Background: Pompe disease is a rare autosomal recessive disorder caused by the deficiency of a lysosomal enzyme, acid alpha-glucosidase (GAA). Early diagnosis and initiation of treatment with enzyme replacement therapy have remarkable effects on the prognosis of Pompe disease. We performed the expanded screening for late onset Pompe disease (LOPD) at eight centers in Korea. Methods: From September 1, 2015, GAA activity were measured from both dried blood spot (DBS) and mixed leukocyte for 188 available patients. For 12 patients with low GAA activity, we performed Sanger sequencing of GAA gene. Results: Among 188 patients, 115 were males. The mean of age of symptom onset and diagnosis were 34.3 years and 41.6 years. Among 12 patients with decreased GAA activity, two patients were confirmed to have LOPD with genetic test (c.1316T>A [p.M439K] + c.2015G>A [p.R672Q], c.1857C>G [p.S619R] + c.546G>C [leaky splicing]). Other two patients had homozygous G576S and E689K mutation, known as pseudodeficiency allele. Conclusions This study is expanded study of LOPD screening for targeted Korean population. We found two patients with LOPD, and the detection rate of LOPD is 1.06%. With application of modified GAA cutoff value (0.4), which was previously reported, there were no false positive results of GAA activity test using DBS. Therefore, it could be an appropriate screening test for LOPD in especially East-Asian population, in which pseudodeficiency allele is frequent.

Background: Pompe disease is a rare autosomal recessive disorder caused by the deficiency of a lysosomal enzyme, acid alpha-glucosidase (GAA). Early diagnosis and initiation of treatment with enzyme replacement therapy have remarkable effects on the prognosis of Pompe disease. We performed the expanded screening for late onset Pompe disease (LOPD) at eight centers in Korea. Methods: From September 1, 2015, GAA activity were measured from both dried blood spot (DBS) and mixed leukocyte for 188 available patients. For 12 patients with low GAA activity, we performed Sanger sequencing of GAA gene. Results: Among 188 patients, 115 were males. The mean of age of symptom onset and diagnosis were 34.3 years and 41.6 years. Among 12 patients with decreased GAA activity, two patients were confirmed to have LOPD with genetic test (c.1316T>A [p.M439K] + c.2015G>A [p.R672Q], c.1857C>G [p.S619R] + c.546G>C [leaky splicing]). Other two patients had homozygous G576S and E689K mutation, known as pseudodeficiency allele. Conclusions This study is expanded study of LOPD screening for targeted Korean population. We found two patients with LOPD, and the detection rate of LOPD is 1.06%. With application of modified GAA cutoff value (0.4), which was previously reported, there were no false positive results of GAA activity test using DBS. Therefore, it could be an appropriate screening test for LOPD in especially East-Asian population, in which pseudodeficiency allele is frequent.

The purpose of this study is to propose learning objectives in social dental hygiene by analyzing and reviewing learning objectives in oral health administration area of the existing public oral health. This study is a cross-sectional study. The subjects of the study selected with convenience extraction were 15 members of the social dental hygiene subcommittee of the Korean Society of Dental Hygiene Science. Data collection was conducted by self-filling questionnaire. The research tool is from 48 items of A division in the book of learning objectives in the dental hygienist national examination, and this study classified each of them into ‘dental hygiene job relevance’, ‘dental hygiene competency relevance’, ‘timeliness’, and ‘value discrimination of educational goal setting’ to comprise 192 items. Also, to collect expert opinions, this study conducted Delphi survey on 7 academic experts. Statistical analysis was performed using the IBM SPSS Statistics ver. 23.0 program (IBM Co., Armonk, NY, USA). Recoding was performed according to the degree of relevance of each learning objective and frequency analysis was performed. This study removed 18 items from the whole learning objectives in the dental hygienist national examination in the oral health administration area of public oral health. Fifteen revisions were made and 15 existing learning objectives were maintained. Forty-five learning objectives were proposed as new social dental hygiene learning objectives. The topics of learning objectives are divided into social security and medical assistance, oral health care system, oral health administration, and oral health policy. As a result of this study, it was necessary to construct the learning objectives of social dental hygiene in response to changing situation at the time. The contents of education should be revised in order of revision of learning objectives, development of competency, development of learning materials, and national examination.
Cross-Sectional Studies ; Data Collection ; Dental Hygienists ; Discrimination (Psychology) ; Education ; Expert Testimony ; Humans ; Hygiene ; Learning* ; Medical Assistance ; Oral Health* ; Oral Hygiene* ; Social Security

