• International Journal of Heart Failure
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• v.6 no.2
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• pp.76-81
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• 2024

Background and Objectives: Real-world clinical data, outside of clinical trials and expert centers, on adverse events related to the use of SyncCardia total artificial heart (TAH) remain limited. We aim to analyze adverse events related to the use of SynCardia TAH reported to the Food and Drug Administration (FDA)'s Manufacturers and User Defined Experience (MAUDE) database. Methods: We reviewed the FDA's MAUDE database for any adverse events involving the use of SynCardia TAH from 1/01/2012 to 9/30/2020. All the events were independently reviewed by three physicians. Results: A total of 1,512 adverse events were identified in 453 "injury and death" reports in the MAUDE database. The most common adverse events reported were infection (20.2%) and device malfunction (20.1%). These were followed by bleeding events (16.5%), respiratory failure (10.1%), cerebrovascular accident (CVA)/other neurological dysfunction (8.7%), renal dysfunction (7.5%), hepatic dysfunction (2.2%), thromboembolic events (1.8%), pericardial effusion (1.8%), and hemolysis (1%). Death was reported in 49.4% of all the reported cases (n=224/453). The most common cause of death was multiorgan failure (n=73, 32.6%), followed by CVA/other non-specific neurological dysfunction (n=44, 19.7%), sepsis (n=24, 10.7%), withdrawal of support (n=20, 8.9%), device malfunction (n=11, 4.9%), bleeding (n=7, 3.1%), respiratory failure (n=7, 3.1%), gastrointestinal disorder (n=6, 2.7%), and cardiomyopathy (n=3, 1.3%). Conclusions: Infection was the most common adverse event following the implantation of TAH. Most of the deaths reported were due to multiorgan failure. Early recognition and management of any possible adverse events after the TAH implantation are essential to improve the procedural outcome and patient survival.

• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.4
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• pp.837-842
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• 1998

Rett syndrome is a progressive neurological disorder that occurs exclusively in females. The syndrome is characterized by regression of language, motor development, and stereotypic hand movement. Autistic behavior, breathing irregularities, gait dyspraxia, scoliosis, and seizure are also accompanied. The cause of Rett syndrome is unknown, however, it is believed that the X-chromosome might playa significant role in the development of the syndrome. Patients with this syndrome have unusual oral and/or digital habits such as abnormal chewing pattern, bruxism, hypersalivation, micrognathia, high vaulted palate, tongue protrusion with lower posture of tongue, hand biting, digit-hand sucking. Dentists who are aware of distinct manifestations of Rett syndrome will be able to aid in early diagnosis and treatment of the syndrome. Prior to dental treatment for a patient with the Rett syndrome under sedation or general anesthesia, one should assess the degree of hypersalivation, apnea, severity of autism, expected life span. Early recognition of the syndrome and also dental treatment with established strict preventive guidelines for patients with the Rett syndrome may obviate the necessity of sedation or general anesthesia. Two cases with the Rett syndome were reported. Both patients had most of the above mentioned typical manifestations of the syndrome. Dental treatment for the case 1(8-year-old) including caries control, stainless steel crown, sealant application was performed under general anesthesia. The case 2 could not be undergone the dental treatment due to poor general conditions.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.23-29
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• 2018

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is a rare autosomal recessive urea cycle disorder characterized by hyperammonemia. CPS1D is caused by mutations in the CPS1 gene on chromosome 2q35. Based on the age of onset, there are two phenotypes: the neonatal type and the delayed-onset type. The severity of clinical manifestation depends on the degree of CPS1 residual enzymatic activity, and can result in hyperammonemia and neurological dysfunction. We report a case of CPS1D in a neonate who developed vomiting, decreased consciousness and hyperammonemia at 25th day after birth. She showed excellent response to treatment including hydration, ammonia-lowering drugs and a low-protein diet without hemodialysis. Her growth, development and neurological outcomes were fair at the last follow-up at 17 months of age.

