• Journal of The Korean Society of Inherited Metabolic disease
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• v.22 no.2
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• pp.58-62
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• 2022

Pompe disease (PD) is an autosomal recessive genetic disorder caused by a deficiency of the lysosomal enzyme acid α-glucosidase (GAA). It is easy to hastily diagnose as patients if they have two pathogenic variants. Clinical pathologists misdiagnosed our infant and her mother as PD. Here, we report a case of pseudodeficiency in a potential late-onset Pompe disease (LOPD) carrier with a double dual variant, each in cis formation in a 3-month infant. The person who has two pathogenic variants was diagnosed as a carrier, not a patient. It was first reported in Korea. The patient had: two likely pathogenic heterozygous mutations on exon #4: c.752C>T (p.Ser251Leu), c.761C>T (p.Ser254Leu), and a heterozygous mutation on exon #12: c.1726G>A (p.Gly576Ser), also with a heterozygous mutation on exon #15: c.2065G>A (p.Glu689Lys). By presenting this case we emphasize the possibility of cis formation of genes which may cause pseudodeficiency, and potential LOPD carrier form. Hereby we suggest that thorough evaluation of GAA gene is essential among whom initially diagnosed as PD.

• Journal of the Korean Orthopaedic Association
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• v.54 no.4
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• pp.327-335
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• 2019

Purpose: To investigate the radiological efficacy of polymethylmethacrylate (PMMA) augmentation of pedicle screw operation in osteoporotic vertebral compression fractures (OVCF) patients. Materials and Methods: Twenty OVCF patients, who underwent only posterior fusion using pedicle screws with PMMA augmentation, were included in the study. The mean follow-up period was 15.6 months. The demographic data, bone mineral density (BMD), fusion segments, number of pedicle screws, and amount of PMMA were reviewed as medical records. To analyze the radiological outcomes, the radiologic parameters were measured as the time serial follow-up (preoperation, immediately postoperation, postoperation 6 weeks, 3, 6 months, and 1 year follow-up). Results: A total of 20 patients were examined (16 females [80.0%]; mean age, 69.1±8.9 years). The average BMD was -2.5±0.9 g/cm². The average cement volume per vertebral body was 6.3 ml. The mean preoperative Cobb angle of focal kyphosis was 32.7°±7.0° and was improved significantly to 8.7°±6.9° postoperatively (p<0.001), with maintenance of the correction at the serial follow-up, postoperatively. The Cobb angle of instrumented kyphosis, wedge angle, and sagittal index showed similar patterns. In addition, the anterior part of fractured vertebral body height averaged 11.0±5.0 mm and was improved to 18.5±5.7 mm postoperatively (p=0.006), with maintenance of the improvement at the 3-month, 6-month, and 1-year follow-up. Conclusion: The reinforcement of pedicle screws using PMMA augmentation may be a feasible surgical technique for OVCF. Moreover, it appears to be appropriate for improving the focal thoracolumbar/lumbar kyphosis and is maintained well after surgery.

• Journal of the Korean Orthopaedic Association
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• v.54 no.2
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• pp.164-171
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• 2019

Purpose: A dedifferentiated chondrosarcoma is a rare lethal tumor characterized by a low grade chondrosarcoma juxtaposed with a high grade dedifferentiated sarcoma, such as osteosarcoma, fibrosarcoma. The aim of our study was to document the clinical manifestation and oncologic outcomes of a dedifferentiated chondrosarcoma. Materials and Methods: This study identified 11 patients who were diagnosed and treated for dedifferentiated chondrosarcoma between January 2007 and December 2016. The identified cohort was then reviewed regarding age, sex, symptom onset, tumor location, magnetic resonance imagings (MRIs), surgical margin, and pathologic diagnosis. The time to local recurrence and/or metastasis, follow-up duration, and the patients' final status were analyzed. Results: The patients were comprised of 7 males and 4 females with a mean age of 54 years (range, 33-80 years). The location of the tumor was in the femur in 6 cases, pelvis in 4 cases, and metatarsal in 1 case. The average tumor diameter was 12.7 cm (range, 6.0-26.1 cm). At the time of diagnosis, 2 patients showed pathologic fracture; 1 patient was Enecking stage IIA, 9 patients were stage IIB, and 1 patient was stage III. Eight patients were classified as a primary dedifferentiated chondrosarcoma and 3 patients were secondary. One of the primary lesions was misinterpreted initially as a low grade chondroid lesion by MRI and underwent curettage. Local recurrence occurred in 8 cases and distant metastasis occurred in 10 cases with a mean duration of 8 months (range, 2-23 months) and 7 months (range, 1-32 months), respectively. The three-year overall survival of patients with dedifferentiated chondrosarcoma was 18%, and 10 patients died due to disease progression. Conclusion: Dedifferentiated chondrosarcoma developed lung metastases in the early period of the clinical courses and the prognosis was dismal.

