• Annals of Clinical Neurophysiology
• /
• v.11 no.2
• /
• pp.74-77
• /
• 2009

We report a 23-year-old woman with adult Sandhoff disease, who presented with motor neuron disease phenotype. The patient had experienced progressive motor weakness in four extremities since 1 year prior to admission. Electrophysiological study revealed wide-spread denervation potentials, and the assay of total hexosaminidase involving A and B activities showed decreased levels of these activities, which was consistent with Sandoff disease. This is the first Korean case of adult Sanhoff disease presented as a motor neuron disease phenotype.

• International Journal of Advanced Culture Technology
• /
• v.9 no.4
• /
• pp.169-179
• /
• 2021

This study was conducted to develop and apply clinical nursing practice training simulation program using Standardized Patient for Nursing Students focused on infectious respiratory disease. This study is descriptive methodological study. Through prior consideration of documents and educational task of infectious respiratory disease was conducted with interview of clinical specialists of infection control managers. Development of educational task for infectious respiratory disease for Nursing Students went through the content validity. Finally, 10 educational tasks are developed 'knowledge of respiratory infections disease', 'hand washing', 'put on mask', 'lead to put on mask to patients and caregiver', 'intravenous injection via 3way', 'surgical aseptic technique', 'sterilization medical instrument', 'management of contaminated linen', 'infected personnel management manual'. The infectious respiratory disease simulation program was developed based on the ADDIE model and proceeded to 4 steps of analysis, design, development, implementation. The infection control education program included lectures (20 min), skill training (20 min), simulation using standardized patient (20 min), and debriefing (40 min), The collected data were analyzed by descriptive statistics with SPSS program for version 23.0. The results of this study confirmed that the clinical nursing practice training simulation program using standardized patients was effective in infectious respiratory disease of the nursing college students in knowledge of infectious respiratory disease and clinical nursing performance. we found this practical training program for nursing college students to improve knowledge and clinical competency of infection control. we expected that this developed program could be applied to practical training for various infectious control.

• Nuclear Medicine and Molecular Imaging
• /
• v.42 no.sup1
• /
• pp.166-171
• /
• 2008

PET of the cerebral metabolic rate of glucose is increasingly used to support the clinical diagnosis in the examination of patients with suspected major neurodegenerative disorders, such as Alzheimer's disease. $^{18}F-FDG$ PET has been reported to have high diagnostic performance, especially, very high sensitivity in the diagnosis and clinical assessment of therapeutic efficacy. According to clinical research data hitherto, $^{18}F-FDG$ PET is expected to be an effective diagnostic tool in early and differential diagnosis of Alzheimer's disease. Since 2004, Medicare covers $^{18}F-FDG$ PET scans for the differential diagnosis of fronto-temporal dementia (FTD) and Alzheimer's disease (AD) under specific requirements; or, its use in a CMS approved practical clinical trial focused on the utility of $^{18}F-FDG$ PET in the diagnosis or treatment of dementing neurodegenerative diseases.

• Genomics & Informatics
• /
• v.16 no.4
• /
• pp.26.1-26.12
• /
• 2018

Shigella spp. constitutes some of the key pathogens responsible for the global burden of diarrhoeal disease. With over 164 million reported cases per annum, shigellosis accounts for 1.1 million deaths each year. Majority of these cases occur among the children of the developing nations and the emergence of multi-drug resistance Shigella strains in clinical isolates demands the development of better/new drugs against this pathogen. The genome of Shigella flexneri was extensively analyzed and found 4,362 proteins among which the functions of 674 proteins, termed as hypothetical proteins (HPs) had not been previously elucidated. Amino acid sequences of all these 674 HPs were studied and the functions of a total of 39 HPs have been assigned with high level of confidence. Here we have utilized a combination of the latest versions of databases to assign the precise function of HPs for which no experimental information is available. These HPs were found to belong to various classes of proteins such as enzymes, binding proteins, signal transducers, lipoprotein, transporters, virulence and other proteins. Evaluation of the performance of the various computational tools conducted using receiver operating characteristic curve analysis and a resoundingly high average accuracy of 93.6% were obtained. Our comprehensive analysis will help to gain greater understanding for the development of many novel potential therapeutic interventions to defeat Shigella infection.

