Incidence and Mortality Trends in Critically Ill Children: A Korean Population-Based Study

Background@#Monitoring mortality trends can help design ways to improve survival, but observation of national mortality trends in critically ill children is lacking for the Korean population Methods: We analyzed the incidence and mortality trends of children younger than 18 years admitted to an intensive care unit (ICU) from 2012 to 2018 using the Korean National Health Insurance database. Neonates and neonatal ICU admissions were excluded. Multivariable logistic regression analyses were performed to estimate the odds ratio of in-hospital mortality according to admission year. Trends in incidence and in-hospital mortality of subgroups according to admission department, age, presence of intensivists, admissions to pediatric ICU, mechanical ventilation, and use of vasopressors were evaluated. @*Results@#The overall mortality of critically ill children was 4.4%. There was a significant decrease in mortality from 5.5% in 2012 to 4.1% in 2018 (Pfor trend < 0.001). The incidence of ICU admission in children remained around 8.5/10,000 population years (Pfor trend = 0.069). In-hospital mortality decreased by 9.2% yearly in adjusted analysis (P < 0.001). The presence of dedicated intensivists (Pfor trend < 0.001, mortality decrease from 5.7% to 4.0%) and admission to pediatric ICU (Pfor trend < 0.001, mortality decrease from 5.0% to 3.2%) were associated with significant decreasing trends in mortality. @*Conclusion@#Mortality among critically ill children improved during the study period, and the improving trend was prominent in children with high treatment requirements. Varying mortality trends, according to ICU organizations, highlight that advances in medical knowledge should be supported structurally.

Factors Associated with the Underuse of Sedatives and Neuromuscular Blocking Agents for Pediatric Emergency Endotracheal Intubation in Korea

Purpose@#Rapid sequence intubation (RSI) using sedatives and neuromuscular blocking agents (NMBAs) is recommended for pediatric emergency endotracheal intubation (ETI), but is not frequently performed in Korea. This study aimed to verify factors associated with the underuse of RSI medications. @*Materials and Methods@#This multicenter retrospective study reviewed patients aged under 18 years who underwent an ETI within 24 hours of arrival at the emergency department between 2016 and 2019. Any cases of ETI during cardiopulmonary resuscitation were excluded. We investigated the characteristics of the patients, intubators, RSI medications, and outcomes. The study cases were classified into no-medication, sedative-only, and sedative-with-NMBA groups. Multivariable logistic regression analysis of RSI medication use was conducted. @*Results@#A total of 334 cases with a median age of 3.4 years were included in this study. Sedatives and NMBAs were used in 63.8% and 32.9%, respectively. In comparing the no-medication (n=121), sedative-only (n=103), and sedative-with-NMBA (n=110) groups, patient age (median; 1.0 year vs. 2.8 years vs. 11.3 years; p<0.001), underlying medical conditions (77.7% vs. 56.3% vs. 36.4%; p<0.001), and pediatricians as intubators (76.9% vs. 54.4% vs. 17.3%; p<0.001) were different. The factors that influenced sedatives with NMBA use were patient age [for a year increment; adjusted odds ratio (aOR), 1.182; 95% confidence interval (CI), 1.120– 1.249], no underlying medical conditions (aOR, 2.109; 95% CI, 1.093–4.070), and intubators other than pediatricians (aOR, 5.123; 95% CI, 2.257–11.626). @*Conclusion@#RSI accounted for 32.9% of pediatric emergency ETI in Korea. The underuse of RSI medications is associated with younger patient age, underlying medical conditions, and pediatricians as intubators.