• Korea Science and Art Forum
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• v.28
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• pp.105-116
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• 2017

This study is a basic research to suggest user-centered general hospital environmental design guidelines, which aims to analyze user's behavior characteristics across generation in general hospital. For this purpose, this study constructed an analysis tool through the literature review with regard to generation and behavior characteristics in general hospital. Besides, an online survey regarding user's behavior in general hospital was conducted targeting from 20s to 60s, 300 persons for each group, total 1,500 persons for about 3 weeks since September 1, 2016. The results of this study are as follows: (1) Based on the generation, there were significant differences in relevant categories of their visiting frequency, visiting purpose, visiting hour, transportation, companion, behavior during the wait and selection of a general hospital. (2) In all generation, they responded that they have visited once or twice per year. People in 20s and 30s responded that their visit for the hospital is to receive specific treatment, while other people in 40s, 50s and 60s visit the hospital majorly for routine check-ups. Therefore, it is imperative for a health check-up center to design an environmental plan that reflects the characteristics of elders in 40s, 50s and 60s. (3) People in 40s, 50s and 60s usually visit a general hospital in the mornings of weekdays, while generations in 20s and 30s responded that they mostly visit the hospital in the mornings of weekend. (4) When they visit a general hospital, people in their 20s are usually using public transportations, while people in their 30s to 60s are using their own vehicle. (5) People in their 20s majorly visited 'lobby'. In older generations, they tend to visit 'outpatient clinic'. Therefore, it is necessary to build an outpatient clinic environment that considers the elderly. (6) Patients majorly responded that they are using their cell phone, while waiting for their clinic call. In elder generations, they responded that they are more likely watching TVs, reading books/magazines or doing nothing. Therefore, it is essential to provide cell-phone related services and environmental supports. Visually attractive media can be utilized for this purpose.

• Journal of Korean Academy of Dental Administration
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• v.5 no.1
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• pp.13-21
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• 2017

Objective: To understand the experience of medical accidents and disputes according to clinical career of dental hygienists. Methods: A self-administrated questionnaire survey was conducted from May 2012 to June 2012 targeting 313 dental hygienists in Gyeongsangnam-do, Republic of Korea. Data were analyzed by descriptive analysis, χ2-test, and one-way analysis of variance (ANOVA). Collected data were analyzed using SPSS 18.0. Results: 1) The group who had more than 6 years of clinical experience experienced complaints from patients (70.3%) and dental hygienists duty (30.7%); 2) Complaints were related to diagnosis, dental treatment instruments and materials, scaling, impression preparation, and prosthodontics. Pediatric patients had significantly more complaints compared to other patients. Overall, there were more frequent cases in the group with over 6 years of clinical career; 3) For the psychological state after experiencing patients' complaints, 'the process is difficult, but I forget after thinking it could be happen' had 160 respondents. Among them, 38.1% had clinical experience of more than 6 years, 37.5% had less than 3 years, and 24.4% had clinical career of 3~6 years; 4) About 73.6% of those had 3~6 years of clinical career answered that 'sometimes it is doubtful' on the issue of medical accidents and conflicts on prevention education of dental hygienists that 'there is a doubt about the issue of medical treatment or the occurrence of disputes'. About 60.0% of those who had less than 3 years of clinical experience answered that it was highly 'necessary but not urgent' on the necessity of preventive education, while 87.1% of those who had more than 6 years of clinical career answered that it was highly 'necessary but not urgent' on the necessity of preventive education. The difference between the two was not statistically significant. Conclusions: It is necessary and urgent to provide education related to prevention and countermeasures against malpractice disputes for dental hygienists.

