Background: To investigate the clinical treatment status, such as treatment regimen, bleeding events, and drug dose, in patients with hemophilia B in South Korea. Methods: In this retrospective chart review, data of patients with hemophilia B from eight university hospitals were collected. Demographic and clinical data, treatment data, such as regimen and number of injections, dose of factor IX concentrate, and bleeding data were reviewed. Descriptive analyses were performed with annual data for 2019, 2020, and 2021, as well as the three years consecutively. Results: The medical records of 150 patients with hemophilia B between January 1, 2019, and December 31, 2021, were collected. Among these, 72 (48.0%) were severe, 47 (31.3%) were moderate, and 28 (18.7%) were mild. The results showed approximately two times more patients receiving prophylaxis as those receiving on-demand therapy, with 66.1% of patients receiving prophylaxis in 2019, 64.9% in 2020, and 72.1% in 2021. Annualized bleeding rates were 2.2% (± 3.1) in 2019, 1.8% (± 3.0) in 2020, and 1.8% (± 2.9) in 2021 among patients receiving prophylaxis.For the doses of factor IX concentrate, patients receiving prophylaxis received an average of 41.6 (± 11.9) IU/Kg/Injection in 2019, 45.7 (± 12.9) IU/Kg/Injection in 2020, and 60.1 (± 24.0) IU/Kg/Injection in 2021. Conclusions Clinically, prophylaxis is more prevalent than reported. Based on insights gained from current clinical evidence, it is expected that the unmet medical needs of patients can be identified, and physicians can evaluate the status of patients and actively manage hemophilia B using more effective treatment strategies.

Background: To investigate the clinical treatment status, such as treatment regimen, bleeding events, and drug dose, in patients with hemophilia B in South Korea. Methods: In this retrospective chart review, data of patients with hemophilia B from eight university hospitals were collected. Demographic and clinical data, treatment data, such as regimen and number of injections, dose of factor IX concentrate, and bleeding data were reviewed. Descriptive analyses were performed with annual data for 2019, 2020, and 2021, as well as the three years consecutively. Results: The medical records of 150 patients with hemophilia B between January 1, 2019, and December 31, 2021, were collected. Among these, 72 (48.0%) were severe, 47 (31.3%) were moderate, and 28 (18.7%) were mild. The results showed approximately two times more patients receiving prophylaxis as those receiving on-demand therapy, with 66.1% of patients receiving prophylaxis in 2019, 64.9% in 2020, and 72.1% in 2021. Annualized bleeding rates were 2.2% (± 3.1) in 2019, 1.8% (± 3.0) in 2020, and 1.8% (± 2.9) in 2021 among patients receiving prophylaxis.For the doses of factor IX concentrate, patients receiving prophylaxis received an average of 41.6 (± 11.9) IU/Kg/Injection in 2019, 45.7 (± 12.9) IU/Kg/Injection in 2020, and 60.1 (± 24.0) IU/Kg/Injection in 2021. Conclusions Clinically, prophylaxis is more prevalent than reported. Based on insights gained from current clinical evidence, it is expected that the unmet medical needs of patients can be identified, and physicians can evaluate the status of patients and actively manage hemophilia B using more effective treatment strategies.