Influenza Vaccine Effectiveness in Children at the Emergency Department during the 2018-2019 Season: the First Season School-aged Children Were Included in the Korean Influenza National Immunization Program

Background@#For the 2018–2019 season, the national influenza immunization program expanded to cover children aged from 6 months to 12 years in Korea. This study aimed to analyze vaccine effectiveness (VE) against influenza in children visiting the pediatric emergency room at a tertiary hospital during the 2018-2019 season. @*Methods@#Patients tested for influenza antigens from October 1st 2018 to May 31st 2019 at the pediatric emergency room of Samsung Medical Center were included. Patients' influenza antigen test results, influenza vaccination history, and underlying medical conditions were reviewed retrospectively. VE was estimated from the test-negative design study. @*Results@#Among the 2,901 visits with influenza test results 1,692 visits of 1,417 patients were included for analysis. Among these 1,417 patients, 285 (20.1%) were positive (influenza A, n = 211, 74.0%; influenza B, n = 74, 26.0%). The VE in all patients was 36.4% (95% confidence interval [CI], 13.9 to 53.1). The VE for influenza A was 37.6% (95% CI, 12.6 to 55.5) and VE for influenza B was 24.0% (−38.5 to 58.3). The VE in the age group 6 months to 12 years was significant with a value of 35.6% (95% CI, 10.5 to 53.7); it was not statistically significant in the age group 13 to 18 years. In a multivariate logistic regression model, patients who received an influenza vaccination were less likely to get influenza infection (OR, 0.6; 95% CI, 0.4 to 0.8; P = 0.001), with significant confounding factors such as age group 13 to 18 years (OR, 0.5; 95% CI, 0.3 to 0.8; P = 0.003) and underlying hematology-oncology disease (OR, 0.3;95% CI, 0.1 to 0.6; P = 0.002). @*Conclusion@#We report moderate effectiveness of influenza vaccination in previously healthy children aged from 6 months to 12 years in the 2018-2019 season.

Effect of coronavirus disease 2019 on febrile children’s visits to the emergency department of a tertiary hospital

Purpose@#During the coronavirus disease 2019 pandemic, many countries have reported sharp drops in the numbers of patients, especially children, seeking emergency care unrelated to the disease. The author investigated the patterns of febrile children’s visits to the emergency department (ED) of a tertiary hospital from 2017 through 2020. @*Methods@#We compared the data of febrile children aged 15 years or younger who visited the ED in 2017-2019 and in 2020 (February through August, each year). Information on the children’s baseline characteristics, the Korean Triage and Acuity Scale 1-2 indicating a high severity, and ED disposition, such as hospitalization, were collected. In addition, we determined the number of children who underwent cancellation, defined as the leave of children without treatment, and the rates of respiratory virus polymerase chain reaction performance and its positivity. @*Results@#In 2020, we noted decreases in the median monthly fever-related ED visits (2017-2019, 326 [interquartile range, 292-425] vs. 2020, 149 [132-182]; P < 0.001) with a shorter median fever duration (2017-2019, 11 [3-27] days vs. 2020, 9 [2-23] days; P < 0.001). However, in the same year, we noted increases in rates of the Korean Triage and Acuity Scale 1-2 (2017-2019, 5.7% vs. 2020, 16.4%; P < 0.001) and hospitalization (2017-2019, 23.5% vs. 2020, 35.7%; P < 0.001). We also noted increases in the number of cancellation and the rate of respiratory virus polymerase chain reaction performance rate, along with a decrease in its positive rate in 2020. @*Conclusion@#During the pandemic, the febrile children’s visits to the ED decreased in number. However, the severity remarkably increased, requiring more medical attention.

Influenza Vaccine Effectiveness in Children at the Emergency Department during the 2018-2019 Season: the First Season School-aged Children Were Included in the Korean Influenza National Immunization Program

