Background: This study aimed to generate a Z score calculation model for coronary artery diameter of normal children and adolescents to be adopted as the standard calculation method with consensus in clinical practice. Methods: This study was a retrospective, multicenter study that collected data from multiple institutions across South Korea. Data were analyzed to determine the model that best fit the relationship between the diameter of coronary arteries and independent demographic parameters. Linear, power, logarithmic, exponential, and square root polynomial models were tested for best fit. Results: Data of 2,030 subjects were collected from 16 institutions. Separate calculation models for each sex were developed because the impact of demographic variables on the diameter of coronary arteries differs according to sex. The final model was the polynomial formula with an exponential relationship between the diameter of coronary arteries and body surface area using the DuBois formula. Conclusion A new coronary artery diameter Z score model was developed and is anticipated to be applicable in clinical practice. The new model will help establish a consensus-based Z score model.

Smoking is the major risk factor for lung squamous cell carcinoma (SCC), although a small number of lung SCCs occurs in never-smokers. The purpose of this study was to compare 50 hotspot mutations of lung SCCs between never-smokers and smokers. We retrospectively reviewed the medical records of patients newly diagnosed with lung SCC between January 1, 2011 and December 31, 2013 in the Seoul National University Hospital. Formalin-fixed, paraffin-embedded tumor samples were used for analysis of hotspot mutations. Fifty cancer-related genes in never-smokers were compared to those in ever-smokers. Of 379 lung SCC patients, 19 (5.0%) were never-smokers. The median age of these 19 patients was 67 years (interquartile range 57–73 years), and 10 of these patients were women (52.5%). The incidence rates of stage I, II, III, and IV disease in this group were 26.4%, 5.3%, 31.6%, and 36.8%, respectively, and sequencing was performed successfully in 14 cases. In the 26 lung SCC tumor samples (12 from never-smokers and 14 from ever-smokers) sequenced using personal genome machine, the most common mutations were in TP53 (75.0%), RAS (66.7%), and STK11 (33.3%), but mutations were also found in EGFR, KIT, and PTEN. The distribution of hotspot mutations in never-smokers was similar to that in ever-smokers. There was no significant difference in overall survival between the 2 groups. The 50 hotspot mutations of lung SCC in never-smokers were similar to those of ever-smokers.
Carcinoma, Squamous Cell* ; Epithelial Cells* ; Female ; Genome ; Humans ; Incidence ; Lung Neoplasms ; Lung* ; Medical Records ; Retrospective Studies ; Risk Factors ; Seoul ; Smoke ; Smoking

PURPOSE: Few studies have reported on predicting prognosis using myocardial perfusion single-photon emission computed tomography (SPECT) during coronary artery disease (CAD) treatment. Therefore, we aimed to assess the clinical implications of myocardial perfusion SPECT during follow-up for CAD treatment. MATERIALS AND METHODS: We enrolled 1153 patients who had abnormal results at index SPECT and underwent follow-up SPECT at intervals ≥6 months. Major adverse cardiac events (MACE) were compared in overall and 346 patient pairs after propensity-score (PS) matching. RESULTS: Abnormal SPECT was associated with a significantly higher risk of MACE in comparison with normal SPECT over the median of 6.3 years (32.3% vs. 19.8%; unadjusted p<0.001). After PS matching, abnormal SPECT posed a higher risk of MACE [32.1% vs. 19.1%; adjusted hazard ratio (HR)=1.73; 95% confidence interval (CI)=1.27–2.34; p<0.001] than normal SPECT. After PS matching, the risk of MACE was still higher in patients with abnormal follow-up SPECT in the revascularization group (30.2% vs. 17.9%; adjusted HR=1.73; 95% CI=1.15–2.59; p=0.008). Low ejection fraction [odds ratio (OR)=5.33; 95% CI=3.39–8.37; p<0.001] and medical treatment (OR=2.68; 95% CI=1.93–3.72; p<0.001) were independent clinical predictors of having an abnormal result on follow-up SPECT. CONCLUSION: Abnormal follow-up SPECT appears to be associated with a high risk of MACE during CAD treatment. Follow-up SPECT may play a potential role in identifying patients at high cardiovascular risk.
Coronary Artery Disease* ; Coronary Vessels* ; Follow-Up Studies* ; Humans ; Ischemia* ; Perfusion* ; Prognosis ; Tomography, Emission-Computed* ; Tomography, Emission-Computed, Single-Photon

