• 보건행정학회지
• /
• 제23권4호
• /
• pp.376-386
• /
• 2013

Purpose: The purpose of study is to estimate the number of chronic disease patients and medical care expenditure at the time baby-boomers belong to 65 years old aging population, and compare with current 65 year-old aging population. Methods: Analysis method used an estimating formula devised by the researcher and estimated the number of chronic disease patients and medical care expenditure of each generation. Results: When comparing the estimated number of chronic diseases patients of each generation, 40.6% of the first generation, 76.4% of the second generation, 95.2% of third generation are expected to get chronic disease. When comparing each generation's total medical care expenditure, based on the estimated number of chronic diseases patients of each generation, the second generation( 1,206,251,224 thousand won) showed higher than other generation. This study compared the number of chronic disease patients and medical care expenditure between the second generation of the elderly and current elder generation. As a result, the second generation patients was higher than the fourth generation in high blood pressure, diabetes, psychological and behavioral disorder, and neurological diseases whereas the fourth generation is only high the number of patients in heart disease. As for total medical care expenditure, the second generation paid more in high blood pressure, psychological and behavioral disorder while the fourth generation in neurological disease and heart disease. Conclusion: It is desired that considering the number of chronic disease patients and medical care expenditure of baby boomers accounting for 14.6% of total population, in-depth follow-up study is carried out that inquires into what are issues with a current chronic disease management project, what business is needed in order to manage these issues, and how to fund to cover increasing medical care expenditure.

• Genomics & Informatics
• /
• 제20권3호
• /
• pp.30.1-30.9
• /
• 2022

Hereditary spastic paraplegia is a not common inherited neurological disorder with heterogeneous clinical expressions. ALDH18A1 (located on 10q24.1) gene-related spastic paraplegias (SPG9A and SPG9B) are rare metabolic disorders caused by dominant and recessive mutations that have been found recently. Autosomal recessive hereditary spastic paraplegia is a common and clinical type of familial spastic paraplegia linked to the SPG11 locus (locates on 15q21.1). There are different symptoms of spastic paraplegia, such as muscle atrophy, moderate mental retardation, short stature, balance problem, and lower limb weakness. Our first proband involves a 45 years old man and our second proband involves a 20 years old woman both are affected by spastic paraplegia disease. Genomic DNA was extracted from the peripheral blood of the patients, their parents, and their siblings using a filter-based methodology and quantified and used for molecular analysis and sequencing. Sequencing libraries were generated using Agilent SureSelect Human All ExonV7 kit, and the qualified libraries are fed into NovaSeq 6000 Illumina sequencers. Sanger sequencing was performed by an ABI prism 3730 sequencer. Here, for the first time, we report two cases, the first one which contains likely pathogenic NM_002860: c.475C>T: p.R159X mutation of the ALDH18A1 and the second one has likely pathogenic NM_001160227.2: c.5454dupA: p.Glu1819Argfs Ter11 mutation of the SPG11 gene and also was identified by the whole-exome sequencing and confirmed by Sanger sequencing. Our aim with this study was to confirm that these two novel variants are direct causes of spastic paraplegia.

• 정신신체의학
• /
• 제24권1호
• /
• pp.28-42
• /
• 2016

연구목적 19세기말부터 근래까지 정신과 및 응급실에서 많이 볼 수 있었던 히스테리아(전환장애)환자를 이제는 더 이상 보기 어려워졌다. 이러한 현상의 원인을 규명해보고자 하였다. 방 법 정신과 외래 및 입원환자의 수가 지난 12년 동안 과연 얼마나 되는지 병록지를 조사하고 증상의 변화, 증상지속기간을 조사하였으며, 지난 2년간 응급실에서 정신과진료에서 본 전환장애 환자수를 조사하였다. 이 결과를 과거의 연구 결과와 비교해보았다. 이를 토대로 히스테리아(전환장애)의 소실 및 증상 변화의 원인을 찾아보았다. 결 과 히스테리아(전환장애)의 환자수가 현저히 줄어들었으며 그 증상도 변화된 것을 확인하였다. 그리고 이러한 변화는 불과 수십 년 사이에 일어났다. 그 간의 연구결과를 통하여 신경질환 진단법의 발달, 진단명과 증상기재 용어의 변화, 감정자극에 따른 뇌기능의 변환, 그리고 진화론적 관점 등이 이 병의 감소 내지는 증상의 변화를 설명할 수 있다. 결 론 히스테리아(전환장애)의 소실 및 증상의 변화가 불과 수십 년 동안에 일어날 수 있는 것은 진화적으로 변화하는 뇌와 환경 사이의 부조화에 따른 충격이 반응으로 나타난다는 가설을 통하여 이유의 많은 부분이 설명된다. 그리고 비록 과거의 히스테리아(전환장애) 환자수는 줄었으나 그 모습이 변화된 환자수는 결코 적지 않다. 정신과의사는 이들 환자에게 적절한 치료를 제공해 주기 위해서 일차 진료의사와 신경과의사와 협동해야 하며 주도적인 역할이 필요하다. 현재 사용하는 진단명이나 증상 기재 용어는 그 기본 내용이나 개념이 일부 변화했을 뿐, 뇌기능의 장애와 연관된 원인 불명의 신체증상이라는 점에서 달라진 바가 없다고 생각한다. 이에 저자는 과거의 용어인 히스테리아(전환장애)를 부활시키는 것이 타당하다는 점을 설명하였다.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
• /
• 제12권1호
• /
• pp.149-156
• /
• 2001

