Hypoplastic left heart syndrome is a rare congenital heart disease. This is a universally fatal leison, and approximately 80% of patients die within the first week of life. The diagnosis should be considered in infants, with the sudden onset of heart failure, systemic hypoperfusion and cyanosis. 2 cases of hypoplastic left syndrome are presented with review of related literatures.
Cyanosis ; Diagnosis ; Heart Defects, Congenital ; Heart Failure ; Humans ; Hypoplastic Left Heart Syndrome* ; Infant

This paper describes the design, development, and implementation of a ubiquitous portfolio (u-portfolio) system aimed at enhancing clinical clerkship education at Inje University College of Medicine from 2016 to 2020. This developmental research employed involved a literature review of 69 papers on electronic portfolios (1990–2013), and as well as a series of focus group interviews and surveys with key stakeholders during the iterative design, evaluation, and revision process. The literature analysis revealed three main purposes of e-portfolios (learning, assessment, and showcase) and nine key elements for a multi-purpose e-portfolio (e.g., quality of student reflection, postgraduate continuous learning, and timely and frequent feedback). The system evolved from paper-based portfolios to address the standardization of students’ clerkship experiences across five teaching hospitals, incorporating competency-based education and enabling efficient data management to promote student learning, assessment, and feedback. Key challenges in transitioning from paper to electronic portfolios included infrastructure issues, user adaptation, and assessment standardization. The resulting u-portfolio system, which allowed the seamless integration of mobile devices, emphasized five core functions: enhanced real-time feedback, transparent evaluation with rubrics, improved reflection quality, real-time assessment and tracking, and mapping between learning and competency achievements. Initially implemented in the internal medicine clerkship in 2016, the system was gradually expanded to all core clerkships by 2018. The success of this system led to its adoption by 19 medical schools through a consortium organized by the Korea Association of Medical Colleges, with 31 schools now collaborating to enhance clerkships through the u-portfolio system.

This paper describes the design, development, and implementation of a ubiquitous portfolio (u-portfolio) system aimed at enhancing clinical clerkship education at Inje University College of Medicine from 2016 to 2020. This developmental research employed involved a literature review of 69 papers on electronic portfolios (1990–2013), and as well as a series of focus group interviews and surveys with key stakeholders during the iterative design, evaluation, and revision process. The literature analysis revealed three main purposes of e-portfolios (learning, assessment, and showcase) and nine key elements for a multi-purpose e-portfolio (e.g., quality of student reflection, postgraduate continuous learning, and timely and frequent feedback). The system evolved from paper-based portfolios to address the standardization of students’ clerkship experiences across five teaching hospitals, incorporating competency-based education and enabling efficient data management to promote student learning, assessment, and feedback. Key challenges in transitioning from paper to electronic portfolios included infrastructure issues, user adaptation, and assessment standardization. The resulting u-portfolio system, which allowed the seamless integration of mobile devices, emphasized five core functions: enhanced real-time feedback, transparent evaluation with rubrics, improved reflection quality, real-time assessment and tracking, and mapping between learning and competency achievements. Initially implemented in the internal medicine clerkship in 2016, the system was gradually expanded to all core clerkships by 2018. The success of this system led to its adoption by 19 medical schools through a consortium organized by the Korea Association of Medical Colleges, with 31 schools now collaborating to enhance clerkships through the u-portfolio system.

This paper describes the design, development, and implementation of a ubiquitous portfolio (u-portfolio) system aimed at enhancing clinical clerkship education at Inje University College of Medicine from 2016 to 2020. This developmental research employed involved a literature review of 69 papers on electronic portfolios (1990–2013), and as well as a series of focus group interviews and surveys with key stakeholders during the iterative design, evaluation, and revision process. The literature analysis revealed three main purposes of e-portfolios (learning, assessment, and showcase) and nine key elements for a multi-purpose e-portfolio (e.g., quality of student reflection, postgraduate continuous learning, and timely and frequent feedback). The system evolved from paper-based portfolios to address the standardization of students’ clerkship experiences across five teaching hospitals, incorporating competency-based education and enabling efficient data management to promote student learning, assessment, and feedback. Key challenges in transitioning from paper to electronic portfolios included infrastructure issues, user adaptation, and assessment standardization. The resulting u-portfolio system, which allowed the seamless integration of mobile devices, emphasized five core functions: enhanced real-time feedback, transparent evaluation with rubrics, improved reflection quality, real-time assessment and tracking, and mapping between learning and competency achievements. Initially implemented in the internal medicine clerkship in 2016, the system was gradually expanded to all core clerkships by 2018. The success of this system led to its adoption by 19 medical schools through a consortium organized by the Korea Association of Medical Colleges, with 31 schools now collaborating to enhance clerkships through the u-portfolio system.

