Long-term Clinical Course and Electroencephalographic Analysis in Children with Benign Childhood Epilepsy with Centrotemporal Spikes / 대한소아신경학회지

PURPOSE: Benign childhood epilepsy with centrotemporal spikes (BECTS) is one of the most common and benign focal epilepsy syndromes during childhood. In this study, we analyzed the clinical features and electroencephalogram (EEG) of BECT patients to determine if there were any predictive factors for seizures or abnormal EEG findings lasting longer than the average. METHODS: We studied 49 patients who were diagnosed with BECTS at the Department of Pediatrics, Chonnam National University Hospital and were 18 years of age or older at the time of the study. Differences in clinical course according to EEG features, treatment duration, abnormal EEG duration, seizure development period, and time to achieving the first normal EEG were analyzed. RESULTS: Average onset age was 8.3±1.9 years and follow-up duration was 4.2±2.4 years. The average seizure-free age was 9.5±2.0 years, and abnormal EEG-free age was 11.6±2.1 years. Younger-onset patients had a longer duration of medication (P=0.04). Patients who needed shorter time to achieving the first normal EEG had a shorter seizure development period (P=0.02). Patients who did not show typical EEG findings consistent with BECTS had a significantly longer duration of medication (P<0.01) and seizure development period (P=0.02), and abnormal EEG duration (P= 0.01). The ratio of abnormal findings in the first three EEGs was significantly correlated with the seizure development period, abnormal EEG duration, and duration of medication (P<0.01). CONCLUSION: Although BECTS is known to take a benign course, the actual clinical course varied from patient to patient, and these variations may be predicted by analyzing clinical factors or EEGs.

Outcomes of Hematopoietic Stem Cell Transplantation by Donor Types in Children with Acute Myeloid Leukemia / 임상소아혈액종양

BACKGROUND: The aim of this study was to compare the outcomes of children with acute myeloid leukemia (AML) who received stem cell transplantation from different donor groups.METHODS: This study included 37 pediatric AML patients who received allogeneic stem cell transplantation from March 1996 to December 2012 at Chonnam National University Hospital and Chonnam National University Hwasun Hospital. The overall survival (OS), event-free survival (EFS), cumulative incidence (CI) of graft versus host disease (GvHD), relapse and transplant-related mortality (TRM) were compared between different donor groups.RESULTS: Transplant donor groups included matched sibling donor (MSD, n=15), unrelated donor (URD=13), unrelated umbilical cord blood (UCB, n=7), or haploidentical donor (HD, n=2). Twenty-six patients survived with a median follow-up of 7.3 years. The 7-year EFS rates were 80.0±10.3% in MSD, 69.2±12.8% in URD and 57.1±18.7% in UCB, and 0% in HD, respectively (P=0.019). The CI of relapse at 5 years was 20.0%, 15.4%, 33.3%, 50%, respectively (P=0.721). The CI of TRM at 2 years was 0%, 15.4%, 16.7%, 50.0%, respectively in each donor group (P=0.017). The CI of grade II-IV acute and extensive chronic GvHD were higher in UCB (P=0.003, P=0.020, respectively). There were no significant differences in OS, EFS, and CI of TRM and relapse between allele-mismatched URD and UCB.CONCLUSION: Despite the limitation of small number of patients, the comparable outcome of pediatric AML patients transplanted from alternative donor with those transplanted from MSD are encouraging. Especially, if a matched donor is not available, allele-mismatched URD or UCB transplant may offer the advantage of prompt availability for patients who urgently require transplantation.

Outcomes of Hematopoietic Stem Cell Transplantation by Donor Types in Children with Acute Myeloid Leukemia / 임상소아혈액종양

