• Journal of Dental Anesthesia and Pain Medicine
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• v.18 no.4
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• pp.245-254
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• 2018

Background: When performing dental treatment under general anesthesia in adult patients who have difficulty cooperating due to intellectual disabilities, anesthesia induction may be difficult as well. In particular, patients who refuse to come into the dental office or sit in the dental chair may have to be forced to do so. However, for adult patients with a large physique, physical restraint may be difficult, while oral sedatives as premedication may be helpful. Here, a retrospective analysis was performed to investigate the effect of oral sedatives. Methods: A hospital-based medical information database was searched for patients who were prescribed oral midazolam or triazolam between January 2009 and December 2017. Pre-anesthesia evaluation, anesthesia, and anesthesia recovery records of all patients were analyzed, and information on disability type, reason for prescribing oral sedatives, prescribed medication and dose, cooperation level during anesthesia induction, anesthesia duration, length of recovery room stay, and complications was retrieved. Results: A total of 97 patients were identified, of whom 50 and 47 received midazolam and triazolam, respectively. The major types of disability were intellectual disabilities, autism, Down syndrome, blindness, cerebral palsy, and epilepsy. Analyses of changes in cooperation levels after drug administration showed that anesthesia induction without physical restraint was possible in 56.0% of patients in the midazolam group and in 46.8% of patients in the triazolam group (P = 0.312). Conclusions: With administration of oral midazolam or triazolam, general anesthesia induction without any physical restraint was possible in approximately 50% of patients, with no difference between the drugs.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.139-144
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• 2016

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that affects ectomesodermal tissues (skin, eyes, adipose tissue, and brain). The neurologic manifestations associated with ECCL are various including seizures. However, ECCL patients very rarely develop brain tumors that originate from the neuroepithelium. This is the first described case of ECCL in combination with dysembryoplastic neuroepithelial tumor (DNET) that presented with intractable seizures. A 7-year-old girl was admitted to our center because of ECCL and associated uncontrolled seizures. She was born with right anophthalmia and lipomatosis in the right temporal area and endured right temporal lipoma excision at 3 years of age. Seizures began when she was 3 years old, but did not respond to multiple antiepileptic drugs. Brain magnetic resonance (MR) imaging performed at 8 and 10 years of age revealed an interval increase of multifocal hyperintense lesions in the basal ganglia, thalamus, cerebellum, periventricular white matter, and, especially, the right temporal area. A nodular mass near the right hippocampus demonstrated the absence of N-acetylaspartate decrease on brain MR spectroscopy and mildly increased methionine uptake on brain positron emission tomography, suggesting low-grade tumor. Twenty-four-hour video electroencephalographic monitoring also indicated seizures originating from the right temporal area. Right temporal lobectomy was performed without complications, and the nodular lesion was pathologically identified as DNET. The patient has been seizure-free for 14 months since surgery. Although ECCL-associated brain tumors are very rare, careful follow-up imaging and surgical resection is recommended for patients with intractable seizures.

• The Korean Journal of Rehabilitation Nursing
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• v.7 no.1
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• pp.96-104
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• 2004

Purpose: The aim of this study is to evaluate the general status and prevalence of cerebral palsy, the complication and the activity of daily living in patients with cerebral palsy in Holt Ilsan Home. Methods: Cross-sectional study was performed on 113 cerebral palsy patients in Holt Ilsan Home with the medical records review, the physical examination, and the Modified Barthel index for the independency of activities of daily living. Results: The mean age of subjects was $28.1{\pm}12.7$ years and the duration of residence was $22.9{\pm}12.8$ years. The most common type of cerebral palsy according to the muscle tone abnormality was spastic type(53.1%). The most frequent condition in which extremities are involved were quadriplegia in 46.0%. The score of Modified Barthel index was significantly lower in mixed type for $16.9{\pm}24.0$ and quadriplegic type for $14.8{\pm}25.5$ compared with others. Conclusion: This study indicates that cerebral palsy patients in rehabilitation facility have severe medical problems such as musculoskeletal complications, visual and language problems, epilepsy and dependency in activities of daily living. Therefore more medical attention like long-term follow-up study and social supports is needed.

