO2retention, recurrent extubation failure, or abnormal breathing sounds such as wheeze or stridor. Optimal cutoff values for each risk factor were also determined.Results:Fifty-eight preterm infants with BPD were enrolled, of whom 29 (50%) had TBM. There were no significant differences in gestational age and birth weight between those with and without TBM. Significantly more of the patients with TBM had severe BPD compared to those without TBM (68.9 vs. 20.6%,p< 0.001). Clinical parameters that were significantly different between the two groups were included in multivariate analysis. Among these factors, severe BPD was the most powerful risk factor for the development of TBM (odds ratio 5.57, 95% confidence interval 1.32–23.5,p= 0.019). The areas under the receiver operating characteristic curves for peak inspiratory pressure (PIP) and the duration of intubation were 0.788 and 0.75, respectively. The best predictive cutoff values of PIP and duration of intubation for TBM were 18.5 mmHg and 82 days, respectively.Conclusion:Preterm infants with severe BPD are at high risk for the development of TBM, and the risk is even higher in those who receive a higher PIP or are intubated for longer. Bronchoscopy examinations should be considered for the early diagnosis and management of TBM in infants with these risk factors.

Percutaneous ultrasound-guided renal biopsy (PURB) is an invasive but essential procedure in establishing the histologic diagnosis of pediatric renal diseases. Large studies which describe PURB complications and its contributory risk factors are scarce in the pediatric literature. Patients who underwent real-time PURB from September 2011 to August 2017 were retrospectively reviewed. Data pertaining to clinical characteristics, histologic diagnosis and biopsy-related complications were collected. In addition, the risk factors for complications were also analyzed. Overall, 183 patients (109 females) were enrolled and 201 biopsies were obtained. The mean age was 14.4 ± 13.7 years. Over 98% of the biopsies were considered adequate in quality. The major complications were perirenal hematoma requiring blood transfusion (4 cases, 2.0%), followed by perirenal abscess (1 case, 0.5%) and arteriovenous fistula (1 case, 0.5%). All patients recovered without sequelae after treatment. Hypertension, low estimated glomerular filtration rate (eGFR) and anemia were more common in patients with complication than in those without. Further logistic regression model analysis demonstrated that eGFR

Background: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an anti-neuronal antibody-mediated inflammatory brain disease that causes severe psychiatric and neurological deficits in previously healthy patients. The aims of this study were to demonstrate the clinical characteristics of patients diagnosed with anti-NMDA receptor encephalitis and to compare the different treatment strategies among these patients. Methods: Patients presenting with newly acquired psychiatric and/or neurological deficits were studied retrospectively from 2009 to 2017. Patients with evidence of anti-NMDA receptor antibodies in serum and/or cerebrospinal fluid were enrolled. The modified Rankin scale was used to assess the initial status and outcomes of the enrolled patients. Details of the clinical presentations and results of investigations were analyzed. Results: All (n = 24) of the patients received first-line immunotherapy (steroids, and/or intravenous immunoglobulin, and/or plasma exchange), and 14 patients received second-line immunotherapy (rituximab and/or cyclophosphamide). The mean time between the first- and second-line treatment was 13 days. During the first 6 months, 20 patients (20/24, 83%) achieved a good outcome (modified Rankin Scale score ≤2) and 15 patients (15/24, 62.5%) completely recovered. Four patients (17.7%) relapsed, and three patients (12.5%) had associated tumors. Conclusion: Immunotherapy is an effective treatment for anti-NMDA receptor encephalitis. Rituximab and/or cyclophosphamide are treatment options for those who cannot tolerate or do not respond to first-line immunotherapy. Prospective studies are necessary to investigate the role of rituximab and cyclophosphamide in anti-NMDA receptor encephalitis. Key Words: cyclophosphamide, anti-NMDAR encephalitis, immunotherapy, intravenous immunoglobulin, rituximab

To compare systemic vascular resistance index (SVRI) as measured by invasive transpulmonary indicator dilution (TPID) and non-invasive Doppler-derived resistive index in septic patients.We measured the snuffbox resistive index (SBRI) in both hands of septic patients who received hemodynamic monitoring by TPID prospectively.Thirty-six patients with septic shock were enrolled (median acute physiology and chronic health evaluation II score: 23; median age: 64 years). Four SBRI values were measured in each patient, for a total of 96 patient days and 951 ultrasound measurements. The correlation coefficients between SVRI and the four SBRI values were all higher than 0.87 (p .001). A higher SVRI was associated with sharp waveforms and reversed diastolic flow. A resistive index (RI) of 0.97 was the lower limit of normal SVRI (1700 dyn*s*cmUsing ultrasound to measure RI is a noninvasive, inexpensive, reliable method to evaluate peripheral vascular resistance in septic patients, and it is highly correlated with SVRI. In addition, SBRI can be used to evaluate peripheral circulatory disturbances in septic patients.

Aim: High-flow nasal cannulas (HFNCs) show potential in the application of positive pressure, improving gas exchange, and decreasing work of breathing in patients with acute respiratory distress. The aims of this study were to elucidate the indications for HFNC therapy in children of all ages and diagnoses, and to evaluate the efficacy and risk factors for failure of HFNC therapy in children with acute respiratory distress with hypoxia in a pediatric intensive care unit.Methods: We conducted this retrospective cohort study at a tertiary pediatric intensive care unit between January 1, 2018 and December 31, 2020. All children, from 1 month to 18 years of age, with acute respiratory distress with hypoxia and HFNC therapy were eligible. The clinical data were reviewed.Results: One hundred and two children met the eligibility criteria for the study, of whom 57 (55.9%) were male, and the mean age was 7.00 ± 6.79 years. Seventy-eight (76.5%) of the children had underlying disorders. The most common indications for the use of HFNC therapy were pneumonia (40, 39.2%), sepsis-related respiratory distress (17, 16.7%), and bronchiolitis (16, 15.7%). The failure rate was 15.7% (16 of 102 children). Higher initial and maximum fraction of inspiration O2 levels and lower initial and lowest SpO2/FiO2 (S/F) ratio were early and possible signs of failure requiring escalation of respiratory support.Conclusion: In our population, we found that HFNC therapy could be initiated as the first-line therapy for various etiologies of acute respiratory distress with hypoxia in a pediatric intensive care unit and for all age groups.

