BACKGROUND AND OBJECTIVES: Despite significant advances in the treatment of acute myocardial infarction (MI), the prevention of sudden cardiac death (SCD), the most common mode of death in patients with MI, remains challenging. Furthermore, previous Korean MI registries did not address the issue of post-MI SCD. Additional risk stratifiers of post-MI SCD are still required to compensate for the limitation of using left ventricular ejection fraction to predict lethal arrhythmic events. SUBJECTS AND METHODS: We designed the first Korean prospective nationwide multicenter registry primarily focused on SCD; the Korean noninvasive Risk Evaluation study for sudden cardiac DEath From INfarction or heart failurE (K-REDEFINE). The registry consists of 2 groups of patients presenting with (1) acute MI or (2) acute heart failure (HF) at 25 tertiary referral cardiovascular centers. The primary endpoint of the MI group study of K-REDEFINE registry is the incidence and risk factors of post-MI SCD. In particular, the association between the risk of SCD and non-invasive Holter-based electrocardiogram (ECG) variables will be evaluated, such as T-wave alternans (marker of repolarization heterogeneity) and heart rate turbulence/variability (a marker of autonomic function). Other secondary study outcomes include atrioventricular arrhythmias, HF-related admission, repeated myocardial ischemic events, stroke, and overall deaths. CONCLUSION AND PERSPECTIVE: The K-REDEFINE registry will provide new prospects for the better management of MI patients with high risk of SCD by clarifying the burden and predictors of SCD and the clinical utility of various non-invasive ambulatory ECG-based variables in risk stratification for SCD in this patient population.
Arrhythmias, Cardiac ; Death, Sudden, Cardiac* ; Electrocardiography ; Heart Failure* ; Heart Rate ; Heart* ; Humans ; Incidence ; Infarction* ; Myocardial Infarction* ; Prospective Studies ; Referral and Consultation ; Registries ; Risk Factors ; Stroke ; Stroke Volume

We found an error in this article. The author's name should be corrected as following: from "Younsuk Koh" to "Younsuck Koh".

BACKGROUND: Critical care physician staffing is a crucial element of the intensive care unit (ICU) organization, and is associated with better outcomes in ICUs. Adult ICUs in Korea have been suffering from inadequate full-time intensivists and nurses because of insufficient reimbursement rates (<50% of the original critical care cost) from the National Health Insurance System. Recently, full-time intensivists have been introduced as a prerequisite for adult ICUs of tertiary hospitals in Korea. The purpose of this study was to examine the perception of intensivist staffing among critical care program directors regarding the barriers and solutions when implementing an intensivist model of critical care in Korea. METHODS: An email survey of critical care program directors in designated teaching hospitals for critical care subspecialty training by the Korean Society of Critical Care Medicine was performed. The survey domains included vision, culture, resources, barriers, and potential solutions to implementing intensivist physician staffing (IPS). RESULTS: Forty-two critical care program directors were surveyed. A total of 28 directors (66.7%) responded to email queries. Of these, 27 directors (96.4%) agreed that IPS would improve the quality of care in the ICU, although half of them reported a negative perception of relevant clinical colleagues for the role of full-time intensivists and poor resources for IPS in their hospitals. Increased financial burden due to hiring full-time intensivists and concerns regarding exclusion from the management of their critically ill patients in the ICU, together with loss of income for primary attending physicians were stated by the respondents to be major barriers to implementing IPS. Financial incentives for the required cost from the health insurance system and enhancement of medical law relevant to critical care were regarded as solutions to these issues. CONCLUSIONS: Critical care program directors believe that intensivist-led critical care can improve the outcome of ICUs. They indicated the financial burden due to IPS and underestimation of a full-time intensivist's role to be major barriers. The program directors agreed that a partnership between hospital leaders and the Ministry of Health and Welfare was needed to overcome these barriers.
Adult* ; Critical Care ; Critical Illness ; Electronic Mail ; Hospitals, Teaching ; Humans ; Insurance, Health ; Intensive Care Units ; Jurisprudence ; Korea ; Motivation ; National Health Programs ; Personnel Staffing and Scheduling ; Surveys and Questionnaires ; Tertiary Care Centers

