Background: Multiple system atrophy (MSA) is a sporadic neurodegenerative disease characterized by various combinations of parkinsonism, cerebellar ataxia, autonomic dysfunction and pyramidal signs. Two clinical subtypes are recognized: MSA with predominant cerebellar ataxia (MSA-C) and MSA with predominant parkinsonism (MSA-P). The aim of this study was to compare pathological features between MSA-C and MSA-P. Methods: Two autopsy confirmed cases with MSA were included from the Pusan National University Hospital Brain Bank. Case 1 had been clinically diagnosed as MSA-C and case 2 as MSA-P. The severity of neuronal loss and gliosis as well as the glial and neuronal cytoplasmic inclusions were semiquantitatively assessed in both striatonigral and olivopontocerebellar regions. Based on the grading system, pathological phenotypes of MSA were classified as striatonigral degeneration (SND) predominant (SND type), olivopontocerebellar degeneration (OPC) predominant (OPC type), or equivalent SND and OPC pathology (SND=OPC type). Results: Both cases showed widespread and abundant α-synuclein positive glial cytoplasmic inclusions in association with neurodegenerative changes in striatonigral or olivopontocerebellar structures, leading to the primary pathological diagnosis of MSA. Primary age-related tauopathy was incidentally found but Lewy bodies were not in both cases. The pathological phenotypes of MSA were MSA-OPC type in case 1 and MSA-SND=OPC type in case 2. Conclusions Our data suggest that clinical phenotypes of MSA reflect the pathological characteristics.

Despite advancements in treating ruptured cerebral aneurysms, an aneurysmal subarachnoid hemorrhage (aSAH) is still a grave cerebrovascular disease associated with a high rate of morbidity and mortality. Based on the literature published to date, worldwide academic and governmental committees have developed clinical practice guidelines (CPGs) to propose standards for disease management in order to achieve the best treatment outcomes for aSAHs. In 2013, the Korean Society of Cerebrovascular Surgeons issued a Korean version of the CPGs for aSAHs. The group researched all articles and major foreign CPGs published in English until December 2015 using several search engines. Based on these articles, levels of evidence and grades of recommendations were determined by our society as well as by other related Quality Control Committees from neurointervention, neurology and rehabilitation medicine. The Korean version of the CPGs for aSAHs includes risk factors, diagnosis, initial management, medical and surgical management to prevent rebleeding, management of delayed cerebral ischemia and vasospasm, treatment of hydrocephalus, treatment of medical complications and early rehabilitation. The CPGs are not the absolute standard but are the present reference as the evidence is still incomplete, each environment of clinical practice is different, and there is a high probability of variation in the current recommendations. The CPGs will be useful in the fields of clinical practice and research.
Aneurysm ; Brain Ischemia ; Cerebrovascular Disorders ; Diagnosis ; Disease Management ; Hydrocephalus ; Intracranial Aneurysm ; Mortality ; Neurology ; Quality Control ; Rehabilitation ; Risk Factors ; Search Engine ; Subarachnoid Hemorrhage ; Surgeons

The National Health Information Standards Committee was established in 2004 in Korea. The practical subcommittee for laboratory test terminology was placed in charge of standardizing laboratory medicine terminology in Korean. We aimed to establish a standardized Korean laboratory terminology database, Korea-Logical Observation Identifier Names and Codes (K-LOINC) based on former products sponsored by this committee. The primary product was revised based on the opinions of specialists. Next, we mapped the electronic data interchange (EDI) codes that were revised in 2014, to the corresponding K-LOINC. We established a database of synonyms, including the laboratory codes of three reference laboratories and four tertiary hospitals in Korea. Furthermore, we supplemented the clinical microbiology section of K-LOINC using an alternative mapping strategy. We investigated other systems that utilize laboratory codes in order to investigate the compatibility of K-LOINC with statistical standards for a number of tests. A total of 48,990 laboratory codes were adopted (21,539 new and 16,330 revised). All of the LOINC synonyms were translated into Korean, and 39,347 Korean synonyms were added. Moreover, 21,773 synonyms were added from reference laboratories and tertiary hospitals. Alternative strategies were established for mapping within the microbiology domain. When we applied these to a smaller hospital, the mapping rate was successfully increased. Finally, we confirmed K-LOINC compatibility with other statistical standards, including a newly proposed EDI code system. This project successfully established an up-to-date standardized Korean laboratory terminology database, as well as an updated EDI mapping to facilitate the introduction of standard terminology into institutions.

