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Shortage of doctors in emergency departments (EDs) is a root issue in maintaining ED-based residency programs. This study describes the efficiency of emergency practice according to board certification; 3 board-certified physicians versus a pediatric resident. Of 342 children, we found no differences as per the board certification in the ED length of stay, acuity, and return visits with more frequent hospitalization by the board-certified physicians. This result suggests that with a proper residency program, both board-certified physicians and residents can make a decision on hospitalization.
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Background@#and Purpose Data regarding the effects of cannabidiol (CBD) on the quality of life (QOL) are currently inadequate. We assessed the QOL of pediatric patients with epilepsy who were treated with CBD. @*Methods@#This prospective, open-label study included pediatric and adolescent patients (aged 2–18 years) with Dravet syndrome or Lennox-Gastaut syndrome. Oral CBD was administered at 10 mg/kg/day. The Korean version of the Quality Of Life in Childhood Epilepsy (QOLCE) questionnaire was administered when CBD treatment began and again after 6 months. Adaptive behavior was measured using the Korean versions of the Child Behavior Checklist (K-CBCL) and the second edition of the Vineland Adaptive Behavior Scales (Vineland-II). @*Results@#This study included 41 patients (11 with Dravet syndrome and 30 with LennoxGastaut syndrome), of which 25 were male. The median age was 4.1 years. After 6 months, 26.8% (11/41) of patients experienced a ≥50% reduction in the number of seizures. The total score for the QOLCE questionnaire did not change from baseline to after 6 months of CBD treatment (85.71±39.65 vs. 83.12±48.01, respectively; p=0.630). The score in the motor skills domain of Vineland-II reduced from 48.67±13.43 at baseline to 45.18±14.08 after 6 months of treatment (p=0.005). No other Vineland-II scores and no K-CBCL scores had changed after 6 months of CBD treatment. @*Conclusions@#CBD is an efficacious antiseizure drug used to treat Dravet syndrome and Lennox-Gastaut syndrome. However, it did not improve the patient QOL in our study, possibly because all of our patients had profound intellectual disabilities.
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Background@#For the first time in Korea, we aimed to study the efficacy and safety of cannabidiol (CBD), which is emerging as a new alternative in treating epileptic encephalopathies. @*Methods@#This study was conducted retrospectively with patients between the ages of 2–18 years diagnosed with Lennox-Gastaut syndrome (LGS) or Dravet syndrome (DS) were enrolled from March to October 2019, who visited outpatient unit at 3 and 6 months to evaluate medication efficacy and safety based on caregiver reporting. Additional evaluations, such as electroencephalogram and blood tests, were conducted at each period also. CBD was administered orally at a starting dose of 5 mg/kg/day, and was maintained at 10 mg/kg/day. @*Results@#We analyzed 34 patients in the LGS group and 10 patients in the DS group between the ages of 1.2–15.8 years. In the 3-month evaluation, the overall reduction of seizure frequency in the LGS group was 52.9% (>50% reduction in 32.3% of the cases), and 29.4% in the 6-month evaluation (more than 50% reduction in 20.6%). In DS group, the reduction of seizure frequency by more than 50% was 30% and 20% in the 3-month and 6-month evaluation, respectively. Good outcomes were defined as the reduction of seizure frequency by more than 50% and similar results were observed in both LGS and DS groups. Adverse events were reported in 36.3% of total patients of which most common adverse events were gastrointestinal problems. However, no life-threatening adverse event was reported in both LGS and DS during the observation period. @*Conclusion@#In this first Korean study, CBD was safe and tolerable for use and could be expected to potentially reduce the seizure frequency in pediatric patients with LGS or DS.