This paper describes the design, development, and implementation of a ubiquitous portfolio (u-portfolio) system aimed at enhancing clinical clerkship education at Inje University College of Medicine from 2016 to 2020. This developmental research employed involved a literature review of 69 papers on electronic portfolios (1990–2013), and as well as a series of focus group interviews and surveys with key stakeholders during the iterative design, evaluation, and revision process. The literature analysis revealed three main purposes of e-portfolios (learning, assessment, and showcase) and nine key elements for a multi-purpose e-portfolio (e.g., quality of student reflection, postgraduate continuous learning, and timely and frequent feedback). The system evolved from paper-based portfolios to address the standardization of students’ clerkship experiences across five teaching hospitals, incorporating competency-based education and enabling efficient data management to promote student learning, assessment, and feedback. Key challenges in transitioning from paper to electronic portfolios included infrastructure issues, user adaptation, and assessment standardization. The resulting u-portfolio system, which allowed the seamless integration of mobile devices, emphasized five core functions: enhanced real-time feedback, transparent evaluation with rubrics, improved reflection quality, real-time assessment and tracking, and mapping between learning and competency achievements. Initially implemented in the internal medicine clerkship in 2016, the system was gradually expanded to all core clerkships by 2018. The success of this system led to its adoption by 19 medical schools through a consortium organized by the Korea Association of Medical Colleges, with 31 schools now collaborating to enhance clerkships through the u-portfolio system.

This paper describes the design, development, and implementation of a ubiquitous portfolio (u-portfolio) system aimed at enhancing clinical clerkship education at Inje University College of Medicine from 2016 to 2020. This developmental research employed involved a literature review of 69 papers on electronic portfolios (1990–2013), and as well as a series of focus group interviews and surveys with key stakeholders during the iterative design, evaluation, and revision process. The literature analysis revealed three main purposes of e-portfolios (learning, assessment, and showcase) and nine key elements for a multi-purpose e-portfolio (e.g., quality of student reflection, postgraduate continuous learning, and timely and frequent feedback). The system evolved from paper-based portfolios to address the standardization of students’ clerkship experiences across five teaching hospitals, incorporating competency-based education and enabling efficient data management to promote student learning, assessment, and feedback. Key challenges in transitioning from paper to electronic portfolios included infrastructure issues, user adaptation, and assessment standardization. The resulting u-portfolio system, which allowed the seamless integration of mobile devices, emphasized five core functions: enhanced real-time feedback, transparent evaluation with rubrics, improved reflection quality, real-time assessment and tracking, and mapping between learning and competency achievements. Initially implemented in the internal medicine clerkship in 2016, the system was gradually expanded to all core clerkships by 2018. The success of this system led to its adoption by 19 medical schools through a consortium organized by the Korea Association of Medical Colleges, with 31 schools now collaborating to enhance clerkships through the u-portfolio system.

OBJECTIVES: The etiology for Kawasaki disease (KD) remains unknown, but several studies have suggested the involvement of immune dysregulation and genetic factors. The purpose of this study is to compare gene expressions before and after an infusion of intravenous immunoglobulin (IVIG) in KD patients. METHODS: Blood was obtained from both acute and sub-acute phases of 4 patients with KD and febrile control children. Blood was collected in PAXgene blood RNA tubes and RNA was extracted using a PAXgene blood RNA isolation kit. Labeled RNAs were analyzed using Roche NimbleGen human whole genome 12-plex array. RESULTS: KD patients prior to IVIG injection showed more than a two-fold increase in the expression of 88 genes and more than a two-fold decrease in the expression of 98 genes compared to the control group. They also showed more than two-fold increase in the expression of 226 genes and more than a two-fold decrease in 117 genes in KD patients after IVIG treatment compared to the patients before IVIG injection. Through microarray evaluation, the expressions of genes involved in proliferation, translation, inflammatory response, immune response, cell adhesion, cell migration, cell differentiation, apoptosis, cell growth, transport, cell cycle, transcription, signal transduction and metastasis were observed. CONCLUSION: Changes in gene expressions in pediatric patients with KD before and after IVIG were observed via microarray evaluation.
Apoptosis ; Cell Adhesion ; Cell Cycle ; Cell Differentiation ; Cell Movement ; Child ; Gene Expression ; Genome ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Microarray Analysis ; Mucocutaneous Lymph Node Syndrome ; Neoplasm Metastasis ; RNA ; Signal Transduction