• The Korean Journal of Physiology and Pharmacology
• /
• v.18 no.6
• /
• pp.447-456
• /
• 2014

Until now, a disease-modifying therapy (DMT) that has an ability to slow or arrest Alzheimer's disease (AD) progression has not been developed, and all clinical trials involving AD patients enrolled by clinical assessment alone also have not been successful. Given the growing consensus that the DMT is likely to require treatment initiation well before full-blown dementia emerges, the early detection of AD will provide opportunities to successfully identify new drugs that slow the course of AD pathology. Recent advances in early detection of AD and prediction of progression of the disease using various biomarkers, including cerebrospinal fluid (CSF) $A{\beta}_{1-42}$, total tau and p-tau181 levels, and imagining biomarkers, are now being actively integrated into the designs of AD clinical trials. In terms of therapeutic mechanisms, monitoring these markers may be helpful for go/no-go decision making as well as surrogate markers for disease severity or progression. Furthermore, CSF biomarkers can be used as a tool to enrich patients for clinical trials with prospect of increasing statistical power and reducing costs in drug development. However, the standardization of technical aspects of analysis of these biomarkers is an essential prerequisite to the clinical uses. To accomplish this, global efforts are underway to standardize CSF biomarker measurements and a quality control program supported by the Alzheimer's Association. The current review summarizes therapeutic targets of developing drugs in AD pathophysiology, and provides the most recent advances in the clinical utility of CSF biomarkers and the integration of CSF biomarkers in current clinical trials.

• Clinical and Experimental Pediatrics
• /
• v.56 no.8
• /
• pp.323-326
• /
• 2013

Quality of life in addition to various medical problems in children with end-stage renal disease (ESRD) should be objectively assessed to accomplish normal growth and development during childhood. However, unfortunately, studies of quality of life (QoL) in children with ESRD have been not popular yet and there are only fewer suitable assessment tools compared with adults. Recently, disease-specific modules to evaluate QoL in children with chronic disease such as ESRD have been developed. This review was made to introduce these QoL instruments for children and help the clinical application of them.

• The korean journal of orthodontics
• /
• v.53 no.3
• /
• pp.150-162
• /
• 2023

Objective: To investigate craniofacial differences in individuals with hypodontia and explore the relationship between craniofacial features and the number of congenitally missing teeth. Methods: A cross-sectional study was conducted among 261 Chinese patients (males, 124; females, 137; age, 7-24 years), divided into four groups (without hypodontia: no teeth missing, mild: one or two missing teeth, moderate: three to five missing teeth, severe: six or more missing teeth) according to the number of congenitally missing teeth. Differences in cephalometric measurements among the groups were analyzed. Further, multivariate linear regression and smooth curve fitting were performed to evaluate the relationship between the number of congenitally missing teeth and the cephalometric measurements. Results: In patients with hypodontia, SNA, NA-AP, FH-NA, ANB, Wits, ANS-Me/N-Me, GoGn-SN, UL-EP, and LL-EP significantly decreased, while Pog-NB, AB-NP, N-ANS, and S-Go/N-Me significantly increased. In multivariate linear regression analysis, SNB, Pog-NB, and S-Go/N-Me were positively related to the number of congenitally missing teeth. In contrast, NA-AP, FH-NA, ANB, Wits, N-Me, ANS-Me, ANS-Me/N-Me, GoGn-SN, SGn-FH (Y-axis), UL-EP, and LL-EP were negatively related, with absolute values of regression coefficients ranging from 0.147 to 0.357. Further, NA-AP, Pog-NB, S-Go/N-Me, and GoGn-SN showed the same tendency in both sexes, whereas UL-EP and LL-EP were different. Conclusions: Compared with controls, patients with hypodontia tend toward a Class III skeletal relationship, reduced lower anterior face height, flatter mandibular plane, and more retrusive lips. The number of congenitally missing teeth had a greater effect on certain characteristics of craniofacial morphology in males than in females.