Background: To investigate the clinical treatment status, such as treatment regimen, bleeding events, and drug dose, in patients with hemophilia B in South Korea. Methods: In this retrospective chart review, data of patients with hemophilia B from eight university hospitals were collected. Demographic and clinical data, treatment data, such as regimen and number of injections, dose of factor IX concentrate, and bleeding data were reviewed. Descriptive analyses were performed with annual data for 2019, 2020, and 2021, as well as the three years consecutively. Results: The medical records of 150 patients with hemophilia B between January 1, 2019, and December 31, 2021, were collected. Among these, 72 (48.0%) were severe, 47 (31.3%) were moderate, and 28 (18.7%) were mild. The results showed approximately two times more patients receiving prophylaxis as those receiving on-demand therapy, with 66.1% of patients receiving prophylaxis in 2019, 64.9% in 2020, and 72.1% in 2021. Annualized bleeding rates were 2.2% (± 3.1) in 2019, 1.8% (± 3.0) in 2020, and 1.8% (± 2.9) in 2021 among patients receiving prophylaxis.For the doses of factor IX concentrate, patients receiving prophylaxis received an average of 41.6 (± 11.9) IU/Kg/Injection in 2019, 45.7 (± 12.9) IU/Kg/Injection in 2020, and 60.1 (± 24.0) IU/Kg/Injection in 2021. Conclusions Clinically, prophylaxis is more prevalent than reported. Based on insights gained from current clinical evidence, it is expected that the unmet medical needs of patients can be identified, and physicians can evaluate the status of patients and actively manage hemophilia B using more effective treatment strategies.

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: The standard morphological evaluation has been widely used for embryo selection, but it has limitations. This study aimed to investigate the correlation between morphologic grading and euploidy rate of in vitro fertilization (IVF) preimplantation genetic screening (PGS) and compare the pregnancy rates in young and old ages. METHODS: This is a retrospective study using the medical records of patients who underwent IVF procedures with PGS between January 2016 and February 2017 in a single center. The embryo grades were categorized into 4 groups: excellent, good, fair, and poor. Basic characteristics, euploidy rates, clinical pregnancy (CP) rates and ongoing pregnancy rates were analyzed. RESULTS: The excellent group had significantly higher rate of euploid embryos than fair group (47.82% vs. 29.33%; P = 0.023) and poor group (47.82% vs. 29.60%; P = 0.005). When the four groups were recategorized into two groups (excellent and good vs. fair and poor), they also showed significant difference in euploidy rates (44.52% vs. 29.53%; P = 0.002). When the patients were divided into two groups by age 35, the CP rates for those under and over 35 years old were 44.74% and 47.83%, respectively, which showed no significant difference. CONCLUSION: The significant differences among the euploidy rates of different morphologic embryo grades demonstrated the positive correlations between the morphologic grading of the embryo and the euploidy rate of PGS. Additionally, there was no significant difference between the younger and older patients' CP rates. These findings emphasize the fact that old age patients might benefit from PGS whatever the indication of PGS is.
Blastocyst* ; Embryonic Structures ; Fertilization in Vitro* ; Genetic Testing* ; Humans ; In Vitro Techniques* ; Medical Records ; Pregnancy ; Pregnancy Rate ; Retrospective Studies

PURPOSE: The purpose of this study was to explore whether or not there is a relationship between doctor's awareness of clinical nutrition service and needs for a clinical dietitian. METHODS: A cross-sectional survey design was used. The research was carried out by using questionnaires that had been specifically designed for the study. The research was conducted from September to October, 2013 for 311 doctors at 43 hospitals (with over 400 beds). Frequency analysis, factor analysis, reliability analysis, confirmatory factor analysis, and bootstrapping analysis were conducted using SPSS 21.0. RESULTS: ‘Implementation of clinical nutritional service’ (p < 0.001) and ‘usefulness on clinical nutrition service’ (p < 0.001) were found to be correlated with ‘importance of clinical nutrition service’ as an independent variable. The correlation between ‘importance of clinical nutrition service’ as a mediating variable and ‘needs for clinical dietitian’ as a dependent variable was also confirmed (p < 0.001). The results of the bootstrapping test showed that the mediating effect of ‘importance of clinical nutrition service’ was significant. The indirect effect value between ‘implementation of clinical nutrition service’ and ‘needs for clinical dietitian’ was 0.040, indirect effect value between ‘usefulness on clinical nutrition service’ and ‘needs for clinical dietitian’ was 0.095. CONCLUSION: The frequency of providing clinical nutritional services, the quality of clinical nutritional services, and the degree of implementation of clinical nutritional services were found to be important for positive perception of clinical nutrition services by doctors. Therefore, proper provision of clinical nutrition services and effective therapeutic effects will be continuously highlighted, which will increase the awareness of the physician and ultimately increase the demand for clinical nutrition service.
Cross-Sectional Studies ; Negotiating* ; Nutritionists* ; Therapeutic Uses