Background: This study aimed to identify the risk factors and predictors of neurologic outcomes and mortality for children with physical abuse and unintentional injury admitted to intensive care unit (ICU).Methods: We retrospectively evaluated the data for children with maltreatments admitted to the pediatric, neurosurgery and trauma ICUs between 2001 and 2019. Clinical factors were analyzed and compared between the physical abuse and the unintentional injury groups, including age, gender, season of admission, identifying settings, injury severity score, etiologies, length of ICU stay, neurologic outcomes, and mortality. Neurologic assessments were conducted with the Pediatric Cerebral Performance Category scale.Results: A total of 2,481 forensic children were collected, and there were 480 victims (19.3%) admitted to the ICus, including 156 physical abused and 324 unintentional injured. The significant difference between the two groups included age, histories of prematurity, clinical outcomes, head injury, neurosurgical interventions, clinical manifestations, brain computed tomography findings, and laboratory findings (all pConclusions: In children with physical abuse and unintentional injuries on ICU admission, initial presentation with shock and spontaneous hypothermia may serve as the powerful predictors for mortality; and shock and convulsion may serve as the powerful predictors for poor neurologic outcomes. In addition, retinal hemorrhage could be a risk factor for poor neurologic outcomes in critical victims with physical abuse, and spontaneous hypothermia could be a risk factor for poor neurologic outcomes in unintentional injuries.

Background:Neonatal splenic rupture/hemorrhage (SRH), an extremely rare and potentially fatal presentation, can spontaneously resolve without surgical treatment; However, treatment approaches remain controversial. The present study aimed to describe and analyze the clinical features and therapies of neonatal SRH and therapeutic approaches.Methods:We present the cases of two patients and review another 37 cases reported in English-literature. The literature search included all articles published in PUBMED from inception between January 1968 and December 2019. Demographic data, precipitating factors, clinical characteristics including presenting symptoms and signs, presenting time, age at SRH presentation, imaging findings, as well as treatments and outcomes were analyzed.Results:In addition to the two cases treated at our hospital, 37 neonates with SRH were reported during the study period. The rate of full-term neonates was 72% (28/39). The cause was idiopathic in most cases, and congenital coagulation disorders were underlying causes in 13% (5/39) of the cases. The most common presenting symptom and sign of neonatal SRH were pallor or anemia, followed by abdominal discoloration/distension. Additionally, 18% (7/39) of the cases presented with scrotal hematoma or swelling. The age at SRH presentation ranged between 3 h and 5 days of age. Abdominal ultrasonography or computed tomography was used as the diagnostic tool. Twenty-seven cases (69%) received surgical management. The prognosis was comparable between the neonates treated with splenectomy and those treated with non-surgical approaches. The mortality rate was 18% (7/39) in the study cohort. SRH presentation at ≤12 h of age was associated with higher mortality compared to SRH presenting time at >12 h of age (odds ratio 25.0, 95% CI 2.514–248.575,p= 0.001).Conclusion:Our literature review revealed that the mortality rate of neonatal SRH was 18% and that the mortality risk was higher in neonates presenting with SRH symptoms and signs at ≤12 h of age.

Aim: To analyze the factors associated with in-hospital mortality of children with acute fulminant myocarditis on venoarterial extracorporeal membrane oxygenation (VA-ECMO). Methods: This was a retrospective cohort study using chart reviews of patients diagnosed with acute fulminant myocarditis at the pediatric intensive care unit of two tertiary medical centers between January 1, 2005 and December 31, 2017. The inclusion criteria for this study were: (1) age from 1 month to 18 years; (2) diagnosed with acute myocarditis; (3) cardiogenic shock and need vasoactive-inotropic score ≥20 within 48 h after the use of vasoactive-inotropic agents; and (4) the need for ECMO placement. Results: Thirty-three children with acute fulminant myocarditis who needed ECMO were included. Clinical parameters were retrospectively reviewed. The overall survival rate was 69.6%. Higher levels of pre-ECMO troponin-I and pre-ECMO lactate, and lower post-ECMO left ventricular ejection fraction (LVEF) were significantly associated with in-hospital mortality in univariate analysis. Only higher pre-ECMO lactate and lower post-ECMO LVEF remained as predictors for in-hospital mortality in multivariate analysis. The areas under the curve of pre-ECMO lactate and post-ECMO LVEF in predicting survival were 0.848 (95% CI, 0.697–0.999, p = 0.002) and 0.824 (95% CI, 0.704–0.996, p = 0.01), respectively. A pre-ECMO lactate level of 79.8 mg/dL and post-ECMO LVEF of 39% were appropriate cutoff points to predict mortality. Conclusion: Pre-ECMO lactate level was associated with mortality in children with acute fulminant myocarditis, with an optimal cutoff value of 79.8 mg/dL. After VA-ECMO implantation, post-ECMO LVEF was associated with mortality, with an optimal cutoff value of 39%. The use of LVADs or urgent heart transplantation should be considered if the post-ECMO LVEF does not improve.

Persistent catecholamine-resistant shock in children causes severe morbidity and mortality. We aimed to analyze the association between hemodynamics and serum lactate at different time points and 28-day mortality in children with persistent catecholamine-resistant shock. Methods. Twenty-six children with persistent catecholamine-resistant shock were enrolled, and their hemodynamics were monitored using the pulse index continuous cardiac output. Serial cardiac index (CI), systemic vascular resistant index (SVRI), and vasoactive-inotropic score (VIS) were analyzed for the first 24 hours. Associations between hemodynamics, serum lactate, and 28-day mortality were analyzed. Results. The 28-day mortality rate was 53.8%. SVRI and VIS were independent predictors of 28-day mortality. The mortality group had lower serial SVRI and higher VIS than the survival group (p<0.05). Serial SVRI had the highest area under the receiver operating characteristic curve (AUC) for predicting mortality during the first 24 hours of persistent catecholamine-resistant shock. Three important hemodynamic parameters, CI, SVRI and perfusion pressure (MAP-CVP), were significantly correlated with lactate, of which SVRI had the best correlation (r=−0.711, p<0.001). According to the AUC, SVRI was a more powerful predictor of mortality than lactate in persistent catecholamine-resistant shock. After 24 hours of treatment for persistent catecholamine-resistant shock, an SVRI lower than 1284 dyn·s·cm-5·m2 was associated with 28-day mortality. Conclusions. SVRI was an early factor associated with mortality in the pediatric patients with persistent catecholamine-resistant shock potentially and had the good correlation with serum lactate. An SVRI more than 1284 dyn·s·cm-5·m2 during the first 24 hours of persistent catecholamine-resistant shock was associated with favorable outcomes. The result should be used with caution due to the small sample size.