Background@#For the 2018–2019 season, the national influenza immunization program expanded to cover children aged from 6 months to 12 years in Korea. This study aimed to analyze vaccine effectiveness (VE) against influenza in children visiting the pediatric emergency room at a tertiary hospital during the 2018-2019 season. @*Methods@#Patients tested for influenza antigens from October 1st 2018 to May 31st 2019 at the pediatric emergency room of Samsung Medical Center were included. Patients' influenza antigen test results, influenza vaccination history, and underlying medical conditions were reviewed retrospectively. VE was estimated from the test-negative design study. @*Results@#Among the 2,901 visits with influenza test results 1,692 visits of 1,417 patients were included for analysis. Among these 1,417 patients, 285 (20.1%) were positive (influenza A, n = 211, 74.0%; influenza B, n = 74, 26.0%). The VE in all patients was 36.4% (95% confidence interval [CI], 13.9 to 53.1). The VE for influenza A was 37.6% (95% CI, 12.6 to 55.5) and VE for influenza B was 24.0% (−38.5 to 58.3). The VE in the age group 6 months to 12 years was significant with a value of 35.6% (95% CI, 10.5 to 53.7); it was not statistically significant in the age group 13 to 18 years. In a multivariate logistic regression model, patients who received an influenza vaccination were less likely to get influenza infection (OR, 0.6; 95% CI, 0.4 to 0.8; P = 0.001), with significant confounding factors such as age group 13 to 18 years (OR, 0.5; 95% CI, 0.3 to 0.8; P = 0.003) and underlying hematology-oncology disease (OR, 0.3;95% CI, 0.1 to 0.6; P = 0.002). @*Conclusion@#We report moderate effectiveness of influenza vaccination in previously healthy children aged from 6 months to 12 years in the 2018-2019 season.

Effect of coronavirus disease 2019 on febrile children’s visits to the emergency department of a tertiary hospital

Purpose@#During the coronavirus disease 2019 pandemic, many countries have reported sharp drops in the numbers of patients, especially children, seeking emergency care unrelated to the disease. The author investigated the patterns of febrile children’s visits to the emergency department (ED) of a tertiary hospital from 2017 through 2020. @*Methods@#We compared the data of febrile children aged 15 years or younger who visited the ED in 2017-2019 and in 2020 (February through August, each year). Information on the children’s baseline characteristics, the Korean Triage and Acuity Scale 1-2 indicating a high severity, and ED disposition, such as hospitalization, were collected. In addition, we determined the number of children who underwent cancellation, defined as the leave of children without treatment, and the rates of respiratory virus polymerase chain reaction performance and its positivity. @*Results@#In 2020, we noted decreases in the median monthly fever-related ED visits (2017-2019, 326 [interquartile range, 292-425] vs. 2020, 149 [132-182]; P < 0.001) with a shorter median fever duration (2017-2019, 11 [3-27] days vs. 2020, 9 [2-23] days; P < 0.001). However, in the same year, we noted increases in rates of the Korean Triage and Acuity Scale 1-2 (2017-2019, 5.7% vs. 2020, 16.4%; P < 0.001) and hospitalization (2017-2019, 23.5% vs. 2020, 35.7%; P < 0.001). We also noted increases in the number of cancellation and the rate of respiratory virus polymerase chain reaction performance rate, along with a decrease in its positive rate in 2020. @*Conclusion@#During the pandemic, the febrile children’s visits to the ED decreased in number. However, the severity remarkably increased, requiring more medical attention.

Clinical Significance of Segmental Chromosomal Aberrations in Patients with Neuroblastoma: First Report in Korean Population

Background@#This study aimed to investigate the incidence and clinical significance of segmental chromosomal aberrations (SCAs) in Korean patients with neuroblastoma. @*Methods@#Patients diagnosed with neuroblastoma from 2012 to 2018 were included for retrospective review. Fluorescence in situ hybridization (FISH) was used to analyze four SCAs (MYCN amplification, 1p deletion, 11q deletion, and 17q gain). Clinical characteristics at diagnosis, early tumor response (reduction in primary tumor volume and neuron-specific enolase level after the first three cycles of chemotherapy), and survival rates were compared according to SCAs. @*Results@#Among 173 patients with FISH results, 92 (53.2%) had at least one of the four SCAs, while 25 (14.5%) had two co-aberrations, and eight (4.6%) had three co-aberrations. SCAs detected in our study were MYCN amplification (n = 17, 9.8%), 1p deletion (n = 26, 15.2%), 11q deletion (n = 44, 25.6%), and 17q gain (n = 46, 27.1%). Patients with MYCN amplification showed a better early response but a worse survival than those without (5-year overall survival: 46.2% ± 13.1% vs. 88.6% ± 3.4%). Furthermore, 1p deletion was associated with a better early response but a worse survival; however, it was not an independent factor for survival. We could not find any prognostic significance associated with 11q deletion or 17q gain. @*Conclusion@#This is the first study investigating SCAs in Korean neuroblastoma patients. Prognostic significance of SCAs other than MYCN amplification was different from those reported in western countries. Further study with a larger cohort and longer follow-up is needed to confirm our findings.

