BACKGROUND: Primary hyperoxaluria (PH), a rare inborn error of glyoxylate meta bolism causing overproduction of oxalate, is classified into three genetic subgroups: type 1–3 (PH1–PH3) caused by AGXT, GRHPR , and HOGA1 gene mutations, respectively. We performed a retrospective case series study of Korean pediatric patients with PH.METHODS: In total, 11 unrelated pediatric patients were recruited and their phenotypes and genotypes were analyzed by a retrospective review of their medical records.RESULTS: Mutational analyses revealed biallelic AGXT mutations (PH1) in nine patients and a single heterozygous GRHPR and HOGA1 mutation in one patient each. The c.33dupC was the most common AGXT mutation with an allelic frequency of 44%. The median age of onset was 3 months (range, 2 months-3 years), and eight patients with PH1 presented with end stage renal disease (ESRD). Patients with two truncating mutations showed an earlier age of onset and more frequent retinal involvement than patients with one truncating mutation. Among eight PH1 patients presenting with ESRD, five patients were treated with intensive dialysis followed by liver transplantation (n=5) with/without subsequent kidney transplantation (n=3).CONCLUSION: Most patients presented with severe infantile forms of PH. Patients with two truncating mutations displayed more severe phenotypes than those of patients with one truncating mutation. Sequential liver and kidney transplantation was adopted for PH1 patients presenting with ESRD. A larger nation-wide multicenter study is needed to confirm the genotype-phenotype correlations and outcomes of organ transplantation.
Age of Onset ; Dialysis ; Genetic Association Studies ; Genotype ; Humans ; Hydrogen-Ion Concentration ; Hyperoxaluria, Primary ; Kidney Failure, Chronic ; Kidney Transplantation ; Liver ; Liver Transplantation ; Medical Records ; Organ Transplantation ; Phenotype ; Retinaldehyde ; Retrospective Studies ; Transplants

BACKGROUND: Accurate volume measurement is important in the management of patients with congestive heart failure or renal insufficiency. A bioimpedance analyser can estimate total body water in litres and has been widely used in clinical practice due to its non-invasiveness and ease of results interpretation. To change impedance data to volumetric data, bioimpedance analysers use equations derived from data from healthy subjects, which may not apply to patients with other conditions. Bioelectrical impedance vector analysis (BIVA) was developed to overcome the dependence on those equations by constructing vector plots using raw impedance data. BIVA requires normal reference plots for the proper interpretation of individual vectors. The aim of this study was to construct normal reference vector plots of bioelectrical impedance for Koreans. METHODS: Bioelectrical impedance measurements were collected from apparently healthy subjects screened according to a comprehensive physical examination and medical history performed by trained physicians. Reference vector contours were plotted on the RXc graph using the probability density function of the bivariate normal distribution. We further compared them with those of other ethnic groups. RESULTS: A total of 242 healthy subjects aged 22 to 83 were recruited (137 men and 105 women) between December 2015 and November 2016. The centers of the tolerance ellipses were 306.3 Ω/m and 34.9 Ω/m for men and 425.6 Ω/m and 39.7 Ω/m for women. The ellipses were wider for women than for men. The confidence ellipses for Koreans were located between those for Americans and Spaniards without overlap for both genders. CONCLUSION: This study presented gender-specific normal reference BIVA plots and corresponding tolerance and confidence ellipses on the RXc graph, which is important for the interpretation of BIA-reported volume status in patients with congestive heart failure or renal insufficiency. There were noticeable differences in reference ellipses with regard to gender and ethnic groups.
Adult ; Blood Volume ; Body Fluid Compartments ; Body Water ; Electric Impedance ; Ethnic Groups ; Female ; Healthy Volunteers ; Heart Failure ; Humans ; Male ; Physical Examination ; Renal Insufficiency

The present erratum notice corrects one figure of the article.