베타 용혈성 A군 연구균(group A beta-hemolytic streptococcus)에 의한 류마치스성 열 이후에 Sydenham 무도병이 발현되는 환자 군에서 강박 증상 또는 틱 증상이 나타난다는 보고가 있은 후, A군 연구균 감염과 강박 장애 또는 틱 장애 사이의 관계가 주목받게 되었다. A군 연구균 감염 후에 강박 증상 또는 틱 증상이 유발되는 환자들은 갑작스런 발병과 극적인 증상 악화를 보이고 사춘기 이전에 발병하며 무도병 모양의 운동 및 독특한 양상의 운동 과잉 등의 신경학적 이상을 보이고 또 그 경과가 악화 및 완화를 반복하는 양상을 보인다. 이런 임상적 특징을 보이는 소아 환자 군을 PANDAS(pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections)라고 부르게 되었다. 이후 현재까지 강박 장애와 틱 장애뿐 아니라 주의력 결핍/과잉운동 장애, 신경성 식욕부전증, 신체이형장애 환자들이 PANDAS 범주에 속한다는 보고들이 있어 왔다. 본 증례는 7세까지 정상적인 발달을 보이다가 A군 연구균 감염 수개월 후부터, 인지 기능, 사회성, 언어 및 의사소통에 장애를 보이고 틱 증상과 함께 얼굴과 손발에 이상 운동을 보이는 환자를 기술하였다. 이에 저자들은 본 증례가 소아기 붕괴성 장애의 양상을 보이는 PANDAS 증례라고 생각되어 이를 문헌 고찰과 함께 보고함으로써, PANDAS가 틱 장애, 강박 장애, 주의력 결핍/과잉운동 장애, 신경성 식욕부전증, 그리고 신체이형장애 등의 양상을 보일 뿐 아니라 소아기 붕괴성 장애의 양상을 보일 수 있다는 사실을 보고하고자 한다.

• 성인간호학회지
• /
• 제20권3호
• /
• pp.489-499
• /
• 2008

Purpose: The purpose of this study was to assess the importance and contribution of 9 nursing outcomes and their indicators that could be applied to cerebrovascular patients. Methods: Data were collected from 175 neurosurgical nurses working at two university affiliated hospitals and five secondary hospitals located in Gwang-ju. The Fehring method was used to estimate outcome content validity(OCV) and outcome sensitivity validity(OSV) of nursing outcomes and their indicators. Stepwise regression was used to evaluate relationship between outcome and its indicators. Results: The core outcomes identified by the OCV were Tissue Perfusion: Cerebral, Nutritional Status, Neurological Status, and Wound Healing: Primary Intention, whereas highly supportive outcomes identified by the OSV were Oral Health, Self-Care: ADL, and Nutritional Status. All the critical indicators selected for Fehring method were not included in stepwise regression model. By stepwise regression analysis, the indicators explained outcomes from 19% to 52% in importance and from 21% to 45% in contribution. Conclusion: This study identified core and supportive outcomes and their indicators which could be useful to assess the physical status of cerebrovascular patients. Further research is needed for the revision and development of nursing outcomes and their indicators at neurological nursing area.

• Child Health Nursing Research
• /
• 제25권3호
• /
• pp.324-332
• /
• 2019

Purpose: Epilepsy is the most common neurological disorder in childhood. Hospital nurses, who are the first to recognize seizures in epilepsy patients in the ward environment, possess expertise related to epilepsy and play a central role in epilepsy management. The purpose of this study was to develop an algorithm-based education program and to improve nurses' knowledge and self-efficacy related to providing nursing care to children with epilepsy. Methods: The education program consisted of lectures on the definition, cause, classification, diagnosis, treatment, and nursing of epilepsy based on a booklet, as well as practice using an algorithm for nursing interventions when a child experiences a seizure. Twenty-seven nurses working at pediatric neurological wards and a pediatric emergency room participated in the education program. The data were analyzed using descriptive statistics and the paired t-test. Results: Nurses' knowledge and self-efficacy showed a statistically significant improvement after participation in the education program on nursing care for children with epilepsy. Conclusion: The application of this education program for hospital setting is expected to improve nurses' capability to care for children with epilepsy, thereby contributing to a higher quality of nursing.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
• /
• 제30권3호
• /
• pp.127-131
• /
• 2019

Many neurologic disorders manifest as psychiatric symptoms. Anti-N-Methyl-D-Aspartate (NMDA) receptor encephalitis is an autoimmune disease of the brain characterized by numerous neurological and psychiatric features. Despite being rare, its prevalence is rapidly increasing and early management is critical in ensuring successful and sustainable recovery. Therefore, the illness should be considered as a differential diagnosis when clinically assessing patients. This report presents a case of a female child who was hospitalized for acute psychiatric manifestations, which was later confirmed as anti-NMDA receptor encephalitis. She recovered relatively successfully after combined neurological and psychiatric treatment. This report provides information on the clinical course of early onset anti-NMDA receptor encephalitis, including treatment strategy and prognosis.