Clinical features of adolescents who visited the emergency department with chest discomfort: the importance of recognizing underlying medical conditions

Purpose@#We aimed to evaluate the clinical features of adolescents who visit emergency departments (EDs) with chest discomfort, and analyze the implications of underlying medical conditions for the development of cardiogenic chest discomfort. @*Methods@#We reviewed the medical records of adolescents (13-18 years) with chest discomfort who visited a tertiary hospital ED in Seoul, Korea from 2014 through 2018. Sex, age, duration and character of the discomfort, symptoms and signs, ED length of stay, abnormal findings of vital signs, chest radiograph, electrocardiogram, and elevated concentrations of cardiac enzymes were reviewed. Final diagnosis was based on cardiac evaluations within 1 year after the index visit. Underlying medical conditions were defined as visits to the cardiology, pulmonology or hematology-oncology clinics at least twice in the preceding year. Initial suspicious clinical findings were defined as palpitation, syncope or high blood pressure. Logistic regression was used to identify predictors for cardiac etiology. @*Results@#Of the 231 patients, 43 (18.6%) and 69 (29.9%) had underlying medical conditions and initial suspicious clinical findings, respectively. The predictors for cardiac etiology were underlying medical conditions (odds ratio, 4.28; 95% confidence interval, 1.09-16.73), initial suspicious clinical findings (4.77; 1.36-16.77), abnormal electrocardiogram (11.54; 3.22-41.32), and elevated concentration of troponin I (66.52; 5.37-823.55). The patients with cardiogenic chest discomfort had a longer median ED length of stay (281.0 minutes [interquartile range, 215.5-369.0] vs. 199.5 [132.8-298.0]; P = 0.004) and a higher hospitalization rate (48.3% vs. 13.4%; P < 0.001) than those with non-cardiogenic chest discomfort. @*Conclusion@#It may be necessary to recognize underlying medical conditions and initial suspicious clinical findings in EDs prior to cardiac evaluation in adolescents with chest discomfort.

Clinical Significance of Segmental Chromosomal Aberrations in Patients with Neuroblastoma: First Report in Korean Population

Background@#This study aimed to investigate the incidence and clinical significance of segmental chromosomal aberrations (SCAs) in Korean patients with neuroblastoma. @*Methods@#Patients diagnosed with neuroblastoma from 2012 to 2018 were included for retrospective review. Fluorescence in situ hybridization (FISH) was used to analyze four SCAs (MYCN amplification, 1p deletion, 11q deletion, and 17q gain). Clinical characteristics at diagnosis, early tumor response (reduction in primary tumor volume and neuron-specific enolase level after the first three cycles of chemotherapy), and survival rates were compared according to SCAs. @*Results@#Among 173 patients with FISH results, 92 (53.2%) had at least one of the four SCAs, while 25 (14.5%) had two co-aberrations, and eight (4.6%) had three co-aberrations. SCAs detected in our study were MYCN amplification (n = 17, 9.8%), 1p deletion (n = 26, 15.2%), 11q deletion (n = 44, 25.6%), and 17q gain (n = 46, 27.1%). Patients with MYCN amplification showed a better early response but a worse survival than those without (5-year overall survival: 46.2% ± 13.1% vs. 88.6% ± 3.4%). Furthermore, 1p deletion was associated with a better early response but a worse survival; however, it was not an independent factor for survival. We could not find any prognostic significance associated with 11q deletion or 17q gain. @*Conclusion@#This is the first study investigating SCAs in Korean neuroblastoma patients. Prognostic significance of SCAs other than MYCN amplification was different from those reported in western countries. Further study with a larger cohort and longer follow-up is needed to confirm our findings.