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Background@#For the first time in Korea, we aimed to study the efficacy and safety of cannabidiol (CBD), which is emerging as a new alternative in treating epileptic encephalopathies. @*Methods@#This study was conducted retrospectively with patients between the ages of 2–18 years diagnosed with Lennox-Gastaut syndrome (LGS) or Dravet syndrome (DS) were enrolled from March to October 2019, who visited outpatient unit at 3 and 6 months to evaluate medication efficacy and safety based on caregiver reporting. Additional evaluations, such as electroencephalogram and blood tests, were conducted at each period also. CBD was administered orally at a starting dose of 5 mg/kg/day, and was maintained at 10 mg/kg/day. @*Results@#We analyzed 34 patients in the LGS group and 10 patients in the DS group between the ages of 1.2–15.8 years. In the 3-month evaluation, the overall reduction of seizure frequency in the LGS group was 52.9% (>50% reduction in 32.3% of the cases), and 29.4% in the 6-month evaluation (more than 50% reduction in 20.6%). In DS group, the reduction of seizure frequency by more than 50% was 30% and 20% in the 3-month and 6-month evaluation, respectively. Good outcomes were defined as the reduction of seizure frequency by more than 50% and similar results were observed in both LGS and DS groups. Adverse events were reported in 36.3% of total patients of which most common adverse events were gastrointestinal problems. However, no life-threatening adverse event was reported in both LGS and DS during the observation period. @*Conclusion@#In this first Korean study, CBD was safe and tolerable for use and could be expected to potentially reduce the seizure frequency in pediatric patients with LGS or DS.
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PURPOSE@#Childhood absence epilepsy (CAE) is a common form of idiopathic generalized epilepsy with onset middle childhood and has typically a good prognosis, but remission rates vary. We aimed to analyze unfavorable prognostic factors in children initially diagnosed with CAE.@*METHODS@#We retrospectively reviewed 48 patients under 13 years of age who were diagnosed with CAE at the Severance Children's Hospital, Seoul, Korea. We analyzed clinical information including comorbidity through neuropsychological test.@*RESULTS@#Thirteen of the 48 patients (27%) showed an unfavorable prognosis, with clinical seizures or seizure waves on electroencephalogram persistent even after 12 months of anticonvulsant therapy. The mean age at absence seizure onset was 6.51±2.36 years. The most commonly used antiepileptic drug (AED) was ethosuximide, and the median duration of initial AEDs was 25.63±24.41 months. The presence of comorbidity and clinical absence seizures after 6 months of AEDs correlated with an unfavorable prognosis. Motor seizures were the most unfavorable prognostic factor during follow-up.@*CONCLUSION@#This study shows that clinical absence seizures after 6 months of AED, comorbidity, and motor seizure are the most important predictive factors of an unfavorable prognosis for absence epilepsy in childhood. This study suggests that when these factors are observed, early intervention needs to be considered.
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PURPOSE@#Mutations in the cyclin-dependent kinase-like 5 (CDKL5) gene are associated with epileptic encephalopathy and severe cognitive impairment. We aim to characterize the association between this gene and treatment efficacy.@*METHODS@#We retrospectively analyzed 10 patients who were treated at Severance Children's Hospital for epileptic encephalopathy who were subsequently diagnosed with a CDKL5 mutation using next-generation sequencing.@*RESULTS@#Electroencephalography (EEG) results showed generalized pattern abnormalities in 60% (6/10) of patients with CDKL5 mutations. We analyzed the effects of three treatments, namely antiepileptic drugs (AEDs), ketogenic diet (KD), and steroids. A more than 50% reduction in seizures was observed in 12% (1/8) of patients treated with clobazam. KD treatment proved ineffective in most cases. In addition, a more than 50% reduction in seizures was observed in 57% (4/7) of patients treated with steroids. EEG analysis of patients treated effectively with steroids revealed that 75% (3/4) showed hypsarrhythmia and 25% (1/4) showed focal epileptiform.@*CONCLUSION@#In this study, as in other studies, AEDs and KD did not effectively control seizures in most patients with a CDKL5 mutation. However, steroid therapy reduced the frequency of seizures in patients who also exhibited hypsarrhythmia. This suggests that steroid treatment is helpful in cases of hypsarrhythmia with CDKL5 mutations.
