Background: There are few long-term large-scale epidemiologic studies on hypertrophic cardiomyopathy (HCM; 10th revision of the International Statistical Classification of Diseases and Related Health Problems codes: I42.1, I42.2). This analysis used the Korean National Health Insurance Service (KNHIS) data between 2006 and 2017 to evaluate the natural history of HCM over a decade. Methods: KNHIS data and death statistics were evaluated according to age, sex, socioeconomic position, and comorbidities. Survival rates (SRs) and adjusted hazard ratio (HR) were compared with death data of the Korean population from 2006 through 2018. Results: The mean age was 47.3 ± 14.9 years in males and 57.5 ± 15.4 years in females (P < 0.001).The male proportion was 58.9%. The most common cause of death was diseases of the circulatory system. The 10-year SR of HCM was higher in males (75.9% vs. 62.5%, P < 0.001).The adjusted HR for different age groups was significantly high in most age group: 3.67 (95% confidence interval [CI], 2.65–5.10) for 0–9 years, and it gradually increased from the 30s to the 80s group (1.39 [95% CI, 1.05–1.83] for 30–39 years and 48.2 [95% CI, 37.0–62.7] for those older than 80 years). The adjusted HR was 1.12 (95% CI, 1.07–1.17) for males, 1.40 (95% CI, 1.33–1.48) for the lower income level, and 1.18 (95% CI, 1.12–1.25) for the medium income level. In patients with comorbidities, the adjusted HRs were 1.23 (95% CI, 1.16–1.30) for diabetes, 1.45 (95% CI, 1.30–1.62) for myocardial infarction, 1.63 (95% CI, 1.52–1.76) for atrial fibrillation, 1.83 (95% CI, 1.68–1.99) for ischemic stroke, 1.66 (95% CI, 1.31–2.10) for hemorrhagic stroke, 2.42 (95% CI, 2.16–2.70) for chronic kidney disease, and 3.18 (95% CI, 2.87–3.52) for malignant neoplasm. Conclusion HCM in Korea showed a higher prevalence and incidence in males. The 10-year SR of HCM was approximately 70% and lower in females than that in males. The risk of death from HCM increased with age and was significantly higher in males, individuals with low income levels, and patients with various comorbidities. These findings should be considered for the long-term management and allocation of healthcare resources for patients with HCM.

Background: There are few long-term large-scale epidemiologic studies on hypertrophic cardiomyopathy (HCM; 10th revision of the International Statistical Classification of Diseases and Related Health Problems codes: I42.1, I42.2). This analysis used the Korean National Health Insurance Service (KNHIS) data between 2006 and 2017 to evaluate the natural history of HCM over a decade. Methods: KNHIS data and death statistics were evaluated according to age, sex, socioeconomic position, and comorbidities. Survival rates (SRs) and adjusted hazard ratio (HR) were compared with death data of the Korean population from 2006 through 2018. Results: The mean age was 47.3 ± 14.9 years in males and 57.5 ± 15.4 years in females (P < 0.001).The male proportion was 58.9%. The most common cause of death was diseases of the circulatory system. The 10-year SR of HCM was higher in males (75.9% vs. 62.5%, P < 0.001).The adjusted HR for different age groups was significantly high in most age group: 3.67 (95% confidence interval [CI], 2.65–5.10) for 0–9 years, and it gradually increased from the 30s to the 80s group (1.39 [95% CI, 1.05–1.83] for 30–39 years and 48.2 [95% CI, 37.0–62.7] for those older than 80 years). The adjusted HR was 1.12 (95% CI, 1.07–1.17) for males, 1.40 (95% CI, 1.33–1.48) for the lower income level, and 1.18 (95% CI, 1.12–1.25) for the medium income level. In patients with comorbidities, the adjusted HRs were 1.23 (95% CI, 1.16–1.30) for diabetes, 1.45 (95% CI, 1.30–1.62) for myocardial infarction, 1.63 (95% CI, 1.52–1.76) for atrial fibrillation, 1.83 (95% CI, 1.68–1.99) for ischemic stroke, 1.66 (95% CI, 1.31–2.10) for hemorrhagic stroke, 2.42 (95% CI, 2.16–2.70) for chronic kidney disease, and 3.18 (95% CI, 2.87–3.52) for malignant neoplasm. Conclusion HCM in Korea showed a higher prevalence and incidence in males. The 10-year SR of HCM was approximately 70% and lower in females than that in males. The risk of death from HCM increased with age and was significantly higher in males, individuals with low income levels, and patients with various comorbidities. These findings should be considered for the long-term management and allocation of healthcare resources for patients with HCM.