Clinical features of adolescents who visited the emergency department with chest discomfort: the importance of recognizing underlying medical conditions

Purpose@#We aimed to evaluate the clinical features of adolescents who visit emergency departments (EDs) with chest discomfort, and analyze the implications of underlying medical conditions for the development of cardiogenic chest discomfort. @*Methods@#We reviewed the medical records of adolescents (13-18 years) with chest discomfort who visited a tertiary hospital ED in Seoul, Korea from 2014 through 2018. Sex, age, duration and character of the discomfort, symptoms and signs, ED length of stay, abnormal findings of vital signs, chest radiograph, electrocardiogram, and elevated concentrations of cardiac enzymes were reviewed. Final diagnosis was based on cardiac evaluations within 1 year after the index visit. Underlying medical conditions were defined as visits to the cardiology, pulmonology or hematology-oncology clinics at least twice in the preceding year. Initial suspicious clinical findings were defined as palpitation, syncope or high blood pressure. Logistic regression was used to identify predictors for cardiac etiology. @*Results@#Of the 231 patients, 43 (18.6%) and 69 (29.9%) had underlying medical conditions and initial suspicious clinical findings, respectively. The predictors for cardiac etiology were underlying medical conditions (odds ratio, 4.28; 95% confidence interval, 1.09-16.73), initial suspicious clinical findings (4.77; 1.36-16.77), abnormal electrocardiogram (11.54; 3.22-41.32), and elevated concentration of troponin I (66.52; 5.37-823.55). The patients with cardiogenic chest discomfort had a longer median ED length of stay (281.0 minutes [interquartile range, 215.5-369.0] vs. 199.5 [132.8-298.0]; P = 0.004) and a higher hospitalization rate (48.3% vs. 13.4%; P < 0.001) than those with non-cardiogenic chest discomfort. @*Conclusion@#It may be necessary to recognize underlying medical conditions and initial suspicious clinical findings in EDs prior to cardiac evaluation in adolescents with chest discomfort.

Clinical Significance of Tyrosine Hydroxylase mRNA Transcripts in Peripheral Blood at Diagnosis in Patients with Neuroblastoma / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: The purpose of this study is to investigate the clinical significance of tyrosine hydroxylase (TH) expression in peripheral blood (PB) at diagnosis in patients with neuroblastoma. MATERIALS AND METHODS: TH mRNA expression in PB was measured by reverse transcription quantitative real-time polymerase chain reaction in 210 patients who were newly diagnosed with neuroblastoma from July 2005 to June 2015 and the clinical significance of TH expression in PB at diagnosis was evaluated. RESULTS: TH expression was positive in 60 patients (28.6%). Fifty of 60 TH-positive patients had metastatic tumors and the remaining 10 had localized tumors. TH expression was associated with high-risk features (i.e., advanced stage, older age, unfavorable pathology, and MYCN amplification) at diagnosis. Among TH-positive patients, higher TH expression level was observed in high-risk patients than in low- or intermediate-risk patients (p=0.035). The probability of 5-year progression-free survival (PFS) was lower in TH-positive patients than in TH-negative patients (63.8±6.9% vs. 94.7±2.1%, p < 0.001). In analysis confined to high-risk patients, the 5-year probability of PFS remained lower in TH-positive patients (55.7±8.2% vs. 89.6±5.8%, p < 0.001). Among TH-positive patients, a higher expression level of TH was associated with a worse outcome. In multivariate analyses, positive TH expression in PB at diagnosis was an independent poor prognostic factor for PFS. CONCLUSION: The treatment intensity should be tailored according to TH expression in PB at diagnosis.

