PURPOSE: The purpose of this study was to examine the molecular basis for steroid-induced cataract and the specific aim was to investigate the potential effects of glucocorticoids on modulation of alpha-crystallin gene expression in lens epithelial cells. METHOD: Bovine lens epithelial explants, B-3 human lens epithelial cells, and alphaTN4 mouse lens epithelial cells were incubated in the absence or presence of dexamethasone. At the indicated time point, total cellular RNA was isolated and subjected to RT-PCR and Northern blot analysis to assess mRNA expression of alphaA- and alphaB-crystallin. RESULTS: The mRNA expression of alphaA-crystallin was drastically downregulated by treatment with dexamethasone in bovine, human, and mouse lens epithelial cells. The mRNA level of alphaA-crystallin was also decreased in response to hydrocortisone but not to estrogen. The stability of the alphaA-crystallin mRNA was decreased after dexamethasone treatment. CONCLUSIONS: These results suggest that the alteration of alphaA-crystallin gene expression by glucocorticoids may result in abnomal structure and differentiation of lens epithelial cells on steroid-induced cataract formation.
alpha-Crystallins* ; Animals ; Blotting, Northern ; Cataract ; Dexamethasone ; Epithelial Cells* ; Estrogens ; Gene Expression* ; Glucocorticoids* ; Humans ; Hydrocortisone ; Mice ; RNA ; RNA, Messenger

To identify the long-term survival rate and prognostic factors of AMI in Korea, total 404 patients who presented between Jan 1984 and mar 1989 at Seoul National University Hospotal were followed for and average of 24.9+/-18.2 months(range 1 to 69 months). 50 patients(12.4%) died during the in-hospital period and 25 patients(6.2%) died after discharge. Among the survivors reinfarction developled in 11 patients(3.3%). Overall survival rates were 0.87, 0.85, 0.83, 0.81, 0.79, 0.77 and event-free survival rates were 0.87, 0.84, 0.83, 0.79, 0.77, 0.72 at 1, 6, 12, 24, 36, 48 months respectively. During the in-hospital period sex, age, peak creatine kinase level, Killip class, Q wave in ECG, heart failure, and AV block in anterior infarction were of prognostic value. After discharge age, exercise duration on pre-discharge treadmill test, cardiac index, ejection fraction, and presence of heart failure were significant prognostic factors. Pre-discharge coronary angiographies were performed in 217 cases. There was no statistically significant difference in survival rate between multiple vessel disease and single vessel disease. But the more the number of involved vessels was, the higher the incidence of reinfarction was. In the group with jeopardy score less than 8, event-free survival rate was signigicantly higher. Overall survival rate was higher and reinfarction rate was lower in the group, but both were not statistically significant. On discriminant analysis of in-hospital prognostic factors, Killip class, heart failure and age were independent prognostic factors, but other factors had no additional prognostic value.
Atrioventricular Block ; Coronary Angiography ; Creatine Kinase ; Disease-Free Survival ; Electrocardiography ; Exercise Test ; Heart Failure ; Humans ; Incidence ; Infarction ; Korea ; Myocardial Infarction* ; Seoul ; Survival Rate* ; Survivors

PURPOSE: Malignant lymphoma is the primary malignant tumor derived from lymphoid organs. It is composed of Hodgkin's disease and non-Hodgkin lymphoma. Recently, survival rate is on the rise due to improved combination chemotherapy, radiotherapy and high dose chemotherapy followed by hematopoietic stem cell transplantation. In South Korea, no epidemiologic studies concerning malignant lymphoma in the pediatric age group has been performed. Therefore, the Korean Society of Pediatric Hematology-Oncology retrospectively analyzed the incidence, pathologic subtypes, treatment strategies, and survival rates of pediatric malignant lymphomas in South Korea. METHOD: Questionnaires were made and sent to a group of training hospitals, with a return of 580 questionnaires from 24 hospitals. Among them, 517 reports were suitable for analysis. RESULTS: Among the 517 cases, Hodgkin's disease accounted for 58 cases and non-Hodgkin's lymphoma for 459 cases. Male to female ratio for malignant lymphoma was 2.7. Mean age at diagnosis was 8.3 years. Among the pathologic subtypes, mixed cellularity was the most frequent subtype for Hodgkin's disease. Most (70.7%) cases of non-Hodgkins lymphoma belonged to high grade NHL. Burkitt lymphoma accounted for 102 cases, and lymphoblastic lymphoma was found in 58 cases. Peripheral lymphadenopathy was the most common presenting sign upon diagnosis. B symptoms were significantly more frequent in Hodgkin's disease patients than in non-Hodgkin lymphoma patients. The Complete response rate was 62.1% for non-Hodgkin's lymphoma, and 82.8% for Hodgkin's disease. Overall 5 year survival rate was 60.0% in non-hodgkin's lymphoma, and 84.8% in Hodgkin's disease. CONCLUSION: The annual incidence of malignant lymphoma in Korea is 4.7 per million. In cases of chemotherapy-sensitive, refractory or relapsed malinant lymphoma, high dose chemotherapy followed by hematopoietic stem cell transplantation is vital for improved survival. For more systematic analysis of epidemiology on malignant lymphomas, better surveillance mechanisms on the occurrence of malignant lymphomas are crucial, and establishment of standardized treatment protocol for malignant lymphoma is required.
