Background: and Purpose The accurate prediction of functional outcomes in patients with acute ischemic stroke (AIS) is crucial for informed clinical decision-making and optimal resource utilization. As such, this study aimed to construct an ensemble deep learning model that integrates multimodal imaging and clinical data to predict the 90-day functional outcomes after AIS. Methods: We used data from the Korean Stroke Neuroimaging Initiative database, a prospective multicenter stroke registry to construct an ensemble model integrated individual 3D convolutional neural networks for diffusion-weighted imaging and fluid-attenuated inversion recovery (FLAIR), along with a deep neural network for clinical data, to predict 90-day functional independence after AIS using a modified Rankin Scale (mRS) of 3–6. To evaluate the performance of the ensemble model, we compared the area under the curve (AUC) of the proposed method with that of individual models trained on each modality to identify patients with AIS with an mRS score of 3–6. Results: Of the 2,606 patients with AIS, 993 (38.1%) achieved an mRS score of 3–6 at 90 days post-stroke. Our model achieved AUC values of 0.830 (standard cross-validation [CV]) and 0.779 (time-based CV), which significantly outperformed the other models relying on single modalities: b-value of 1,000 s/mm2 (P<0.001), apparent diffusion coefficient map (P<0.001), FLAIR (P<0.001), and clinical data (P=0.004). Conclusion The integration of multimodal imaging and clinical data resulted in superior prediction of the 90-day functional outcomes in AIS patients compared to the use of a single data modality.

Background and Objectives: Vascular smooth muscle cell (SMC) proliferation and migration play a critical role in neointimal formation. Focal adhesion is involved in cell proliferation and migration, and talin is known to be a key regulator of these processes. We synthesized a new talin modulator that binds to the talin protein, and investigated its effects on SMCs and neointimal formation after vascular injury. Methods: Human aortic SMCs (HAoSMCs) were treated with a newly synthesized talin modulator. Apolipoprotein E knockout (ApoE KO) mice were subjected to left femoral arterial injury and orally administered with the talin modulator daily. Laser Doppler imager was used to compare the blood flow, and injured femoral arteries and blood serum were analyzed after 28 days. Results: The talin modulator significantly inhibited cell proliferation in a concentration-dependent manner and suppressed the migration of HAoSMCs. Treatment with a talin modulator resulted in a significant reduction in the phosphorylation of focal adhesion molecules and downstream signaling molecules related to cell proliferation and migration. The effects of the talin modulator in HAoSMCs were found to be reversible, as evidenced by the reactivation of signaling pathways upon its removal. After 28 days of administration of the talin modulator, an improvement in the blood flow and reduction in neointimal formation in the injured femoral arteries were observed. Conclusions We demonstrated the inhibitory effects of a talin modulator on SMC proliferation and migration, and that were associated with downregulation of signaling pathways, resulting in the attenuation of neointimal formation in ApoE KO mice.

Hepatocellular carcinoma (HCC) is one of the major cancers with a high incidence and mortality in Korea. A Korean multidisciplinary collaborative committee consisting of hepatologists, radiologists, epidemiologists and family medicine doctors systematically reviewed clinical practice guidelines in the world and literatures. The level of evidence for each recommendation was assessed and discussed to reach a consensus. Meta-analysis was also conducted to evaluate the grade of recommendation for the five key questions. Several randomized controlled studies and cohort studies showed a survival gain associated with surveillance for those at risk of developing HCC. The target populations for HCC surveillance were identified as hepatitis B virus or hepatitis C virus carriers and cirrhotic patients, since numerous studies revealed that these patients have significantly higher risk of HCC compared with non-infected or non-cirrhotic controls. Individual surveillance strategy according to treatment history or degree of fibrosis in patients with viral hepatitis remains to be settled. Based on several cohort and randomized studies, a surveillance interval of six months was recommend. The starting age of surveillance was determined as 40 years from the epidemiologic data. Although ultrasonography (US) is the mainstay for detection of HCC, its sensitivity is not fully accepted. Measurement of serum alpha-fetoprotein can complement US examination, increasing the sensitivity of HCC detection. The recommendation for HCC surveillance is that those with hepatitis B virus (or hepatitis C virus) infection or cirrhosis should have liver US and serum alpha-fetoprotein measurement every six months from 40 years of age or at the time of diagnosis of cirrhosis.
alpha-Fetoproteins ; Carcinoma, Hepatocellular* ; Cohort Studies ; Complement System Proteins ; Consensus ; Diagnosis ; Fibrosis ; Health Services Needs and Demand ; Hepacivirus ; Hepatitis ; Hepatitis B virus ; Hepatitis C ; Humans ; Incidence ; Korea ; Liver ; Mortality ; Ultrasonography