• Korean Journal of Clinical Laboratory Science
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• v.38 no.3
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• pp.224-230
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• 2006

Up to now, there have been rare clinical studies on leaders and aerobics athletes. To get the useful data for protecting from auditory disorder, we selected 15 female aerobics leaders (experimental group) and 15 females (control group) unexperienced in aerobics and a without neurological and octolaryngological disorder. The average age was $34.87{\pm}8.80$ (experimental group) and $34.07{\pm}8.45$ (control group) years, and the average career of an aerobics leader (experimental group) was $8.33{\pm}4.73$ years. We measured the auditory evoked potential (AEP) of the two groups treated with 70, 75 and 85 dB intensity from January 2006 to May 2006 and analyzed the absolute latency (AL) and interpeak latency (IPL) by the SPSS/pc+ 12.0 program. In the comparison of the AL between the experimental group and the control group according to intensity, both ears treated with 70 and 75 dB had a significant difference (p<0.05) in the I, III, V wave and in the I, V wave respectively, and the experimental group treated with 85 dB showed a difference in the I, III, V wave (left ear) and in the I wave (right ear) respectively. The IPL of the two groups treated with various intensities had no prolongation. In the comparison of the AL between the experimental group and the control group according to ages, the experimental group in their 20s treated with 70 dB showed a significant difference (p<0.05) in the V wave (left ear) and in the I, III, V wave (right ear), and the experimental group in their 20s treated with 75 dB in the I, III wave (left ear) and in I, III, V wave (right ear), and experimental group in their 20s treated with 85 dB in the V wave (left ear) and in the III, V wave (right ear). The experimental group in their 30s treated with 70 dB had a significant difference (p<0.05) only in the V wave (right ear). Only in the IPL of subjects in their 20s treated with 85 dB, III-V and I-V of both ears was extended. In the comparison of the AL and IPL according to career, there was no significant difference between the two groups. From this results, we concluded that the lower sound intensity (70 dB) showed a more significant difference in the experimental group than the control group. We concluded that the leader of aerobics exposed to louder sounds than normal people are affected by auditory neurological and octolaryngological disorders. So we think that the leaders of aerobics need to control the noise level for protecting themselves against a disease.

• Korean Journal of Psychosomatic Medicine
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• v.29 no.2
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• pp.207-212
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• 2021

Anti-N-methyl-D-aspartate receptor (Anti-NMDAR) encephalitis is a neuroinflammatory disease mediated by autoantibodies to NMDAR. In the initial clinical stages of anti-NMDAR encephalitis, psychiatric symptoms like delusions, perceptual disturbances, and disorganized speech or behaviors are pronounced even without obvious neurological symptoms. Early treatments like immunotherapy and/or tumor removal are central to good clinical outcomes. Hence, it is important to diagnose early anti-NMDAR encephalitis, distinguishing it from mental disorder. In the present case study, the authors described psychiatric symptoms assessed with Positive and Negative Syndrome Scale (PANSS) of Ms. A, a 26-year-old woman, in the early phase of anti-NMDAR encephalitis. We will discuss the characteristic psychopathology of anti-NMDAR encephalitis toward prompt diagnosis and treatment. Ms. A showed a higher negative subscale score than positive one on the PANSS. Compared with mental disorder, negative symptoms and cognitive impairment would be more prominent in the early stage of anti-NMDAR encephalitis. Rituximab and teratoma removal were effective, and quetiapine showed good tolerability. It is recommended to evaluate anti-NMDAR encephalitis when negative symptoms, cognitive impairment, catatonia, changes in consciousness level, and neurological symptoms are observed, especially in young women.

• Therapeutic Science for Rehabilitation
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• v.4 no.1
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• pp.63-73
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• 2015

Objective : The purpose of this study was to investigate the effects of sensory diet home program for children with autism spectrum disorder who have sensory defensiveness and explore how this intervention affect to sensory modulation and social interaction ability. Methods : The subject of this case study was a 12-year-old girl with autism spectrum disorder who was struggling with her sensory modulation problem. Parent interview and evaluations were conducted and subject-tailored sensory diet home program was made by the therapist. Parent education for the home program was provided to parents and everyday, 3 times, 30minutes of home program activities were conducted to the child for 23 days. Adolescent/Adult Sensory Profile and Social Maturity Scale were measured at pre and post intervention and parent wrote daily observation record about child during the intervention. Results : After the intervention, Low Registration, Sensory Sensitivity, Sensation Avoiding area of Adolescent/Adult Sensory Profile included to the normal range and the scores of the Social Maturity Scale improved. Conclusions : Child's sensory defensiveness was relieved and sensory modulation and controling emotion ability were improved. While there was significant improvement for sensory modulation and social interaction ability, this was a case study and there was only one subject. So we need to study with larger sample size to generalize this intervention's effectiveness.

