Essential thrombocythemia in childhood is a rare clonal myeloproliferative disorder in the multipotent stem cell origin and is associated with an increased risk of thrombohemorrhagic complications. The one of diagnostic criteria is a platelet count of more than 600,000/mm3. We diagnosed this disease in 8 year old boy incidentally and treated with hydroxyurea. We report a case of essential thrombocythemia to summarize the current trends in the diagnosis and management with a brief review of related literatures.
Child ; Diagnosis ; Humans ; Hydroxyurea ; Male ; Multipotent Stem Cells ; Myeloproliferative Disorders ; Platelet Count ; Thrombocythemia, Essential*

PURPOSE: Abnormality in the composition of lipid metabolism is well known to be a main cause of atherosclerosis. Accordingly the abnormality in lipid metabolism after suffering from Kawasaki disease may lead to premature coronary atherosclerosis. The aim of this study is to investigate the abnormalities of lipid metabolism in patients with Kawasaki disease. We studied 67 patients with Kawasaki disease. METHODS: We serially measured total cholesterol, HDL-cholesterol, and triglyceride(period 1 : at admission, period 2 : 24-48 hours after the defervescence, period 3 : 2-3 weeks after the onset of disease, period 4 : 2-3 months after the onset of disease, period 5 : 6 months after the onset of disease, period 6 : 1 year after the onset of disease, period 7 : 2 years after the onset of disease). RESULTS: In patients with Kawasaki disease, HDL was relatively low(P=0.07) in the acute stage (period 2) compared with control group, but recovered in the subacute stage(period 3) and stayed the same level. Total cholesterol was low(P=0.02) in the acute stage(period 1) compared with control group, but recovered in the subacute stage(period 3) and stayed at the same level. CONCLUSION: Kawasaki disease, a systemic vasculitis of unknown etiology, may cause abnormalities in lipid metabolism, especially at the acute stage by affecting endothelial metabolism. To understand the long-term effect of this metabolic abnormalities, we have to study more patients for a longer period of time.
Atherosclerosis ; Cholesterol ; Coronary Artery Disease ; Humans ; Lipid Metabolism ; Metabolism ; Mucocutaneous Lymph Node Syndrome* ; Systemic Vasculitis

PURPOSE: Anemia frequently develops during intensive chemotherapy in childhood cancer patients and requires transfusion. But transfusions are restricted because of many side effects. We prospectively evaluated the efficacy of the recombinant human erythropoietin (rhEPO) in childhood cancer patients who receiving chemotherapy. The safety was also evaluated. METHPDS: rhEPO was administered subcutaneously, 150 U/kg/day, 3 times a week for 12 consecutive weeks in 19 childhood cancer patients from June, 1999 to March, 2000 in the Department of Pediatrics, Kyungpook National University Hospital, Taegu, Korea. The mean age of rhEPO group was 5.2 (1~13) years and the control group was 6.9 (1~13) years. There were 9 hematologic cancers and 10 solid cancers in rhEPO group and there were 17 hematologic cancers and 16 solid cancers in control group. Serum erythropoietin level was measured before treatment and hemoglobin weekly. Packed red cell transfusion was performed in patients with hemoglobin less than 8 g/dL. The safety and adverse reactions (hypertension, headache, facial flushing, redness and pain in injection site, etc) was assessed in rhEPO group. The mean duration of follow up was 24 (6~40) weeks. RESULTS: The baseline hemoglobin (rhEPO vs control:10.1 vs 10.2 g/dL) and serum erythropoietin levels (rhEPO vs control:182.82 vs 133.92 mU/mL) were similar in two groups. The mean hemoglobin level was statistically higher in rhEPO group (11.4 g/dL) than the control (10.1 g/dL) from 5 weeks after treatment (P=0.025). These statistically different levels were sustained until week 13 (rhEPO vs control: 11.6 vs 10.3 g/dL, P=0.037). The mean transfusion requirements per patient in rhEPO group (4.2 times) were less than control (5.9 times) during the follow up periods, but this was not statistically significant. No serious adverse reactions were observed after the administration of rhEPO. CONCLUSION: rhEPO is expensive, but is a safe and effective means of increasing hemoglobin level and reducing blood requirements in children with cancer receiving intensive chemotherapy.
Anemia* ; Child ; Daegu ; Drug Therapy* ; Erythropoietin* ; Flushing ; Follow-Up Studies ; Gyeongsangbuk-do ; Headache ; Humans* ; Korea ; Pediatrics ; Prospective Studies