Acute kidney injury (AKI) is a common complication of perinatal asphyxia and is associated with poorer short-term and long-term outcomes. This retrospective study describes the incidence of AKI in asphyxiated neonates who have received therapeutic hypothermia using the proposed modified Kidney Diseases: Improving Global Outcomes (KDIGO) definition and investigates clinical markers that would allow earlier recognition of at-risk neonates. We included asphyxiated neonates who underwent therapeutic hypothermia between the period of January 2011 and May 2018 in our study. The serum creatinine levels within a week of birth were used in establishing AKI according to the modified KDIGO definition. Demographic data, resuscitation details, laboratory results and use of medications were collected and compared between the AKI and non-AKI groups to identify variables that differed significantly. A total of 66 neonates were included and 23 out of them (35%) were found to have AKI. The neonates with AKI had a lower gestational age (p = 0.006), lower hemoglobin level (p = 0.012), higher lactate level before and after therapeutic hypothermia (p = 0.013 and 0.03 respectively) and higher troponin-I level after therapeutic hypothermia (p p = 0.025). The receiver operating curve showed that troponin-I after therapeutic hypothermia had an area under curve of 0.858 at the level 0.288 ng/ml. Our study concludes that the incidence of AKI among asphyxiated newborns who received therapeutic hypothermia is 35% and an elevated troponin-I level after therapeutic hypothermia is independently associated with an increased risk of AKI in asphyxiated newborns.

Traumatic brain injury (TBI) is a leading cause of pediatric morbidity and mortality and is categorized as abusive head trauma (AHT) and accidental head injury. A retrospective chart review of 124 children aged

Objective: Valproic acid is the most high-risk teratogenic antiepileptic drug, and it may lead to fetal major congenital malformations. However, it is still used in women of childbearing age with epilepsy. The aim of this study was to report our experience of discontinuing or lowering valproic acid by adding levetiracetam, a low-risk teratogenic antiepileptic drug. Methods: We reviewed the medical records of childbearing age female patients with epilepsy who were treated with valproic acid initially and then switched to levetiracetam. The clinical profiles were recorded. The primary outcome was successful switching, which was defined as a decrease in the daily valproic acid dosage, after levetiracetam had been added. Results: Twenty-four female patients were enrolled (median age 22 years). The successful switching rate was 83.3% (20/24), and 55% (11/20) discontinued valproic acid after levetiracetam had been added. There were no significant differences between the successful and unsuccessful groups in etiology, electroencephalogram, and magnetic resonance imaging findings. Pharmacoresistant to levetiracetam was much higher in the unsuccessful group (45 vs. 100%). The median switching duration was 19.5 months in the successful group. There were improvements in metrorrhagia and alopecia in all of the patients in the successful group after valproic acid had been tapered. Conclusions: Our experience supports switching valproic acid to levetiracetam in childbearing age women with epilepsy as an effective strategy to lower the teratogenic rate and adverse effects. A long switching period was noted in this study. We suggest starting early in childbearing age women with epilepsy.

In children with rhombencephalitis, neuroimaging abnormalities have been infrequently correlated with clinical outcome. We investigated whether magnetic resonance (MR) neuroimaging studies could predict clinical outcomes and disturbance of consciousness in patients with rhombencephalitis. We retrospectively analyzed the MR studies of 19 pediatric patients with rhombencephalitis (median age: 4.2 years, range 0.5-17; sex: 32% male). Fluid-attenuated inversion recovery imaging and diffusion-weighted imaging findings were graded to create imaging scores according to the extent of imaging abnormality. Clinical outcomes in the first week and 12th month were graded by using Glasgow Outcome Scale scores (1-5) and dichotomized to unfavorable or favorable outcome. Correlations of the imaging scores with the clinical outcomes and with disturbance of consciousness were assessed by using multivariate logistic regression analysis. No significant correlation was found between fluid-attenuated inversion recovery score or diffusion-weighted imaging score ( P = .608, P = .132, respectively) and disturbance of consciousness. In the first week, the unfavorable outcome group (n = 11) had significantly higher diffusion-weighted imaging score than did the favorable outcome group (n = 8) (Mann-Whitney U test, P = .005). Multivariate logistic regression analysis showed that the diffusion-weighted imaging score (odds ratio, 18.182; 95% confidence interval: 1.36, 243.01; P = .028) was significantly associated with unfavorable outcome. In the 12th month, the fluid-attenuated inversion recovery score or diffusion-weighted imaging score ( P = .994, P = .997, respectively) were not significantly associated with unfavorable outcome. Patients with rhombencephalitis who have a higher diffusion-weighted imaging score are more likely to have an unfavorable 1-week clinical outcome.

The one month survival rate after out-of-hospital cardiac arrest (OHCA) in the paediatric population remains low. Improving survival in paediatric OHCA by enhancing the quality of cardiopulmonary resuscitation (CPR) is important. In this study, we aimed to analyse the factors associated with CPR quality by using a real-time feedback device. Participants were prospectively divided into 4 groups: paediatric research fellows, paediatric residents, medical students (clerks), and paediatric critical care nurses. Then, the participants were asked to perform 5 cycles of CPR on a paediatric simulation manikin without prompts from feedback devices, and to repeat another 5 cycles of CPR after education with the 2015 paediatric advanced life support guidelines. A total of 75 participants were evaluated. In the overall analysis, an improvement in the percentage of participants meeting the target compression rate was observed (from 49.82% to 71.23%, P P = 0.005). Among the 4 groups, the residents showed the most significant improvement in both compression rate (from 48.41% to 86.57%, P P , the real-time CPR feedback device may help clinical physicians and nurses in improving the quality of chest compression. Excessive CPR compression rate may be a major cause of inadequate CPR performance.

Aim This study investigated the efficacy and safety of perampanel (PER) adjunctive therapy in pediatric patients with epilepsy whose seizures are pharmacoresistant to existing antiepileptic drugs. Methods A clinical retrospective study was conducted from 2016 to 2017 in the pediatric neurology clinic at a tertiary children's hospital. We reviewed the data obtained from 66 children whose seizures were pharmacoresistant to more than two antiepileptic drugs, and could be followed up for a minimum of 3 months after PER adjunctive therapy initiation. The efficacy was estimated by the PER response rate at 3-, 6-, and 12-month follow-up evaluations, and adverse events were also recorded. Results The rate of seizure reduction of > 50% was 30.3%, 37.5%, and 34.7% for all seizure types at 3, 6, and 12 months, in which 7.6%, 8.9%, and 14.3% of the patients became seizure-free at these time points, respectively. No significant differences were found between enzyme-inducing and nonenzyme-inducing antiepileptic drugs in combination with PER with regard to the responder rate. Five patients with Dravet syndrome were included in the study. Four of them (80%) exhibited 50% seizure reduction at the last visit, at which point, two patients (40.0%) were seizure-free. The retention rate was 51% at 12 months. Adverse events were documented in 25 patients (35.7%) and led to PER discontinuation in eight patients (12.1%). The most common adverse events comprised irritability, skin rash, dizziness, and somnolence; however, all were transient and successfully managed after PER dose reduction or discontinuation. Conclusion The current data support the value of adjunctive PER in child and adolescent patients with pharmacoresistant epilepsy in daily clinical practice. Perampanel was efficacious and generally well-tolerated as an add-on treatment for epilepsy.