• The Journal of Health Technology Assessment
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• v.6 no.2
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• pp.142-147
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• 2018

Objectives: The purpose of this study is to evaluate the safety and effectiveness of Robot-Assisted Brain Stereotactic Surgery with a systematic review. Methods: Electronic literature was searched using KoreaMed, Ovid-MEDLINE, Ovid-EMBASE, and Cochrane Library on 6th April 2017. Two authors screened 1218 citations. Duplicated articles of 456 excluded, the remaining 762 articles were reviewed with title and abstract. Results: A total of 8 studies were selected in this review. The device used in all studies was $ROSA^{TM}$. In one cohort study comparing the intervention ($ROSA^{TM}$) with the control (conventional stereotactic surgery), hematoma was reported no significant difference between groups. In six descriptive studies, one study reported hematoma 10% (10/100) and temporary nerve impairment 6% (6/100) using the ROSA; while five descriptive study did not report any complications. In one cohort, the localization precision were 1.2 mm in the intervention group and 1.1 mm in the control group; the localization success rate as 78.2% in the intervention group and 76.2% in the control group in one cohort; and the average time for surgery as 130 min for the intervention group and 352 min for the control group in one cohort. Four studies reported the localization success rate as 100%; two out of three articles reported the overall time for surgery as 56 min and 90 min, while one article reported the time as less than one hour in 50% of patients (50/100); two articles reported in epilepsy patients, the condition after the surgery was Engel level I in 66.2%, 75% patients, Engel level II-III in 25%, 26.5% patients, and Engel level 4 in 7.3% patients. Conclusion: Robot-Assisted Brain Stereotactic Surgery is a safe and accurate technique that can significantly reduce the time for the brain stereotactic surgery. However, further studies are needed to generalize the results.

• Clinical and Experimental Pediatrics
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• v.62 no.4
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• pp.131-137
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• 2019

Purpose: Respiratory syncytial virus (RSV) infection can cause various neurological complications. This study aimed to investigate the RSV-associated neurologic manifestations that present with seizures. Methods: We retrospectively reviewed the medical records of patients aged less than 15 years with laboratory-confirmed RSV infections and seizures between January 2011 and December 2016 in a regional hospital in South Korea. Results: During this period, 1,193 patients with laboratory-confirmed RSV infection were identified. Of these, 35 (35 of 1,193, 2.93%; boys, 19; girls, 16; mean age: $20.8{\pm}16.6months$) presented with seizure. Febrile seizure was the most common diagnosis (27 of 35, 77.1%); simple febrile seizures in 13 patients (13 of 27, 48.1%) and complex febrile seizures in 14 (14 of 27, 51.9%). Afebrile seizures without meningitis or encephalopathy were observed in 5 patients (5 of 35, 14.3%), seizures with meningitis in 2 (2 of 35, 5.7%), and seizure with encephalopathy in 1 (1 of 35, 2.9%) patient. Lower respiratory symptoms were not observed in 8 patients. In a patient with encephalopathy, brain diffusion-weighted magnetic resonance imaging revealed transient changes in white matter, suggesting cytotoxic edema as the mechanism underlying encephalopathy. Most patients recovered with general management, and progression to epilepsy was noted in only 1 patient. Conclusion: Although febrile seizures are the most common type of seizure associated with RSV infection, the proportion of patients with complex febrile seizures was higher than that of those with general febrile seizures. Transient cytotoxic edema may be a pathogenic mechanism in RSV-related encephalopathy with seizures.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.57-61
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• 2016

Isovaleric acidemia (IVA) is an autosomal recessively inherited organic acid disorder due to a defect of the enzyme isovaleryl-CoA dehydrogenase in the leucine metabolic pathway. Deficiency of this enzyme results in the accumulation of derivatives of isovaleryl-CoA. In acute illness in IVA, isovaleric acid and its derivatives accumulate and profound metabolic acidosis with ketosis, characteristic pungent body odor, hypoglycemia, and hyperammonemia can be developed. Additionally, recurrent vomiting, failure to thrive, developmental delay, epilepsy and mental retardation are chronic presenting symptoms and signs for IVA. On the result of newborn screening for inherited metabolic disorders, increased levels of isovalerylcarnitine (C5) are shown. However, C5 elevation can be accompanied with short/branched-chain acyl-CoA dehydrogenase (SBCAD) and therapy with certain antibiotics containing pivalic acid. Quantitative measurement of organic acids in urine and acylcarnitine profiles in plasma are necessary to differential diagnosis. Molecular genetic analysis of the IVD gene for IVA and ACADSB is also helpful to confirm IVA and SBCAD deficiency, respectively. Considering that IVA can be associated with significant morbidity and mortality at acute presentation of metabolic crisis, early diagnosis prior to the onset of symptoms by newborn screening enable to introduction of early treatment and prevention of acute and chronic complications.

