Maple syrup urine disease(MSUD) is an autosomal recessive disorder involving the metabolism of the branched-chain amino acids(BCAA) such as leucine, isoleucine and valine. The disorder is due to a defect in branched-chain alpha-ketoacid dehydrogenase(BCKAD) and the classic form causes rapid progressive and overwhelming illness beginning in the first weeks of life, present with poor feeding, lethargy, change in muscle tone, acidosis, seizures and coma. The goal of therapy in acutely ill patients with MSUD is an immediate reduction in the plasma levels of the BCAAs and branched-chain ketoacids. In this report, we describe an infant with MSUD who was treated by dietary therapy alone. During the therapy, acrodermatitis enteropathica-like syndrome developed with low plasma isoleucine concentration while she was receiving a formula deficient in BCAAs.
Acer* ; Acidosis ; Acrodermatitis* ; Amino Acids, Branched-Chain ; Coma ; Diet Therapy* ; Diet* ; Humans ; Infant ; Isoleucine ; Lethargy ; Leucine ; Maple Syrup Urine Disease* ; Metabolism ; Plasma ; Seizures ; Valine

OBJECTIVES: This study was conducted to investigate the status and need for nutrition and dietary life education among nutrition teachers at schools. These characteristics were analyzed if they were different between elementary schools and middle-high schools. METHODS: Subjects were 151 nutrition teachers from 70 elementary schools, 41 middle schools and 40 high schools in 17 cities nationwide selected by two-stage stratified cluster sampling process. Survey questionnaires included the items on general characteristics, status and need assessment for nutrition and dietary life education. Chi-square test or t-test was used for data analysis by school groups. RESULTS: Nutrition education was implemented at 65.7% of elementary schools and 51.9% of middle-high schools. Nutrition education was mainly performed in 'discretionary activities·extracurricular activities' at elementary school and through 'newsletters, school homepage, foodservice bulletin board' at middle-high school (p<0.001). The most needed topic for nutrition education in nutrition teachers was 'healthy dietary habits and table manners' and this was not significantly different by school groups. Responses on adequate frequency (p<0.01), methods used for nutrition education (p<001), materials for nutrition education (p<0.001), information sources for nutrition education (p<0.001) were significantly different by school groups. Major tasks for activating nutrition education included 'securing the time for implementing nutrition education by reducing work loads' and 'developing standardized nutrition education materials' in schools. CONCLUSIONS: Nutrition education at schools might be activated by improving working conditions of nutrition teachers and developing the practical programs that reflect the needs of nutrition teachers.
Adolescent ; Child ; Education* ; Food Habits ; Humans ; Needs Assessment ; Statistics as Topic

PURPOSE: The purpose is to correlate computed tomographic findings classified according to the degree of disease severity(grading A-E) with clinical course of acute pancreatitis. MATERIALS AND METHODS: in a retrospective review of 42 patients with acute pancreatitis, computed tomographic scans were classifed according to the degree of disease severity, and were correlatd with the clinical course. RESULTS: Pancreatic abscesses were seen in 14.3% and occured in 35.7% of grade D and E patients. Three patients with abscess died. Fatty infiltration of the liver was noted in 16.7%, pleural effusion in 21.4%.. stones and thickened wall of the gallbladder were present in 7.1% and in 9.5%, respectively. CONCLUSION: Our data indicate that phlegmonous extrapancreatic spread on initial CT scan had a high predictive value of the patients, prognosis.
Abscess ; Cellulitis ; Gallbladder ; Humans ; Liver ; Pancreatitis* ; Pleural Effusion ; Prognosis ; Retrospective Studies ; Tomography, X-Ray Computed