Coronary artery fistula draining into lung parenchymal vasculature has not been reported. Herein, we describe a case of an 81-year-old woman who presented with a localized pulmonary edema on right upper lobe associated with coronary fistula emptied into vasculature in right upper lobe. She underwent transcatheter closure of the fistula with an Amplatzer Vascular Plug 4, which resulted in complete occlusion and improved localized pulmonary edema.
Aged, 80 and over ; Coronary Vessels* ; Female ; Fistula* ; Humans ; Lung* ; Pulmonary Edema*

PURPOSE: This study aimed to evaluate the autonomic imbalance in syncope by comparing the baseline heart rate variability (HRV) between healthy children and those with vasovagal syncope. METHODS: To characterize the autonomic profile in children experiencing vasovagal syncope, we evaluated the HRV of 23 patients aged 7-18 years and 20 healthy children. These children were divided into preadolescent (<12 years) and adolescent groups. The following time-domain indices were calculated: root mean square of the successive differences (RMSSD); standard deviation of all average R-R intervals (SDNN); and frequency domain indices including high frequency (HF), low frequency (LF), normalized high frequency, normalized low frequency, and low frequency to high frequency ratio (LF/HF). RESULTS: HRV values were significantly different between healthy children and those with syncope. Student t test indicated significantly higher SNDD values (60.46 ms vs. 37.42 ms, P=0.003) and RMSSD (57.90 ms vs. 26.92 ms, P=0.000) in the patient group than in the control group. In the patient group, RMSSD (80.41 ms vs. 45.89 ms, P=0.015) and normalized HF (61.18 ms vs. 43.19 ms, P=0.022) were significantly higher in adolescents, whereas normalized LF (38.81 ms vs. 56.76 ms, P=0.022) and LF/HF ratio (0.76 vs. 1.89, P=0.041) were significantly lower in adolescents. In contrast, the control group did not have significant differences in HRV values between adolescents and preadolescents. CONCLUSION: The results of this study indicated that children with syncope had a decreased sympathetic tone and increased vagal tone compared to healthy children. Additionally, more severe autonomic imbalances possibly occur in adolescents than in preadolescents.
Adolescent* ; Child* ; Heart Rate* ; Humans ; Syncope ; Syncope, Vasovagal*

Scrub typhus is an acute febrile illness caused by Orientia tsutsugamushi that is characterized by focal or disseminated vasculitis and perivasculitis. Scrub typhus can have fatal complications, such as acute respiratory distress syndrome, septic shock, and acute kidney injury. However, there are few reports of fatal myocarditis caused by scrub typhus. We present a case of acute fatal myocarditis combined with acute kidney injury complicating scrub typhus.
Acute Kidney Injury ; Myocarditis* ; Orientia tsutsugamushi ; Respiratory Distress Syndrome, Adult ; Scrub Typhus* ; Shock, Septic ; Vasculitis

PURPOSE: Glucose uptake and glycolytic metabolism are enhanced in cancer cells, and increased expression of glucose transporter 1 (GLUT1) has also been reported. The aim of this study was to investigate GLUT1 expression in human breast tissues and invasive ductal carcinomas. METHODS: We used tissue microarrays consisting of normal breast tissue, ductal hyperplasia, ductal carcinoma in situ, invasive ductal carcinoma, and lymph node metastases. We examined GLUT1 expression in the microarrays by immunohistochemistry, reviewed the medical records and performed a clinicopathological analysis. RESULTS: Membranous GLUT1 expression was observed in normal and tumor cells. GLUT1 expression was higher in ductal carcinoma in situ, invasive ductal carcinoma, and lymph node metastasis than in normal tissue and ductal hyperplasia (p=0.002). Of 276 invasive ductal carcinomas, 106 (38.4%) showed GLUT1 expression. GLUT1 expression was correlated with higher histologic grade (p<0.001), larger tumor size (p=0.025), absence of estrogen receptor (p<0.001), absence of progesterone receptor (p<0.001), and triple-negative phenotype (p<0.001). In univariate survival analysis, patients with GLUT1 expression had poorer overall survival and disease-free survival (p=0.017 and p=0.021, respectively, log-rank test). In multivariate survival analysis with the Cox proportional hazards model, GLUT1 expression was an independent prognostic factor of poorer overall survival and disease-free survival (p=0.017 and p=0.019, respectively). CONCLUSION: GLUT1 expression seems to play an important role in malignant transformation, and the glycolytic phenotype in invasive ductal carcinoma may indicate aggressive biological behavior and a worse prognosis.
Breast ; Carcinoma, Ductal ; Carcinoma, Intraductal, Noninfiltrating ; Disease-Free Survival ; Estrogens ; Glucose ; Glucose Transport Proteins, Facilitative ; Humans ; Hyperplasia ; Immunohistochemistry ; Lymph Nodes ; Medical Records ; Neoplasm Metastasis ; Phenotype ; Prognosis ; Proportional Hazards Models ; Receptors, Progesterone