BACKGROUND AND OBJECTIVES: Sudden cardiac death (SCD) is one of the most common causes of death in patients with heart failure (HF). However, there are no available data on SCD in previous Korean HF registries. Additionally, although widely used, the utility of left ventricular (LV) ejection fraction (EF) in risk stratification for SCD is limited. SUBJECTS AND METHODS: The Korean non-invasive Risk Evaluation study for sudden cardiac DEath From INfarction or heart failurE (KREDEFINE) is the first Korean prospective, nationwide multicenter registry, primarily focused on SCD. The registry consists of 2 groups of patients presenting with (1) acute HF or (2) acute myocardial infarction (MI) at 25 tertiary referral cardiovascular centers. Using the HF-group data of the K-REDEFINE registry, the incidence and risk factors of SCD in patients with HF will be assessed. In particular, the efficacy of Holter-based ECG variables, such as T-wave alternans (marker of repolarization heterogeneity) and heart rate turbulence/ variability (maker of autonomic function), in risk stratification for SCD will be evaluated. Other cardiovascular outcomes will also be analyzed, including atrioventricular arrhythmias, HF-related admission, stroke, and overall deaths. CONCLUSION AND PERSPECTIVE: The K-REDEFINE registry will pave the way for better management of patients with HF at high risk of SCD by elucidating the burden and risk factors of SCD and the clinical utility of various non-invasive ambulatory ECG-based parameters in risk stratification for SCD in this patient population.
Arrhythmias, Cardiac ; Cause of Death ; Death, Sudden, Cardiac* ; Electrocardiography ; Heart Failure* ; Heart Rate ; Heart* ; Humans ; Incidence ; Infarction* ; Myocardial Infarction ; Prospective Studies ; Referral and Consultation ; Registries ; Risk Factors ; Stroke

BACKGROUND: Critical care physician staffing is a crucial element of the intensive care unit (ICU) organization, and is associated with better outcomes in ICUs. Adult ICUs in Korea have been suffering from inadequate full-time intensivists and nurses because of insufficient reimbursement rates (<50% of the original critical care cost) from the National Health Insurance System. Recently, full-time intensivists have been introduced as a prerequisite for adult ICUs of tertiary hospitals in Korea. The purpose of this study was to examine the perception of intensivist staffing among critical care program directors regarding the barriers and solutions when implementing an intensivist model of critical care in Korea. METHODS: An email survey of critical care program directors in designated teaching hospitals for critical care subspecialty training by the Korean Society of Critical Care Medicine was performed. The survey domains included vision, culture, resources, barriers, and potential solutions to implementing intensivist physician staffing (IPS). RESULTS: Forty-two critical care program directors were surveyed. A total of 28 directors (66.7%) responded to email queries. Of these, 27 directors (96.4%) agreed that IPS would improve the quality of care in the ICU, although half of them reported a negative perception of relevant clinical colleagues for the role of full-time intensivists and poor resources for IPS in their hospitals. Increased financial burden due to hiring full-time intensivists and concerns regarding exclusion from the management of their critically ill patients in the ICU, together with loss of income for primary attending physicians were stated by the respondents to be major barriers to implementing IPS. Financial incentives for the required cost from the health insurance system and enhancement of medical law relevant to critical care were regarded as solutions to these issues. CONCLUSIONS: Critical care program directors believe that intensivist-led critical care can improve the outcome of ICUs. They indicated the financial burden due to IPS and underestimation of a full-time intensivist's role to be major barriers. The program directors agreed that a partnership between hospital leaders and the Ministry of Health and Welfare was needed to overcome these barriers.
Adult ; Critical Care ; Critical Illness ; Electronic Mail ; Hospitals, Teaching ; Humans ; Insurance, Health ; Intensive Care Units ; Jurisprudence ; Korea ; Motivation ; National Health Programs ; Personnel Staffing and Scheduling ; Surveys and Questionnaires ; Tertiary Care Centers

We found an error in this article. The author's name should be corrected as following: from "Younsuk Koh" to "Younsuck Koh".

Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is an autosomal recessive hereditary metabolic disorder of mitochondrial fatty acid beta-oxidation. It is characterized by hypoketotic hypoglycemia, hyperammonemia, seizure, coma, and sudden infant death syndrome-like illness. The most frequently isolated mutation in the acyl-CoA dehydrogenase, medium-chain (ACADM) gene of Caucasian patients with MCADD is c.985A>G, but ethnic variations exist in the frequency of this mutation. Here, we describe 2 Korean pediatric cases of MCADD, which was detected during newborn screening by tandem mass spectrometry and confirmed by molecular analysis. The levels of medium-chain acylcarnitines, including octanoylcarnitine (C8), hexanoylcarnitine (C6), and decanoylcarnitine (C10), were typically elevated. Molecular studies revealed that Patient 1 was a compound heterozygote for c.449_452delCTGA (p.Thr150ArgfsX4) and c.461T>G (p.L154W) mutations, and Patient 2 was a compound heterozygote for c.449_452delCTGA (p.Thr150ArgfsX4) and c.1189T>A (p.Y397N) mutations. We detected asymptomatic patients with MCADD by using a newborn screening test and confirmed it by ACADM mutation analysis. This report presents evidence of the biochemical and molecular features of MCADD in Korean patients and, to the best of our knowledge, this is the first report of the c.461T>G mutation in the ACADM gene.
Acyl-CoA Dehydrogenase/chemistry/deficiency/genetics ; Asian Continental Ancestry Group/*genetics ; Base Sequence ; Biological Markers/blood ; Carnitine/analogs & derivatives/blood ; DNA Mutational Analysis ; Exons ; Female ; Gene Deletion ; Heterozygote ; Humans ; Infant, Newborn ; Lipid Metabolism, Inborn Errors/diagnosis/genetics ; Male ; Mutation ; Neonatal Screening ; Republic of Korea ; Tandem Mass Spectrometry