Burkitt Lymphoma ; Child* ; Clinical Protocols ; Diagnosis ; Drug Therapy ; Drug Therapy, Combination ; Epidemiologic Studies ; Epidemiology* ; Female ; Hematopoietic Stem Cell Transplantation ; Hodgkin Disease ; Humans ; Incidence ; Korea* ; Lymphatic Diseases ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Surveys and Questionnaires ; Radiotherapy ; Retrospective Studies* ; Survival Rate

PURPOSE: Wilms tumor is the most common malignant renal tumor in children. We investigated the epidemiology, clinical features and treatment outcome of the children with Wilms tumor in Korea during the recent 10 years. METHODS: Two hundred forty six patients were enrolled between January 1991 and December 2000 from 26 major hospitals in Korea. The data regarding the clinical features including sex, age, pathologic type, prognostic factor and treatment outcome of patients were analyzed retrospectively by review of patient's medical records. Kaplan-Meier survival curves were constructed, The differences between groups were analyzed by log-rank test. RESULTS: There were 130 males and 116 females. The incidence between the age of 1~4 years was the highest with 66.2%. The annual incidence rate per 1, 000, 000 population varied from 1.9 to 2.1. The 10 years overall survival rate according to sex, clinical stage, pathologic type and relapse were as follows: 88.6% in male, 90.9% in female, 100% in stage I, 94.7% in stage II, 92.1% in stage III, 63.4% in stage IV, 85.7% in stage V, 95.3% in favorable histology, 64.1% in unfavorable histology, 94.8% in non-relapse, and 40.9% in relapse. The relapse rate was 12%. The 10 years overall survival rate of 246 patients were 89.1%. CONCLUSION: Our results could provide the most recent and important clinical information on Wilms tumor of children in Korea.
Child ; Epidemiology* ; Female ; Humans ; Incidence ; Kaplan-Meier Estimate ; Korea* ; Male ; Medical Records ; Recurrence ; Retrospective Studies ; Survival Rate ; Treatment Outcome ; Wilms Tumor*

PURPOSE: The annual incidence of aplastic anemia (AA) in Asian countries is higher than in Western countries. The pathogenesis in AA has been investigated in hematopoiesis and immunology. Recently, the survival rate and the quality of life of the patients with AA have been steadily improved by the development of a variety of treatments such as the immunosuppressive therapy (IST), and hematopoietic stem cell transplantation (HSCT). The Korean Society of Pediatric Hematology-oncology retrospectively investigated the incidence, treatment strategies, survival rate, and time to become independent from transfusion in patients with AA, who were diagnosed from January 1st, 1991 to December 31st, 2000 in Korea. METHODS: All the questionnaires were sent to a group of training hospitals, and we collected about 600 questionnaire forms from 27 hospitals. However, 493 reports were available for data analysis. RESULTS: The male and female ratio in AA is 1.1 (259 males vs. 234 female). The median age at diagnosis is 9 years old (range; 0.8~16 years old). The annual incidence of children with AA in Korea is 4.5 per million on the basis of Korean pediatric population. In etiology, there are 20 cases (4.1%) in congenital and others in acquired AA. In acquired AA, the cause of illness was not identifiable for most of the patients, but 1 patient had hepatitis-associated AA, and 3 patients developed the illness after medication. According to the initial laboratory data at diagnosis, the peripheral blood findings showed that hemoglobin is 7.1+/-2.4 g/dL, white blood cell 3, 200/microL (200~16, 550), absolute neutrophil counts 670/microL (0~12, 487), platelets 19, 000/microL (1, 000~500, 000), and corrected reticulocytes 0.18% (0.0~4.7). The bone marrow examination revealed that cellularity was below 