Iron deficiency anemia is a common disorder during infancy and childhood. Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common disorder of mitochondrial fatty acid oxidation. However, this metabolic disorder is extremely rare among Asians. Generally MCAD deficiency patients are healthy until initial presentation of hypoketogenic hypoglycemia and encephalopathy which is predisposed by an intercurrent illness and/or a period of poor oral intake. The first attack causes a high risk of mortality or permanent neurologic sequelae. The authors report a suspect case of MCAD deficiency with iron deficiency anemia, with a brief review of related literatures.
Acyl-CoA Dehydrogenase* ; Anemia, Iron-Deficiency* ; Asian Continental Ancestry Group ; Humans ; Hypoglycemia ; Iron* ; Mortality

Fusarium species are common soil saprophytes and plant pathogens. In humans, several species have been recognized as agents of superficial infections. Disseminated Fusariosis have been increasingly described in immunocompromised patients, especially in neutropenic patients. The prognosis is very poor despites antifungal therapy. This is the report of Fusarium oxysporum infection in a 6-year-old patient with relapsed acute leukemia and prolonged neutropenia. The patient presented with persistent fever and multiple erythematous papules with central necrosis or vesicle. Fuasrium oxysporum was isolated and cultured from a skin biopsy specimen. Initially, the patient failed to respond to conventional amphotericin B but recovered after treatment was switched to liposomal amphotericin B and voriconazole.
Amphotericin B* ; Biopsy ; Child* ; Drug Therapy* ; Fever ; Fusariosis* ; Fusarium ; Humans ; Immunocompromised Host ; Leukemia ; Necrosis ; Neutropenia ; Plants ; Prognosis ; Skin ; Soil

Pleuropulmonary blastoma (PPB) is a rare primary malignant neoplasm with poor prognosis in children. PPB originates from the lung, the pleura, or the mediastinum. Histologically, it is characterized by a primitive, mixed blastematous, sarcomatous appearance and the absence of epithelial cell. Initial presenting symptoms are cough, fever and dyspnea. We experienced two cases of PPB (type I and type II). Complete surgical removal is always required for the treatment and chemotherapy and radiotherapy is needed as adjuvant therapy. We report two cases of pleuropulmonary blastoma treated with surgical removal, chemotherapy (vincristine, actinomycin D, cyclophosphamide) and radiotherapy.
Child* ; Cough ; Dactinomycin ; Drug Therapy ; Dyspnea ; Epithelial Cells ; Fever ; Humans ; Lung ; Mediastinum ; Pleura ; Prognosis ; Radiotherapy

PURPOSE: The aim of this study is to find out the role of HEPA filter equipped lamina air flow room reverse isolation for the treatment of anticancer drug induced febrile neutropenic patients. METHODS: Antibiotics and antifungal agents were promptly administered to twenty six patients with febrile neutropenic following chemotherapy from January 2003 to July 2005 at the Department of Pediatrics, Kyungpook National University Hospital, Daegu, Korea. And if possible, they were treated in the aseptic room. RESULTS: Nineteen patients recovered and seven patients died of infectious causes among twenty six patients. Fourteen patients had microbiologically defined infection, seven patients had clinically defined infection, and five patients had unexplained fever. The causes of infection were sepsis, pneumonia and urinary tract infection. The etiologic pathogens were Gram negative bacilli, 6 (42.9%) ; Gram positive cocci, 3 (21.4%) ; and fungus, 5 (35.7%). The dead group showed lower leukocyte count and higher CRP than the survived group. All dead patients showed disseminated intravascular coagulation syndrome (DIC). Nine of the ten patients (90%) who were treated in the aseptic room survived and ten of the sixteen patients (62.5%) in the general ward survived. CONCLUSION: The infection-related mortality of febrile neutropenic patients following chemotherapy is influenced by the early and adequate use of antimicrobial agents, DIC, neutrophil count and CRP. The isolation in the aseptic room increased the survival rate, but it was not statistically significant.
Anti-Bacterial Agents ; Anti-Infective Agents ; Antifungal Agents ; Dacarbazine ; Daegu ; Disseminated Intravascular Coagulation ; Drug Therapy ; Fever ; Fungi ; Gram-Positive Cocci ; Gyeongsangbuk-do ; Humans ; Korea ; Leukocyte Count ; Mortality ; Neutrophils ; Patients' Rooms ; Pediatrics ; Pneumonia ; Sepsis ; Survival Rate ; Urinary Tract Infections

