Purpose: In this study, we evaluated 66 patients diagnosed with adenoid cystic carcinoma (ACC) enrolled in two Korean Cancer Study Group trials to investigate the response and progression patterns in recurrent and/or metastatic ACC treated with vascular endothelial growth factor receptor tyrosine kinase inhibitors (VEGFR-TKIs). Materials and Methods: We evaluated 66 patients diagnosed with ACC who were enrolled in the Korean Cancer Study Group trials. The tumor measurements, clinical data, treatment outcomes, and progression patterns of therapy were analyzed. Results: In the 66 patients (53 receiving axitinib and 13 receiving nintedanib), the disease control rate was 61%, and three patients achieved partial response. The median follow-up, median progression-free survival (PFS), overall survival, and 6-month PFS rate were 27.6%, 12.4%, and 18.1% months and 62.1%, respectively. Among 42 patients who experienced progression, 27 (64.3%) showed target lesion progression. Bone metastasis was an independent poor prognostic factor. Conclusion Overall, most patients demonstrated stable disease with prolonged PFS; however, prominent target lesion progression occurred in some patients. Thus, PFS may capture VEGFR-TKI efficacy better than the objective response rate.

Despite the availability of direct-acting antivirals (DAAs) for chronic hepatitis C virus (HCV) infection in Korea, need remains for pangenotypic regimens that can be used in the presence of hepatic impairment, comorbidities, or prior treatment failure. We investigated the efficacy and safety of sofosbuvir–velpatasvir and sofosbuvir–velpatasvir–voxilaprevir for 12 weeks in HCV-infected Korean adults. Methods: This Phase 3b, multicenter, open-label study included 2 cohorts. In Cohort 1, participants with HCV genotype 1 or 2 and who were treatment-naive or treatment-experienced with interferon-based treatments, received sofosbuvir–velpatasvir 400/100 mg/day. In Cohort 2, HCV genotype 1 infected individuals who previously received an NS5A inhibitor-containing regimen ≥ 4 weeks received sofosbuvir–velpatasvir–voxilaprevir 400/100/100 mg/day. Decompensated cirrhosis was an exclusion criterion. The primary endpoint was SVR12, defined as HCV RNA < 15 IU/mL 12 weeks following treatment. Results: Of 53 participants receiving sofosbuvir–velpatasvir, 52 (98.1%) achieved SVR12. The single participant who did not achieve SVR12 experienced an asymptomatic Grade 3 ASL/ALT elevation on day 15 and discontinued treatment. The event resolved without intervention. All 33 participants (100%) treated with sofosbuvir–velpatasvir–voxilaprevir achieved SVR 12. Overall, sofosbuvir–velpatasvir and sofosbuvir–velpatasvir–voxilaprevir were safe and well tolerated. Three participants (5.6%) in Cohort 1 and 1 participant (3.0%) in Cohort 2 had serious adverse events, but none were considered treatment-related. No deaths or grade 4 laboratory abnormalities were reported. Conclusions: Treatment with sofosbuvir–velpatasvir or sofosbuvir–velpatasvir–voxilaprevir was safe and resulted in high SVR12 rates in Korean HCV patients.

Isolated extramedullary relapse (EMR) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in acute myeloid leukemia (AML) is relatively rare and the clinical significance is still not well characterized, particularly in children. During or after chemotherapy, an uncommon event termed lineage switch, in which the lineage at onset of leukemia converts to another lineage at a later time, is observed in a subset of leukemia patients, but it is a rare event. We report a 10-year-old boy with isolated mediastinal EMR after allo-HSCT for AML who subsequently experienced BM relapse with the cell lineage switching from AML to T-cell acute lymphoblastic leukemia.

Purpose: Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. Materials and Methods: Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. Results: Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). Conclusion This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.

Background: Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. Methods: We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. Results: A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. Conclusion This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

BACKGROUND: von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. However, the number of patients who register to the Korea Hemophilia Foundation (KHF) is much lower than the expected prevalence rate and only few hospitals perform tests for diagnosis autonomously. Thus, we surveyed practical realities of VWD in Yeungnam region. METHODS: Patients with VWD (N=267) who were diagnosed at eleven university hospitals from March 1995 to March 2018 were enrolled in this study. We evaluated the medical records from each hospital retrospectively. RESULTS: Two hundred and twenty-eight children and 39 adults met the diagnostic criteria for VWD. Seventy-eight (57.4%) patients had the blood type O. Fifty-eight patients were definite type 1 (21.7%), 151 were possible type 1 (56.6%), and the others were type 2. Abnormal laboratory findings were the most common factor for the diagnosis in children. VWF mutations were detected in 17 patients. Patients with a family history showed age of diagnosis of 9 y, which is higher than in those with no family history (6 yr), and also showed a higher rate of significant bleeding (32.1% vs. 14.2%). VWF:RCo and VWF:Ag tests were performed in-hospital at only 1 of 11 hospitals. Twelve of 267 patients were enrolled at the KHF (4.5%). CONCLUSION: A high rate of out-sourcing studies may result in inaccurate diagnosis. The registration rate to the KHF is still lower than the prevalence rate. A comprehensive nationwide registration system is necessary in order to identify the actual prevalence rate and promote the diagnosis of VWD in Korea.
Adult ; Child ; Diagnosis ; Hemophilia A ; Hemorrhage ; Hospitals, University ; Humans ; Korea ; Medical Records ; Prevalence ; Retrospective Studies ; von Willebrand Diseases