• Therapeutic Science for Rehabilitation
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• v.6 no.1
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• pp.45-54
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• 2017

Objective : The purpose of this study was to investigate the effects of an Interactive Metronome on increasing attention and impulsivity control for children with attention deficit hyperactivity disorder(ADHD). Methods : This study was conducted through the ABA'design among single-case research methods with one child with ADHD. From May to July, 2014, 3 sessions of baseline phase(A), 15 sessions of intervention phase(B), and 3 sessions of post baseline(A') were conducted. The intervention was IM training, and each session carried out short form assessment(SFA) of IM and concentration tasks. In addition, long form assessment(LFA) of IM and Self-Control Rating Scale (SCRS) were conducted at baseline, intervention and post baseline. Results : After IM training as the intervention, the participation showed an increase in attention and controled in impulsivity. After the intervention, it was demonstrated that the effects were maintained or improved in the post baseline. Conclusions : IM training can be useful for increasing attention and impulsivity control of children with ADHD. The results demonstrated the efficacy of IM training as a new approach for children with ADHD.

• Nuclear Medicine and Molecular Imaging
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• v.40 no.6
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• pp.302-308
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• 2006

Purpose: Although several neuroanatomical models of panic disorder have been proposed, little is known regarding the neurological mechanisms underlying cognitive-behavioral therapy (CBT) in patients with panic disorder. This study was performed to identify the brain structures that show changes of regnioal cerebral blood flow (rCBF) after CBT in patients with panic disorder. Materials and Methods: Seven patients who were diagnosed as panic disorder by DSM-IV were treated with CBT for 8 weeks and twelve healthy volunteers joined in this study. Serial $^{99m}Tc-ECD$ brain perfusion SPECT images were acquisited and PDSS-SR (Self-Report version of Panic Disorder Severity Scale) and ACQ (Agoraphobic Cognitive Question) scores were measured just before and after CBT in all patients. Data were analyzed using SPM2. Results: Subjective symptoms were improved, and PDSS-SR and ACQ scores were significantly reduced ($14.9{\pm}3.9\;vs.\;7.0{\pm}1.8$, p<0.05; $30.3{\pm}8.5\;vs.\;21.6{\pm}3.4$, p<0.05, respectively) after CBT in panic patients. Before CBT, a significant increase of rCBF was found in the cingulate gylus, thalamus, midbrain, both medial frontal and temporal lobes of the panic patients compared to the normal volunteers. After CBT, we observed a significant rCBF decrease in the left parahippocamus, right insula and cingulate gyrus, both frontal and temporal lobes, and a significant rCBF increase in both the occipital lobes, left insula, both frontal and left parietal lobes. Conclusion: These data suggested that CBT is effective for panic disorder and diminish the activity of the brain areas associated with fear in panic disorder.

• Therapeutic Science for Rehabilitation
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• v.7 no.4
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• pp.57-66
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• 2018

Objective: The purpose of this study is to investigate the effect of interactive metronome (IM) training on attention among children on the autistic spectrum. Methods: This is a single case study, using ABA design, of one child with autism spectrum disorder (ASD) who had attentional difficulties. A total of 20 sessions were conducted: 3 baseline sessions (A), 14 intervention sessions (B), and 3 follow-up baseline sessions (A'). During the intervention period (B), IM training was performed. Inattention and attention activities were measured as the dependent variables in all three phases (A, B, and A'). Results: Compared to baseline, the subject's inattention decreased in the IM training mediation period, and the performance of activities requiring attention also improved. The intervention effect was maintained even during the follow-up baseline period. Conclusion: The results of this study suggest that IM training positively influences attention among children with ASD with attention deficit symptoms. Further, it provides the clinical basis of IM training as an intervention for children with ASD.

• The Journal of Internal Korean Medicine
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• v.39 no.4
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• pp.802-813
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• 2018

This case report describes a patient with olivopontocerebellar atrophy accompanied by sleep disorder and gait disturbance whose condition was improved by treatment with Korean medicine. The 61-year-old woman, who was diagnosed with olivopontocerebellar atrophy (Multiple Systemic Atrophy-Cerebellum), was admitted to hospital twice and treated with Korean medicine (acupuncture and herbal medicine) and rehabilitation. The Korean medicine was Gwibiondam-tang-gami and Jaeumgeonbi-tang-gami. Clinical symptoms were assessed by the Modified Bathel index, functional independent measurement, Berg balance scale, and Unified Multiple System Atrophy rating scale. A brain MRI at the one-year follow up after onset showed similar progress but clinical symptoms were improved after treatment, and the evaluation index score increased. Multiple system atrophy, a type of degenerative neurological disease, has no targeted treatment. In this situation, although this report describes a single case, Korean medicine treatment could provide a meaningful improvement in the sleep disturbance and gait disorder symptoms of patients with olivopontocerebellar atrophy.