BACKGROUND: Psoriasis is an immune-mediated, chronic inflammatory disease affecting multiple aspects of patients' lives. Its epidemiology varies regionally; however, nationwide epidemiologic data on psoriasis depicting profile of Korean patients has not been available to date. OBJECTIVE: To understand nationwide epidemiologic characteristics and clinical features of adult patients with psoriasis visited university hospitals in Korea. METHODS: This multicenter, non-interventional, cross-sectional study recruited 1,278 adult patients with psoriasis across 25 centers in Korea in 2013. Various clinical data including PASI, BSA, DLQI, SF-36 and PASE were collected. RESULTS: A total of 1,260 patients completed the study (male:female=1.47:1). The mean age was 47.0 years with a distribution mostly in the 50s (24.9%). Early onset (<40 years) of psoriasis accounted for 53.9% of patients. The mean disease duration was 109.2 months; mean body mass index was 23.9 kg/m²; and 12.7% of patients had a family history of psoriasis. Plaque and guttate types of psoriasis accounted for 85.8% and 8.4%, respectively. Patients with PASI ≥10 accounted for 24.9%; patients with body surface area ≥10 were 45.9%. Patients with DLQI ≥6 accounted for 78.8%. Between PASI <10 and PASI ≥10 groups, significant difference was noted in age at diagnosis, disease duration, blood pressure, waist circumference of female, and treatment experiences with phototherapy, systemic agents, and biologics. CONCLUSION: This was the first nationwide epidemiologic study of patients with psoriasis in Korea and provides an overview of the epidemiologic characteristics and clinical profiles of this patient population.
Adult ; Biological Products ; Blood Pressure ; Body Mass Index ; Body Surface Area ; Cross-Sectional Studies* ; Diagnosis ; Epidemiologic Studies ; Epidemiology ; Female ; Hospitals, University ; Humans ; Korea* ; Phototherapy ; Psoriasis* ; Waist Circumference

PURPOSE: In outbreaks of infectious disease, medical students are easily overlooked in the management of healthcare personnel protection although they serve in clinical clerkships in hospitals. In the early summer of 2015, Middle East respiratory syndrome (MERS) struck South Korea, and students of Sungkyunkwan University School of Medicine (SKKUSOM) were at risk of contracting the disease. The purpose of this report is to share SKKUSOM's experience against the MERS outbreak and provide suggestions for medical schools to consider in the face of similar challenges. METHODS: Through a process of reflection-on-action, we examined SKKUSOM's efforts to avoid student infection during the MERS outbreak and derived a few practical guidelines that medical schools can adopt to ensure student safety in outbreaks of infectious disease. RESULTS: The school leadership conducted ongoing risk assessment and developed contingency plans to balance student safety and continuity in medical education. They rearranged the clerkships to another hospital and offered distant lectures and tutorials. Five suggestions are extracted for medical schools to consider in infection outbreaks: instant cessation of clinical clerkships; rational decision making on a school closure; use of information technology; constant communication with hospitals; and open communication with faculty, staff, and students. CONCLUSION: Medical schools need to take the initiative and actively seek countermeasures against student infection. It is essential that medical schools keep constant communication with their index hospitals and the involved personnel. In order to assure student learning, medical schools may consider offering distant education with online technology.
Clinical Clerkship ; Communicable Diseases ; Communicable Diseases, Emerging ; Coronavirus Infections* ; Decision Making ; Delivery of Health Care ; Disease Outbreaks ; Education ; Education, Medical ; Humans ; Korea ; Leadership ; Learning ; Lectures ; Middle East* ; Risk Assessment ; Schools, Medical* ; Students, Medical

BACKGROUND/AIMS: Helicobacter pylori is a major risk factor for atrophic gastritis (AG) and gastric cancer. The correlation between H. pylori, AG and colorectal neoplasm (CRN) has only been examined in a limited number of studies, and findings have been inconclusive. We aimed to investigate the association between H. pylori infection status, AG and advanced CRN. METHODS: This cross-sectional study investigated the relationship between the presence of serum anti-H. pylori IgG antibodies, AG, and advanced CRN in 6,351 consecutive asymptomatic subjects who underwent a screening colonoscopy. RESULTS: A total of 316 participants (5.0%) had advanced CRN. H. pylori seropositivity was 61.3%. In a univariate analysis, the presence of H. pylori infection was associated with advanced CRN (odds ratio [OR], 1.49; 95% confidence interval [CI], 1.17 to 1.91; p=0.001). H. pylori infection was associated with an increased risk of advanced CRN after adjusting for clinically relevant confounders (OR, 1.34; 95% CI, 1.04 to 1.72; p=0.023). H. pylori-related AG was significantly associated with the risk of advanced CRN (OR, 1.40; 95% CI, 1.03 to 1.91; p=0.030), whereas H. pylori infection without AG was not. CONCLUSIONS: H. pylori infection increased the risk of advanced CRN, especially when it was combined with AG. Strict colonoscopy screening and surveillance may be warranted in those with H. pylori-positive AG.
Antibodies ; Atrophy ; Colon* ; Colonic Neoplasms* ; Colonoscopy ; Colorectal Neoplasms ; Cross-Sectional Studies ; Gastritis ; Gastritis, Atrophic* ; Helicobacter pylori* ; Helicobacter* ; Immunoglobulin G ; Mass Screening ; Risk Factors* ; Stomach Neoplasms