Hematologic Recovery after Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Children with High-Risk Solid Tumors

Although the number of studies using tandem high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT) for the treatment of high-risk pediatric solid tumors has been increasing, documentation of hematologic recovery after tandem HDCT/autoSCT is very limited. For this reason, we retrospectively analyzed the hematologic recovery of 236 children with high-risk solid tumors who underwent tandem HDCT/autoSCT. The median numbers of CD34+ cells transplanted during the first and second HDCT/autoSCT were 4.3 x 10(6)/kg (range 0.6-220.2) and 4.1 x 10(6)/kg (range 0.9-157.6), respectively (P = 0.664). While there was no difference in neutrophil recovery between the first and second HDCT/autoSCT, platelet and RBC recoveries were significantly delayed in the second HDCT/autoSCT (P < 0.001 and P < 0.001, respectively). Delayed recovery in the second HDCT/autoSCT was more prominent when the number of transplanted CD34+ cells was lower, especially if it was < 2 x 10(6)/kg. A lower CD34+ cell count was also associated with increased RBC transfusion requirements and a higher serum ferritin level after tandem HDCT/autoSCT. More CD34+ cells need to be transplanted during the second HDCT/autoSCT in order to achieve the same hematologic recovery as the first HDCT/autoSCT.

Human bone marrow-derived mesenchymal stem cell gene expression patterns vary with culture conditions

BACKGROUND: Because of the heterogeneity of human mesenchymal stem cells (MSCs), methods for cell expansion in culture and the effects on gene expression are critical factors that need to be standardized for preparing MSCs. We investigated gene expression patterns of MSCs with different seeding densities and culture times. METHODS: Bone marrow-derived MSCs were plated at densities from 200 cells/cm2 to 5,000 cells/cm2, and the gene expression patterns were evaluated over time using a reverse-transcription polymerase chain reaction assay. RESULTS: The mRNA levels of factors that play a critical role in cell migration and tissue regeneration, such as podocalyxin-like protein (PODXL), alpha4-integrin, alpha6-integrin, and leukemia inhibitory factor (LIF), were higher in MSCs plated at 200 cells/cm2 than in MSCs plated at 5,000 cells/cm2. The mRNA levels of these factors gradually increased for 10 days and then decreased by day 15 in culture. MSCs seeded at 200 cells/cm2 that were cultured for 10 days expressed high levels of Oct-4 and Nanog. Indoleamine 2,3-dioxygenase, cyclooxygenase-1, and hepatocyte growth factor expression were upregulated in the presence of the proinflammatory cytokine interferon-gamma in these cells. CONCLUSION: We found differences in the gene expression patterns of MSCs under different culture conditions. MSCs from 10-day cultures seeded at a low density were efficiently expanded, expressed PODXL, alpha6-integrin, alpha4-integrin, and LIF, and maintained properties like stemness and immunomodulation. Therefore, ex vivo expansion of MSCs maintained for an adequate culture time after plating at low cell density can provide an effective regenerative medicinal strategy for cell therapies using MSCs.

Hematopoietic stem cell transplantation in children with acute leukemia: similar outcomes in recipients of umbilical cord blood versus marrow or peripheral blood stem cells from related or unrelated donors / 소아과

PURPOSE: This study compared outcomes in children with acute leukemia who underwent transplantations with umbilical cord blood (UCB), bone marrow, or peripheral blood stem cells from a human leukocyte antigen (HLA)-matched related donor (MRD) or an unrelated donor (URD). METHODS: This retrospective study included consecutive acute leukemia patients who underwent their first allogeneic hematopoietic stem cell transplantation (HSCT) at Samsung Medical Center between 2005 and 2010. Patients received stem cells from MRD (n=33), URD (n=46), or UCB (n=41). RESULTS: Neutrophil and platelet recovery were significantly longer after HSCT with UCB than with MRD or URD (P<0.01 for both). In multivariate analysis using the MRD group as a reference, the URD group had a significantly higher risk of grade III to IV acute graft-versus-host disease (GVHD; relative risk [RR], 15.2; 95% confidence interval [CI], 1.2 to 186.2; P=0.03) and extensive chronic GVHD (RR, 6.9; 95% CI, 1.9 to 25.2; P<0.01). For all 3 donor types, 5-year event-free survival (EFS) and overall survival were similar. Extensive chronic GVHD was associated with fewer relapses (RR, 0.1; 95% CI, 0.04 to 0.6; P<0.01). Multivariate analysis showed that lower EFS was associated with advanced disease at transplantation (RR, 3.2; 95% CI, 1.3 to 7.8; P<0.01) and total body irradiation (RR, 2.1; 95% CI, 1.0 to 4.3; P=0.04). CONCLUSION: Survival after UCB transplantation was similar to survival after MRD and URD transplantation. For patients lacking an HLA matched donor, the use of UCB is a suitable alternative.