• Journal of mucopolysaccharidosis and rare diseases
• /
• 제5권1호
• /
• pp.1-7
• /
• 2021

Gaucher disease (GD, OMIM #230800 OMIM#230800) is a rare, autosomal recessive inherited metabolic disorder caused by mutation in GBA1 encoding the lysosomal enzyme, glucocerebrosidase. The deficiency of glucocerebrosidase leads to an accumulation of its substrate, glucosylceramide in macrophages of various tissues. Common clinical manifestations include cytopenia, splenomegaly, hepatomegaly, and bone lesions. The phenotype of GD is classified into three clinical categories: Type 1 (non-neuronopathic) is characterized by involvements on the viscera, whereas types 2 and 3 (neuronopathic) are associated with not only visceral symptoms but also neurological impairment, either severe in type 2 or variable in type 3. A diagnosis of GD can be confirmed by demonstrating the deficiency of acid glucocerebrosidase activity in leukocytes. Mutations in the GBA1 should be identified as they may be of prognostic value in some cases. Biomarkers including Chitotriosidase, CCL18, and glucosylsphingosine (lyso-GL1) are useful in diagnosis and treatment monitoring. Currently available disease-specific treatment in Korea consists of intravenous enzyme replacement therapy and substrate reduction therapy. For enhancing long-term prognosis, the onset of Parkinson's disease and Lewy body dementia, or the occurrence of a blood disease or cancer (hepatocellular carcinoma) should be monitored in older patients. The development of new strategies that can modify the neurological phenotype are expected, especially in Asia including Korea, where the prevalence of neuronopathic GD is relatively higher than that in western countries.

• 대한한방소아과학회지
• /
• 제24권2호
• /
• pp.1-12
• /
• 2010

Objectives Taking detailed patient history helps earlier diagnosis and treatment of developmental disability. In this study we analyzed the clinical questionnaire to find out the clinical characteristics of those with five-retardation, five-limpness, or five-stiffness. Methods The data was collected from 484 children under the age of six who have visited H oriental medicine clinic for developmental delay. The clinical questionnaire was filled out by their parents and the data was analyzed statistically. Results 436 children showed symptoms of five-retardation, 90 children suffered from five-stiffness, 54 children showed five-limpness and 7 children suffered from five-stiffness and five-limpness complex. Generally, boys had higher chance to show disease symptoms than the girls (2.32:1) and 40 children (8.26%) reported family history of developmental disability. Cerebral palsy ranks the most common familial disease, followed by developmental delay, mental retardation, autistic disorder and language disorder. Among the children we have studied, 285 children (63.19%) showed delayed unassisted walk while 192 children (42.57%) had language disorder. Also, 138 children (28.51%) had both walk and language disorders. The children in this study also showed delayed toilet training and half of them had little stranger anxiety when they were infants. It was also found that 120 children (24.79%) experienced epilepsy. This study reaffirmed that low birth weight, premature birth, and suffocation are major risks causing neurological damage. Conclusions They had history which including family history, problems at birth, epilepsy, face recognition, muscle tone disorder, delayed walking without assistance, language ability, and toilet training.

• 정신신체의학
• /
• 제12권2호
• /
• pp.103-121
• /
• 2004

감각 및 운동 이상 증상을 동반하면서 꾀병이 의심되는 환자를 가려내는 방법에 대해서 약술하고 몇 개의 증례를 예시하였다. 꾀병을 규명하기 위해서는 우선 전환 장애, 가장성 장애를 차례로 증명하여 제외시켜야 한다. 이를 위한 방법으로는 자세한 병력 수집과 조사, 면밀한 신경학적 검사, 심리검사, 장시간의 면담 그리고 광범위한 행동 관찰이 필요하다. 그러나 증상 형성의 동기나 의도의 본질을 파악하기란 어려워서 환자의 고백에 의해 알게되는 경우도 있다. 신경 심리검사를 포함한 임상심리 검사를 통하여 꾀병의 가능성을 가려내고, 질병행동 특성을 파악하거나 기계적인 측정도구를 이용하기도 한다. 그러나 아직 객관적으로 타당한 검사 방법은 부족하다. 최근, 통증 차단제를 이용한 진성(眞性) 통증 구별법은 객관적인 검사법의 하나이다. 사고나 보상 문제가 겹친 만성 통증 환자는 꾀병의 가능성이 엿보인다 하더라도 많은 수에서 시간 경과와 환경의 영향으로 인해서 정신사회적 합병증을 동반한다. 향후 임상 심리검사법이 발달되고 행동과 의도성에 관한 뇌 기능의 연구론 통해 신체 증상이나 행위의 진실성에 대한 구별력은 더욱 향상될 것이다.