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.

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

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

PURPOSE: This exploratory study was undertaken to analyze the information needs of family members of terminal cancer patients, collected through the telephone counselling service by National Cancer Information Center. METHODS: The study included 113 family members of terminal cancer patients who had enrolled at the National Cancer Information Center for the period from June, 2007 through March, 2008 and had agreed to the survey. RESULTS: The subjects (n=113) consisted of grown-up children (n=82) and spouses (n=8) of patients'. Those in their 40's (n=40) and 30's (n=36) accounted for the majority of the sample. The questions raised most were about the information on treatment methods (n=117), management of terminal cancer patients (n=46), terminal cancer patients' life (n=27), deathbed and prediction of remaining life (n=18), hospitalization (n=16), and financial support (n=15). Most of the subjects were satisfied with the telephone counseling services, and 69% of the subjects had come to know about the telephone counseling service via Internet, and 10.6% of them stated that the PR for the service was poor. CONCLUSION: It is deemed essential for the government to use the mass media for PR of the hospice services, since family members of terminal cancer patients' are less aware of the hospice conducive to enhancement of patients' remaining quality of life, being involued too deeply in their treatment.
Child ; Counseling ; Financial Support ; Hospices ; Hospitalization ; Humans ; Information Centers ; Information Services ; Internet ; Mass Media ; Quality of Life ; Spouses ; Telephone

Cauda equina syndrome is a well-known but rare complication of spinal anesthesia. An 80-year-old man was scheduled for both herniorrhaphy. Spinal anesthesia was performed at the L3-4 interspinous space with 0.5% hyperbaric bupivacaine 12 mg. Eight hours after anesthesia, the patient complained bilateral sensorimotor deficits of the lower extremities and peroneal region. Urinary and fecal incontinence were also observed. MRI and myelography showed severe central spinal stenosis at L3-4 and L4-5. EMG showed cauda equina syndrome. Seven weeks after the procedure, left decompressive subtotal laminectomy L2-L5 was done. The patient still complains the neuropathic pain in the both lower extremities and ambulates using a walker. The local anesthetic was injected into thecal sac between maximum stenoses, and it is likely that there was poor upward spread leading to maldistribution of local anesthetic and resultant local anesthetic toxicity.
Aged, 80 and over ; Anesthesia ; Anesthesia, Spinal ; Bupivacaine ; Cauda Equina ; Constriction, Pathologic ; Fecal Incontinence ; Herniorrhaphy ; Humans ; Laminectomy ; Lower Extremity ; Myelography ; Neuralgia ; Polyradiculopathy ; Spinal Stenosis ; Walkers

Herpes zoster in childhood is uncommon, but it is more common in association with immunosuppression. Maternal varicella infection during pregnancy and varicella occurring in the newborn represent risk for childhood herpes zoster. However, some controversies persist on risk factors, diagnosis, and the natural history of childhood disease. We report a 10-year-old healthy boy with shingles and review the risk factors, prognosis, and treatment of pediatric zoster.
Chickenpox ; Child ; Herpes Zoster ; Humans ; Immunosuppression ; Infant, Newborn ; Natural History ; Pregnancy ; Prognosis ; Risk Factors