BACKGROUND: Bcl-xL and bax act as checkpoints of the apoptotic cell death. Although apoptosis is one of major mechanism of cell death in renal allografts inflicted by various events, the role of bcl-xL and bax in kidney transplantation has not been characterized yet. We therefore studied intragraft expression of bcl-x and bax and its clinical significance in renal transplantation. METHODS: We localized the expression of bcl-x, bcl-xS and bax proteins by immunohistochemistry, and measured the magnitude of the gene transcription of bax and bcl-xL by semi-quantitative RT-PCR in 37 implantation allograft biopsies(18 from 9 cadaveric donors, 19 from living donors), and 17 biopsies from patients undergoing acute rejection(AR). RESULTS: Immunoreactivities for bax, bcl-x, and bcl-xS were observed in tubular epithelial cells but not in glomeruli and vessels in implantation and AR biopsies. The infiltrating lymphocytes in AR expressed bax and bcl-xS but not for bcl-x. Comparing the intragraft gene transcript level of each allograft of a pair of recipients, who received graft from the same cadaveric donor, showed a higher bcl-xL in the patients with a higher concentration of postoperative 7th day serum creatinine. The transcript level of bcl-xL was higher in the Banff grade II and III AR biopsies than in the borderline or grade I AR, and also higher in steroid-resistant AR than in steroid-responsive patients. CONCLUSION: These results implicated the apoptotic death of infiltrating lymphocytes during rejection, and the compensatory up-regulation of bcl-xL in response to various apoptotic stimuli occurring in renal allografts.
Allografts* ; Apoptosis ; bcl-2-Associated X Protein ; Biopsy ; Cadaver ; Cell Death ; Creatinine ; Epithelial Cells ; Gene Expression* ; Humans ; Immunohistochemistry ; Kidney Transplantation ; Lymphocytes ; Tissue Donors ; Transplantation ; Transplants ; Up-Regulation*

BACKGROUND: Bcl-xL and bax act as checkpoints of the apoptotic cell death. Although apoptosis is one of major mechanism of cell death in renal allografts inflicted by various events, the role of bcl-xL and bax in kidney transplantation has not been characterized yet. We therefore studied intragraft expression of bcl-x and bax and its clinical significance in renal transplantation. METHODS: We localized the expression of bcl-x, bcl-xS and bax proteins by immunohistochemistry, and measured the magnitude of the gene transcription of bax and bcl-xL by semi-quantitative RT-PCR in 37 implantation allograft biopsies(18 from 9 cadaveric donors, 19 from living donors), and 17 biopsies from patients undergoing acute rejection(AR). RESULTS: Immunoreactivities for bax, bcl-x, and bcl-xS were observed in tubular epithelial cells but not in glomeruli and vessels in implantation and AR biopsies. The infiltrating lymphocytes in AR expressed bax and bcl-xS but not for bcl-x. Comparing the intragraft gene transcript level of each allograft of a pair of recipients, who received graft from the same cadaveric donor, showed a higher bcl-xL in the patients with a higher concentration of postoperative 7th day serum creatinine. The transcript level of bcl-xL was higher in the Banff grade II and III AR biopsies than in the borderline or grade I AR, and also higher in steroid-resistant AR than in steroid-responsive patients. CONCLUSION: These results implicated the apoptotic death of infiltrating lymphocytes during rejection, and the compensatory up-regulation of bcl-xL in response to various apoptotic stimuli occurring in renal allografts.
Allografts* ; Apoptosis ; bcl-2-Associated X Protein ; Biopsy ; Cadaver ; Cell Death ; Creatinine ; Epithelial Cells ; Gene Expression* ; Humans ; Immunohistochemistry ; Kidney Transplantation ; Lymphocytes ; Tissue Donors ; Transplantation ; Transplants ; Up-Regulation*

The aim of this study was to investigate time trends in the public awareness of stroke and its predicting factors. The target population was 9,600 community-dwelling adults, aged 19–79 years, in 16 metropolitan cities and provinces in Korea. The survey samples in 2012 and 2014 were selected separately (entirely different sets of subjects) using a proportionate quota sampling method. Information concerning knowledge of stroke and demographics was collected by trained telephone interviewers using random digit dialing. After excluding subjects with a non-response or refusal to answer any question, the analyses included 8,191 subjects in 2012 and 8,127 subjects in 2014. Respondents’ awareness of stroke warning signs (numbness or weakness, difficulty speaking or understanding speech, dizziness, visual impairment, and severe headache) was highest for difficulty speaking or understanding speech (80.9% in 2012 and 86.4% in 2014). There were significant increases in the proportion of respondents understanding the appropriate action (i.e., calling an ambulance) at the time of stroke occurrence (59.6% to 67.1%), and in the proportion aware of the general need for prompt treatment (86.7% to 89.8%). In multivariable logistic regression analysis, older age, higher education level, higher household income, current non-smoking, exposure to stroke-related public relations materials, and experience of stroke education were significantly associated with both high knowledge of stroke warning signs and awareness of the need for prompt treatment. Between 2012 and 2014, the public’s awareness of stroke increased significantly. More specialized interventions, including public relations materials and education, should focus on subgroups who have lower stroke knowledge.
Adult ; Demography ; Dizziness ; Education ; Family Characteristics ; Health Services Needs and Demand ; Humans ; Interviews as Topic ; Korea* ; Logistic Models ; Methods ; Public Relations ; Stroke* ; Surveys and Questionnaires ; Telephone* ; Vision Disorders