Hematopoietic stem cell transplantation in children with acute leukemia: similar outcomes in recipients of umbilical cord blood versus marrow or peripheral blood stem cells from related or unrelated donors / 소아과

PURPOSE: This study compared outcomes in children with acute leukemia who underwent transplantations with umbilical cord blood (UCB), bone marrow, or peripheral blood stem cells from a human leukocyte antigen (HLA)-matched related donor (MRD) or an unrelated donor (URD). METHODS: This retrospective study included consecutive acute leukemia patients who underwent their first allogeneic hematopoietic stem cell transplantation (HSCT) at Samsung Medical Center between 2005 and 2010. Patients received stem cells from MRD (n=33), URD (n=46), or UCB (n=41). RESULTS: Neutrophil and platelet recovery were significantly longer after HSCT with UCB than with MRD or URD (P<0.01 for both). In multivariate analysis using the MRD group as a reference, the URD group had a significantly higher risk of grade III to IV acute graft-versus-host disease (GVHD; relative risk [RR], 15.2; 95% confidence interval [CI], 1.2 to 186.2; P=0.03) and extensive chronic GVHD (RR, 6.9; 95% CI, 1.9 to 25.2; P<0.01). For all 3 donor types, 5-year event-free survival (EFS) and overall survival were similar. Extensive chronic GVHD was associated with fewer relapses (RR, 0.1; 95% CI, 0.04 to 0.6; P<0.01). Multivariate analysis showed that lower EFS was associated with advanced disease at transplantation (RR, 3.2; 95% CI, 1.3 to 7.8; P<0.01) and total body irradiation (RR, 2.1; 95% CI, 1.0 to 4.3; P=0.04). CONCLUSION: Survival after UCB transplantation was similar to survival after MRD and URD transplantation. For patients lacking an HLA matched donor, the use of UCB is a suitable alternative.

Iron Overload during Follow-up after Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Patients with High-Risk Neuroblastoma

Multiple RBC transfusions inevitably lead to a state of iron overload before and after high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT). Nonetheless, iron status during post-SCT follow-up remains unknown. Therefore, we investigated post-SCT ferritin levels, factors contributing to its sustained levels, and organ functions affected by iron overload in 49 children with high-risk neuroblastoma who underwent tandem HDCT/autoSCT. Although serum ferritin levels gradually decreased during post-SCT follow-up, 47.7% of the patients maintained ferritin levels above 1,000 ng/mL at 1 yr after the second HDCT/autoSCT. These patients had higher serum creatinine (0.62 vs 0.47 mg/mL, P = 0.007) than their counterparts (< 1,000 ng/mL). Post-SCT transfusion amount corresponded to increased ferritin levels at 1 yr after the second HDCT/autoSCT (P < 0.001). A lower CD34+ cell count was associated with a greater need of RBC transfusion, which in turn led to a higher serum ferritin level at 1 yr after HDCT/autoSCT. The number of CD34+ cells transplanted was an independent factor for ferritin levels at 1 yr after the second HDCT/autoSCT (P = 0.019). Consequently, CD34+ cells should be transplanted as many as possible to prevent the sustained iron overload after tandem HDCT/autoSCT and consequent adverse effects.