• Korean Journal of Psychosomatic Medicine
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• v.31 no.1
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• pp.10-18
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• 2023

Objectives : The purpose of this study is to investigate the characteristics of patients referred for delirium and depressive disorder and to find direction of improvement of consultation-liason psychiatry in general hospital. Methods : We performed a retrospective computed chart review of the 4,966 inpatients hospitalized at Konkuk University Hospital who were referred to the Department of Psychiatry from August 1, 2005 to December 31, 2011. Results : Depressive disorder shows the order of frequency of consultation type Parallel-Complementary-Mending. Delirium shows the order of frequency of consultation type Mending-Parallel-Complementary. When comparing 'follow up consultation' and 'without follow up consultation' group within the depressive disorder, the proportion of men in the 'follow up consultation' group was higher. In the analysis of the consultation type, the 'follow up consultation' group showed the order of consultation type Parallel-Mending- Complementary, and type Parallel-Complementary-Mending in the 'without follow up consultation' group. When comparing 'follow up consultation' and 'without follow up consultation' group within the delirium, the proportion of the surgical field in the 'follow up consultation' group was higher. In the analysis of the consultation type, both group showed the order of consultation type Mending-Parallel- Complementary. Conclusions : Doctors in each department and psychiatrists should pay attention to delirium symptoms that may occur in surgical inpatients and preventive measures should be taken. Screening tests should be conducted in medical patients to properly evaluate coexisting psychiatric diseases. Risk factors of Delirium and Depressive disorder should be identified from the time of hospitalization, and actively discussing treatment plans and early interventions could improve the quality of medical services.

• Korean Journal of Social Welfare Studies
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• v.41 no.4
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• pp.189-224
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• 2010

Nowadays Korean society has a fast growing population of North Korean immigrant youth. Especially, North Korean immigrant youth, who are discontinuing formal education, experience difficulties in social exclusion and adaptation. Therefore, the purpose of this study was to understand the experiences of adaptation and social support of North Korean immigrant youth discontinuing formal education, and to seek affirmative supporting plans and solutions for their adaptation. The methodology utilized for this study was a case study research. On the basis of the results and categorization, 'a network between overall categories and of early adaptation' was derived. The major findings of this study are summarized as follows. First, the participants of this study experienced major difficulties during 'economical adaptation', and their economical adaptation was strongly interrelated with their 'educational adaptation'. The discontinuing of their education resulting from their fleeing period caused 'academic differential and age disparity.' Thus they could not enroll in the formal education system, and their insufficient educational background resulted in low wages and overwork due to manual labor jobs. Second, participants recognized 'interpersonal relations' as the most essential factor in adaptation, and 'social support' from the interpersonal relations played an important role in adaptation to and comfort in Korean society. 'Interactions with South Koreans' was recognized as a significant resource for gaining informational and appraisal support, but was not activated satisfactorily. On the other hand, interaction with North Korean immigrants was avoided by the reason that 'there's nothing to learn', however emotional support from North Korean immigrants of close relationship played an important role in participants' adaptation, especially in all aspects of psychological adaptation. Third, participants experienced a lot of difficulties and stresses in their 'physical health and psychological adaptation'. Their chronic diseases worsened by remaining untreated, and female perceived symptoms were observed more often than in male. Meanwhile, excessive 'stresses' from the unfamiliar environment of South Korea negatively affected their psychological adaptation. However, they were satisfied with sense of liberty and security from living in a democratic society.

