No abstract available.
Brain Neoplasms* ; Brain*

Improved treatment strategies and better supportive care have resulted in increased survival rates for childhood cancers. However, most of the survivors may have complex, long-term health issues. In 2004, Childhood Cancer Survivorship Study of the United States confirmed that both survivors and the medical community need to be educated about the late effects of childhood cancer treatment. Korea, with an estimated number of childhood cancer survivors of 20,000 to 25,000, faces similar challenges that the United States had experienced earlier. Despite of the tight budgetary situation on the part of government and hospitals, nationwide cohort study for Korean childhood cancer survivors was proposed and the versions of instruments to measure the quality of life of childhood cancer survivors were already validated. Nationwide registry of long-term survivors as well as the multi-center study is to be developed not only for the care of the survivors but also to raise the patient's awareness of the importance of regular follow-up. In addition to education of primary care providers regarding the survivorship, the need for special education program in the school system is emphasized.
Cohort Studies ; Education, Special ; Follow-Up Studies ; Humans ; Korea ; Primary Health Care ; Quality of Life ; Survival Rate ; Survivors ; United States

Posttransplant lymphoproliferative disease(PTLD) has emerged as a potential life-threatening complication of immunosuppressive therapy after organ transplantation. The occurrence of PTLD is usually associated with an Epstein-Barr virus(EBV) infection in patients who are treated by aggressive immunosuppressive therapy. PTLD is represented by diverse manifestations ranging from reactive lymphoid hyperplasia to high grade malignant lymphoma. This is a case report of a late PTLD in a child. The patient is a 14-year-old girl, who presented as malignant lymphoma 44 months after successful renal transplantation. There was no evidence of EBV infection. On bone marrow study, many neoplastic lymphoid cells were detected. Aggressive chemotherapy for PTLD had resulted in clinical remission. However the patient expired from uncontrolled sepsis and septic shock after 77 days.
Adolescent ; Bone Marrow ; Child* ; Drug Therapy ; Epstein-Barr Virus Infections ; Female ; Herpesvirus 4, Human ; Humans ; Kidney Transplantation* ; Lymphocytes ; Lymphoma ; Organ Transplantation ; Pseudolymphoma ; Sepsis ; Shock, Septic ; Transplants

We report two cases of atypical teratoid/rhabdoid tumor(AT/RT) in the central nervous system. Primary central nervous system AT/RT is an extremely rare malignant tumor affecting infants and young children. These tumors have been diagnosed previously as primitive neuroectodermal tumors(PNETs) because AT/RT contains fields indistinguishable from classic PNETs. Separation of these two tumor types is crucial because the prognosis for AT/RT is poor even when treatment includes surgery with or without radiation therapy and/or chemotherapy. Clinical, radiological and histopathological features of AT/RT are presented.
Central Nervous System* ; Child ; Drug Therapy ; Humans ; Infant ; Neural Plate ; Neuroectodermal Tumors, Primitive ; Prognosis

Kaposiform hemangio-lymphangiomatosis is an extremely rare and locally aggressive vascular neoplasm. This neoplasm histologically shows dilated vascular spaces lined by flat endothelium-like cells and areas of spindle cells forming slit-like vascular spaces similar to those described in Kaposi's sarcoma. We report a case of this neoplasm which originated from the mediastinum infiltrating other adjacent soft tissues such as thymus and pulmonary interstitium without the evidence of distant metastasis. In spite of interferon-alpha2b therapy and excision of this neoplasm, the patient expired due to respiratory failure caused by aggravating interstitial pneumonia and progression of the neoplasm.
Hemangioma ; Humans ; Lung Diseases, Interstitial ; Lymphangioma ; Mediastinum ; Neoplasm Metastasis ; Respiratory Insufficiency ; Sarcoma, Kaposi ; Thymus Gland ; Vascular Neoplasms