BACKGROUND/AIMS: Helicobacter pylori is a major risk factor for atrophic gastritis (AG) and gastric cancer. The correlation between H. pylori, AG and colorectal neoplasm (CRN) has only been examined in a limited number of studies, and findings have been inconclusive. We aimed to investigate the association between H. pylori infection status, AG and advanced CRN. METHODS: This cross-sectional study investigated the relationship between the presence of serum anti-H. pylori IgG antibodies, AG, and advanced CRN in 6,351 consecutive asymptomatic subjects who underwent a screening colonoscopy. RESULTS: A total of 316 participants (5.0%) had advanced CRN. H. pylori seropositivity was 61.3%. In a univariate analysis, the presence of H. pylori infection was associated with advanced CRN (odds ratio [OR], 1.49; 95% confidence interval [CI], 1.17 to 1.91; p=0.001). H. pylori infection was associated with an increased risk of advanced CRN after adjusting for clinically relevant confounders (OR, 1.34; 95% CI, 1.04 to 1.72; p=0.023). H. pylori-related AG was significantly associated with the risk of advanced CRN (OR, 1.40; 95% CI, 1.03 to 1.91; p=0.030), whereas H. pylori infection without AG was not. CONCLUSIONS: H. pylori infection increased the risk of advanced CRN, especially when it was combined with AG. Strict colonoscopy screening and surveillance may be warranted in those with H. pylori-positive AG.
Antibodies ; Atrophy ; Colon* ; Colonic Neoplasms* ; Colonoscopy ; Colorectal Neoplasms ; Cross-Sectional Studies ; Gastritis ; Gastritis, Atrophic* ; Helicobacter pylori* ; Helicobacter* ; Immunoglobulin G ; Mass Screening ; Risk Factors* ; Stomach Neoplasms

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

Along with the development of innovative stent designs, preclinical trials in animal models are essential. Many animal models have been used and appear to yield comparable results to clinical trials despite substantial criticisms about their validity. Among the animal models, porcine coronary artery models have been the standard models for the preclinical evaluation of endovascular devices. However, rapid growth rate, high body weight potential, and the propensity to develop granulomatous inflammatory reactions are major limitations of the porcine coronary artery model. Compared with porcine coronary artery models, the comparative rabbit iliac artery model has the advantages of being small and easy to handle and relatively inexpensive. Furthermore, the rabbit model has been known to reliably reflect human restenosis histopathologically and have major advantages such as pairwise comparison, which makes each animal serve as its own control subject, therefore, maximizing its statistical power for comparative testing. However, despite the widespread use of this model, a systematic description of the procedure and harvest protocols has never been published. This article describes the surgical procedure, stent implantation procedure, method for tissue harvesting, and how measurements are performed. Although the results of animal models may not perfectly extrapolate to humans, the comparative rabbit iliac artery model may be a useful tool for assessing and comparing the efficacy of new coronary stents with conventional stent systems. This thorough description of the techniques required for vascular access, stent implantation, tissue preparation, and measurement, should aid investigators wishing to begin using the comparative rabbit iliac artery model.
Animals ; Body Weight ; Coronary Vessels ; Humans ; Iliac Artery ; Models, Animal ; Rabbits ; Research Personnel ; Stents* ; Tissue and Organ Harvesting

The aim of our study was to determine the impact of vascular access on in-hospital major bleeding (IHMB) in acute coronary syndrome (ACS). We analyzed 995 patients with non-ST elevation myocardial infarction and unstable angina at the Can Rapid risk stratification of Unstable angina patients Suppress ADverse outcomes with Early implementation of the ACC/AHA guidelines (CRUSADE) moderate- to very high-bleeding risk scores in trans-radial intervention (TRI) retrospective registry from 16 centers in Korea. A total of 402 patients received TRI and 593 patients did trans-femoral intervention (TFI). The primary end-point was IHMB as defined in the CRUSADE. There were no significant differences in in-hospital and 1-yr mortality rates between two groups. However, TRI had lower incidences of IHMB and blood transfusion than TFI (6.0% vs 9.4%, P = 0.048; 4.5% vs 9.4%, P = 0.003). The patients suffered from IHMB had higher incidences of in-hospital and 1-yr mortality than those free from IHMB (3.1% vs 15.0%, P < 0.001; 7.2% vs 30.0%, P < 0.001). TRI was an independent negative predictor of IHMB (odds ratio, 0.305; 95% confidence interval, 0.109-0.851; P = 0.003). In conclusions, IHMB is still significantly correlated with in-hospital and 1-yr mortality. Our study suggests that compared to TFI, TRI could reduce IHMB in patients with ACS at moderate- to very high-bleeding risk.
Acute Coronary Syndrome/mortality/*pathology ; Aged ; Female ; Femoral Artery ; *Hemorrhage ; Hospital Mortality ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Odds Ratio ; Percutaneous Coronary Intervention ; Radial Artery ; Retrospective Studies ; Risk Factors ; Stents ; Treatment Outcome