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PURPOSE@#Acquired epileptic aphasia (AEA) accompanied by electroencephalogram (EEG) abnormality is a rare disease; therefore, there are few studies investigating the prognostic factors and treatment efficacy. We aimed to determine the therapeutic effects and prognostic factors for clinical seizure and neuropsychological function in acquired aphasia patients.@*METHODS@#We retrospectively studied cases of AEA diagnosed at Severance Children's Hospital from January 2013 to October 2017. We evaluated the efficacy of antiepileptic drugs, steroids, and ketogenic diets (KD) in treating acquired aphasia. The EEG patterns and prognostic factors were predicted by the background EEG and frequency of spike and wave during sleep (SWS).@*RESULTS@#The study analyzed 20 patients, 11 male and 9 female, with AEA. Aphasia most commonly occurred at 4 years of age, and clinical seizure was most likely to occur between 2 and 4 years of age and focal seizures were the most common seizure type. KD was shown to be the best treatment for clinical seizure in AEA patients. Patients with normal EEG background showed better responses to clinical seizure treatment and improvements in neuropsychological function.@*CONCLUSION@#KD and steroids generate the best therapeutic effects for clinical seizure in AEA patients. Improvements in neuropsychological function in AEA patients may be related to the EEG background and the SWS patterns. Additionally, the results suggest that the response of clinical seizure to antiepileptic drugs may also be related to the EEG background. However, the current study had some limitations and further research is needed.
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We report the case of a 12-year-old girl who had mild encephalopathy with a reversible splenial lesion (MERS) associated with acutepyelonephritis caused by Escherichia coli. The patient was admitted with a high fever, and she was diagnosed with acute pyelonephritis based on pyuria and the results of urine culture, which detected cefotaxime-sensitive E. coli. Although intravenous cefotaxime and tobramycin were administered, her fever persisted and her C-reactive protein level increased to 307 mg/L. On day 3 of admission, she demonstrated abnormal neuropsychiatric symptoms, such as delirium, ataxia, and word salad. Magnetic resonance imaging (MRI) of the brain performed on day 4 showed marked hyperintensities in the bilateral corpus callosum and deep white matter on diffusion-weighted images, with corresponding diffusion restriction on apparent diffusion coefficient mapping. No abnormalities or pathogens were detected in the cerebrospinal fluid; however, lipopolysaccharides (LPS, endotoxin) were detected in plasma (41.6 pg/mL), associated with acute neurological deterioration. Her clinical condition gradually improved, and no neurological abnormalities were observed on day 6. Follow-up brain MRI performed 2 weeks later showed near-disappearance of the previously noted hyperintense lesions. In this patient, we first proved endotoxemia in a setting of MERS. The release of LPS following antibiotic administration might be related to the development of MERS in this patient. The possibility of MERS should be considered in patients who present with acute pyelonephritis and demonstrate delirious behavior.
Subject(s)
Child , Female , Humans , Ataxia , Brain , Brain Diseases , C-Reactive Protein , Cefotaxime , Cerebrospinal Fluid , Corpus Callosum , Delirium , Diffusion , Endotoxemia , Escherichia coli , Fever , Follow-Up Studies , Lipopolysaccharides , Magnetic Resonance Imaging , Plasma , Pyelonephritis , Pyuria , Tobramycin , White MatterABSTRACT
Alice in wonderland syndrome (AWS) is a paroxysmal disorder with distorted body image, including altered perceptions of size, mass, or shape. In fact, altered body image is the classic symptom of AWS. However, young children and adolescents younger than 15 years with AWS usually show visual symptoms, including micropsia or teleopsia. The most common visual symptom with AWS is micropsia. There are several theories of the etiology of AWS, the most popular of which is migraine. The second most frequently mentioned etiologic theory of AWS is that it is infection-induced. Finally, abnormal brain electrical activity, such as epilepsy, is another possible cause of AWS. We herein report a case of 9-year-old boy who was admitted with visual disturbances characterized by microteleopsia and pelopsia. He denied headache, but his mother had a history of migraine. His general physical examination was unremarkable. Brain magnetic resonance imaging did not show any abnormal findings, but an electroencephalogram showed abnormal discharges. The patient was prescribed an antiepileptic drug, which improved his symptoms.