OBJECTIVES: The purpose of this study was to investigate how clinical nutrition services is provided at a long term care hospital in Korea and to investigate job satisfaction levels of the clinical dietitians. METHODS: Survey questionnaire was sent to dietitians working at a long term care hospital in Korea. The participating hospitals (n=240) were randomly selected from 1,180 long- term care hospitals using a stratified sampling method. A total of 134 long term care hospital s and 223 dietitians completed the survey of clinical nutrition service s and job satisfaction questionnaires The job satisfaction questionnaire included 27 job satisfaction questions on task, stability vision, working conditions, and relationship areas. RESULTS: The average nutritional screening rate was 17.9% and the rate of computerized nutritional screening system was 9.7% in the participating hospitals. Nutritional intervention rate was only 3.2% of all patients. KOIHA (Korea Institute for Healthcare Accreditation) accreditated hospitals showed only 50% performance rate of nutrition service evaluation area. This shows that after achieving KOIHA accredition, many hospitals do not emphasize the performance of nutritional services. The job satisfaction scores in all four areas ranged from 2/5 to 3/5, implying generally low job satisfaction level in hospital dietitians. Linear regression analysis results showed that the "hospital adequacy grade" type was a significant predictor of job satisfaction level for two areas (working conditions & relationship). CONCLUSIONS: There is a need to provide proper standardized clinical nutrition services as a primary treatment and we observed large variations in the quality of nutritional service s in long term care hospitals. Therefore, government and local hospitals have to work on implementing nutritional programs and policies for improved service and care.
Delivery of Health Care ; Hospitals ; Humans ; Job Satisfaction ; Korea ; Linear Models ; Long-Term Care* ; Mass Screening ; Nutritionists ; Surveys and Questionnaires

BACKGROUND: A number of anesthetic factors may influence the risk of postoperative nausea and vomiting (PONV) throughout various mechanisms. This study analyzed the effect of different anesthetics and opioids with regards to PONV. METHODS: Ninety ASA physical status 1 and 2 patients, who were scheduled for minor orthopedic or plastic surgery, were randomly assigned for three groups to receive either propofol-fentanyl (Group PF), propofol-femifentanil (Group PR) or sevoflurane-fentanyl (Group SF). Anesthesia was induced with propofol or pentothal sodium, rocuronium and fentanyl (2microg/kg) or remifentanil (1microg/kg). Anesthesia was maintained with BIS (bispectral index) value in the range of 40-60 and blood pressure and heart rate within 20% of baseline. The incidence and severity score of PONV, the administration of metoclopramide, visual analogue scale (VAS) for pain were assessed at 2, 12, 24 hours postoperatively. RESULTS: The incidence of PONV in SF group was significantly higher than in PF and PR group during 0-2 hours period (P < 0.05). The incidence of PONV in SF and PF group was significantly higher than in PR group during 2-12 h period (P < 0.05). There were no significant differences among the groups in gender, the administration of antiemetics and postoperative VAS. CONCLUSIONS: With propofol based anesthesia, short-cting remifentanil resulted in a lower incidence of PONV during 2-12 h period than fentanyl. Propofol-fentanyl anesthesia decreases the incidence of PONV compared with sevoflurane-entanyl during 0-2 hours postoperatively.
Analgesics, Opioid* ; Anesthesia ; Anesthesia, General* ; Anesthetics* ; Antiemetics ; Blood Pressure ; Fentanyl ; Heart Rate ; Humans ; Incidence ; Metoclopramide ; Orthopedics ; Postoperative Nausea and Vomiting* ; Propofol ; Sodium ; Surgery, Plastic ; Thiopental