• Journal of The Korean Dental Society of Anesthesiology
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• v.8 no.1
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• pp.22-28
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• 2008

Background: Cerebral palsy (CP) is non-progressive disorder of motion and posture. In CP patient, there are difficulties in dental treatment because of uncontrolled movement of limb and head, and conjoined disabilities such as cognitive impairment, sensory loss, seizures, communication and behavioral disturbances. It is reported that CP patients have high incidence in caries and a higher prevalence of periodontal disease. But, despite the need for oro-dental care, these patients often are unlikely to receive adequate treatment without sedation or general anesthesia because of uncontrolled movements of the trunk or head. Methods: We reviewed the 58 cases of 56 patients with CP who underwent outpatient general anesthesia for dental treatment at the clinic for the disabled in Seoul National University Dental Hospital. Results: The mean age was 19 (2-54) years. The number of male patient was 40 and that of female was 18. They all had severe spastic cerebral palsy and 22 had sever mental retardation, 15 epilepsy, 8 organic brain disorder, 1 blindness, 2 deafness and cleft palate. For anesthesia induction, 14 cases was needed physical restriction who had sever mental retardation and cooperation difficulty, but 44 cases showed good or moderate cooperation. Drugs used for anesthesia induction were thiopental (37 cases), sevoflurane (14 cases), ketamine (3 cases ) and propofol (4 cases). All patients except one were done nasotracheal intubation for airway management and 4 cases were needed difficult airway management and 1 patient already had tracheostomy tube. Mean total anesthetic time was $174{\pm}56$ min and staying time at PACU was $88{\pm}39$ min. There was no death or long term hospitalization because of severe complications. Conclusion: If general anesthesia is needed, pertinent diagnostic tests and workup about anomaly, and appropriate anesthetic planning are essential for safety.

• Pediatric Infection and Vaccine
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• v.14 no.1
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• pp.43-46
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• 2007

Purpose : Neonatal bacterial meningitis has been known that its mortality and morbidity is high and its neurologic complications are common. This study was performed to review of the etiology of neonatal bacterial meningitis in Busan, Korea. Methods : We retrospectively analyzed 21 medical records of patients who were diagnosed neonatal bacterial meningitis in four university hospitals in Busan from January 1997 to December 2005. We analyzed age at onset of symptoms, symptoms at admission, causative organisms, and complications. Results : There were 11 male patients and 10 female patients. One premature neonate was included. 7 patients had symptoms at 2-3 weeks after birth and 6 patients at 1-2 weeks after birth. 18 cases had fever, 8 cases had seizure and some had poor feeding, lethargy and irritability. The most common organism causing neonatal bacterial meningitis was Group B streptococcus (GBS). 11 cases were due to GBS, 3 cases due to E. coli, 3 cases were due to Klebsiella pneumoniae, and 2 cases due to Streptococcus pneumoniae. one Listeria monocytogenes and one Enterobacter cloacae were included. 18 patients were alive and 3 patients died. Mental retardation, developmental delay and epilepsy occurred in a living patient. Conclusions : Although this was a retrospective study, we found that Group B streptococcus (GBS) was the most common organism causing neonatal bacterial meningitis and this data is nearly same as others in the world.

• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1047-1051
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• 2008

Purpose : This study aims to evaluate the efficacy and safety of sedatives for pediatric patients using noninvasive procedures. Methods : We performed a prospective study in 446 (aged 1 month-21 y) consecutive pediatric patients undergoing sedation to study noninvasive sedation techniques from February to August 2007. We reviewed demographic data, sedative drugs, dosage, complications, and successful rates of sedation according to the underlying diseases. Results : The overall successful rate of sedation was 435/446 (97.5%). The overall rate of successful sedation using chloral hydrate was 99.1% (420/424), and was 70.6% (12/17) and 60.0% (3/5) with ketamine and midazolam, respectively. Of the neurologic patients (n=172, aged 1 month to 21 years), 136 patients were sedated for EEGs, 5 patients for renal scans, and 31 patients for neuroimaging studies such as brain CT or MRI. All non-neurological patients (n=274, aged 1 month to 5 years) were diagnosed with urinary tract infection and sedated for renal scan. The overall success rate of sedation for this group was 99.6% (273/274). A total of 14 adverse events were observed (3.1%). Most adverse reactions were mild in severity and clinically insignificant. Conclusion : Using chloral hydrate alone has enough effect to sedate non-neurologic patients. However, neurologic patients in the severe course group, especially those suffering from intractable epilepsy, autism, or severe cerebral palsy, must be medicated with chloral hydrate 2 times at most; instead, injections of ketamine or midazolam in the early stage may result in a more promising outcome.