BACKGROUND: The aim of this study was to compare the outcomes of children with acute myeloid leukemia (AML) who received stem cell transplantation from different donor groups. METHODS: This study included 37 pediatric AML patients who received allogeneic stem cell transplantation from March 1996 to December 2012 at Chonnam National University Hospital and Chonnam National University Hwasun Hospital. The overall survival (OS), event-free survival (EFS), cumulative incidence (CI) of graft versus host disease (GvHD), relapse and transplant-related mortality (TRM) were compared between different donor groups. RESULTS: Transplant donor groups included matched sibling donor (MSD, n=15), unrelated donor (URD=13), unrelated umbilical cord blood (UCB, n=7), or haploidentical donor (HD, n=2). Twenty-six patients survived with a median follow-up of 7.3 years. The 7-year EFS rates were 80.0±10.3% in MSD, 69.2±12.8% in URD and 57.1±18.7% in UCB, and 0% in HD, respectively (P=0.019). The CI of relapse at 5 years was 20.0%, 15.4%, 33.3%, 50%, respectively (P=0.721). The CI of TRM at 2 years was 0%, 15.4%, 16.7%, 50.0%, respectively in each donor group (P=0.017). The CI of grade II-IV acute and extensive chronic GvHD were higher in UCB (P=0.003, P=0.020, respectively). There were no significant differences in OS, EFS, and CI of TRM and relapse between allele-mismatched URD and UCB. CONCLUSION: Despite the limitation of small number of patients, the comparable outcome of pediatric AML patients transplanted from alternative donor with those transplanted from MSD are encouraging. Especially, if a matched donor is not available, allele-mismatched URD or UCB transplant may offer the advantage of prompt availability for patients who urgently require transplantation.

Prognostic Factors and Outcome in Childhood and Adolescent Osteosarcoma: Single Institution Experience / 임상소아혈액종양

BACKGROUND: Osteosarcoma is the most common primary malignant bone tumor in children and adolescents. The aim of this study was to evaluate survival and the prognostic factors in children and adolescent osteosarcoma.METHODS: Twenty-seven patients with osteosarcoma diagnosed at Chonnam National University Hwasun Hospital between Apr. 2004 and Feb. 2013 were retrospectively reviewed.RESULTS: Fourteen patients were males and the median age at diagnosis was 13.0 years. The most common tumor site was distal femur and 5 patients had metastasis at diagnosis. All patient but one who underwent definitive surgery at diagnosis received preoperative chemotherapy. The 5-year overall survival (OS) and event free survival (EFS) rate for all patients were 65.7+/-10.2% and 55.6+/-11.0%, respectively. Ten patients (37.0%) experienced relapse or progression of the disease. Among them, 3 patients are alive without any evidence of disease at the time of this writing. Seven among 8 dead patients died of relapse/progression of the disease. The 5-year EFS rate was significantly higher for patients with tumor volume <100 cm3 (n=14) at diagnosis than others (n=10) (70.7+/-14.6% vs. 37.5+/-16.1%, P=.031). Age, histologic type, metastasis at diagnosis and histologic response to preoperative chemotherapy did not have a significant effect on OS and EFS.CONCLUSION: Although chemotherapy has improved outcomes of osteosarcoma, relapse or progression is the most common cause of treatment failure. A higher tumor volume at diagnosis was identified as a poor prognostic factor. Future studies incorporating a larger number of patients are required to further delineate the prognostic factors in osteosearcoma.

Prognostic Factors and Outcome in Childhood and Adolescent Osteosarcoma: Single Institution Experience / 임상소아혈액종양

BACKGROUND: Osteosarcoma is the most common primary malignant bone tumor in children and adolescents. The aim of this study was to evaluate survival and the prognostic factors in children and adolescent osteosarcoma. METHODS: Twenty-seven patients with osteosarcoma diagnosed at Chonnam National University Hwasun Hospital between Apr. 2004 and Feb. 2013 were retrospectively reviewed. RESULTS: Fourteen patients were males and the median age at diagnosis was 13.0 years. The most common tumor site was distal femur and 5 patients had metastasis at diagnosis. All patient but one who underwent definitive surgery at diagnosis received preoperative chemotherapy. The 5-year overall survival (OS) and event free survival (EFS) rate for all patients were 65.7+/-10.2% and 55.6+/-11.0%, respectively. Ten patients (37.0%) experienced relapse or progression of the disease. Among them, 3 patients are alive without any evidence of disease at the time of this writing. Seven among 8 dead patients died of relapse/progression of the disease. The 5-year EFS rate was significantly higher for patients with tumor volume <100 cm3 (n=14) at diagnosis than others (n=10) (70.7+/-14.6% vs. 37.5+/-16.1%, P=.031). Age, histologic type, metastasis at diagnosis and histologic response to preoperative chemotherapy did not have a significant effect on OS and EFS. CONCLUSION: Although chemotherapy has improved outcomes of osteosarcoma, relapse or progression is the most common cause of treatment failure. A higher tumor volume at diagnosis was identified as a poor prognostic factor. Future studies incorporating a larger number of patients are required to further delineate the prognostic factors in osteosearcoma.