• Korean Journal of Psychosomatic Medicine
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• v.31 no.2
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• pp.89-99
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• 2023

Objectives : In this study, elderly hospitalized patients aged 65 years or older who were admitted to a university hospital and referred to the department of psychiatry were classified into youngest-old, middle-old, and oldest-old. It was conducted to find out what factors contribute to the agitation pattern by age group, whether there is a difference in the factors, and whether there is a difference in the severity of the agitation pattern. Methods : From July 1, 2021 to December 31, 2021, the medical records of patients aged 65 years or older who were referred to the department of psychiatry were retrospectively reviewed. Age, gender, route of hospitalization, department of referral, reason for referral, treatment method, presence of internal and surgical diseases, and hematological test data were investigated. Results : There was a significant correlation with the RASS score in cases of surgery referral and high CRP levels for youngest-old aged 65 to 74 years, in cases of surgery referral, dementia and hyponatremia for middle-old aged 75 to 84 years, in case of dementia for oldest-old aged 85 years old or older. In addition, there were differences in the severity of agitation patterns between age groups. Conclusions : As the age group increases, the agitation of delirium patients becomes more severe, and various factors contributing to the agitation pattern also differ by age group. Therefore, when treating elderly patients with delirium, attention should be paid to factors that may affect agitation depending on age.

• Korean Journal of Psychosomatic Medicine
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• v.31 no.2
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• pp.165-172
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• 2023

Objectives : The purpose of this study is to determine whether there is a significant difference in the neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio according to the history of suicide attempt in patients with psychiatric diseases. Methods : A medical record review was conducted on patients who had been hospitalized in Konyang University Hospital since 2021-03-01 to 2023-02-28 to collect demographic and clinical characteristics. T-test for continuous variables and Chi-square test for categorical variables were performed to determine demographic differences according to the history of suicide attempt, and the ANCOVA test was performed to compare the average value of peripheral inflammatory marker according to the history of suicide attempt with gender and age as covariates. One-way variance analysis was performed to determine whether the number of suicide attempt causes significant difference of the peripheral inflammatory marker. Results : The final analysis target of this study was 266 patients, 101 had history of suicide attempt, and 165 had no history of suicide attempt. The neutrophil-to-lymphocyte ratio (p<0.001) and platelet-to-lymphocyte ratio (p<0.001) were higher in patients with the history of suicide attempt than patients without the history of suicide attempt, but the neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio were not significantly increased depending on the serial increase of number of suicide attempts. Conclusions : This study suggests that peripheral inflammatory markers are meaningful and easily accessible indicators for predicting the risk of suicide attempt in psychiatric patients. We expect that prospective follow-up study will be conducted with more subjects and controlled potential confounding variables.

• Pediatric Infection and Vaccine
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• v.30 no.3
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• pp.111-120
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• 2023

Purpose: Human rhinovirus (HRV) infections can result in lower respiratory tract infections (LRTIs). We aimed to investigate the characteristics of severe HRV LRTI in young children. Methods: Medical records were reviewed retrospectively in patients who were hospitalized for HRV LRTIs from 2016 to 2020 at the Samsung Medical Center in Seoul, Korea. Patients aged 90 days or older and younger than 5 years were included. Patients with co-infections with other respiratory pathogens were excluded. Severe HRV LRTI was defined as the following: the need for high-flow oxygenation, mechanical ventilation, or intensive care unit admission. Results: A total of 115 cases were identified. The median age was 17 months (range, 3-56 months) and the median hospital days were 4 days (range, 2-31 days). Of the 115 cases, 18 patients (15.7%) developed severe HRV LRTI. The median age was younger in the severe group compared to the non-severe group (9.5 months vs. 19.0 months, P=0.001). Of 18 patients with severe HRV LRTI, 11 (61.1%) had underlying diseases - chronic lung diseases accounted for the largest proportion (63.6%). Six patients (33.3%) required mechanical ventilation. Of note, 7 previously healthy children were diagnosed with severe HRV LRTI. Of those 7 children, 4 of them were diagnosed with asthma later. When the 115 cases were divided into previously healthy (n=60) and underlying disease (n=55) groups, severe courses of HRV LRTI were observed in 11.7% and 20.0% of children, respectively (P=0.219). Conclusions: HRV can cause severe LRTI even in previously healthy children as well as in children with comorbidities.