OBJECTIVES: Due to their developmental characteristics, adolescents have a higher probability than other age groups of experiencing injuries caused by accidents, violence, and intentional self-harm. The severity and characteristics of injuries vary by the intentionality and mechanism of injury; therefore, there is a need for a national-level estimate of the scale and the severity of injuries in adolescents that takes these factors into account. METHODS: By using data from the Emergency Department-based Injury In-depth Surveillance Data, National Emergency Department Information System, the Korean National Hospital Discharge In-depth Injury Survey, and cause of death statistics, we calculated the emergency department (ED) visit rate, hospitalization rate, and death rate of injuries per 100 000 adolescents for each injury mechanism. The calculated rates were used to generate the injury pyramid ratio (ratio of death rate to hospitalization rate to ED visit rate) to visualize the scale and the severity of the injury. RESULTS: The mortality rate in adolescents due to injury was 10/100 000; the corresponding rates for hospitalization and ED visits were 1623 and 4923, respectively, resulting in an injury pyramid ratio with the general pyramid form, with a 1:162:492 ratio of deaths to hospitalizations to ED visits. The mortality rate due to suicide/intentional self-harm was 5/100 000, while 35 were hospitalized for this reason and 74 made ED visits. The pyramid ratio of 1:7:15 for intentional self-harm/suicide showed a steep pyramidal form, indicating considerable lethality. The mortality rate due to motor vehicle collisions (MVCs) was 3/100 000; 586 were hospitalized for this reason, while 1023 made ED visits. The pyramid ratio of 1:195:341 for MVCs showed a gradual pyramid form, indicating that the lethality was low and the scale of injury was high. CONCLUSIONS: The main categories of injuries in adolescents were visualized in pyramid form, contributing to an understanding of the scale of each injury by mechanism in terms of levels of death, hospitalization, and ED visits. These findings will be helpful for understanding how to prioritize injuries in adolescents.
Adolescent* ; Cause of Death ; Emergencies ; Emergency Service, Hospital ; Hospitalization ; Humans ; Information Systems ; Intention ; Mortality ; Motor Vehicles ; Violence

No abstract available.
Cardiopulmonary Resuscitation* ; Education*

PURPOSE: The incidence and severity of injuries differs between each region of Korea due to socioeconomic differences. The comparative analysis between national injury data and the evaluation of regional epidemiologic characteristics is useful in establishing an injury prevention strategy for a regional community. This study was conducted in order to provide basic data for the establishment of an injury prevention strategy by priority through comparison between national injury data collected by National Emergency Department Information System (NEDIS) and injury data from regional emergency centers located in small cities, in Korea. METHODS: The study subjects were the injury patients who visited a total of 117 regional, specialized and local emergency centers from April 2009 to March 2010, as well as those who visited Konkuk University Chungju Hospital during the same period. We collected national data from the Injury Surveillance Report published by Korea Centers for Disease Control and Prevention and compared these with study hospital data collected by our own injury registration system to include gender, ratio, age, and mechanism and severity of patient injury. RESULTS: Of 3,931,573 patients who visited 117 emergency centers and 23,671 patients who visited the study hospital during the study year, the number of injury patients was 786,006 (20.0%) versus 6,177 (26.1%), respectively. 485,521 (62.0%) of the nation-wide subjects versus 4,046 (65.5%) of the study hospital subjects were male, and 298,197 (38.0%) of the nation-wide subjects versus 2,128 (34.5%) of the study hospital subjects were female. The comparison by age of the injury subjects was as follows: below 9 years (22.4% versus 16.8%), twenties (14.5% versus 16.3%) and forties (14.5% versus 15.2%). In the comparison of injury mechanism, blunt injury was higher (20.4%) in nation-wide subjects followed by slip injury (20.0%) and transport accident (19.0%). Transport accidents produced the highest cause of injury (26.0%) in the study subjects followed by slip injury (20.1%) and bunt injury (16.1%). In the severity comparison, 641,344(81.6%) versus 4797(77.8%) were mild injury patients, 139,260(17.7%) versus 1299(21.1%) were severely injured, and 3114 (0.4%) versus 69(1.1%) were fatal injuries. The causes of severe injury nation-wide included intoxication (46.3%), falls (34.8%) and machine accidents (33.3%) versus machine (46.0%), asphyxia (44.4%), intoxication (39.3%) in the study hospital, and the causes of fatal injuries nation-wide were intoxication (5.2%), drowning (4.3%), and asphyxia (4.2%), versus drowning (11.1%), asphyxia (5.6%) and intoxication (4.9%) in the study hospital. CONCLUSION: Through the comparison between study hospital with national injury data, we found that the percentage of severe and fatally injured patients was higher in the nationally with transportation related accidents being highest in terms of mechanism. The construction of a regional injury data system with ongoing comparison with national injury data may be useful in the establishment of a regional injury intervention strategy by priority.
Accident Prevention ; Asphyxia ; Centers for Disease Control and Prevention (U.S.) ; Drowning ; Emergencies ; Female ; Humans ; Incidence ; Information Systems ; Korea ; Male ; Population Surveillance ; Transportation ; Wounds, Nonpenetrating