This nationwide, prospective cohort study evaluated pulmonary function and radiological sequelae according to infection severity in 73 survivors from the 2015 Middle East respiratory syndrome (MERS) outbreak in Korea. Patients with severe pneumonia in MERS-coronavirus infection had more impaired pulmonary function than those with no or mild pneumonia at the 1-year follow-up, which was compatible with the radiological sequelae. Severe pneumonia significantly impairs pulmonary function and makes long radiological sequelae in MERS.
Cohort Studies ; Coronavirus ; Coronavirus Infections* ; Follow-Up Studies ; Humans ; Korea ; Middle East* ; Pneumonia* ; Prospective Studies ; Survivors

BACKGROUND AND OBJECTIVES: The recurrence rate of patients with Graves' disease (GD) is estimated to be 50-55% after withdrawal of antithyroid drug therapy, and relapse is frequent in the first year after discontinuing the medication. Follow-up examination of these patients frequently reveals laboratory findings consistent with subclinical thyrotoxicosis in the first year after stopping the antithyroid agents. We investigated the risk of recurrence of GD among patients with resurfacing subclinical thyrotoxicosis state after remission of initial GD with antithyroid treatments. MATERIALS AND METHODS: We reviewed the patients diagnosed with GD who visited the Department of Endocrinology at two tertiary medical centers: Wonju Severance Christian Hospital and Gangneung Asan Hospital. We enrolled patients whose GD was completely treated after initial treatment with antithyroid agents who then developed subclinical thyrotoxicosis after discontinuation of antithyroid agents. RESULTS: We reviewed a total of 44 patients (29 females, 15 males; age, 48.93±18.04; range, 17-85 years). The recurrence rate was 27.3% (12/44 patients), and recurrence occurred 3 months to 12 months later resurfacing of subclinical thyrotoxicosis. Patients with recurred GD was significantly older than non-recurred patients (44.63±17.75 years vs. 58.58±15.48 years, p=0.02). Other clinical parameters measured at the time of initial diagnosis were not different between the two groups. CONCLUSION: The recurrence rate of GD in patients with resurfacing subclinical thyrotoxicosis after initial remission of the disease was less than 30%. A close monitoring is recommended in these subgroup patients, especially in older patients.
Antithyroid Agents* ; Chungcheongnam-do ; Diagnosis ; Drug Therapy ; Endocrinology ; Female ; Follow-Up Studies ; Gangwon-do ; Graves Disease* ; Humans ; Male ; Recurrence* ; Thyrotoxicosis*

Since Zika virus has been spreading rapidly in the Americas from 2015, the outbreak of Zika virus infection becomes a global health emergency because it can cause neurological complications and adverse fetal outcome including microcephaly. Here, we report clinical manifestations and virus isolation findings from a case of Zika virus infection imported from Brazil. The patient, 43-year-old Korean man, developed fever, myalgia, eyeball pain, and maculopapular rash, but not neurological manifestations. Zika virus was isolated from his semen, and reverse-transcriptase PCR was positive for the virus in the blood, urine, and saliva on the 7th day of the illness but was negative on the 21st day. He recovered spontaneously without any neurological complications. He is the first case of Zika virus infection in Korea imported from Brazil.
Adult ; Brazil ; Humans ; Male ; Microscopy, Electron, Transmission ; RNA, Viral/analysis/blood/urine ; Republic of Korea ; Reverse Transcriptase Polymerase Chain Reaction ; Saliva/virology ; Semen/virology ; Travel ; Zika Virus/genetics/*isolation & purification ; Zika Virus Infection/*diagnosis/virology

Up-to-date imaging modalities such as three-dimensional dynamic contrast-enhanced CT (3D CT) and MRI may contribute to detection of hypervascular nodules in the liver. Nevertheless, distinguishing a malignancy such as hepatocellular carcinoma from benign hypervascular hyperplastic nodules (HHN) based on the radiological findings is sometimes difficult. Multiple incidental liver masses were detected via abdominal ultrasonography (US) in a 65-year-old male patient. He had no history of alcohol intake and no remarkable past medical history or relevant family history, and his physical examination results and laboratory findings were normal. 3D CT and MRI showed numerous enhanced nodules with hypervascularity during the arterial phase. After US guided liver biopsy, the pathological diagnosis was HHN. To date, several cases of HHN have been reported in patients with chronic alcoholic liver disease or cirrhosis. Herein, we report on a case of HHN in a patient with no history of alcoholic liver disease or cirrhosis.
Abdomen/diagnostic imaging ; Aged ; Alcoholism/pathology ; Chronic Disease ; Diagnosis, Differential ; Focal Nodular Hyperplasia/*diagnosis ; Humans ; Imaging, Three-Dimensional ; Liver/diagnostic imaging/*pathology ; Liver Diseases/pathology ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed ; Ultrasonography