No abstract available.
Glioma*

We describe a girl with Diamond-Blackfan anemia with accompanying red cell enolase deficiency. At the age of 9 yr old, the patient received allogeneic bone marrow transplantation from her HLA-identical sister who had normal red cell enolase activity. While the post transplant DNA analysis with short tandem repeat has continuously demonstrated a stable mixed chimerism on follow-up, the patient remains transfusion independent and continues to show a steady increase in red cell enolase activity for over two and a half years following bone marrow transplantation.
*Anemia, Diamond-Blackfan/blood/enzymology/surgery ; Bone Marrow Cells/cytology/metabolism ; *Bone Marrow Transplantation ; Child ; Child, Preschool ; Erythrocytes/*enzymology ; Female ; Humans ; Infant ; Phosphopyruvate Hydratase/genetics/*metabolism ; *Transplantation, Homologous

PURPOSE: This study was undertaken to investigate the clinical characteristics and prognostic predictors of myelodysplastic syndrome (MDS) in childhood. Method: The characteristics and laboratory findings of 20 patients seen at Asan Medical Center for the past 10 years from September 1989 to August 1998 were reviewed retrospectively with regard to the new International Prognostic Scoring System (IPSS) proposed by International MDS Risk Analysis Workshop. RESULTS: 1) In 20 children with MDS we studied, there was no age or sex predilection unique to the subgroups of MDS. 2) 19 cases (95%) out of the 20 had pallor at the time of diagnosis. Other major clinical findings were bleeding tendency in 11 (55%), fever in 8 (40%), hepatosplenomegaly in 8 (40%), and lymphadenopathy in 3 (15%). 3) The hemoglobin level was less than 10 g/dL in all cases and absolute neutrophil count (ANC) was decreased in 11 cases, thrombocytopenia in 15 cases. Pancytopenia was noted in 8 cases (40%). 4) Of the 20 cases, 9 had refractory anemia (RA), 3 refractory anemia with excess blasts (RAEB), 3 refractory anemia with excess blasts in transformation (RAEBIT), and 5 juvenile chronic myelogenous leukemia (JCML). 5) All RA patients were Intermediate (INT)-1 risk group, and all RAEB children were INT-2 risk group. The 3 cases of RAEBIT fell into INT-1, INT-2, and high risk group. Three cases of JCML were INT-1 group, and 2 cases INT-2 group. 6) Seven cases out of 13 INT-1 group had mean survival of 20.2 month (6~57 month), but only 1 out of 6 INT-2 survived. One case of high risk group succumbed to disease 50 months after diagnosis. CONCLUSION: These results showed that there was no age or sex predilection for the specific subgroup of childhood MDS. All the FAB subtypes of the childhood MDS except RA subgroup had poor survival. In this study, we found the IPSS seemed to be a prognostic predictor in childhood MDS but more cases are needed to confirm the validity of IPSS.
Anemia, Refractory ; Anemia, Refractory, with Excess of Blasts ; Child ; Chungcheongnam-do ; Diagnosis ; Education ; Fever ; Hemorrhage ; Humans ; Leukemia, Myelomonocytic, Juvenile ; Lymphatic Diseases ; Myelodysplastic Syndromes* ; Neutrophils ; Pallor ; Pancytopenia ; Prognosis ; Retrospective Studies ; Thrombocytopenia