PURPOSE: Discordance with expectation there are a few differences in hematopoietic stem cells according to their source. The purpose of this study is to compare the cryopreservative potential of hematopoiectic stem cell containing fractions between cord blood (CB) and mobilized peripheral blood (mPB) during long term cryopreservation. METHODS: Nineteen CB and seven mPB were frozen with a programed freezer and stored in liquid phase of nitrogen from 1 to 4 years. After thawing the viability of mononuclear cells (MNCs) and the recovery rate of MNC, CD34 positive cell, colony form unit-granulocyte/monocyte was measured by CD34 flow cytometry and colony formation in semisolid methycellulose culture. CD34 positive cell was purified from cryopreserved-thawed or fresh mPB using magnetic associated cell sorting (MACS) CD34 selection kit. RESULTS: Though there is no difference in the viability and the recovery rate of CFU-GM between cryopreserved-thawed CB and mPB, the recovery rate of MNCs and CD34 positive cells is much higher in CB. Cell aggregation during the thawing process of long term cryopreserved mPB was effectively prevented by using high viscosity thawing buffer like as 10% dextran and 20% human serum albumin. We can also purify the CD34 positive cells from the long term cryopreserved mPB in the purity of more than 90%. CONCLUSION: Contrary to mPB long term cryopreserved CB can maintain the hematopoietic stem cell fraction without considerable loss, so it can be clinically used for hematopoietic stem cell transplantation.
Cell Aggregation ; Cryopreservation ; Dextrans ; Fetal Blood* ; Flow Cytometry ; Granulocyte-Macrophage Progenitor Cells ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells* ; Humans ; Nitrogen ; Serum Albumin ; Stem Cells ; Viscosity

PURPOSE: Valproic acid (VPA) has been used as an anticonvulsant for a long time. Recently, there are many reports on VPA activity with regards to intracellular signal transduction, including differentiation, proliferation, and apoptosis. We experienced several hematologic toxicities during the long-term use of VPA. Therefore, we investigated whether VPA has effects on short-term or long-term hematopoiesis with respect to differing concentrations. METHODS: We obtained bone marrow mononuclear cells (BMMNC) from a 5 week old female C3H/He strain mouse. The BMMNC were cultured in semi-solid media mixed with VPA according to the concentrations of colony forming unit for granulocyte-monocytes (CFU-GM). The concentrations of VPA were used as follows: 0.01 mM, 0.1 mM, 1 mM, and 10 mM (therapeutic level: 0.07~1.1 mM). We performed long-term liquid culture under VPA to compare the frequency of long-term culture initiating cells (LTC-IC) according to various VPA levels. RESULTS: The number of CFU-GM was highest with 1 mM of VPA (45.2+/-13.5), with higher therapeutic level than control (25.7+/-11.9), in 0.01 mM of VPA (26.5+/-12.1) and in 0.1 mM of VPA (26.6+/-12.2). In 10 mM of VPA, a toxic level of VPA, was the lowest at 1.6+/-1.1 (P< 0.01). In long-term culture, the frequency of LTC-IC was increased in 0.1 mM of VPA (67.7+/-16.3%), lower therapeutic level than in control (5.5+/-10.6%). In 1 mM of VPA, the high therapeutic level decreased to 81.6+/-9.3%. With toxic levels of VPA, 10 mM, there was no hematopoiesis. CONCLUSION: The VPA might enhance short-term hematopoiesis at high therapeutic levels, while preserving LTC-IC in long-term hematopoiesis under low therapeutic concentrations. Therefore, we suggest that VPA to be used within a low therapeutic level to escape from hematopoietic suppression when using VPA as long-term medication for seizure control.
Animals ; Apoptosis ; Bone Marrow ; Female ; Granulocyte-Macrophage Progenitor Cells ; Hematopoiesis ; Humans ; Mice* ; Seizures ; Signal Transduction ; Stem Cells ; United Nations ; Valproic Acid*