Background: There are few long-term large-scale epidemiologic studies on hypertrophic cardiomyopathy (HCM; 10th revision of the International Statistical Classification of Diseases and Related Health Problems codes: I42.1, I42.2). This analysis used the Korean National Health Insurance Service (KNHIS) data between 2006 and 2017 to evaluate the natural history of HCM over a decade. Methods: KNHIS data and death statistics were evaluated according to age, sex, socioeconomic position, and comorbidities. Survival rates (SRs) and adjusted hazard ratio (HR) were compared with death data of the Korean population from 2006 through 2018. Results: The mean age was 47.3 ± 14.9 years in males and 57.5 ± 15.4 years in females (P < 0.001).The male proportion was 58.9%. The most common cause of death was diseases of the circulatory system. The 10-year SR of HCM was higher in males (75.9% vs. 62.5%, P < 0.001).The adjusted HR for different age groups was significantly high in most age group: 3.67 (95% confidence interval [CI], 2.65–5.10) for 0–9 years, and it gradually increased from the 30s to the 80s group (1.39 [95% CI, 1.05–1.83] for 30–39 years and 48.2 [95% CI, 37.0–62.7] for those older than 80 years). The adjusted HR was 1.12 (95% CI, 1.07–1.17) for males, 1.40 (95% CI, 1.33–1.48) for the lower income level, and 1.18 (95% CI, 1.12–1.25) for the medium income level. In patients with comorbidities, the adjusted HRs were 1.23 (95% CI, 1.16–1.30) for diabetes, 1.45 (95% CI, 1.30–1.62) for myocardial infarction, 1.63 (95% CI, 1.52–1.76) for atrial fibrillation, 1.83 (95% CI, 1.68–1.99) for ischemic stroke, 1.66 (95% CI, 1.31–2.10) for hemorrhagic stroke, 2.42 (95% CI, 2.16–2.70) for chronic kidney disease, and 3.18 (95% CI, 2.87–3.52) for malignant neoplasm. Conclusion HCM in Korea showed a higher prevalence and incidence in males. The 10-year SR of HCM was approximately 70% and lower in females than that in males. The risk of death from HCM increased with age and was significantly higher in males, individuals with low income levels, and patients with various comorbidities. These findings should be considered for the long-term management and allocation of healthcare resources for patients with HCM.

Background: There are few long-term large-scale epidemiologic studies on hypertrophic cardiomyopathy (HCM; 10th revision of the International Statistical Classification of Diseases and Related Health Problems codes: I42.1, I42.2). This analysis used the Korean National Health Insurance Service (KNHIS) data between 2006 and 2017 to evaluate the natural history of HCM over a decade. Methods: KNHIS data and death statistics were evaluated according to age, sex, socioeconomic position, and comorbidities. Survival rates (SRs) and adjusted hazard ratio (HR) were compared with death data of the Korean population from 2006 through 2018. Results: The mean age was 47.3 ± 14.9 years in males and 57.5 ± 15.4 years in females (P < 0.001).The male proportion was 58.9%. The most common cause of death was diseases of the circulatory system. The 10-year SR of HCM was higher in males (75.9% vs. 62.5%, P < 0.001).The adjusted HR for different age groups was significantly high in most age group: 3.67 (95% confidence interval [CI], 2.65–5.10) for 0–9 years, and it gradually increased from the 30s to the 80s group (1.39 [95% CI, 1.05–1.83] for 30–39 years and 48.2 [95% CI, 37.0–62.7] for those older than 80 years). The adjusted HR was 1.12 (95% CI, 1.07–1.17) for males, 1.40 (95% CI, 1.33–1.48) for the lower income level, and 1.18 (95% CI, 1.12–1.25) for the medium income level. In patients with comorbidities, the adjusted HRs were 1.23 (95% CI, 1.16–1.30) for diabetes, 1.45 (95% CI, 1.30–1.62) for myocardial infarction, 1.63 (95% CI, 1.52–1.76) for atrial fibrillation, 1.83 (95% CI, 1.68–1.99) for ischemic stroke, 1.66 (95% CI, 1.31–2.10) for hemorrhagic stroke, 2.42 (95% CI, 2.16–2.70) for chronic kidney disease, and 3.18 (95% CI, 2.87–3.52) for malignant neoplasm. Conclusion HCM in Korea showed a higher prevalence and incidence in males. The 10-year SR of HCM was approximately 70% and lower in females than that in males. The risk of death from HCM increased with age and was significantly higher in males, individuals with low income levels, and patients with various comorbidities. These findings should be considered for the long-term management and allocation of healthcare resources for patients with HCM.