In childhood encephalitis, perfusion abnormalities have been infrequently reported to associate with clinical status. We investigated whether perfusion abnormalities correlated with seizure and clinical outcome in encephalitis. We retrospectively analyzed the MR studies of 77 pediatric patients with encephalitis. Pseudo-continuous arterial spin-labeling (ASL) imaging was performed on a 3-T scanner. The patients were divided into five groups according to ASL perfusion imaging pattern: normal perfusion (NP), focal hypoperfusion (Lf), extreme global hypoperfusion (LE), focal hyperperfusion (Hf), and extreme global hyperperfusion (HE). Clinical outcome at 3 weeks was dichotomized to unfavorable or favorable outcome according to the Glasgow outcome scale. Multivariate logistic regression was conducted to predict unfavorable outcome and presence of seizure separately, based on explanatory variables including age, sex, and ASL pattern. Twenty-seven (35%) patients were designated as in group Hf, five (7%) in group Lf, 11 (14%) in group LE, none in group HE, and 34 (44%) in group NP. Multivariate logistic regression analysis showed that ASL pattern was significantly associated with unfavorable outcome (P = 0.005) and with presence of seizure (P = 0.005). For ASL pattern, group LE was 17.31 times as likely to have an unfavorable outcome as group NP (odds ratio confidence interval [CI] 3.084, 97.105; P = 0.001). Group Hf was 6.383 times as likely to have seizure as group NP (CI 1.765, 23.083; P = 0.005). In childhood encephalitis, patients with extreme global hypoperfusion had poor neurological outcome and those with focal hypoperfusion were more likely to have seizure.

Background Perinatal stroke is a common cause of established neurological sequelae. Although several risk factors have been identified, many questions regarding causes and clinical outcomes remain unanswered. This study investigated the clinical manifestations and outcomes of perinatal stroke and identified its etiologies in Taiwan. Methods We searched the reports of head magnetic resonance imaging and computed tomography performed between January 2003 and December 2012. The medical records of enrolled infants with perinatal stroke were also reviewed. Results Thirty infants with perinatal stroke were identified; 10 infants had perinatal arterial ischemic stroke (PAIS) and 20 had perinatal hemorrhagic stroke (PHS). Neonatal seizure was the most common manifestation and presented in 40% of infants with PAIS and 50% of infants with PHS. All survivors with PAIS and 77% of the surviving infants with PHS developed neurological sequelae. Acute seizure manifestation was associated with poststroke epilepsy in infants with PHS but not in infants with PAIS (86% vs. 0%, p =0.005). PAIS was mostly caused by dysfunctional hemostasis (20%) and embolism (20%), whereas PHS was mostly attributable to birth asphyxia (30%). Conclusion Perinatal stroke is associated with high mortality and morbidity rates in infants. Clinically, it can be difficult to distinguish PAIS and PHS. One should keep a high level of suspicion, especially for PHS, if infants develop unexplained seizure, cyanosis, conscious change, anemia, and/or thrombocytopenia. A systematic diagnostic approach is helpful in identifying the etiologies of perinatal stroke.

Septic shock and cardiogenic shock are the two most common types of shock in children admitted to pediatric intensive care units (PICUs). The aim of the study was to investigate which hemodynamic variables were associated with mortality in children with shock. We retrospectively analyzed 50 children with shock (37 septic shock cases and 13 cardiogenic shock cases) in the PICU and monitored their hemodynamics using transpulmonary thermodilution from 2003 to 2016. Clinical factors were analyzed between the patients with septic and cardiogenic shock. In addition, hemodynamic parameters associated with mortality were analyzed. The 28-day mortality was significantly higher in the septic group than in the cardiogenic group (p=0.016). Initially, the parameters of cardiac output and cardiac contractility were higher in the septic group (p<0.05) while the parameters of preload and afterload were all higher in the cardiogenic group (p<0.05). Cardiac index was significantly lower in the nonsurvivors of cardiogenic shock at the time of initial admission and after the first 24 hours (bothp<0.05), while systemic vascular resistance index (SVRI) was significantly lower in the nonsurvivors of septic shock (p<0.001). Therefore, during the first 24 hours after intensive care, SVRI and cardiac index are the most important hemodynamic parameters associated with mortality.

Traumatic brain injury (TBI) is the leading cause of pediatric morbidity and mortality worldwide, and half of all fatalities occur in infants aged less than 1 year. We analyzed 129 infants diagnosed with TBI complicated with intracranial hemorrhage confirmed by brain computed tomography. We defined delta hemoglobin (ΔHB) as nadir HB - age specific mean HB, and the ratio of HB (%) as ΔHB/age specific mean HB x 100. Infants with poor neurologic outcomes had a lower admission HB and ΔHB (p +), 8.24; negative likelihood ratio (LR−), 0.34); the AUC was 0.88 in infants aged from 6–12 months and the optimal cutoff was −20.6% (sensitivity, 89%; specificity, 79%; LR+, 4.13; LR−, 0.15).

Enterovirus A71 (EV-A71) is one of the causative pathogens of hand, foot, and mouth disease (HFMD), which may cause severe neurological and cardiopulmonary complications in children. In this review, we discuss the pathogenesis, clinical manifestations, management strategy, and clinical outcomes of cardiopulmonary failure (CPF) in patients with EV-A71 infection.The pathogenesis of CPF involves both catecholamine-related cardiotoxicity following brainstem encephalitis and vasodilatory shock due to cytokine storm. Sympathetic hyperactivity, including tachycardia and hypertension, are the early clinical manifestations of cardiopulmonary involvement, which may progress to pulmonary edema/hemorrhage and/or CPF. The management strategy comprises multidisciplinary supportive treatment, including fluid management, positive pressure ventilation support, and use of milrinone, vasopressors, and inotropes. Some patients may require extracorporeal membrane oxygenation. Major neurological sequelae are almost inevitable once a child develops life-threatening illness. Long-term care of these children is an important medico-social issue.

