No abstract available.

BACKGROUND: Hemophagocytic lymphohistiocytosis(HLH) is a rare and fatal disorder in children. Recently its clinical characteristics and etiology of uncontrolled activation of cellular immune system in genetically predirected patients have keen elucidated. The authors analyzed the clinical characteristics and response to immunosuppressive agents of HLH patients in single institute. METHODS: The authors retrospectively analyzed various clinical data including CSF and bone marrow examination at diagnosis and follow up period in the 6 patients who were diagnosed as HLH at Asan Medical Center during last 2 years. Antithymocyte globulin(ATG : 10 mg/kg/day) and methylprednisolone(methyl-PD: 5 mg/kg/day) for 5 consecutive days as induction treatment and cyclosporin A(CsA) as maintenance treatment after induction with weekly intrathecal methotrexate for 5-6 weeks were given to the recently diagnosed 3 patients. RESULTS: All the patients except one were infants. Persistent fever, hepatosplenomegaly and pancytopenia were observed in all the patients. Family history of suspicious HLH was observed in 4 patients(67%). The characteristic elevated serum triglyceride(TG) level and/or decreased fibrinogen level were noted in all. Mild to moderate CSF pleocytosls with increased lymphocytes and monocytes was also observed in all during the disease course. Variable degree of nonqr-Langerhans cell histiocytic infiltration and hemophagocytosis were observed in all the cases. Of the 3 patients treated with ATG and methyl-PD, one achieved complete remission and the others achieved partial remission within 2 weeks of treatment, but all expired after 5 months, remission with CsA. Regardless of treatment regimen, all the 6 patients expired due to CNS sequelae of HLH. CONCLUSIONS: HLH mainly developed in infants. Persistant fever, hepatosplenomegaly and pancytopenia were observed in most cases with the characteristic change of serum TG and/or fibrinogen level. HLH should be included in the differential diagnosis in patients with these features especially when the family history of suspicious HLH is present, and histologic comfirmation of HLH could be easily accomplished with bone marrow study in most cases. Remission induction of HLH could be achieved with immunosuppressive treatment but it was difficult to maintain long term remission.
Bone Marrow ; Bone Marrow Examination ; Child ; Chungcheongnam-do ; Cyclosporine ; Diagnosis ; Diagnosis, Differential ; Fever ; Fibrinogen ; Follow-Up Studies ; Humans ; Immune System ; Immunosuppressive Agents ; Infant ; Lymphocytes ; Lymphohistiocytosis, Hemophagocytic* ; Methotrexate ; Monocytes ; Pancytopenia ; Remission Induction ; Retrospective Studies

BACKGROUND: Despite the development of empiric antibiotics in treatment of neutropenic fever(NF) in pediatric cancer patients, bacterial infection is still the most important cause of death in these patients. In this study the authors analyzed clinical characteristics and tried to find out the possible risk factors of the pediatric cancer patients who succumbed to the documented bacterial infection after episodes of neutropenic fever. METHODS: 17 pediatric cancer patients expired after episodes of neutropenic fever(NF) in the pediatric department of Asan Medical Center from Mar. 1990 to Feb. 1996 were grouped by the results of bacterial culture. 7 cases (Group A) had documented bacterial culture results and 10 cases (Group B) had negative culture results. The clinical characteristics of these two groups were analyzed retrospectively. RESULTS: There were no differences in the types of cancer between the two groups. All the documented bacteria were gram negative organisms and all cultured from the blood. There were no differences between the two groups in the treatment duration, known risk factors of infection before and at the onset of fever, antibiotics administered, and interval from the onset of NF to the administration of antibiotics. The response rate of initial empiric antibiotics was lower in group A(14%) compared to Group B(40%), even though all the cultured organisms except 2 cases in Group A were sensitive to initial empirical antibiotics. Septic shock was the cause of death in 86% of Group A patients, but only in 10% of Group B patients. The other causes of death were progression of cancer, bleeding, hepatic failure, adult respiratory distress syndrome and multiple organ failure, which showed no difference between the 2 groups. The interval from the onset of fever to death was significantly shorter in Group A(6.2+/-2.3 days) compared to Group B(24.9+/-18.6 days). CONCLUSIONS: Bacterial infection is still the most frequent cause of death after NF. Most patients succumbed to the documented bacterial infection showed rapid progression of bacteremia to septic shock despite administration of sensitive antibiotics. The known risk factors of infection before the onset of NF and other treatment factors shows no differences between the two groups in this study. These results suggest that the bacteremia is obvious risk factor of poor outcome after NF episode, and antibiotics alone may be insufficient to prevent the rapid progression of septic shock in these patients.
Anti-Bacterial Agents ; Bacteremia ; Bacteria ; Bacterial Infections ; Cause of Death* ; Chungcheongnam-do ; Fever* ; Hemorrhage ; Humans ; Liver Failure ; Multiple Organ Failure ; Respiratory Distress Syndrome, Adult ; Retrospective Studies ; Risk Factors ; Shock, Septic

No abstract available.