Solitary fibrous tumors (SFTs) are spindle-cell tumors that rarely arise within extrathoracic area. Massive SFTs involving the pterygopalatine fossa are extremely rare and surgical excision represents a multidisciplinary surgical challenge. We present a case of 64-year-old female with a huge mass originating from the pterygopalatine fossa invading the skull base. Distinct microscopic findings and a positive nuclear staining of signal transducer and activator of transcription 6 (STAT-6) pathologically confirmed SFT. The right internal maxillary artery branch was embolized preoperatively and a surgical excision was performed through a combined technique of transpalatal, mid-facial degloving and endoscopic approach. Postoperative radiotherapy successfully removed the remnant tumor adjacent to the carvenous sinus. Follow- up MR images showed no evidence of recurrence for two years. To our knowledge, there has been no previous report of the successful treatment of this vast extent of SFT in the pterygopalatine region via endoscopic-external-combined approach and radiotherapy.

No abstract available.
Gastrectomy* ; Hyperhomocysteinemia ; Obesity, Morbid* ; Thrombophilia*

OBJECTIVE: The aim of this study was to analyze the long-term survival of subjects with peripheral artery disease (PAD). METHODS: The data included 415 Korean PAD patients aged ≥20 years hospitalized from 1994 through 2004 at a single tertiary center in Korea. Death data were obtained from all participants between 1994 and 2009. RESULTS: The mean of age was 64.4±9.3 years in PAD. The proportion of peripheral vascular bypass operation (re-vascularized) was about 50%. The proportion of males was 90.6% in PAD. Five- and 10- year survival rates were 79.2% and 60.5% in PAD, respectively. The 5- and 10- year survival rates were 83.0% and 64.1% in re-vascularized group, and 75.5% and 56.3% in non-revascularized group (p<0.05). For PAD, the adjusted hazard ratios (HRs) were 1.75 (95% confidence interval (CI) 1.17-2.68) in over 65 years, 1.53 (95% CI 1.05-2.27) in diabetes, and 2.21 (95% CI 1.51-3.23) in chronic kidney disease (CKD). Interestingly, HRs in PAD were 0.55 (95% CI 0.34-0.84) in overweight and 0.45 (95% CI 0.25-0.76) in obesity. CONCLUSIONS: The 5- and 10- year survival rates were 79.2% and 60.5% in PAD. The survival rate in re-vascularized group was higher than that in non-revascularized group. Independent predictors of mortality were age, diabetes, and CKD in PAD. Obesity showed improved survival rates.
Humans ; Korea ; Male ; Mortality ; Obesity ; Overweight ; Peripheral Arterial Disease* ; Renal Insufficiency, Chronic ; Survival Rate*

We describe two cases of pulmonary arterial hypertension (PAH) that occurred under dasatinib treatment and were resolved after dasatinib discontinuation. Two patients with chronic phase chronic myeloid leukemia (CML) were switched to dasatinib therapy because of hematological progress while receiving imatinib. These patients had New York Heart Association (NYHA) functional class II dyspnea with elevated right ventricular systolic pressure (RVSP), which progressed under dasatinib treatment. After dasatinib treatment was discontinued, subjective symptoms were improved to NYHA functional class I and the follow-up transthoracic Doppler echocardiography showed improved RVSP. Treatment with an alternate tyrosine kinase inhibitor was initiated and had been continued without development of dyspnea or elevation of RVSP. This report suggests that dasatinib can cause the reversible PAH, therefore, routine cardiopulmonary evaluation before and during treatment with dasatinib may be needed in CML patients with clinical manifestations.
Blood Pressure ; Dyspnea ; Echocardiography, Doppler ; Follow-Up Studies ; Heart ; Humans ; Hypertension* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Protein-Tyrosine Kinases