PURPOSE: The purpose of this study was to identify trends for studies published in the Journal of Korean Academy of Nursing and journals published by member societies from inaugural issues to 2010. METHODS: A total of 6890 studies were analyzed using descriptive statistics. RESULTS: Quantitative studies accounted for 83.6% while qualitative studies accounted for 14.4%. Most frequently used research designs were quasi-experimental (91.1%) for experimental research and survey (85.2%) for non-experimental research. Most frequent study participants were healthy people (35.8%), most frequent nursing interventions, nursing skills (53.5%), and 39.8% used knowledge, attitude and behavior outcomes for dependent variables. Most frequently used keyword was elderly. Survey studies decreased from 1991 to 2010 by approximately 50%, while qualitative studies increased by about 20%. True experimental research (1.2%) showed no significant changes. Studies focusing on healthy populations increased from 2001-2005 (37.5%) to 2006-2010 (41.0%). From 1970 to 2010, studies using questionnaire accounted for over 50% whereas physiological measurement, approximately 5% only. Experimental studies using nursing skill interventions increased from 1970-1980 (30.4%) to 2006-2010 (64.0%). No significant changes were noted in studies using knowledge, attitude and behavior (39.9% ) as dependent variables. CONCLUSION: The results suggest that further expansion of true experimental, qualitative studies and physiological measurements are needed.
Asian Continental Ancestry Group ; Humans ; Nursing Research/ethics/*trends ; *Publishing ; *Qualitative Research ; Republic of Korea ; Research Design

BACKGROUND: The aim of this study was to evaluate antibiotic consumption by adult patients at a single university hospital in Korea between 2001 and 2012. METHODS: We used the 2004 World Health Organization Anatomical Therapeutic Chemical Classification System definition of defined daily doses (DDD) per 1,000 patient-days to calculate the annual antibiotic consumption for 18 antibiotic groups. Chi-square linear-by-linear analysis was performed to evaluate antibiotic consumption trends for each group. RESULTS: Average annual antibiotic consumption during 2001-2012 was 644.6 DDD/1,000 patient-days (standard deviation, 33.3 DDD/1,000 patient-days). Although no statistically significant change was observed during the study period, consumption of first- and second-generation cephalosporins, and aminoglycosides was significantly decreased, while that of beta-lactam/beta-lactamase inhibitors, fourth-generation cephalosporins, carbapenem, glycopeptide, linezolid, colistin, and quinolone increased significantly. CONCLUSION: The total amount of prescribed antibiotics did not change, but the use of broad-spectrum antibiotics increased during the study period.
Adult ; Aminoglycosides ; Anti-Bacterial Agents ; Cephalosporins ; Classification ; Colistin ; Hospitals, University ; Humans ; Korea ; World Health Organization ; Linezolid

BACKGROUND: While there is an evidence that the anti-inflammatory properties of spironolactone can attenuate proteinuria in type 2 diabetes, its effects on vascular endothelial growth factor (VEGF) expression in diabetic nephropathy have not been clearly defined. In this study, we examined the effects of spironolactone, losartan, and a combination of these two drugs on albuminuria, renal VEGF expression, and inflammatory and oxidative stress markers in a type 2 diabetic rat model. METHODS: Thirty-three Otsuka-Long-Evans-Tokushima-Fatty (OLETF) rats were divided into four groups and treated with different medication regimens from weeks 25 to 50; OLETF diabetic controls (n=5), spironolactone-treated (n=10), losartan-treated (n=9), and combination of spironolactone- and losartan-treated (n=9). RESULTS: At week 50, the albumin-to-creatinine ratio was significantly decreased in the losartan and combination groups compared to the control OLETF group. No decrease was detected in the spironolactone group. There was a significant reduction in renal VEGF, transforming growth factor (TGF)-beta, and type IV collagen mRNA levels in the spironolactone- and combination regimen-treated groups. Twenty-four hour urine monocyte chemotactic protein-1 levels were comparable in all four groups but did show a decreasing trend in the losartan and combination regimen groups. Twenty-four hour urine malondialdehyde levels were significantly decreased in the spironolactone- and combination regimen-treated groups. CONCLUSION: These results suggest that losartan alone and a combined regimen of spironolactone and losartan could ameliorate albuninuria by reducing renal VEGF expression. Also, simultaneous treatment with spironolactone and losartan may have protective effects against diabetic nephropathy by decreasing TGF-beta and type IV collagen expression and by reducing oxidative stress in a type 2 diabetic rat model.
Albuminuria ; Animals ; Chemokine CCL2 ; Collagen Type IV ; Diabetic Nephropathies ; Losartan ; Malondialdehyde ; Oxidative Stress ; Proteinuria ; Rats ; RNA, Messenger ; Spironolactone ; Transforming Growth Factor beta ; Transforming Growth Factors ; Vascular Endothelial Growth Factor A