3-methylcrotonyl-CoA carboxylase deficiency is an autosomal recessive disorder characterized by a defect in leucine catabolism. We report the case of an 80-day-old patient with 3-methylcrotonyl-CoA carboxylase deficiency who had elevated levels of 3-hydroxyisovalerylcarnitine (45.56 micromol/L; reference range, <0.65 micromol/L), which was detected using tandem mass spectrometry during newborn screening, and elevated levels of 3-hydroxyisovaleric acid (375.75 mmol/mol Cr) and 3-methylcrotonylglycine (502.36 mmol/mol Cr ), which were detected in urine organic acid analysis. We performed direct sequence analysis of all the exons of the MCCC1 and MCCC2 genes. No mutations were detected in the direct sequence analysis of MCCC1. However sequencing of the MCCC2 gene revealed a mutation caused by a heterozygous G to C transversion [c.313G>C (p.Gly105Arg)] at nucleotide position 313 and a mutation caused by a heterozygous A to T transversion [c.1252A>T (p.lle418Phe)] at nucleotide position 1252. Identification of these 2 novel MCCC2 gene mutations in our patient suggested that analysis of the MCCC1 and MCCC2 genes might prove useful in the diagnosis of 3-methylcrotonyl-CoA carboxylase deficiency.
Carnitine ; Exons ; Glycine ; Humans ; Infant, Newborn ; Leucine ; Mass Screening ; Reference Values ; Sequence Analysis ; Tandem Mass Spectrometry ; Valerates

Despite remarkable progress in understanding and treating gastrointestinal stromal tumors (GISTs) during the past two decades, the pathological characteristics of GISTs have not been made clear yet. Furthermore, concrete diagnostic criteria of malignant GISTs are still uncertain. We collected pathology reports of 1,227 GISTs from 38 hospitals in Korea between 2003 and 2004 and evaluated the efficacy of the NIH and AFIP classification schemes as well as the prognostic factors among pathologic findings. The incidence of GISTs in Korea is about 1.6 to 2.2 patients per 100,000. Extra-gastrointestinal GISTs (10.1%) are more common in Korea than in Western countries. In univariate analysis, gender, age, tumor location, size, mitosis, tumor necrosis, vascular and mucosal invasions, histologic type, CD34 and s-100 protein expression, and classifications by the NIH and AFIP criteria were found to be significantly correlated with patient's survival. However, the primary tumor location, stage and classification of the AFIP criteria were prognostically significant in predicting patient's survival in multivariate analysis. The GIST classification based on original tumor location, size, and mitosis is more efficient than the NIH criteria in predicting patient's survival, but the mechanism still needs to be clarified through future studies.

Blood, saliva, and nail samples were collected from 54 dogs and 151 cats and analyzed for the presence of Bartonella henselae with a novel nested polymerase chain reaction (PCR) method. Bartonella (B.) henselae was detected in feral cat blood (41.8%), saliva (44.1%), and nail (42.7%) samples. B. henselae was also detected in pet cat blood (33.3%), saliva (43.5%), and nail (29.5%) samples and in pet dog blood (16.6%), saliva (18.5%), and nail (29.6%) samples. Nine samples were infected with B. clarridgeiae and 2 were co-infected with B. henselae and B. clarridgeiae of blood samples of dogs. This report is the first to investigate the prevalence of B. henselae and B. clarridgeiae in dogs and cats in Korea, and suggests that dogs and cats may serve as potential Bartonella reservoirs.
Animals ; Bartonella/*classification ; Bartonella Infections/blood/epidemiology/microbiology/*veterinary ; Cat Diseases/blood/epidemiology/*microbiology ; Cats ; Disease Reservoirs/veterinary ; Dog Diseases/epidemiology/*microbiology ; Dogs ; Hoof and Claw/microbiology ; Korea/epidemiology ; Prevalence ; Saliva/microbiology