• The Journal of Internal Korean Medicine
• /
• v.39 no.4
• /
• pp.612-623
• /
• 2018

Objective: The aim of this research was to investigate the current status and limitations of the clinical use of a pulse tonometry device (PTD). Methods: We searched online medical databases, including Oriental Medicine Advanced Searching Integrated System (OASIS), Research Information Sharing Service (RISS), DataBase Periodical Information Academic (DBpia) and PubMed, for clinical studies of metabolic diseases that used PTD. We selected articles on the clinical application of the PTD but excluded duplicate articles, clinical studies without the PTD, and non-clinical studies. In the first screening, 551 articles were selected. Ultimately, we found 10 articles and classified the articles according to the following diseases: hyperlipidemia, diabetes, overweight and obesity, hypertension, and metabolic disease. Results: Of the 10 selected articles, 4 were focused on hypertension. Two articles were focused on overweight or obesity and two articles were focused on the metabolic disease. Conclusions: This study was the first attempt to conduct a systematic review on clinical studies of metabolic disease using the PTD as a primary outcome. We suggest that the standard of pulse wave parameters and operating procedures for the PTD should be further developed. The results of this study are expected to be used as basic information for the planning and progression of clinical studies of metabolic syndrome using PTD.

• Quality Improvement in Health Care
• /
• v.10 no.2
• /
• pp.204-215
• /
• 2003

Background : ICD-10 Classification, which is used domestically as well as internationally, has limited use in the clinical practice since it is developed for at disease statistics and epidemiology. Therefore, the purposes of this study were to improve the quality of diagnosis by constructing a new disease classification based on the diagnoses doctors currently make in the clinical setting and connecting this classification with OCS and EMR, and to meet the demands of doctors for high quality medical study data in medical research. Methods : The specialists in each ophthalmic subfield collected clinical diagnoses and abbreviations based on the ophthalmology textbooks and confirmed the classifications. Total number of clinical diagnoses collected was totaled 672, for which ideal diagnoses had been selected and a new model of disease classification model in connection with ICD-10 was constructed. The constructed classification of clinical diagnoses consisted of six steps: the first step was the classification by ophthalmic subspecialty field; the second to fifth steps were the detailed classification by each specialty field; the sixth step was the classification by site. Results : After introducing the new disease classification, research on the use and a pre-post comparison was conducted. The result from the research on the use of the clinical diagnoses in inpatient and outpatient care has shown a gradually increasing tendency. From the pre-post comparison of EMR discharge summary diagnoses, the result demonstrated that the diagnosis was stated correctly and in detail. Since the diagnosis was stated correctly, code classification became correct as well, which makes it possible to construct high quality medical DB. Conclusion : This construction of clinical diagnoses provides the medical team with high quality medical information. It is also expected to increase the accuracy and efficiency of service in the department of medical record and department of insurance investigation. In the future, if hospitals wish to construct a classification of clinical diagnosis and a standard proposal of clinical diagnosis is presented by a medical society, the standardization of diagnosis seems to be possible.

• Annals of Clinical Neurophysiology
• /
• v.5 no.1
• /
• pp.11-15
• /
• 2003

Backgrounds: Kennedy disease is a X-linked recessive disease characterized by bulbar symptoms, proximal muscle weakness, and gynecomastia. Methods: We analyzed clinical symptoms and performed electrodiagnostic studies on 6 patients. Results: We found following features: 1) proximal muscle weakness 2) bulbar symptoms, as dysarthria, facial and tongue atrophy 3) hyporeflexia or areflexia 4) fasciculations, predominantly on face, and proximal upper extremities 5) decreased sensory nerve action potentials(SNAPs) 6) chronic neurogenic changes in needle EMG. Conclusions: Kennedy disease is characterized by degenerative process of anterior horn cell and dorsal root ganglion without upper motor neuron dysfunction. Increased triple nucleotide CAG repeats(>38) in androgen receptor gene of Xp21 will confirm early stage of this disease.