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.

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.
Diagnosis* ; Disease-Free Survival ; Humans ; Multivariate Analysis ; Neuroblastoma* ; Pathology ; Polymerase Chain Reaction ; Real-Time Polymerase Chain Reaction ; Reverse Transcription ; RNA, Messenger* ; Tyrosine 3-Monooxygenase* ; Tyrosine*

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.
Biopsy ; Brain Neoplasms ; Child ; Early Diagnosis ; Far East ; Female ; Germ Cells ; Germinoma ; Headache ; Humans ; Japan ; Korea ; Male ; Medical Records ; Neoplasms, Germ Cell and Embryonal ; Retrospective Studies ; Biomarkers, Tumor

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.
Antigens, CD34/metabolism ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Benzoic Acids/therapeutic use ; Blood Transfusion/*adverse effects ; Child ; Child, Preschool ; Creatinine/blood ; Ferritins/blood ; Follow-Up Studies ; Humans ; Infant ; Iron Chelating Agents/therapeutic use ; Iron Overload/*etiology ; Neuroblastoma/drug therapy/*therapy ; Retrospective Studies ; Risk Factors ; *Stem Cell Transplantation ; Transplantation, Autologous ; Triazoles/therapeutic use

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.
Antigens, CD34/metabolism ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Benzoic Acids/therapeutic use ; Blood Transfusion/*adverse effects ; Child ; Child, Preschool ; Creatinine/blood ; Ferritins/blood ; Follow-Up Studies ; Humans ; Infant ; Iron Chelating Agents/therapeutic use ; Iron Overload/*etiology ; Neuroblastoma/drug therapy/*therapy ; Retrospective Studies ; Risk Factors ; *Stem Cell Transplantation ; Transplantation, Autologous ; Triazoles/therapeutic use

BACKGROUND AND OBJECTIVES: he ischemic mitral regurgitation (MR) is known as a clinically important prognostic factor of acute myocardial infarction, and it is important to understand the mechanism of ischemic MR for successful treatment of ischemic MR. However, the mechanism of ischemic MR has not been known clearly. The purposes of this study were to evaluate the role of papillary muscle ischemia and LV dysfunction in the development of ischemic MR and to relate structural and functional changes of LV with severity of ischemic MR. METHODS: Left thoracotomy was performed in ten mongrel dogs. In group 1 (n=5), first and second obtuse marginal (OM) branches were ligated, and in group 2 (n=5), second and third OM branches were ligated. Epicardial echocardiography was performed at baseline, during infusion of esmolol, and during ligation of OM branches to measure end diastolic and end systolic area, fractional area shortening of LV, incomplete leaflet closure distance and area of mitral valve, and jet area of MR. Myocardial contrast echocardiography was performed to evaluate perfusion of papillary muscle and to measure risk area during ligation of OM branches. RESULTS: 1)There was no significant difference in decrease of fractional area shortening between infusion of esmolol and ligation of OM branches, but the significant MR developed in only one dog during esmolol infusion (p=NS), and in four dogs during ligation of OM branches (p<0.05). 2)Ischemia of papillary muscle was provoked in none of group 1, but four of five dogs in group 2 (p<0.05). Ischemic MR developed in all four dogs with provocation of papillary muscle ischemia, but in none of six dogs with maintained perfusion of papillary muscle (p=0.005). 3)Only incomplete leaflet closure area was significantly related with the severity of ischemic MR (r=0.71, p<0.05). CONCLUSIONS: Ischemia of papillary muscle is the important etiologic factor in development of ischemic MR in acute posterior myocardial infarction. It will be useful to assess and restore the perfusion of papillary muscles for the treatment of ischemic MR.
Animals ; Dogs ; Echocardiography ; Ischemia ; Ligation ; Mitral Valve ; Mitral Valve Insufficiency* ; Myocardial Infarction ; Papillary Muscles ; Perfusion ; Thoracotomy