PURPOSE: Pneumonia is rather common and benign disease in children but its course is various. Many clinicians used the empirical antibiotics to treat pneumonia without identification of causative organism. This study was performed to find the pathogenic organism from the fluid culture by bronchoscopy with BAL (bronchoalveolar lavage) in severe pneumonia patients. METHODS: We studied 21 cases (male 15, female 6) who were admitted with severe pneumonia in the Department of Pediatrics, Sunlin Hospital from March to October in 1999. These patients had no underlying disease such as immunologic deficiency. We took laboratory tests including CBC, CRP, ESR, PB smear, mycoplasmal antibody and blood culture at admission day. We performed bronchoscopy with BAL, and wet smear and culture of that fluid. RESULTS: Organisms were cultured in nineteen cases out of 21 cases. Seven cases of Streptococcus mitis, five of Stenotrphomonas maltophilia, five cases of Streptococcus oralis, two of Moraxella species, two of Acinetobacter junii, one of Acinetobacter spesies, one Staphylococcus hominus, one alpha-h-Streptococcus, one Klebsiella pneumoniae, one Pseudomonas aeruginosa, one Enterobacter cloacae. Two organisms were cultured in nine cases. CONCLUSION: The positive rate of BALF culture was very high (90.5%). But, further studies are necessary for the patients with severe pneumonia preceded the use of antibiotics.
Acinetobacter ; Anti-Bacterial Agents ; Bronchoalveolar Lavage* ; Bronchoscopy ; Child ; Enterobacter cloacae ; Female ; Humans ; Klebsiella pneumoniae ; Moraxella ; Pediatrics ; Pneumonia* ; Pseudomonas aeruginosa ; Staphylococcus ; Streptococcus mitis ; Streptococcus oralis

PURPOSE: Tuberculosis may cause a serious complication in children with cancer who are receiving the chemotherapy. But its diagnosis is not easy if we do not suspect the disease in patients with uncontrolled persistent fever. We studied retrospectively the importances of prevention and early diagnosis of tuberculosis in cancer patients. METHPDS: Twelve patients were diagnosed as having tuberculosis during cancer chemotherapy in Kyungpook National University Hospital from May, 1981 to May, 1998. We reviewed their clinical features, diagnostic methods, treatment and prevention. RESULTS: The median age of the patients was 14 (2~18) years. The underlying diseases were seven acute lymphoblastic leukemia (ALL), two acute undifferentiated leukemia (AUL), one acute nonlymphoblastic leukemia (ANLL), one mixed-lineage leukemia, and one Burkitt's lymphoma. The disease categories of tuberculosis were seven pulmonary tuberculosis, two tuberculous pleurisy, one miliary tuberculosis, one bone and endotracheal tuberculosis and one tuberculous meningitis. The family history of tuberculosis is positive in one case. The clues of the suspicion of tuberculosis infections were 9 cases of persistent fever despite broad spectrum of antibiotics and/or antifungal agent therapy, 2 chronic cough and 1 chest pain. We could diagnose four by AFB culture, three cases by AFB smear, two by polymerase chain reaction (PCR), one by pleural biopsy, one by transbronchial lung biopsy and one by chest X-ray and CSF study. We treated pulmonary tuberculosis and tuberculous pleurisy by triple therapy (isoniazid, rifampin, pyrazinamide) and miliary, bone, endotracheal tuberculosis and tuberculous meningitis by quadriple therapy (isoniazid, rifampin, pyrazinamide, streptomycin or kanamycin). The mean duration of defervescence after treatment was 15.4 days. One died of fulminant hepatitis probably by hepatitis B after completion of cancer chemotherapy, one died of adult respiratory distress syndrome, two died of DIC, three died of relapse of underlying disease, but no one died of tuberculosis infection itself. CONCLUSION: The early diagnosis of tuberculosis is an important factor for decreasing the mortality rates of cancer patients, so we should have a suspicion of this disease in patients with persistent fever in spite of appropriate antibiotic and antifungal agents. Isoniazid prophylaxis may be needed in childhood cancer patients with chemotherapy in Korea.
Anti-Bacterial Agents ; Antifungal Agents ; Biopsy ; Burkitt Lymphoma ; Chest Pain ; Child ; Cough ; Dacarbazine ; Diagnosis ; Drug Therapy* ; Early Diagnosis ; Fever ; Gyeongsangbuk-do ; Hepatitis ; Hepatitis B ; Humans ; Isoniazid ; Korea ; Leukemia ; Leukemia, Myeloid, Acute ; Lung ; Mortality ; Polymerase Chain Reaction ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Pyrazinamide ; Recurrence ; Respiratory Distress Syndrome, Adult ; Retrospective Studies ; Rifampin ; Streptomycin ; Thorax ; Tuberculosis* ; Tuberculosis, Meningeal ; Tuberculosis, Miliary ; Tuberculosis, Pleural ; Tuberculosis, Pulmonary