Background No previous study has investigated the relationship between middle cerebral artery (MCA) flow velocity and the severity of hypoxic ischemic encephalopathy (HIE) evaluated by magnetic resonance imaging (MRI). The aim of this study was to assess the correlation between cerebral blood flow as assessed by transcranial Doppler sonography and the severity of MRI brain injury in asphyxiated neonates with clinical HIE who received therapeutic hypothermia. Material and methods This retrospective cohort study was conducted in the neonatal intensive care unit at Chang Gung Memorial Hospital between April 2011 and May 2014. All neonates with HIE who received therapeutic hypothermia, transcranial Doppler examinations, and brain MRI were eligible. Brain MRI was performed at 11 days of age (interquartile range: 8.5–15 days) and the severity of MRI brain injuries was evaluated using the MR scoring system proposed by Barkovich et al. Serial transcranial Doppler examinations were performed in pre-hypothermia, hypothermia, and post-hypothermia phases. Results Twenty-six neonates met the eligibility criteria for this study. Neonates with an abnormal MCA mean flow velocity (MFV) during the hypothermia phase had a higher risk of brain MRI abnormalities (77.8% vs. 22.2%, p = 0.017) and neonates with abnormal high MFV of MCA had higher MR scores of basal ganglia (p = 0.022). However, there were no statistical differences between abnormal MFV of MCA and brain MRI abnormalities during pre- and post-hypothermia phases. Conclusions During therapeutic hypothermia, mean cerebral blood flow velocity of the MCA was associated with the severity of MRI brain injury in the neonates with clinical HIE.

Pediatric myocarditis symptoms can be mild or as extreme as sudden cardiac arrest. Early identification of the severity of illness and timely provision of critical care is helpful; however, the risk factors associated with mortality remain unclear and controversial. We undertook a retrospective review of the medical records of pediatric patients with myocarditis in a tertiary care referral hospital for over 12 years to identify the predictive factors of mortality. Demographics, presentation, laboratory test results, echocardiography findings, and treatment outcomes were obtained. Regression analyses revealed the clinical parameters for predicting mortality. During the 12-year period, 94 patients with myocarditis were included. Of these, 16 (17%) patients died, with 12 succumbing in the first 72 hours after admission. Fatal cases more commonly presented with arrhythmia, hypotension, acidosis, gastrointestinal symptoms, decreased left ventricular ejection fraction, and elevated isoenzyme of creatine kinase and troponin I levels than nonfatal cases. In multivariate analysis, troponin I > 45 ng/mL and left ventricular ejection fraction < 42% were significantly associated with mortality. Pediatric myocarditis had a high mortality rate, much of which was concentrated in the first 72 hours after hospitalization. Children with very high troponin levels or reduced ejection fraction in the first 24 hours were at higher risk of mortality, and targeting these individuals for more intensive therapies may be warranted.

Background Pneumatosis intestinalis and portomesenteric venous gas are usually caused by necrotizing enterocolitis; however they can occur secondary to abusive abdominal trauma with bone fractures and bruising. It is difficult to recognize initially if there is no bruising on the skin or bone fractures. Case presentation We report a 1-year-old child with no obvious history of trauma who presented with conscious disturbance. Abdominal computed tomography showed acute ischemic bowel complicated with pneumatosis intestinalis and portomesenteric venous gas. The first impression was septic shock with acute ischemic bowel. Two weeks after admission, brain magnetic resonance imaging showed subdural hemorrhage of different stages over bilateral fronto-parietal convexities and diffuse axonal injury, suggesting abusive head trauma. He was subsequently diagnosed with occult child abuse. Conclusion Pneumatosis intestinalis and portomesenteric venous gas are rare except in cases of prematurity. Occult abusive abdominal trauma should be considered as a differential diagnosis in patients with pneumatosis intestinalis and portomesenteric venous gas, even without any trauma on the skin or bone fractures.

Background Myasthenia gravis is the most common disease affecting the neuromuscular junction. The most common etiology among patients with juvenile myasthenia gravis is the production of antibodies against the acetylcholine receptor. However, the clinical outcome in relation to serum levels of anti-acetylcholine receptor antibodies in juvenile myasthenia gravis has rarely been discussed. We aimed to analyze the correlation between the presence of anti-acetylcholine receptor antibodies and outcome in juvenile myasthenia gravis. Methods Patients diagnosed with juvenile myasthenia gravis younger than of 20 years of age were retrospectively recruited from January 1995 to February 2017 in a tertiary referral medical center. According to the Myasthenia Gravis Foundation of America outcome scale, the primary outcome was complete symptom remission and cessation of medications for at least 1 year measured 2 years after diagnosis. Secondary outcome was complete symptom remission at the last outpatient clinic. Results A total of 54 patients were followed up for over 2 years. Nine patients (9/54, 16.7%) achieved complete remission without medication use at 2 years after diagnosis. Thirteen (24.1%) patients achieved complete remission during longer follow-up periods. Those with negative anti-acetylcholine receptor antibodies were more likely to achieve complete remission at 2 years (6/15 [40%] vs. 3/39 [7.7%], 95% Confidence interval [CI] 1.670 to 38.323) and at the last outpatient clinic follow-up (8/15 [53.3%] vs. 5/39 [12.8%], 95% CI 2.367 to 20.704). Thirteen patients with comorbid autoimmune thyroid diseases were older than those without disease (11.8 ± 5.8 years old vs. 8.0 ± 6.3 years old, 95% CI 0.018 to 7.33). Moreover, patients negative for anti-acetylcholine receptor antibodies were less likely comorbid with autoimmune thyroid disease (1/35 [2.9%] vs. 12/71 [16.9%], 95% CI 0.018 to 1.161). Conclusions Juvenile myasthenia gravis patients without anti-acetylcholine antibodies exhibited significantly increased complete remission rates and a reduced likelihood of comorbid autoimmune thyroid diseases compared with those with anti-acetylcholine receptor antibodies among Chinese. Electronic supplementary material The online version of this article (10.1186/s12883-019-1397-0) contains supplementary material, which is available to authorized users.

Background Nonketotic hyperglycinemia (NKH) is a rare, inherited disease, with very poor outcome. It is difficult to confirm the diagnosis due to nonspecific presentations and rapid progression. The incidence was reported in a few countries. We report the clinical and genetic features of typical neonatal NKH with novel splicing mutation, c.1058+3A>C, in the intron 7 of the glycine decarboxylase (GLDC) gene. Furthermore, this study aimed to delineate the estimated incidence and clinical characteristics of NKH in the Taiwanese population. Methods Reports of Health Promotion Administration, Ministry of Health and Welfare of Taiwan, during the period from 2000 to 2013; the Human Gene Mutation Database; and literature regarding NKH in Taiwan were reviewed. Demographic information, age of onset, clinical characteristics, genetic analysis, electroencephalography examinations, and outcome of the patients were analyzed. Results The estimated incidence of NKH in the Taiwanese population was 7.2 cases per 1,000,000 live births. Among the 12 cases reported in Taiwan, more than 90% were of neonatal type. Fifty-five percent of affected patients died within 5 years, and all survivors had severe neurologic outcomes. Only three infants underwent genetic analysis during the study period. Two neonatal NKH infants had mutation in the GLDC gene, and the other one, who had late-onset NKH, had mutation in the glutaredoxin 5 gene. Conclusion Compared with other countries, the estimated incidence of NKH was relatively rare in the Taiwanese population. It is important to characterize all index cases at the genetic level. With more awareness of NKH, increased knowledge of gene mutation, and improvement of diagnostic tools, NKH can be diagnosed more accurately.