PURPOSE: The use of greatly intensified anticancer drugs enhances the long-term survival of patients with childhood acute lymphoblastic leukemia (ALL). However, the intensified chemotherapy regimen commonly results in increased long-term morbidity. Dexamethasone, which is more potent than prednisone for its antileukemic effects, has been suspected to more vigorously provoke osteonecrosis (ON). We performed this study to investigate the prevalence and clinical features of ON in the high-risk ALL patients who were treated with high dose corticosteroids. METHODS: We investigated ON in 18 patients who had completed high-risk ALL chemotherapy (CCG-1882, regimen A). We compared the ALL patients with ON and the ALL patients without ON for the clinical features of ALL and for the factors that are related to the development of ON. In addition, the clinical features of ON were evaluated. RESULTS: For the entire study group, 3 (16.7%) of 18 patients had ON. The average age at the time of diagnosis of ON was 10.3 years compared with 6.0 years average age for the total enrolled patients. Among the three patients, 1 (33.3%) was male and 2 (66.7%) were female. The average time that passed from the initiation of chemotherapy to the diagnosis of ON was 23.7 months. A total of 7 joints were involved, with an average of 2.3 joints per patient diagnosed with ON. The affected joints were 3 (42.9%) hips and 4 (57.1%) ankles. CONCLUSION: The result of this study was similar to the previous reports. ON, which could be prevented and treated by decreasing the corticosteroid dose and with instituting early orthopedic intervention, is relatively commonly seen in the older children who are treated with high dose corticosteroid for ALL. In this regard, to minimize the risk of ON, newer chemotherapy protocols for older children should be administered with a reduced dose of dexamethasone.
Adrenal Cortex Hormones* ; Ankle ; Child ; Dexamethasone ; Diagnosis ; Drug Therapy* ; Female ; Hip ; Humans ; Joints ; Male ; Orthopedics ; Osteonecrosis* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prednisone ; Prevalence

PURPOSE: Intracranial atypical teratoid/rhabdoid tumor (ATRT) is an extremely rare and aggressive tumor of early childhood. In this study, we evaluated the clinical characteristics, therapeutic approaches and outcomes of children who were treated at Seoul National University Children's Hospital (SNUCH). METHODS: We reviewed the clinical records of seven patients who were diagnosed as ATRT at SNUCH between January 2000 and July 2005. RESULTS: Of the seven patients, three patients were male and four were female. Median age at diagnosis was 13 months ranging from 3 months to 67 months. The tumors occurred in the infratentorial area in six and at multiple sites in one patient. Metastatic disease at diagnosis was present in two patients. One showed cerebrospinal fluid (CSF) dissemination and the other showed bony metastasis. Tumor excision was performed in all patients, and with the exception of two cases that refused further treatment, five patients received postoperative chemotherpay. One patient with CSF dissemination received radiotherapy and intrathecal chemotherapy as well. Of all the patients who received chemotherapy, two patients died during treatment because of tumor progression. The chemotherapy regimen was changed in three patients during treatment because the tumor showed poor response to chemotherapy. The median length of follow-up for five patients receiving chemotherapy was 6 months and the overall survival (OS) and event free survival (EFS) were 33.3% and 0%, respectively. CONCLUSION: Though various therapeutic approaches have led to improved survival in ATRT, the prognosis of ATRT is dismal compared with other brain tumors. A precise pathologic diagnosis is crucial and intensified treatment modalities should be considered according to the extent of tumor. To establish optimal treatment guidelines, a cooperative prospective study is needed and the efficacy of individual regimens should be analyzed.
Brain Neoplasms ; Cerebrospinal Fluid ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Female ; Follow-Up Studies ; Humans ; Male ; Neoplasm Metastasis ; Prognosis ; Radiotherapy ; Retrospective Studies* ; Seoul

No abstract available.
Glioma*

No abstract available.
Brain Neoplasms* ; Brain* ; Child* ; Humans ; Infant*