BACKGROUND: Transfusion-related adverse reaction is detected based on patients' adverse signs or symptoms during or after transfusion. We analyzed the actual incidence of transfusion-related adverse reactions by investigating diagnosed cases among reported signs or symptoms, and reexamined our transfusion-related adverse reaction reporting system. METHODS: From January to June, 2015, there were 4,234 cases of transfusion and 18,191 units of blood product were used. During transfusion, patients' signs or symptoms were checked and reported by the medical team at least three times, 5 minutes after transfusion started, during transfusion, and after transfusion, using the electronic reporting system in the blood bank. A laboratory medicine doctor investigated reported signs or symptoms by reviewing patients' electronic medical records, diagnosed transfusion-related adverse reaction by textbook definition, and surveyed actual incidence. In addition, incidence of transfusion-related signs or symptoms and transfusionrelated adverse reaction by each blood product was determined. RESULTS: Out of 1,091 transfusion-related signs or symptoms, only 226 cases (20.71%) were diagnosed with transfusion-related adverse reaction. Among these, most common cases were febrile nonhemolytic reaction with incidence of 0.91%, followed by allergic reaction with 0.32%. The incidence of transfusion-related adverse reaction by each blood product was highest for leukocyte-reduced red blood cells 3.41% and apheresis platelets 2.59%. Febrile nonhemolytic reaction was mainly related to red blood cells and allergic reaction was mainly related to platelets. CONCLUSION: The actual incidence of transfusion-related adverse reaction was only 20% of transfusion-related signs or symptoms. Therefore, reforming the reporting system and transfusion-related clinical inspection and education are required.
Blood Banks ; Blood Component Removal ; Education ; Electronic Health Records ; Erythrocytes ; Hypersensitivity ; Incidence*

The Rh blood group D antigen is the most immunogenic of all antigens, next to ABO antigens. Anti-D immunization is clinically important since it may cause clinical problems, such as severe hemolytic transfusion reactions and hemolytic disease of the newborn. DEL is an extremely weak D variant that cannot be detected by basic serologic typing and is typed as D-negative without the absorption-elution techniques and RHD genotyping. Of the DEL phenotype, RHD (c.1227G>A) allelic variant is the most common in Korea. The DEL phenotype has been considered to carry only a few D antigens to induce anti-D immunization, but a few cases have reported that this allelic variant is capable of inducing anti-D immunization in a D-negative recipient, for which it is clinical significant. Herein, we present a case of primary anti-D alloimmunization in a RhD negative patient after receiving RHD (c.1227G>A) DEL red cell transfusion identified by serological and molecular tests, including RHD genotyping.
Erythrocyte Transfusion* ; Erythrocytes* ; Humans ; Immunization ; Infant, Newborn ; Korea ; Phenotype ; Transfusion Reaction

PURPOSE: The purposes of this study were to identify the level of health literacy and to determine its impact on disease-related knowledge and adherence to self-care in adults with hypertension. METHODS: This was a cross-sectional, descriptive study in which patients with hypertension were recruited from outpatient hypertension clinics in Seoul, Korea from December, 2009 to February, 2010. A total 186 patients completed series of questionnaires to assess health literacy, disease-related knowledge, and adherence to self-care. Hierarchical linear regression was used to determine whether health literacy was associated with disease-related knowledge and adherence to self-care. RESULTS: Seventy-one (38.2%) and seventy-two (38.7%) patients had inadequate and marginal health literacy, respectively. In hierarchical linear regression, health literacy independently predicted disease-related knowledge (beta= .43, p < .001) and adherence to self-care (beta= .37, p < .001) after controlling for age, education level, having a job or not, and body mass index. CONCLUSION: These findings show that health care providers need to pay attention to patients with hypertension who have inadequate health literacy. Further, it is recommended to develop and implement new strategies for assessing health literacy in clinical practices. Interventions to improve health literacy could promote disease-related knowledge and adherence to self-care in patients with hypertension.
Adult ; Health Literacy ; Health Personnel ; Health Status ; Humans ; Hypertension ; Korea ; Linear Models ; Outpatients ; Surveys and Questionnaires ; Self Care

PURPOSE: This study was done to identify the prevalence of depression and determine the relationship of body mass index and perceived health status to depression for elderly women who live alone in the community. METHODS: A total of 175 adults aged over 60 participated in this cross-sectional descriptive study. Perceived heath status was measured using a self-report one-item questionnaire. Body mass index was calculated as weight in kilograms divided by the square of height in meters. Depression was assessed using the Korean short version of the geriatric depression scale. Hierarchical linear regression was used to identify associations between variables. RESULTS: Approximately 61.7% of elderly women who lived alone were depressed. The mean score for depression was 7.4 (SD 3.39). Hierarchical linear regression showed body mass index (beta=.25, p<.001) and perceived health status (beta=-.26, p<.001) were independently associated with depression adjusting for general characteristics. These two predictors accounted for 12% of the variance in depression. CONCLUSION: These results suggest that obesity and perceived health status are risk factors for depression in elderly women living alone. Therefore, these factors should be considered when developing intervention program for elderly women with depression.
Adult ; Aged* ; Body Mass Index* ; Depression* ; Female ; Humans ; Linear Models ; Obesity ; Prevalence ; Surveys and Questionnaires ; Residence Characteristics ; Risk Factors