BACKGROUND/AIMS: With the development of self-expanding metallic stents, colonic obstruction can be relieved without the need for surgery. The results of preoperative placement of stents for malignant colorectal obstruction might be different according to the obstructing lesion. The objective of this study was to compare clinical improvement rates and operative results after preoperative placement of stents for malignant colorectal obstruction according to the location of the obstructing lesion. METHODS: This is a retrospective study including 57 patients who underwent self-expanding metallic stent insertion for obstructing resectable colorectal cancers. Patients were classified into three groups according to the location of the lesion as follows: proximal to the sigmoid colon (Group A), sigmoid colon (Group B), and rectum (Group C). RESULTS: The number of patients in A, B, and C groups was 13, 22, and 22, respectively. No significant differences in age, gender, stent type, and accompanying diseases among the three groups were observed. There were no significant differences in stent-related complications, clinical improvement rates, and one-stage resection rates among the three groups. The postoperative complications, the requirement rate of ICU care, the period of ICU stay, postoperative hospital stay, and hospital mortality did not significantly differ among the three groups. CONCLUSIONS: Clinical improvement rates and operative results after successful placement of stents for obstructing resectable colorectal cancers are not different according to the location of the obstructing lesion, suggesting that preoperative stenting for one-stage curative resection is useful, irrespective of the location of lesion.
Aged ; Aged, 80 and over ; Colorectal Neoplasms/*diagnosis/surgery ; Female ; Hospital Mortality ; Humans ; Intensive Care Units ; Intestinal Obstruction/*surgery ; Length of Stay ; Male ; Middle Aged ; Preoperative Care ; Retrospective Studies ; *Stents

BACKGROUND: To avoid the adverse effects of cardiopulmonary bypass and to overcome late vein graft failure, we routinely performed off-pump total arterial coronary revascularization. MATERIAL AND METHOD: From July 2000 to August 2001, 104 consecutive patients underwent first elective off-pump total arterial coronary revascularization. Both internal mammary, radial and gastroepiploic arteries were used. Sequential and composite grafts were used to achieve complete revascularization. Perioperative adverse events and postoperative angiograms were analyzed. RESULT: A total of 252 arterial conduits were used with an average of 2.47 grafts per patient. A total of 326 distal anastomosis were performed with a mean of 3.13 distal anastomosis per patient. Cross over to on-pump occurred in seven patients (6.7%). Of these 4 were due to unstable hemodynamics during lateral or posterior wall stabilization as a result of cardiomegaly and 3 were due to uncontrolled bleeding during dissection of diffusely dimunitive deeply placed intramyocardial coronary arteries. There were no opeartive deaths. Two cases of perioperative myocardial infarction and transient neurologic complications occurred, respectively. Of the 312 distal anastomoses, 308 (98.7%) were compatible with Fitz-Gibbon A or B patency grading. CONCLUSION: Off-pump total arterial coronary revascularization was technically feasible in most elective cases with satisfactory early results. However, on-pump coronary bypass surgery should be considered in difficult circumstances, such as cardiomegaly or unfavorable anatomy of the target coronary artery.
Cardiomegaly ; Cardiopulmonary Bypass ; Coronary Artery Bypass ; Coronary Vessels ; Gastroepiploic Artery ; Hemodynamics ; Hemorrhage ; Humans ; Myocardial Infarction ; Transplants ; Veins

Measurement of regional cerebral blood flow with single photon emission computed tomography(SPECT) was performed using 99mTc-HMPAO in 16 patients with traumatic subdural hygroma, and we investigated the relationships between the results and the initial clinical findings and clinical outcomes on 4-month after injury. In patients with complex subdural hygroma, improvement of perfusion on follow up SPECT was correlated with the clinical outcome. But in patients with simple subdural hygroma, the clinical outcomes were good regardless of cerebral perfusion on initial SPECT and cerebral hypoperfusion was improved on follow up SPECT in all patients. These results suggested that associated parenchymal injury, initial perfusion of the frontal cortex, and improvement of perfusion on follow up SPECT were good indicators of clinical outcome. In conclusion, HMPAO-SPECT can be helpful in predicting the prognosis and assessing the effect of surgical treatment in patients with traumatic subdural hygroma.
Follow-Up Studies ; Humans ; Perfusion ; Prognosis ; Subdural Effusion* ; Technetium Tc 99m Exametazime* ; Tomography, Emission-Computed, Single-Photon*

Open heart surgery via right thoracotomy can be accomplished in atrial septal defects, and mitral valve diseases. Recently, thoracoscopic atrial septal defect closure, mitral valve repair, Maze operation, and minimal invasive direct coronary artery bypass (MIDCAB) are accomplished with AESOP 3000. However, there is no report of thoracoscopic aortic valve replacement in Korea. We report a successful thoracospic aortic valve replacement assisted with AESOP 3000 in a 31-year-old female patient.
Adult ; Aortic Valve* ; Coronary Artery Bypass ; Endoscopes* ; Female ; Heart Septal Defects, Atrial ; Humans ; Korea ; Mitral Valve ; Surgical Procedures, Minimally Invasive ; Thoracic Surgery ; Thoracoscopy ; Thoracotomy