Subject(s)
Adolescent , Child , Humans , Male , Alice in Wonderland Syndrome , Body Image , Brain , Electroencephalography , Epilepsy , Headache , Magnetic Resonance Imaging , Migraine Disorders , Mothers , Physical Examination , Vision DisordersABSTRACT
Juvenile idiopathic arthritis (JIA) can develop extra-articular manifestations, including growth retardation, osteopenia and chronic uveitis. However, pleuropulmonary involvement is rare. Approximately 40% of patients with JIA have abnormal pulmonary function tests without pulmonary symptoms, with the commonest abnormality in carbon monoxide diffusing capacity, but clinically evident pulmonary parenchymal disease in JIA is extremely uncommon. We describe a 15-year-old male with JIA who presented with dyspnea due to interstitial lung disease.
Subject(s)
Adolescent , Humans , Male , Arthritis , Arthritis, Juvenile , Bone Diseases, Metabolic , Carbon Monoxide , Dyspnea , Lung Diseases , Lung Diseases, Interstitial , Lung , Respiratory Function Tests , UveitisABSTRACT
OBJECTIVE: Kawasaki disease is a systemic vascular disease which is caused by an immunologic response. The purpose of this study is to see how a high IgE level affects Kawasaki disease, in two groups of high IgE level and low IgE level. METHODS: A retrospective study was done from 2008 to 2010, among patients, who were admitted in Severance Children's Hospital for Kawasaki disease with IgE levels checked. Age groups with an IgE level above reference ranges and those with normal ranges were done. Also, clinical characteristics were analyzed. Statistical method was done by SPSS 18. RESULTS: A total of 198 Kawasaki patients were analyzed from 2008 to 2010. Among them 123 (62%) patients showed elevated IgE levels. Patients with high IgE had a significantly higher lymphadenopathy prevalence (p=0.006), however they had no connection with quantitative values. Patients with BCG site redness appeared to have lower IgE levels than patients without redness. Coronary complication had no relation with IgE levels. There was no correlation between laboratory results and IgE levels. CONCLUSION: 62% of Kawasaki disease patients show high IgE levels in our study. The symptoms of BCG site redness aged less than 1 year seemed to be related with IgE level. To understand the pathophysiology of Kawasaki disease, more studies should be performed on the role of IgE.
Subject(s)
Aged , Humans , Immunoglobulin E , Immunoglobulins , Lymphatic Diseases , Mucocutaneous Lymph Node Syndrome , Mycobacterium bovis , Prevalence , Reference Values , Retrospective Studies , Vascular DiseasesABSTRACT
OBJECTIVE: Kawasaki disease is a systemic vascular disease which is caused by an immunologic response. The purpose of this study is to see how a high IgE level affects Kawasaki disease, in two groups of high IgE level and low IgE level. METHODS: A retrospective study was done from 2008 to 2010, among patients, who were admitted in Severance Children's Hospital for Kawasaki disease with IgE levels checked. Age groups with an IgE level above reference ranges and those with normal ranges were done. Also, clinical characteristics were analyzed. Statistical method was done by SPSS 18. RESULTS: A total of 198 Kawasaki patients were analyzed from 2008 to 2010. Among them 123 (62%) patients showed elevated IgE levels. Patients with high IgE had a significantly higher lymphadenopathy prevalence (p=0.006), however they had no connection with quantitative values. Patients with BCG site redness appeared to have lower IgE levels than patients without redness. Coronary complication had no relation with IgE levels. There was no correlation between laboratory results and IgE levels. CONCLUSION: 62% of Kawasaki disease patients show high IgE levels in our study. The symptoms of BCG site redness aged less than 1 year seemed to be related with IgE level. To understand the pathophysiology of Kawasaki disease, more studies should be performed on the role of IgE.