Background/Aims: Subepithelial tumors (SETs) are small, mostly asymptomatic lesions with normal overlying mucosa, usually identified incidentally on endoscopy. The aim of this study was to evaluate the pathologic diagnosis of SETs, and to assess the diagnostic yield and impact of endoscopic submucosal dissection (ESD) biopsy on the management of patients with SETs. Materials and Methods: We included 52 subepithelial lesions in this study during the study period. Inclusion criteria included size of the SET >2 cm, and a gastrointestinal stromal tumor (GIST) that cannot be excluded using EUS. We performed an endoscopic biopsy of each SET using the ESD technique. Results: The mean diameter of the lesions was 24.15±6.0 mm. The diagnostic yield of this method was 96.15%. Among the 52 participants, 45 were located in the stomach, four in the esophagus, and three in the duodenum. The pathologic diagnoses included: 17 leiomyomas, 13 GISTs, 11 ectopic pancreases, two carcinomas, two inflammatory fibroid polyps, two Brunner’s gland hyperplasia, two lipomas, one glomus tumor, and two remained undiagnosed. The mean duration of the procedure was 13.44±2.41 minutes. Three complications were associated with the procedure. Conclusions Deep biopsy via ESD is useful in determining the histopathologic nature of SETs. This method minimizes the need for unnecessary surgery in benign SETs.

Background/Aims: Subepithelial tumors (SETs) are small, mostly asymptomatic lesions with normal overlying mucosa, usually identified incidentally on endoscopy. The aim of this study was to evaluate the pathologic diagnosis of SETs, and to assess the diagnostic yield and impact of endoscopic submucosal dissection (ESD) biopsy on the management of patients with SETs. Materials and Methods: We included 52 subepithelial lesions in this study during the study period. Inclusion criteria included size of the SET >2 cm, and a gastrointestinal stromal tumor (GIST) that cannot be excluded using EUS. We performed an endoscopic biopsy of each SET using the ESD technique. Results: The mean diameter of the lesions was 24.15±6.0 mm. The diagnostic yield of this method was 96.15%. Among the 52 participants, 45 were located in the stomach, four in the esophagus, and three in the duodenum. The pathologic diagnoses included: 17 leiomyomas, 13 GISTs, 11 ectopic pancreases, two carcinomas, two inflammatory fibroid polyps, two Brunner’s gland hyperplasia, two lipomas, one glomus tumor, and two remained undiagnosed. The mean duration of the procedure was 13.44±2.41 minutes. Three complications were associated with the procedure. Conclusions Deep biopsy via ESD is useful in determining the histopathologic nature of SETs. This method minimizes the need for unnecessary surgery in benign SETs.

The optimal dose of beta blockers after acute myocardial infarction (MI) remains uncertain. We evaluated the effectiveness of low-dose nebivolol, a beta1 blocker and a vasodilator, in patients with acute MI. A total of 625 patients with acute MI from 14 teaching hospitals in Korea were divided into 2 groups according to the dose of nebivolol (nebistol®, Elyson Pharmaceutical Co., Ltd., Seoul, Korea): low-dose group (1.25 mg daily, n=219) and usual- to high-dose group (≥2.5 mg daily, n=406). The primary endpoints were major adverse cardiac and cerebrovascular events (MACCE, composite of death from any cause, non-fatal MI, stroke, repeat revascularization, rehospitalization for unstable angina or heart failure) at 12 months. After adjustment using inverse probability of treatment weighting, the rates of MACCE were not different between the low-dose and the usual- to high-dose groups (2.8% and 3.1%, respectively; hazard ratio: 0.92, 95% confidence interval: 0.38 to 2.24, p=0.860). The low-dose nebivolol group showed higher rates of MI than the usual- to high-dose group (1.2% and 0%, p=0.008). The 2 groups had similar rates of death from any cause (1.1% and 0.3%, p=0.273), stroke (0.4% and 1.1%, p=0.384), repeat PCI (1.2% and 0.8%, p=0.428), rehospitalization for unstable angina (1.2% and 1.0%, p=0.743) and for heart failure (0.6% and 0.7%, p=0.832). In patients with acute MI, the rates of MACCE for low-dose and usual- to high-dose nebivolol were not significantly different at 12-month follow-up.