BACKGROUND: The limit and the optimal method of the cryopreservation of platelets have not been determined. Moreover, the functional changes platelets after cryopreservation were not clearly defined. This study was conducted to determine the limit and optimal method for cryopreservation of platelet concentrates. METHODS: We compared the recovery, expression of membrane GpIb, GpIIb/IIIa, and aggregatory function of the platelets preserved in three different conditions. Platelet samples were collected from four healthy volunteer donors by apheresis, and placed in 22degrees C agitator for standard preservation. For cryopreservation, after treating 5% DMSO, platelets were either inserted directly in -80degrees C freezer or in liquid nitrogen after computer-controlled rate freezing. After storage for 5 days, 1 week, 2 weeks, 3 weeks, 4 weeks, and 12 weeks, platelets were thawed and analyzed for the evaluation of in vitro functions. RESULTS: Platelets preserved at 22degrees C or cryopreserved with each condition displayed equivalent recovery (90%). With each cryopreservation procedures, platelets showed moderate loss of GpIb and retained more than 90% of GpIIb/IIIa in comparison with fresh platelets. At the third week, loss of GpIb in the directly frozen platelets was augmented compared with those of controlled rate frozen group. The aggregatory response to ristocetin began to decrease drastically after storage for 5 days in platelets frozen by each procedures and to less than 5% at 12 weeks of storage. However, controlled rate frozen platelets retained more aggregatory response to ristocetin and surface GpIb expression than those of directly frozen platelets at 3, 4, 12 weeks of storage. CONCLUSION: This study showed the possibility of moderate preservation of in vitro functions of frozen-thawed platelets after 12 weeks of storage compared with those of the liquid stored 5-day old platelets.
Blood Component Removal ; Blood Platelets ; Cryopreservation* ; Dihydroergotamine ; Dimethyl Sulfoxide ; Freezing ; Healthy Volunteers ; Humans ; Membrane Glycoproteins ; Membranes ; Nitrogen ; Ristocetin ; Tissue Donors

This study attempted to calculate and investigate the incidence of hospitalized acute myocardial infarction (AMI) and stroke in Korea. Using the National Health Insurance claim data, we investigated patients whose main diagnostic codes included AMI or stroke during 2006 to 2010. As a result, we found out that the number of AMI hospitalized patients had decreased since 2006 and amounted to 15,893 in 2010; and that the number of those with stroke had decreased since 2006 and amounted to 73,501 in 2010. The age-standardized incidence rate of hospitalized AMI, after adjustment for readmission, was 41.6 cases per 100,000-population in 2006, and had decreased to 29.4 cases in 2010 (for trend P < 0.001). In the case of stroke was estimated at 172.8 cases per 100,000-population in 2006, and had decreased to 135.1 cases in 2010 (for trend P < 0.001). In conclusion, the age-standardized incidence rates of both hospitalized AMI and stroke in Korea had decreased continuously during 2006 to 2010. We consider this decreasing trend due to the active use of pharmaceuticals, early vascular intervention, and the national cardio-cerebrovascular disease care project as the primary and secondary prevention efforts.
Acute Disease ; Adolescent ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; Female ; Hospitalization/*trends ; Humans ; Incidence ; Infant ; Infant, Newborn ; Male ; Middle Aged ; Myocardial Infarction/*epidemiology ; Patient Readmission ; Republic of Korea/epidemiology ; Sex Factors ; Stroke/*epidemiology ; Young Adult

BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients. METHODS: We analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale. RESULTS: Clinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls. CONCLUSIONS: In CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction.
Action Potentials ; Axons ; Charcot-Marie-Tooth Disease ; Cohort Studies ; Humans ; Muscles ; Neural Conduction

The publisher wishes to apologize for incorrectly displaying the author (Seok Beom Gwon) name. We correct his name from Seok Beom Gwon to Seok Beom Kwon.