BACKGROUND: We describe herein the application of whole exome sequencing (WES) for the molecular genetic diagnosis of a large Korean family with dominantly inherited myopathy. CASE REPORT: The affected individuals presented with slowly progressive proximal weakness and ankle contracture. They were initially diagnosed with limb-girdle muscular dystrophy (LGMD) based on clinical and pathologic features. However, WES and subsequent capillary sequencing identified a pathogenic splicing-site mutation (c.1056+1G>A) in COL6A1, which was previously reported to be an underlying cause of Bethlem myopathy. After identification of the genetic cause of the disease, careful neurologic examination revealed subtle contracture of the interphalangeal joint in the affected members, which is a characteristic sign of Bethlem myopathy. Therefore, we revised the original diagnosis from LGMD to Bethlem myopathy. CONCLUSIONS: This is the first report of identification of COL6A1-mediated Bethlem myopathy in Korea, and indicates the utility of WES for the diagnosis of muscular dystrophy.
Ankle ; Capillaries ; Contracture ; Diagnosis* ; Exome* ; Humans ; Joints ; Korea ; Molecular Biology* ; Muscular Diseases* ; Muscular Dystrophies ; Muscular Dystrophies, Limb-Girdle ; Neurologic Examination

Enteropathy-type T-cell lymphoma (ETL) or enteropathy-associated T-cell lymphoma is a very rare malignant intestinal tumor. ETL is usually diagnosed by surgery. Endoscopic findings of ETL are not well known, and there are few reports of findings from endoscopy that has been performed only using white light. Additionally, there are no definite treatment guidelines for ETL. Therefore, we report a case of ETL diagnosed by enteroscopy with imaging-enhanced endoscopy and also review recently developed treatment options.
Endoscopy ; Enteropathy-Associated T-Cell Lymphoma ; Humans ; Light ; Lymphoma, T-Cell ; T-Lymphocytes

The most common recurrent cytogenetic abnormalities in T-lymphoblastic leukemia (T-acute lymphoblastic leukemia [T-ALL]) involve T-cell receptor (TCR) loci and a variety of partner genes, including HOX11, HOX11L2, MYC, and TAL1. In this report, we present a rare case involving simultaneous translocation of the TCR alpha/delta loci with different partner loci (Xq22 and 12p13); this resulted in a poor prognosis. Chromosomal analysis showed 46,Y,t(X;14)(q22;q11.2),t(12;14)(p13;q11.2) and FISH analysis by using a T-cell receptor alpha delta DNA probe, Split Signal (DakoCytomation, Denmark), showed translocations at the same TCR alpha/delta locus on both chromosomes. FISH with 2 bacterial artificial chromosome clones showed break apart signal, which suggests involvement of the IRS4 gene. To our knowledge, this is the first report of T-ALL in which both TCR alpha/delta loci were translocated with different partner loci, and 1 of the partner loci, Xq22, was a rare translocation partner locus that included IRS4 gene.
Adult ; Chromosomes, Human, Pair 12 ; Chromosomes, Human, Pair 14 ; Chromosomes, Human, X ; Genetic Loci ; Humans ; Insulin Receptor Substrate Proteins/genetics ; Karyotyping ; Male ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma/*genetics/pathology ; Receptors, Antigen, T-Cell/*genetics ; *Translocation, Genetic

PURPOSE: This study was undertaken to investigate the therapeutic effects of topiramate on pediatric patients with migraine, especially migraine accompanied by aura. METHODS: From January. 2004 to December. 2006, we reviewed the medical records of 27 patients who were diagnosed as migraine and treated with topiramate. And we analysed to see whether there was any improvement of symptoms based on the migraine criteria by International Headache Society. RESULTS: There was a correlation between the migraine criteria and the improvement of symptoms after the treatment by topiramate. The symptoms of nausea, vomiting and hypersensitivity to light and sound were not associated with the improvement of symptoms after the treatment by topiramate. The symptom of aura was related with the improvement of symptoms, moreover, closely related with disappearance of symptoms after the treatment by topiramate. CONCLUSION: It is estimated that more accurate diagnosis coupled with the presence of aura is a condition to improve the treatment effects of topiramate.
Diagnosis ; Epilepsy* ; Headache ; Humans ; Hypersensitivity ; Medical Records ; Migraine Disorders* ; Nausea ; Vomiting