Mutations in the calreticulin gene, CALR, have recently been discovered in subsets of patients with essential thrombocythemia (ET) or primary myelofibrosis (PMF). We investigated Korean patients with ET and PMF to determine the prevalence, and clinical and laboratory correlations of CALR/JAK2/MPL mutations. Among 84 ET patients, CALR mutations were detected in 23 (27.4%) and were associated with higher platelet counts (P=0.006) and lower leukocyte counts (P=0.035) than the JAK2 V617F mutation. Among 50 PMF patients, CALR mutations were detected in 11 (22.0%) and were also associated with higher platelet counts (P=0.035) and trended to a lower rate of cytogenetic abnormalities (P=0.059) than the JAK2 V617F mutation. By multivariate analysis, triple-negative status was associated with shorter overall survival (HR, 7.0; 95% CI, 1.6-31.1, P=0.01) and leukemia-free survival (HR, 6.3; 95% CI, 1.8-22.0, P=0.004) in patients with PMF. The type 1 mutation was the most common (61.1%) type among all patients with CALR mutations, and tended toward statistical predominance in PMF patients. All 3 mutations were mutually exclusive and were never detected in patients with other myeloid neoplasms showing thrombocytosis. CALR mutations characterize a distinct group of Korean ET and PMF patients. Triple-negative PMF patients in particular have an unfavorable prognosis, which supports the idea that triple-negative PMF is a molecularly high-risk disease.
Adult ; Aged ; Aged, 80 and over ; Calreticulin/*genetics ; Disease-Free Survival ; Female ; Gene Frequency ; Genetic Association Studies ; Humans ; Janus Kinase 2/*genetics ; Male ; Middle Aged ; Mutation/genetics ; Primary Myelofibrosis/*genetics/mortality ; Receptors, Thrombopoietin/*genetics ; Republic of Korea ; Thrombocythemia, Essential/*genetics/mortality ; Young Adult

BACKGROUND: Efforts to overcome poor outcomes in patients with adult acute lymphoblastic leukemia (ALL) have focused on combining new therapeutic agents targeting immunophenotypic markers (IPMs) with classical cytotoxic agents; therefore, it is important to evaluate the clinical significance of IPMs. METHODS: Baseline characteristics and clinical outcomes of patients with adult ALL were retrospectively analyzed. The percentage of blasts expressing IPMs at diagnosis was measured by multicolor flow cytometry analysis. Samples in which > or =20% of blasts expressed an IPM were considered positive. RESULTS: Among the total patient population (N=230), almost all (92%) were in first or second hematological complete remission (HCR) and 54% received allogeneic hematopoietic cell transplant (allo-HCT). Five-year hematologic relapse-free survival (HRFS) and overall survival (OS) rates were 36% and 39%, respectively, and 45.6% and 80.5% of patients were positive for the IPMs CD20 and terminal deoxynucleotidyl transferase (TdT), respectively. Expression of CD20, CD13, CD34, and TdT was associated with HRFS rate, and expression of CD20 and CD13 was associated with OS rate, as was the performance of allo-HCT. In multivariate analysis, positivity for CD20 (HRFS: hazard ratio [HR], 2.21, P<0.001; OS: HR, 1.63, P=0.015) and negativity for TdT (HRFS: HR, 2.30, P=0.001) were both significantly associated with outcomes. When patients were categorized into three subgroups according to positivity for CD20 and TdT, there were significant differences in HRFS and OS among the subgroups. CONCLUSION: Positivity for CD20 and TdT expression and clinical risk group were prognostic factors in adult ALL.
Adult* ; Cytotoxins ; Diagnosis ; DNA Nucleotidylexotransferase ; Flow Cytometry ; Humans ; Multivariate Analysis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Retrospective Studies ; Transplants

Danon disease is characterized clinically by the triad of cardiomyopathy, myopathy and mental retardation. It was originally reported as a lysosomal glycogen storage disease with normal acid maltase by Danon. Danon disease results from mutations in lysosome associated membrane protein-2 (LAMP-2) gene. The LAMP-2 gene is located on Xq24-25. We report a case of suspected Danon disease in patient who had hypertrophic cardiomyopathy and mental retardation along with abnormal findings in electromyography.
alpha-Glucosidases ; Cardiomyopathies ; Cardiomyopathy, Hypertrophic ; Glycogen Storage Disease ; Glycogen Storage Disease Type IIb ; Humans ; Intellectual Disability ; Lysosomes ; Membranes ; Muscular Diseases