BACKGROUND AND OBJECTIVES: We sought to determine parameters to guide the decision of retreatment in patients with Kawasaki disease (KD) who remained febrile after initial intravenous immunoglobulin (IVIG). SUBJECTS AND METHODS: A total of 129 children with KD were studied prospectively. Patients were treated with IVIG 2 to 9 days after the onset of disease. Laboratory measures, such as white blood cell (WBC), percentage of neutrophils, C-reactive protein (CRP), and N-terminal pro-brain natriuretic peptide (NT-proBNP), were determined before and 48 to 72 hours after IVIG treatment. Patients were classified into IVIG-responsive and IVIG-resistant groups, based on the response to IVIG. RESULTS: Of a total of 129 patients, 107 patients (83%) completely responded to a single IVIG therapy and only 22 patients (17%) required retreatment: 14 had persistent fever and 8 had recrudescent fever. There was no significant difference between the groups in age, gender distribution, and duration of fever to IVIG initiation, but coronary artery lesions developed significantly more often in the resistant group than in the responsive group (31.8% vs. 2.8%, p=0.000). Compared with pre-IVIG data, post-IVIG levels of WBC, percentage of neutrophils, CRP, and NT-proBNP decreased to within the normal range in the responsive group, whereas they remained high in the resistant group. Multivariate logistic regression indicated that neutrophil counts, CRP, and NT-proBNP were independent parameters of retreatment. CONCLUSION: Additional therapy at an early stage of the disease should be administered for febrile patients who have high values of CRP, NT-proBNP, and/or neutrophil counts after IVIG therapy.
C-Reactive Protein ; Child ; Coronary Vessels ; Fever ; Humans ; Immunization, Passive ; Immunoglobulins ; Immunoglobulins, Intravenous ; Leukocytes ; Logistic Models ; Mucocutaneous Lymph Node Syndrome ; Natriuretic Peptide, Brain ; Neutrophils ; Peptide Fragments ; Prospective Studies ; Reference Values ; Retreatment

PURPOSE: The incidence of Kawasaki disease (KD) is rare in young infants (less than 3 months of age), who present with only a few symptoms that fulfill the clinical diagnostic criteria. The diagnosis for KD can therefore be delayed, leading to a high risk of cardiac complications. We examined the clinical characteristics and measured the serum levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) levels of these patients for assessing its value in the early detection of KD. METHODS: We retrospectively reviewed the data of young infants diagnosed with KD from 2004 to 2012. The control group included 20 hospitalized febrile patients. Laboratory data, including NT-proBNP were obtained for each patient in both groups. RESULTS: Incomplete KD was observed in 21/24 patients (87.5%). The mean fever duration on admission was 1.36+/-1.0 days in the KD group. Common symptoms included erythema at the site of Bacille Calmette-Guerin inoculation (70.8%), skin rash (50.0%), changes of oropharyngeal mucosa (29.1%), and cervical lymphadenopathy (20.8%). The mean number of major diagnostic criteria fulfilled was 2.8+/-1.4. Five KD patients (20.8%) had only one symptom matching these criteria. The incidence of coronary artery complications was 12.5%. The mean serum NT-proBNP level in the acute phase, in the KD and control groups, were 4,159+/-3,714 pg/mL and 957+/-902 pg/mL, respectively, which decreased significantly in the convalescent phase. CONCLUSION: Incomplete KD was observed in 87.5% patients. Serum NT-proBNP might be a valuable biomarker for the early detection of KD in febrile infants aged <3 months.
Coronary Vessels ; Diagnosis ; Erythema ; Exanthema ; Fever ; Humans ; Incidence ; Infant* ; Lymphatic Diseases ; Mucocutaneous Lymph Node Syndrome* ; Mucous Membrane ; Natriuretic Peptide, Brain ; Retrospective Studies

PURPOSE: Incomplete Kawasaki disease (KD) is frequently associated with delayed diagnosis and treatment. Delayed diagnosis leads to increasing risk of coronary artery aneurysm. Anterior uveitis is an important ocular sign of KD. The purpose of this study was to assess differences in laboratory findings, including echocardiographic measurements, clinical characteristics such as fever duration and treatment responses between KD patients with and those without uveitis. METHODS: We conducted a prospective study with 110 KD patients from January 2008 to June 2013. The study group (n=32, KD with uveitis) was compared with the control group (n=78, KD without uveitis). Laboratory data were obtained from each patient including complete blood count (CBC), erythrocyte sedimentation rate (ESR), platelet count, and level of alanine aminotransferase, aspartate aminotransferase, serum total protein, albumin, C-reactive protein (CRP), and N-terminal probrain natriuretic peptide (NT-pro BNP). Echocardiographic measurements and intravenous immunoglobulin responses were compared between the two groups. RESULTS: The incidence of uveitis was 29.0%. Neutrophil counts and patient age were higher in the uveitis group than in the control group. ESR and CRP level were slightly increased in the uveitis group compared with the control group, but the difference between the two groups was not significant. No significant differences in coronary arterial complication and treatment responses were observed between the two groups. CONCLUSION: Uveitis is an important ocular sign in the diagnosis of incomplete KD. It is significantly associated with patient age and neutrophil count.
Alanine Transaminase ; Aneurysm ; Aspartate Aminotransferases ; Blood Cell Count ; Blood Sedimentation ; C-Reactive Protein ; Coronary Vessels ; Delayed Diagnosis ; Diagnosis ; Early Diagnosis* ; Echocardiography ; Fever ; Humans ; Immunoglobulins ; Incidence ; Mucocutaneous Lymph Node Syndrome* ; Natriuretic Peptide, Brain ; Neutrophils ; Platelet Count ; Prospective Studies ; Uveitis* ; Uveitis, Anterior