BACKGROUND: Although several hospital-based stroke studies were published, there has not been any reliable data representing the clinical characteristics of stroke in Korea. We analyzed the clinical characteristics of patients with ischemic stroke registered in the Korean Stroke Registry (KSR), which is the largest prospective hospital-based nation-wide stroke registry in Korea. METHODS: The KSR provided standardized protocols for collecting data, which includes the data of demographics, subtypes of stroke, risk factors, and neurological outcome at discharge. The brain imaging studies, including CT or MRI, were performed in all cases. RESULTS: KSR registered 10,811 patients of acute ischemic stroke between Nov. 2002 and Jun. 2004. The large-artery atherosclerosis was the most common subtype (37.3%), followed by small vessel occlusion (30.8%). Hypertension (65.4%) was the most common risk factor, followed by smoking (34.5%) and diabetes (28.3%). Although most of the hypertensive and diabetic patients had been diagnosed before the stroke, less than 45.4% and 32.5% of them were under regular control. The steno-occlusive lesion of extracranial carotid artery was only 29.3% and the ratio of intra- to extracranial artery disease was more than 2 in KSR. Only 20.5% of patients were admitted within 3 hours after stroke onset and 2.1% were treated with intravenous thrombolysis. In-hospital case-fatality was 5.2%, which is relatively comparable to those of previous studies. CONCLUSIONS: The KSR provided informative data in understanding the clinical characteristics of ischemic stroke in Korea. Further analysis of KSR will facilitate clinical trials and development of guidelines for the management of stroke patients.
Arteries ; Atherosclerosis ; Carotid Arteries ; Cerebrovascular Disorders ; Demography ; Epidemiology ; Humans ; Hypertension ; Korea ; Magnetic Resonance Imaging ; Neuroimaging ; Prospective Studies* ; Registries ; Risk Factors ; Smoke ; Smoking ; Stroke*

BACKGROUND: A rapid increase of antimicrobial-resistant bacteria has become a serious problem in many countries. The aim of this study was to determine the prevalence of resistance among frequently isolated gram-positive and -negative bacteria in Korea. METHODS: Data of routine antimicrobial susceptibility test for medically important bacteria, isolated during 3 months of 2002, were collected from 12 university and 1 commercial laboratories in Korea. RESULTS: The proportions of methicillin-resistant Staphylococcus aureus (MRSA) were 60-88%, but vancomycin-resistant S. aureus was not detected. Among the Enterococcus faecium isolates, the resistance rate to vancomycin was 29%. The resistance rates of Escherichia coli and Klebsiella pneumoniae: 11% and 24% to cefotaxime, respectively, and 12% and 21% to cefoxitin, respectively. The resistance rates of Citrobacter freundii, Enterobacter cloacae, and Serratia marcescens: 28%, 34% and 21% to cefotaxime, respectively, <1%, 8% and 14% to cefepime, respectively. The resistance rates of Acinetobacter baumannii and Pseudomonas aeruginosa were: 65% and 37% to piperacillin, 64% and 19% to ceftazidime, 13% and 20% to imipenem, respectively. The resistant rates varied according to the hospital size. The resistance rates were generally higher among the isolates in the hospitals with more than 1,000 beds. The rates of penicillin-nonsusceptible Streptococcus pneumoniae were 58-90%. Among the Haemophilus influenzae isolates, 55-68% were resistant to ampicillin. CONCLUSIONS: Antimicrobial resistant strains were prevalent among the medically important clinical isolates, especially, MRSA, vancomycin-resistant enterococci, extended-spectrum -lactamase- or AmpC -lactamase-producing E. coli and K. pneumoniae, third generation cephalosporin-resistant C. freundii, E. cloacae and S. marcescens, imipenem-resistant A. baumannii and P. aeruginosa, penicillin-nonsusceptible S. pneumoniae and ampicillin-resistant H. influenzae. The antimicrobial resistance has become a serious problem in Korea.
Acinetobacter baumannii ; Ampicillin ; Bacteria* ; Cefotaxime ; Cefoxitin ; Ceftazidime ; Citrobacter freundii ; Cloaca ; Enterobacter cloacae ; Enterococcus faecium ; Escherichia coli ; Haemophilus influenzae ; Health Facility Size ; Imipenem ; Influenza, Human ; Klebsiella pneumoniae ; Korea* ; Methicillin-Resistant Staphylococcus aureus ; Piperacillin ; Pneumonia ; Prevalence ; Pseudomonas aeruginosa ; Serratia marcescens ; Streptococcus pneumoniae ; Vancomycin

Many endocrinologic diseases can induce muscular diseases. Myopathy caused by exogenous steroid is a common problem in patients prescribed steroids as therapy. We report a case of iatrogenic steroid myopathy in a 55-year-old female who had taken steroids under her own volition at a local pharmacy for more than 3 months due to skin rash and itching. She complained of severe proximal muscle wasting and weakness in the lower extremities and also exhibited other stigmata of Cushing's syndrome such as moon face, buffalo hump or easy bruising. Needle electromyography showed the typical pattern of myopathy. Muscle biopsy revealed intermixed numerous, markedly atrophic and angulated basophilic fibers and a few fat cells without inflammation. In addition there was marked and selective atrophy of type II fiber on ATPase staining in pH 9.4 buffer. After discontinuation of steroid treatment, she has experienced slow improvement through physical therapy, including isotonic exercise.
Adenosine Triphosphatases ; Adipocytes ; Atrophy ; Basophils ; Biopsy ; Buffaloes ; Christianity ; Cushing Syndrome* ; Electromyography ; Exanthema ; Female ; Humans ; Hydrogen-Ion Concentration ; Inflammation ; Lower Extremity ; Middle Aged ; Muscular Diseases* ; Needles ; Pharmacy ; Pruritus ; Steroids ; Volition