Background Serum lactate was used to predict the severity and outcome of neonatal hypoxic ischemic encephalopathy (HIE) before the era of therapeutic hypothermia (TH). There is no report on neurodevelopment (ND) outcome of neonates with HIE treated with TH in Taiwan. Methods Between April 2011 and December 2012, newborn infants admitted to Chang Gung Memorial Hospital (CGMH), with gestational age > 35 weeks and birth weight ≥ 1800 g, who had acute perinatal events, evidence of significant fetal compromise, and ongoing clinical encephalopathy were prospectively enrolled for TH. Whole body cooling method was used to maintain the affected neonate's esophageal temperature at 33.5 ± 0.5°C for 72 hours. Demographic data were recorded and hemogram, biochemical parameters, serum lactate, and creatine kinase (CK) were measured as well. Brain magnetic resonance imaging (MRI) was performed between 7 and 14 days of life. ND outcome of infants was evaluated by Bayley Scales of Infant Development, third edition (BSID-III) at 24 months of corrected age. Poor ND (PND) outcome was defined as infants surviving with either disability or ND delay. Results Seventeen patients were enrolled. Fifty-nine percent of babies (10/17) were born through cesarean section and 77% of babies (13/17) were transferred from outside hospitals. Six babies were moderate HIE and 11 babies were severe HIE. Among the 14 surviving patients, eight infants had PND outcome. There was no difference in demographic data between infants with and without PND. Serum level of lactate (mg/dL) after 72 hours of TH was higher (35.6 vs. 13.8, p = 0.042) in infants with PND. Neonates with abnormal brain MRI findings were also associated with PND ( p = 0.01). Conclusion This is the first report on ND outcome of neonates with HIE treated with TH in Taiwan. Higher serum level of lactate following TH and abnormal results of brain MRI are associated with poor ND outcome.

Background Nitrous oxide (N2O) is a commonly used inhaled anesthetic in outpatient dental procedures. However, the increasing recreational use of N2O may result in vitamin B12 deficiency-related neurologic and psychiatric symptoms. The aim of this study was to demonstrate the clinical features of chronic N2O abuse in pediatric patients. Methods Patients under 20 years of age who were diagnosed with N2O-induced subacute combined degeneration of the spinal cord from 2012 to 2018 were enrolled in this study. Clinical presentations, laboratory, imaging, ancillary studies, treatments and outcomes were analyzed. Results Nine patients were included, all of whom presented with symptoms of myeloneuropathy including limb numbness, limb weakness or unsteady gait. Six patients had low or low-normal vitamin B12 (cyanocobalamin) levels. Eight patients had evidence of subacute combined degeneration of the spinal cord via neuroimaging studies. All of the patients received vitamin B12 supplementation as treatment. All had full recovery of muscle power within 2 months. Five patients had persistent sensory deficits. Conclusion Chronic N2O abuse can cause permanent neurological damage if not treated promptly. Clinical staff should be aware of the various presentations of neurotoxicity related to N2O abuse.

The role of methylprednisolone pulse therapy as adjuvant treatment of Streptococcus pneumoniae meningitis complicated by cerebral infarction has rarely been reported. We reported a case report and also performed a systematic literature review. A 1-year 2-month-old boy who presented with high fever, status epilepticus, and septic shock was diagnosed with cerebral infarction caused by Streptococcus pneumoniae meningitis on magnetic resonance imaging (MRI). He was treated with methylprednisolone pulse therapy and his clinical condition gradually improved thereafter. At the follow-up visit 1 year after discharge, he was able to sit without support, but he had moderate delays in speech and developmental milestones and epilepsy sequelae. In severe cases, the use of high-dose methylprednisolone should be considered to modulate the inflammatory response in patients with severe cerebral infarction caused by Streptococcus pneumoniae meningitis.

Encephalitis is a common central nervous system disorder in children and refers to inflammation and swelling of the brain. The pathogenesis is often caused by either a direct viral infection or an immune-mediated process. It is an important cause of acute symptomatic seizures and subsequent postencephalitic epilepsy. Encephalitis-related seizures may present as a single seizure, repetitive seizures, refractory status epilepticus, or even intractable epilepsy.

1. The occurrence of trivial exercise-induced rhabdomyolysis in the absence of infectious diseases, electrolyte imbalances, drug use, and endocrine disorders was indicative of metabolic myopathy. 2. Increased serum lactate and ammonia levels on the ischemic forearm exercise test (IFET) and elevated serum lactate level on the oral glucose lactate stimulation test (OGLST) pointed to mitochondrial myopathy, which was confirmed by the identification of a mitochondrial DNA mutation. 3. The failure to generate muscular energy during exercise due to an impairment of mitochondrial function by a genetically defective synthesis of electron transport chain protein contributes to muscle injury. 4. Prohibition of overexertion with proper exercise training, avoidance of mitochondrion-toxic drugs and pharmacologic therapy, such as respiratory chain cofactor and carnitine, are the mainstays of the treatment for mitochondrial myopathy.

Aim of the study The aim of the present study was to assess the cerebral flow in children receiving therapeutic hypothermia after resuscitation. The prognostic value of transcranial Doppler findings was correlated with the clinical outcomes in these children. Methods A retrospective cohort study was conducted at the paediatric intensive care unit of Chang Gung Children's Hospital between January 2011 and December 2012. All children from 1 month to 18 years of age who received therapeutic hypothermia after resuscitation were eligible. Serial transcranial Doppler examinations were performed and the findings were reviewed. Results Seventeen children met the eligibility criteria for this study. Fourteen patients (82.3%) were asphyxial in aetiology, and 12 (70.5%) of these cases occurred outside of the hospital. Eight patients (47.1%) had a Paediatric Cerebral Performance Score of 1 or 2 at 3 months after the events. Reversal diastolic or undetectable flow patterns during therapeutic hypothermia were associated with unfavourable prognosis. Normal mean flow velocity in the rewarming phase and normal pulsatility index in the hypothermia and rewarming phases were associated with favourable outcome. Conclusion The transcranial Doppler examinations provided additional information for cerebral perfusion during therapeutic hypothermia, which may in the future be used to guide changes to hypothermia management. Mean cerebral blood flow velocity and pulsatility index by transcranial Doppler sonography can serve as a prognostic factor for children who receive therapeutic hypothermia after resuscitation.