PURPOSE: To investigate the care patterns for radiation therapy and to determine inter-hospital differences for patients with laryngeal carcinoma in Korea. MATERIALS AND METHODS: A total of 237 cases of laryngeal carcinoma (glottis, 144; supraglottis, 93) assembled from 23 hospitals, who underwent irradiation in the year of 1998 and 1999, were retrospectively analyzed to investigate inter-hospital differences with respect to radiotherapy treatment. We grouped the 23 hospitals based on the number of new patients annually irradiated in 1998; and designated them as group A (> or =900 patients), group B (> or =400 patients and <900 patients), and group C (<400 patients). RESULTS: The median age of the 237 patients was 62 years (range, 25 to 88 years), of which 216 were male and 21 were female. The clinical stages were distributed as follows: for glottis cancer, I; 61.8%, II; 21.5%, III; 4.2%, IVa; 11.1%, IVb; 1.4%, and in supraglottic cancer, I; 4.3%, II; 19.4%, III; 28.0%, IVa; 43.0%, IVb; 5.4%, respectively. Some differences were observed among the 3 groups with respect to the dose calculation method, radiation energy, field arrangement, and use of an immobilization device. No significant difference among 3 hospital groups was observed with respect to treatment modality, irradiation volume, and median total dose delivered to the primary site. CONCLUSION: This study revealed that radiotherapy process and patterns of care are relatively uniform in laryngeal cancer patients in Korean hospitals, and we hope this nationwide data can be used as a basis for the standardization of radiotherapy for the treatment of laryngeal cancer.
Female ; Glottis ; Humans ; Immobilization ; Korea ; Laryngeal Neoplasms ; Male ; Retrospective Studies

A 48-year-old woman visited the emergency department with shock due to a urinary tract infection. The patient, who had a history of hypertension and diabetes mellitus, presented with precordial ST-segment elevation and Q waves, along with an increase of cardiac enzymes. An echocardiography showed moderately reduced systolic function, severe apical left ventricular ballooning, and a dynamic left ventricular outflow tract obstruction with a pressure gradient of 109 mmHg. Coronary angiography demonstrated normal coronary arteries. At the 1-month echocardiographic follow-up, the apical ballooning and left ventricular systolic function had recovered completely. There was no residual left ventricular intra-cavity gradient at rest, but it was induced in low-dose dobutamine stress-echocardiography. We demonstrated that dynamic left midventricular obstruction in the setting of either increased catecholamine stress or hypovolemia could develop Tako-tsubo cardiomyopathy.
Coronary Angiography ; Coronary Vessels ; Diabetes Mellitus ; Dobutamine ; Echocardiography ; Echocardiography, Stress ; Emergencies ; Female ; Follow-Up Studies ; Humans ; Hypertension ; Hypovolemia ; Middle Aged ; Shock ; Takotsubo Cardiomyopathy ; Urinary Tract Infections ; Ventricular Outflow Obstruction

BACKGROUND AND OBJECTIVES: Right ventricular (RV) dysfunction is associated with a poor prognosis in patients with an acute pulmonary embolism (APE). We studied the role of electrocardiography and biomarkers for early detection and recovery of right ventricular dysfunction (RVD) in APE. SUBJECTS AND METHODS: The medical records of 48 consecutive patients diagnosed with APE using CT-angiography, at the Kangdong Sacred Heart Hospital, between January 2004 and February 2008 were reviewed retrospectively. RVD was assessed by serial echocardiography (ECG). Patients with one of the following were considered to have RVD: 1) RV dilatation (enddiastolic diameter >30 mm in the parasternal long axis view), 2) RV free wall hypokinesia, and 3) paradoxical septal systolic motion. We compared the electrocardiographic findings and the biomarkers for the early detection of RVD. RESULTS: The electrocardiographic findings showed T-wave inversion (TWI) in leads V1 to V3 with a sensitivity of 75% and a specificity of 95%, and a diagnostic accuracy of 80% for the detection of RVD, with positive and negative predictive values of 95.5% and 73.1%, respectively; these results were better than the biomarkers such as cardiac enzymes or B-type natriuretic peptide (BNP) for the early detection of RVD. TWIs persisted throughout the period of RVD, in contrast to a transient S1Q3T3 pattern detected during the acute phase only. CONCLUSION: TWIs in leads V1 to V3 had the greatest sensitivity and diagnostic accuracy for early detection of RVD, and normalization of the TWIs was associated with recovery of RVD in APE.
Axis, Cervical Vertebra ; Biomarkers ; Dilatation ; Echocardiography ; Electrocardiography ; Heart ; Hominidae ; Humans ; Hypokinesia ; Medical Records ; Natriuretic Peptide, Brain ; Prognosis ; Pulmonary Embolism ; Retrospective Studies ; Sensitivity and Specificity ; Ventricular Dysfunction, Right

Nasopharyngeal carcinoma (NPC) is one of the infrequently occurring malignant tumors in childhood. It is different from the adult form of the disease by its close association with Epstein-Barr virus infection, lymphoepithelial histology, and the high incidence of locoregional advancement of the disease. However, these tumors are associated with higher cure rates as they are very sensitive to radiation. We report two cases of pediatric nasopharyngeal carcinoma with locoregional advancement.
Adult ; Herpesvirus 4, Human ; Humans ; Incidence ; Nasopharyngeal Neoplasms ; Pediatrics