Prolongation of QTc interval associated with Takotsubo cardiomyopathy (TC) has previously been reported in published case series. We report an unusual case of a patient who presented with TC associated with long-QT syndrome and developed cardiac arrest secondary to torsade de pointes. Since QT prolongation and bradycardia persisted after the resolution of TC, the patient received permanent pacemaker. Since then additional event did not occur. QT prolongation and bradycardia could be persistent even after recovery of TC, and permanent pacemaker insertion may be a treatment option of long QT syndrome related with TC.
Aged ; Bradycardia/diagnosis/therapy ; Cardiac Pacing, Artificial ; Coronary Angiography ; Diagnosis, Differential ; Electrocardiography ; Female ; Heart Arrest/diagnosis/etiology ; Humans ; Long QT Syndrome/*diagnosis/etiology ; Takotsubo Cardiomyopathy/complications/*diagnosis/ultrasonography ; Torsades de Pointes/*diagnosis/etiology

Klinefelter's syndrome (KFS) is a gonosomal aberration disease that occurs in males, and is characterized by 47, XXY karyotype, hypogonadism and a lack of secondary sexual characteristics. A potential link between this hormonally deficient syndrome and autoimmune disease, particularly systemic lupus erythematosus (SLE), has been reported. On the other hand, KFS is rarely reported to be accompanied by rheumatoid arthritis (RA), and there are no Korean cases reported. We report the first Korean case of a KFS patient with sero-positive RA and discuss the role of the pathogenesis of RA with KFS.
Aluminum Hydroxide ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Carbonates ; Hand ; Humans ; Hypogonadism ; Karyotype ; Klinefelter Syndrome ; Lupus Erythematosus, Systemic ; Male ; X Chromosome

BACKGROUND AND OBJECTIVES: Morning hypertension is closely related to target organ damage and cardiovascular events. Little data is available concerning the baseline characteristics and comprehensive blood pressure analysis of hypertensive patients on treatment with morning hypertension. SUBJECTS AND METHODS: We evaluated 1,087 hypertensive patients who had taken stable anti-hypertensive medication at least 6 months. The enrolled patients measured their home blood pressure for 7 days. Baseline characteristics and the laboratory data were analyzed. Morning hypertension was defined as a morning blood pressure > or =135/85 mm Hg and systolic or diastolic blood pressure difference between morning and evening exceeding 10 mm Hg. RESULTS: One hundred seventy three patients with morning hypertension showed a preponderance of males, older patients, alcohol consumers, and greater waist circumference and waist-to-hip ratio despite the same body mass index. Impaired fasting glucose and metabolic syndrome were more prevalent in the patients with morning hypertension. The morning hypertensives took more anti-hypertensive drugs and displayed higher blood pressure in the clinic and at home. CONCLUSION: The worse clinical variables and relatively poorly controlled blood pressure of those with morning hypertension supports a potential relationship of morning hypertension with poor cardiovascular outcome. Morning blood pressure should be monitored at home for the optimal treatment of hypertension.
Antihypertensive Agents ; Blood Pressure ; Blood Pressure Monitoring, Ambulatory ; Body Mass Index ; Fasting ; Glucose ; Humans ; Hypertension ; Male ; Waist Circumference ; Waist-Hip Ratio