Background Febrile infections are an important cause of paediatric refractory status epilepticus, and immune-mediated mechanisms and inflammatory processes have been associated with neurological manifestations in such patients. The aim of this study was to investigate the effects of immunotherapy as adjuvant treatment for febrile refractory status epilepticus. Methods We retrospectively reviewed cases of febrile refractory status epilepticus in a paediatric intensive care unit between January 2000 and December 2013 and analysed their clinical characteristics. Patients positive for antineuronal antibodies against surface antigens were excluded. Results We enrolled 63 patients (38 boys), aged 1–18 years, all of whom received multiple antiepileptic drugs. Twenty-nine (46%) of the patients received intravenous immunoglobulin alone, 16 (25.4%) received a combination of intravenous immunoglobulin and methylprednisolone pulse therapy, and 18 (28.6%) did not receive immunotherapy treatment. Overall, 12 (19%) patients died within 1 month. After 6 months, 12 (20%) patients had good neurological outcomes, including two who returned to baseline and 13 (29.5%) who had favourable seizure outcomes. We compared the outcomes of the different treatments, and found that a combination of intravenous immunoglobulin and methylprednisolone pulse therapy had the best neurological and seizure outcomes at 6 months compared to intravenous immunoglobulin alone and no immunotherapy. Conclusions Our observational study showed that a combination of intravenous immunoglobulin and methylprednisolone pulse therapy as adjuvant treatment for febrile refractory status epilepticus was associated with better neurological and seizure outcomes. Further prospective studies are needed to confirm these findings.

Objective We aimed to determine the prevalence and risk factors for electrographic seizures in neonates and children requiring extracorporeal membrane oxygenation support. Design Prospective quality improvement project. Setting Quaternary care pediatric institution. Patients Consistent with American Clinical Neurophysiology Society electroencephalographic monitoring recommendations, neonates and children requiring extracorporeal membrane oxygenation support underwent clinically indicated electroencephalographic monitoring. Interventions We performed a 2-year quality improvement study from July 2013 to June 2015 evaluating electrographic seizure prevalence and risk factors. Main results Ninety-nine of 112 patients (88%) requiring extracorporeal membrane oxygenation support underwent electroencephalographic monitoring. Electrographic seizures occurred in 18 patients (18%), of whom 11 patients (61%) had electrographic status epilepticus and 15 patients (83%) had exclusively electrographic-only seizures. Electrographic seizures were more common in patients with low cardiac output syndrome (p = 0.03). Patients with electrographic seizures were more likely to die prior to discharge (72% vs 30%; p = 0.01) and have unfavorable outcomes (54% vs 17%; p = 0.004) than those without electrographic seizures. Conclusions Electrographic seizures occurred in 18% of neonates and children requiring extracorporeal membrane oxygenation support, often constituted electrographic status epilepticus, and were often electrographic-only thereby requiring electroencephalographic monitoring for identification. Low cardiac output syndrome was associated with an increased risk for electrographic seizures. Electrographic seizures were associated with higher mortality and unfavorable outcomes. Further investigation is needed to determine whether electrographic seizures identification and management improves outcomes.

Background The recovery of diaphragmatic function is vital for successful extubation from mechanical ventilation. Recent studies have detected diaphragm atrophy in ventilated adults by using ultrasound, but no similar report has been conducted in children. In the current study, we hypothesized that mechanically ventilated children may also develop diaphragm atrophy and diaphragmatic dysfunction. Materials and methods Children who were admitted to the pediatric intensive care unit and were newly intubated for mechanical ventilation were enrolled into this prospective case–control study. Diaphragm ultrasound assessments were performed daily to evaluate diaphragmatic function in the enrolled children until their discharge from the pediatric intensive care unit. Diaphragm thickness and the diaphragmatic thickening fraction (DTF) were measured through these assessments. Results A total of 31 patients were enrolled, and overall, 1389 ultrasound assessments were performed. Immediately after intubation, the initial diaphragm thickness and DTF were measured to be 1.94 ± 0.44 mm and 25.85% ± 3.29%, respectively. In the first 24 hours of mechanical ventilation, diaphragm thickness and the DTF decreased substantially and decreased gradually thereafter. After extubation, the DTF was significantly different between the successful and failed extubation groups (P < 0.001), and a DTF value of

Background Contactin-associated protein 2 (Caspr2) antibody is a neuronal surface antibody (NSAb) capable of causing disorders involving central and peripheral nervous systems (PNS). Thymoma can be found in patients with Caspr2 antibodies and is most frequently associated with PNS symptoms. Myasthenia gravis can be found in these patients, but Hashimoto thyroiditis (HT) has not been reported. Methods A 76-year-old woman presented with sub-acute-onset changes in mental status. Further investigations revealed thymoma and HT. The presence of NSAb was tested by immunofluorescence on human embryonic kidney-293 cells. Treatment included corticosteroids, azathioprine, thyroxine, plasmapheresis, and thymectomy. Results Caspr2 antibody was positive in serum but absent in CSF. Brain magnetic resonance imaging (MRI) showed diffuse cortical atrophy, but did not change significantly after treatments. Brain positron emission tomography (PET) revealed diffuse hypometabolism over the cerebral cortex. The patient's mental status only partially improved. Conclusions In Caspr2 antibody-associated syndromes, thymoma can occur in patients presenting only with LE, and HT can be an accompanying disease. Brain MRI and PET may not show specific lesions in limbic area. Patients with Caspr2 antibodies and thymoma may not have good prognosis.

Background Headache is a common complaint in children and is one of the most common reasons for presentation at a pediatric emergency department (PED). This study described the etiologies of patients with headache seen in the PED and determined predictors of intracranial pathology (ICP) requiring urgent intervention. A secondary objective was to develop rapid, practical tools for screening headache in the PED. Methods We conducted a retrospective chart review of children who presented with a chief complaint of headache at the PED during 2008. First, we identified possible red flags in the patients' history or physical examination and neurological examination findings. Then, we recorded the brain computed tomography results. Results During the study period, 43,913 visits were made to the PED; in 409 (0.9%) patients, the chief complaint was headache. Acute viral, respiratory, and febrile illnesses comprised the most frequent cause of headache (59.9%). Six children (1.5%) had life-threatening ICP findings. In comparison with the group without ICP, the group with ICP had a significantly higher percentage of blurred vision ( p = 0.008) and ataxia ( p = 0.002). Conclusion Blurred vision and ataxia are the best clinical parameters to predict ICP findings.