PURPOSE: The aim of this study was to investigate the clinical and laboratory findings of hereditary spherocytosis comparing those of different age groups. METHODS: The clinical and laboratory findings of hereditary spherocytosis from June 1989 to August 1998 at Asan Medical Center were analyzed retrospectively according to two different age groups, Group I (9 patients diagnosed under 10 years of age) and Group II (19 patients diagnosed at or over 10 years of age). RESULTS: 1) Mean age at diagnosis was 2.4+/-1.97 and 28.2+/-18.81 years, and family history was positive in 44% and 47% in Group I and II patients respectively. 2) Splenectomy was carried out in 33% and 79% of Group I and II patients respectively, and accessory spleen was found in 100% and 20% of splenectomized patients respectively. 3) Gallstone was found in 11% and 42% of Group I and II patients respectively, and aplastic crisis developed in 0% and 10% respectively. 4) Post-splenectomy hematological parameters improved as follows: Group I; from hemoglobin at diagnosis of 8.5+/-3.59 g/dL to post-splenectomy level of 12.6+/-0.86 g/dL, hematocrit 24.5+/-10.25% to 38.1+/-4.86%, corrected reticulocyte 9.0+/-4.16% to 1.2+/-0.84%, total bilirubin 3.2+/-1.53 mg/dL to 2.2+/-1.34 mg/dL. Group II ; from hemoglobin at diagnosis of 8.9+/-2.95 g/dL to post-splenectomy level of 12.6+/-1.27 g/dL, hematocrit 24.9+/-7.85% to 37.4+/-2.89%, corrected reticulocyte 4.8+/-2.74% to 2.0+/-1.12%, total bilirubin 5.2+/-5.05 mg/dL to 1.1+/-0.49 mg/dL. CONCLUSION: There were no age related differences in hematologic findings at diagnosis and many of the patients with milder form of the disease could be detected later in adult life. The frequency of gallstone was up to 42% in patients whose diagnosis was delayed after 10 years of age, and aplastic crisis was a rare complication. Splenectomy was an effective treatment leading to normal hemoglobin concentrations in all patients. Accessory spleen was found in 33% of splenectomized patients, which emphasizes the necessity of spleen scan before splenectomy.
Adult ; Bilirubin ; Chungcheongnam-do ; Diagnosis ; Gallstones ; Hematocrit ; Humans ; Reticulocytes ; Retrospective Studies ; Spleen ; Splenectomy

PURPOSE: The aim of this study was to review the clinical characteristics and treatment outcome of pancreatitis developed in 19 children with leukemia and lymphoma in Asan Medical Center. METHODS: Hospital and outpatient records of 19 children either with leukemia or lymphoma who developed acute pancreatitis were reviewed. Clinical characteristics of these patients along with serologic data were analysed. RESULTS: 1. Median age at diagnosis of pancreatitis in 19 patients was 11 years of age. 2. Patients had acute lymphocytic leukemia (12 cases; 53%), acute myelocytic leukemia (4 cases; 21%), non-Hodgkins lymphoma (3 cases; 16%). 3. The etiologies of pancreatitis were L-asparaginase (16 cases) therapy, continuous Ara-C therapy (2 cases) and gallbladder stone (1 case). 5. L-asparaginase realated pancreatitis developed during the course of CCG 1882 induction (7 cases), CCG 1901 onsolidation (4 cases), CCG 1901 induction (1 case), and ADCOMP induction (1 case). 6. All patients experienced abdomial pain. Nausea, fever, vomiting, abdominal distention and diarrhea were also manifested clinically. 7. Hypocalcemia, sepsis, ascites, hyperglycemia, diabetic ketoacidosis, pancreatic pseudocysts and fistula were complicating events. 8. 6 patients were dead. The causes of death were from progression of lymphoma/ leukemia itself in 5 cases. One patient died of regimen related toxicity. The period of follow-up ranged from 2 months to 6.6 years with median follow-up of 28 months. CONCLUSION: 1. It is neccessary to monitor the level of serum amylase and lipase or to perform radiologic evaluation in patients who develop abdominal pain during L-asparaginase and Ara-C therapy especially in the course of CCG 1882 induction and CCG 1901 consolidation. 2. The outcome of chemotherapy induced pancreatitis is favorable in most instances but in some patients chronic pancreatitis may remain. The delay of chemotherapy due to pancreatitis may be responsible for the relapse of cancer. Therefore, prompt diagnosis and aggressive supportive therapy are important.
Abdominal Pain ; Amylases ; Ascites ; Cause of Death ; Child* ; Chungcheongnam-do ; Cytarabine ; Diabetic Ketoacidosis ; Diagnosis ; Diarrhea ; Drug Therapy ; Fever ; Fistula ; Follow-Up Studies ; Gallbladder ; Humans ; Hyperglycemia ; Hypocalcemia ; Leukemia* ; Leukemia, Myeloid, Acute ; Lipase ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Nausea ; Outpatients ; Pancreatic Pseudocyst ; Pancreatitis* ; Pancreatitis, Chronic ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Recurrence ; Sepsis ; Treatment Outcome ; Vomiting