Iron Overload during Follow-up after Tandem High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Patients with High-Risk Neuroblastoma

Multiple RBC transfusions inevitably lead to a state of iron overload before and after high-dose chemotherapy and autologous stem cell transplantation (HDCT/autoSCT). Nonetheless, iron status during post-SCT follow-up remains unknown. Therefore, we investigated post-SCT ferritin levels, factors contributing to its sustained levels, and organ functions affected by iron overload in 49 children with high-risk neuroblastoma who underwent tandem HDCT/autoSCT. Although serum ferritin levels gradually decreased during post-SCT follow-up, 47.7% of the patients maintained ferritin levels above 1,000 ng/mL at 1 yr after the second HDCT/autoSCT. These patients had higher serum creatinine (0.62 vs 0.47 mg/mL, P = 0.007) than their counterparts (< 1,000 ng/mL). Post-SCT transfusion amount corresponded to increased ferritin levels at 1 yr after the second HDCT/autoSCT (P < 0.001). A lower CD34+ cell count was associated with a greater need of RBC transfusion, which in turn led to a higher serum ferritin level at 1 yr after HDCT/autoSCT. The number of CD34+ cells transplanted was an independent factor for ferritin levels at 1 yr after the second HDCT/autoSCT (P = 0.019). Consequently, CD34+ cells should be transplanted as many as possible to prevent the sustained iron overload after tandem HDCT/autoSCT and consequent adverse effects.

Effect of Preoperative Chemotherapy on Outcome of Wilms Tumor / 임상소아혈액종양

BACKGROUND: Wilms tumor is the most common renal tumor in children. The aim of this study was to assess the effect of preoperative chemotherapy after needle biopsy on outcome of Wilms tumor and suggest an optimal treatment scheme on Wilms tumor.METHODS: We reviewed the medical records of 52 patients who were diagnosed with Wilms tumor from 1995 to 2010. Before 2000, primary nephrectomy was usually done. After 2000, preoperative chemotherapy was usually done.RESULTS: Preoperative chemotherapy was given to 39 cases while primary nephrectomy was done in 13 cases. Five year survival rate and five year event-free survival rate were 96.2+/-2.7% and 87.9+/-4.7%, respectively. Tumor spillage occurred more frequently in primary nephrectomy group than in preoperative chemotherapy after needle biopsy group (P=0.014). There was no significant difference in survival between the two groups (P=0.599).CONCLUSION: Preoperative chemotherapy after needle biopsy improved diagnostic accuracy and decreased tumor spillage while strengthened chemotherapy intensity. Further studies are needed to optimize chemotherapy intensity.

Clinical Characteristics of Intracranial Germ Cell Tumors in Children / 임상소아혈액종양

BACKGROUND: Intracranial germ cell tumors are higher in the East Asia such as Korea and Japan than any other Western countries. By analyzing common clinical features of intracranial germ cell tumors in children, we will prevent from misdiagnosing and delaying in the establishment of diagnosis. Furthermore, we can choose appropriate therapeutic plans to improve patient's prognosis.METHODS: We retrospectively reviewed the medical records of 68 patients to investigate and analyze clinical characteristics of intracranial germ cell tumors in children.RESULTS: The average age of 68 patients was 14.8 years old, and the male to female ratio in all patients was 3:1. The most common symptom presented by 30 patients was headache regarded as a nonspecific symptom in brain tumors. Sixty four patients were diagnosed by histologic method called biopsy and most of them were come out into germinoma. Thirty five patients were included in low-risk group and 30 patients were in high-risk group. Intracranial germ cell tumors in this study were most commonly located in the pineal gland.CONCLUSION: There are a variety of types in intracranial germ cell tumors, and they have been accurately diagnosed by radiologic, histologic methods and elevated tumor markers. We concluded that it is necessary for early diagnosis to evaluate exhaustively in patients suspected of brain tumors.

Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor in an Adolscence, Manifested as Isolated Cervical Mass / 임상소아혈액종양

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PPNET) arises from bone or soft tissue of many sites of body, but ES/PPNET, as primary lesion, in the soft tissue of neck is very uncommon. The authors experienced a case of ES/PPNET in a 17-year-old girl, which was manifested as isolated cervical mass in the soft tissue without any other suspected primary lesion or metastasis. We report the case with a brief review of the literature.