OBJECTIVES: Emotion perception deficit has long been suggested to be one of the core features of schizophrenia. Although there have been several studies examining responses to facial expressions, few studies addressed music emotion recognition. The aim of this study was to examine the difference in emotion recognition in music between normal subjects and patients with schizophrenia. METHODS: Twenty pieces of music were presented to 43 patients with chronic schizophrenia and 82 healthy controls. The set of music consisted of ten pieces of sad music and ten pieces of cheerful music. The subjects were asked to answer whether each piece of music was sad or cheerful. RESULTS: The correct response rate of music emotion recognition was lower in patients with schizophrenia compared with that in healthy controls (76.67+/-19.03% vs. 93.54+/-8.18%, p<0.001). When subgroup analyses was done by type of music ('sad' or 'cheerful'), patients showed a significantly lower correct response rate in recognizing both sad and cheerful emotion in music than healthy controls (sad emotion : 65.12+/-31.15% vs. 91.95+/-15.27%, cheerful emotion : 88.0+/-16.83% vs. 95.12+/-8.64%). The significant correlations between the clinical symptom severity of schizophrenia and music emotion recognition were not found to be. CONCLUSION: The results suggest that patients with schizophrenia might have difficulties in recognizing musical nuances.
Facial Expression ; Humans ; Music ; Schizophrenia

In 2008, the Joint United Nations Programme on HIV/AIDS (UNAIDS) estimated that about 430,000 children worldwide became infected with HIV, mostly through mother-to-child transmission (MTCT) during pregnancy, labor, delivery, or breast-feeding. The MTCT prevention program proved to be feasible and effective in reduction of perinatal HIV transmission. Three babies born from HIV-infected mothers were admitted to the National Medical Center in 2009. Only two women received antiretroviral (ARV) therapy during pregnancy, labor, and after delivery, and their infants received zidovudine (AZT) for 6 weeks. The outcome, after a follow-up period of 4 months to 16 months, was favorable in all patients. Thus, we emphasize the need for expansion of antenatal HIV screening of pregnant women, implemented for early HIV diagnosis and effective ARV therapy for reduction of perinatal HIV transmission.
Child ; Female ; Follow-Up Studies ; HIV ; Humans ; Infant ; Joints ; Mass Screening ; Mothers ; Pregnancy ; Pregnant Women ; United Nations ; Zidovudine

PURPOSE:Yellow fever, a mosquito-borne viral hemorrhagic fever, is one of the most lethal diseases. Recently there have been an increasing number of Korean children who have travelled to yellow fever endemic zones and were administered yellow fever vaccine (YFV). Therefore, we carried out this study to provide child travelers with safety information of YFV. METHODS:This study was conducted at the International Clinic of National Medical Center in Seoul between April 2007 and June 2008 for the evaluation of adverse events of YFV. One hundred twenty- five children received YFV (17-DD) and were prospectively monitored for adverse events through telephone interviews on day 3, 6, 9, 16, 23 and 30 after vaccination. RESULTS:Adverse events were observed in 31 (24.8%) of 125 child travelers who received the YFV. The mean age was 12.5+/-5.0 years. Sixty-six of the child travelers (52.8%) were males. The common adverse events were pain in 11 (8.8%), swelling in 8 (6.4%) and redness in 7 children (5.6%) at the injection site. The systemic adverse events included mild fever in 5 (4.0%), headache in 5 (4.0%), cough in 4 (3.2%), abdominal pain in 3 (2.4 %), and vomiting in 2 children (1.6%). Most of the adverse events were detected within 7 days of administration and there were no differences in adverse events by gender or age. All travelers who had complained of symptoms improved spontaneously or following symptomatic treatment. CONCLUSION:This study showed that YFV is well-tolerated and there were no reports of severe adverse events. Studies are ongoing to clarify the cause and risk factors for rare adverse events.
Abdominal Pain ; Child ; Cough ; Fever ; Headache ; Hemorrhagic Fevers, Viral ; Humans ; Interviews as Topic ; Male ; Prospective Studies ; Risk Factors ; Vaccination ; Vomiting ; Yellow Fever ; Yellow Fever Vaccine