Background Evidence for the beneficial effect of therapeutic burst-suppression coma in pediatric patients with febrile refractory status epilepticus is limited, and the clinical outcomes of this treatment strategy are largely unknown. Therefore, the aim of this study was to explore the outcomes of therapeutic burst-suppression coma in a series of children with febrile refractory status epilepticus. Methods We retrospectively reviewed consecutive pediatric patients with febrile refractory status epilepticus admitted to our pediatric intensive care unit between January 2000 and December 2013. The clinical characteristics were analyzed. Results Thirty-five patients (23 boys; age range: 1–18 years) were enrolled, of whom 28 (80%) developed super-refractory status epilepticus. All of the patients received the continuous administration of intravenous antiepileptic drugs for febrile refractory status epilepticus, and 26 (74.3%) achieved therapeutic burst-suppression coma. All of the patients received mechanical ventilatory support, and 26 (74.3%) received inotropic agents. Eight (22.9%) patients died within 1 month. The neurologically functional outcomes at 6 months were good in six (27.3%) of the 22 survivors, of whom two returned to clinical baseline. The patients with therapeutic burst-suppression coma were significantly associated with hemodynamic support than the patients with electrographic seizures control ( p = 0.03), and had a trend of higher 1-month mortality rate, worse 6 months outcomes, and a longer duration of hospitalization. Conclusions Our results suggest that therapeutic burst-suppression coma to treat febrile refractory status epilepticus may lead to an increased risk of hemodynamic instability and a trend of worse outcomes.

BackgroundPeritonsillar abscess is an acute infection located between the capsule of the palatine tonsil and the superior constrictor muscle of the pharynx. The reports regarding pediatric cases have been scanty.MethodsA retrospective study was conducted to investigate the patients less than 18 years of age hospitalized at a medical center with a final diagnosis of peritonsillar abscess from January 1999 through December 2009. Only those who were confirmed by the drainage of pus from the peritonsillar spaces or confirmed by a computed tomography (CT) scan were enrolled.ResultsIn total, 56 children, 31 male and 25 female, were included. The mean age was 12.9 ± 4.6 years, ranging from 9 months to 17.9 years. Nineteen (34%) were less than 12 years of age. All 37 children ≧12 years of age complained of sore throat, but only 68% of children less than 12 years of age complained of sore throat. Thirty-one patients (55%) with a characteristic presentation of fever and sore throat plus an asymmetric swollen/bulging tonsil with or without uvular deviation suggestive of the diagnosis received surgical drainage at emergency department immediately, and 87% of them were older than 12 years of age. Twenty-five (45%) children needed a CT scan to confirm the diagnosis. The most common empirical antibiotics were penicillin-containing regimens. The predominant organisms identified were Streptococcus species. Eight children were treated successfully with antibiotics alone. No case was fatal.ConclusionTwo-thirds of the children with peritonsillar abscess were ≥12 years of age. Not every patient

Decreased ADAMTS 13 activity has been reported in severe sepsis and in sepsis-induced disseminated intravascular coagulation. This study aimed to investigate the role of ADAMTS 13 in different pediatric sepsis syndromes and evaluate its relationship with disease severity and outcome. We prospectively collected cases of sepsis treated in a pediatric intensive care unit, between July 2012 and June 2014 in Chang Gung Children's Hospital in Taoyuan, Taiwan. Clinical characteristics and ADAMTS-13 activity were analyzed. All sepsis syndromes had decreased ADAMTS 13 activity on days 1 and 3 of admission compared to healthy controls. Patients with septic shock had significantly decreased ADAMTS 13 activity on days 1 and 3 compared to those with sepsis and severe sepsis. There was a significant negative correlation between ADAMTS 13 activity on day 1 and day 1 PRISM-II, PELOD, P-MOD, and DIC scores. Patients with mortality had significantly decreased ADAMTS 13 activity on day 1 than survivors, but not on day 3. Different pediatric sepsis syndromes have varying degrees of decreased ADAMTS 13 activity. ADAMTS 13 activity is strongly negatively correlated with disease severity of pediatric sepsis syndrome, whereas decreased ADAMTS 13 activity on day 1 is associated with increased risk of mortality.

Guillain-Barré syndrome is characterized by acute progressive weakness, areflexia, and maximal motor disability that occur within 4 weeks of onset. Various clinical subtypes have been described since the original description of the syndrome. This study aimed to identify characteristics of clinical variants of Guillain-Barré syndrome through retrospective review of cases in Chang Gung Children's Hospital from 2000-2010. Forty-three Guillain-Barré syndrome patients were evaluated based on clinical presentations and an electrodiagnostic study. The most frequent variant of Guillain-Barré syndrome was demyelinating polyneuropathy (67.4%), followed by acute axonal neuropathy (7.0%), Miller Fisher syndrome (7.0%), Bickerstaff brainstem encephalitis (7.0%), pharyngo-cervical-brachial variant (4.7%), and polyneuritis cranialis (4.7%). Follow-up revealed that 35 recovered satisfactorily, eight were persistently disabled, and none died during hospitalization. At the earliest stage, differentiating clinical variants from typical Guillain-Barré syndrome was difficult. Children with clinical variants of Guillain-Barré syndrome are more likely to manifest rapid onset from disease onset to nadir, increasing the severity of disability, cranial nerve involvement, urine incontinence, respiratory impairment, and need for ventilator support than in typical Guillain-Barré syndrome.

SUMMARY Purpose: Mycoplasma pneumoniae, a common respiratory pathogen, has been implicated as an etiology of encephalitis, but there are few reports about it and postencephalitic epilepsy. This study aimed to investigate clinical factors, electroencephalography, and neuroradiologic features of M. pneumoniae–related encephalitis in a series of children with postencephalitic epilepsy and to examine possible prognostic factors. Methods: Cases of M. pneumoniae–related encephalitis between January 2001 and June 2010 were retrospectively reviewed. Systematic clinical data were evaluated. Key Findings: The 99 enrolled patients with M. pneumoniae–related encephalitis were all positive by serology and 47 (47.5%) of them developed postencephalitic epilepsy. During the acute phase, 53 patients (53.5%) had seizures, the most common type of which was primary focal with secondary generalized tonic–clonic seizure (39.6%). The most common initial electroencephalography was focal/ diffuse cortical dysfunction (37.4%) and focal epileptiform discharge (26.4%). The time of follow-up ranged from 6–131 months. At the end of the study, 19 (40.4%) of the 47 children with postencephalitic epilepsy had intractable seizures. Significance: Postencephalitic epilepsy is not a rare complication of M. pneumoniae–related encephalitis. Seizures in the acute phase and focal epileptiform discharges in initial electroencephalography are significant prognostic factors.