25% in 348 patients, and over 25% in 105 patients. In the available data, 269 patients (54.6%) were diagnosed of severe aplastic anemia (SAA) and 224 patients of non-SAA (NSAA). HSCT were done for 96 patients (19.5%) and others received another treatments such as the IST. The anti-thymocyte globulin (ATG) or anti-lymphocyte globulin (ALG) treatment was done for 263 cases, corticosteroids for 259, cyclosporine A (CSA) for 215, and anabolic steroids for 138. The combination IST including ATG or ALG plus corticosteroid plus CSA were applied to 154 children with AA, and transfusion only in 37, as conservative care. In case of those patients with HSCT, the time from diagnosis to transplantation was 12 months (1~144 months) and the sources of stem cells were bone marrow in 82 cases, growth factor mobilized peripheral blood in five, and cord blood in six. There were 57 patients transfused below 40 units of blood products before HSC transplantation. Graft rejection was identified from 16 patients, and booster transplantations were done for 12 patients among them. In complications of HSCT, the graft versus host disease was developed in 20 patients and viral diseases in 12 cases including the CMV, herpetic infection, and hepatitis. Also, one patient suffered from veno-occlusive disease. The overall survival rate in children with AA is 64.3%. The survival rate in HSCT is better than that IST (76.9% vs. 62.6%, P< 0.05). In IST, overall survival rate in very SAA showed lower than SAA and NSAA, and in SAA lower than NSAA in case of absolute neutrophil count below 200/microL (P< 0.05). There was no significant difference in terms of the sex, age at beginning of treatment. In HSCT, transfusion was not related to the survival rate. However, overall survival rate is better in short interval between diagnosis and HSCT than in long interval (P< 0.05). There was no significant difference in the probability of transfusion independence according to treatment strategies, even though it was 71.0% in HSCT and 12.8% in immunosuppressive therapy at the end point of survey (P=0.47). The response pattern was as follows. There were 155 cases of complete response, 110 of partial response and 120 of no response in spite of various treatments. The relapse after treatment was found in 11 patients after IST, of which 6 patients experienced more than 2nd relapse. The median time between the end of treatment and relapse was 16 months (6~84 months). Only three cases developed into other diseases (1 case into acute myeloid leukemia and 2 cases into myelodysplastic syndrome). The median time from diagnosis to the end of treatment was 62 months (0.5~174 months). In fatal cases, the median time between diagnosis and death was 29 months (0~144 months) despite several therapeutic strategies. CONCLUSION: In Korea, the annual incidence of children with AA is 4.5 per million. This result is similar to the ones reported in other Asian countries, but higher than those in Western countries. Although a lot of children with AA received various therapies including IST or HSCT, new treatment strategies have to be developed to improve the survival rate and the quality of life of children with AA
Adrenal Cortex Hormones ; Allergy and Immunology ; Anemia, Aplastic* ; Antilymphocyte Serum ; Asian Continental Ancestry Group ; Bone Marrow ; Bone Marrow Examination ; Child* ; Cyclosporine ; Diagnosis ; Epidemiology* ; Female ; Fetal Blood ; Graft Rejection ; Graft vs Host Disease ; Hematopoiesis ; Hematopoietic Stem Cell Transplantation ; Hepatitis ; Humans ; Incidence ; Korea* ; Leukemia, Myeloid, Acute ; Leukocytes ; Male ; Neutrophils ; Quality of Life ; Surveys and Questionnaires ; Recurrence ; Reticulocytes ; Retrospective Studies* ; Statistics as Topic ; Stem Cells ; Steroids ; Survival Rate ; Virus Diseases