BACKGROUND AND OBJECTIVES: The objective of this study was to analyze and compare risk factors for peripheral artery disease (PAD) and coronary artery disease (CAD). SUBJECTS AND METHODS: The sample included 7936 Korean patients aged > or =20 years who were hospitalized from 1994 to 2004. Of the 7936 subjects, PAD (n=415), CAD (n=3686), and normal controls (Control) (n=3835) were examined at the Health Promotion Center, Samsung Medical Center. RESULTS: The mean age (years) of PAD subjects was 64.4 (+/-9.3), while CAD subjects was 61.2 (+/-9.9), and Control subjects was 59.9 (+/-9.1) (p<0.01). The proportion of males was 90.6% for PAD, 71.4% for CAD, and 75.5% for Control subjects (p<0.01). The adjusted odds ratios (ORs) for hypertension, diabetes mellitus, hypercholesterolemia, smoking, metabolic syndrome and chronic kidney disease were significantly higher in subjects with PAD or CAD compared to those in Control. However, the ORs for high density lipoprotein, being overweight, and being obese were significantly lower in PAD subjects compared to those in Control. CONCLUSION: We found that cardiovascular risk factors were in fact risk factors for both PAD and CAD.
Aged ; Coronary Artery Disease ; Coronary Vessels ; Diabetes Mellitus ; Electrolytes ; Health Promotion ; Humans ; Hypercholesterolemia ; Hypertension ; Lipoproteins ; Male ; Odds Ratio ; Overweight ; Peripheral Arterial Disease ; Renal Insufficiency, Chronic ; Risk Factors ; Smoke ; Smoking

PURPOSE: In this study, we analyzed a cohort of children with chronic graft-versus-host disease (GvHD) according to the NIH consensus classification (NCC) in order to observe whether global assessment at diagnosis correlates with GvHD-specific endpoints. We then studied the clinical course of these patients, specifically with regards to episodes of GvHD exacerbation requiring treatment escalation. MATERIALS AND METHODS: Recipients of either allogeneic bone marrow transplantation (BMT) or peripheral blood stem cell transplantation (PBSCT) from January 2006 to August 2008 at the Department of Pediatrics, The Catholic University of Korea were evaluated for chronic GvHD, which was diagnosed according to the NCC. The course of chronic GvHD in these patients was then followed. RESULTS: Of 59 evaluable patients, 23 developed chronic GvHD for a cumulative incidence of 39.3%. Upon multivariate analysis, previous acute GvHD (> or =grade II) had a significant impact on chronic GvHD incidence. With a median duration of systemic treatment for chronic GvHD of 501 days, no significant relationship was found between initial global severity of chronic GvHD and either duration of immunosuppressive treatment or final clinical response to treatment. Fifteen patients (65%) experienced at least one episode of chronic GvHD exacerbation during the period of follow-up, with a median of four exacerbations in the subgroup of patients who experienced such events. Lung GvHD resulted in the highest number of exacerbations per diagnosed patient, followed by oral GvHD. CONCLUSION: Analysis of this small cohort indicates that global assessment as proposed by the NCC may have limited correlations with GvHD-specific endpoints, possibly due to the favorable response of children to treatment.
Adolescent ; Bone Marrow Transplantation/adverse effects ; Child ; Child, Preschool ; Chronic Disease ; Cohort Studies ; Consensus Development Conferences, NIH as Topic ; Female ; Graft vs Host Disease/classification/*diagnosis/drug therapy/etiology ; Humans ; Immunosuppressive Agents/administration & dosage ; Infant ; Male ; National Institutes of Health (U.S.) ; Peripheral Blood Stem Cell Transplantation/adverse effects ; Prognosis ; Republic of Korea ; Risk Factors ; United States