PURPOSE: To describe soft-tissue radiographic and high-resolution CT findings of radiation-induced lung injury of rabbit over time and to correlate them with pathologic findings. MATERIALS AND METHODS: 15 rabbits were irradiated in the right lung with one fraction of 2000 cGy. After 4, 6, 12, 20, 24 weeks, 3 rabbits in each group were sacrificed and soft-tissue radiographs and high-resolution CT of their lung tissue were obtained. Radiological findings were correlated with pathologic findings. RESULTS: On soft- tissue radiogram, radiation pneumonitis shown as consolidation with air-bronchogram occurred in 3 cases after 6 weeks, and in 1 case after 12 weeks of irradiation. In addition, pneumonic consolidation with adjacent pleural contraction was seen in 2 cases after 12 weeks of irradiation. Fibrotic changes indicated by decreased volume occurred after 20 weeks and combined bronchiectatic change and bronchial wall thickening appeared after 20 weeks(N=1), and 24 weeks(N=3). HRCT findings of radiation pneumonitis were homogeneous, increased attenuation after 4 weeks(N=3), 6 and 12 weeks (each N=I), patchy consolidation after 6 and 12 weeks(each N=2), discrete consolidation after 12, 20 and 24 weeks(each N=I) and solid consolidation after 20 and 24 weeks(each N=2). Pathologically radiation pneumonitis and pulmonary congestion were seen after 4 and 6 weeks. After 6 weeks, collagen and reticulin fibers were detected along alveolar wall. Mixed radiation pneumonitis and fibrosis were detected after 12 weeks. 20 weeks after irradiation, fibrosis was well defined in interstitium and in 24 weeks, decreased number of alveoli and thickening of bronchial wall were defined. CONCLUSION: Radiation pneumonitis was provoked 4 weeks after irradiation on rabbit lung and progressed into radiation fibrosis 20 weeks after irradiation on soft-tissue radiographs and high-resolution CT. High-resolution CT is more precise in detecting early radiation pneumonitis and detailed pathologic findings.
Collagen ; Estrogens, Conjugated (USP) ; Fibrosis ; Lung Injury ; Lung* ; Rabbits ; Radiation Pneumonitis ; Reticulin

Forty cases of infiltrating duct carcinoma of the breast were examined immunohistochemically for expression of TGF-beta and angiogenesis in order to analyze significant correlation with prognostic parameters including tumor size, axillary lymph node metastasis, clinical stage, histologic grade, estrogen receptor and progesterone receptor status. The TGF-beta expression was observed in tumors center and advancing edges of tumors. To determine microvessel density for angiogenesis, we stained endothelial cells for Factor VIII related antigen and counted microvessel within tumor. The results were as follows: 1) The strong immunohistochemical expression of TGF-beta and higher counts of microvessels were observed in advancing edges of tumors (p<0.05). 2) The TGF-beta expression in the advancing edges of tumors was closely related to clinical stage and presence of axillary lymph node metastasis (p<0.05). 3) The mean microvessel counts were significantly higher in tumors from patients with axillary lymph node metastasis and increased with increasing clinical stage (p<0.05). 4) The TGF-beta expression was not related to histologic grade, estrogen receptor and progesterone receptor status(p>0.05). Therefore, the results suggested that the TGF-beta expression and angiogenesis in infiltrating duct carcinoma of the breast may play an important part in prognostic factors, closely related to the lymph node metastasis and clinical stage.
Neoplasm Metastasis

No abstract available.
Disabled Persons* ; Female ; Humans ; Menarche*

No abstract available.
Panniculitis*

Down syndrome is the most common autosomal chromosomal abnormality characterized by mental and growth retardation, and by various typical features including prominent epicanthal fold, oblique palpebral fissure, flat nasal bridge, short and broad hand, wide toe interspace, etc. The overall incidence has been shown to be 1:800 deliveries, increasing with advancing maternal age. However, twin cases are extremely rare, and thus far only 500 cases were reported worldwide. We have recently observed 10-day-old male monozygotic twins with Down syndrome, born to a mother of 30 years of age with one normal child. Both have VSD confirmed by 2D-echocardiography, in addition to various typical features. Cytogenetic examination revealed that both have karyotypes of 47, XY, +21. This is the first report in Korea as the authors are aware of.
Child ; Chromosome Aberrations ; Cytogenetics ; Down Syndrome ; Hand ; Humans ; Incidence ; Incontinentia Pigmenti* ; Karyotype ; Korea ; Male ; Maternal Age ; Mothers ; Toes ; Twins, Monozygotic

BACKGROUND: Total intravenous anesthesia(TIVA) with various kinds of sedatives and synthetic analgesics has many advantages such as no air pollution, no hepatic or renal toxicity and good postoperative pain relief compared with inhalation anesthesia. METHODS: TIVA with fentanyl and propofol was applied to the 25 patients in ASA class I and II. The changes of hemodynamics, arterial blood gas, blood sugar level, hepatorenal function and complications were evaluated perioperatively. RESULTS: Systolic blood pressure and mean arterial pressure decreased after induction, but intraoperatively systolic blood pressure became preoperative value while mean arterial pressure increased compared with preoperative value. Heart rate increased after induction and intraoperatively. At recovery room there were no clinically significant changes in the data of arterial blood gas. The blood sugar level slightly increased during postoperative period. There were no clinically significant changes in hepatic or renal function test of postoperative 3rd day compared with preoperative one. The chief complications of TIVA during anesthesia were pain on injection site and slight movement, while nausea or vomiting, dizziness and urinary retention were the major complications at postoperative period. CONCLUSIONS: TIVA with fentanyl and propofol is considered to have good controllability and it can be applied as one of the general anesthesia methods in the case of contraindications to N2O and volatile anesthetics, but further study will be required to quantify the appropriate dosage of propofol or fentanyl to minimize perioperative hemodynamic change and respiratory depression.
Air Pollution ; Analgesics ; Anesthesia ; Anesthesia, General ; Anesthesia, Inhalation ; Anesthesia, Intravenous* ; Anesthetics ; Arterial Pressure ; Blood Glucose ; Blood Pressure ; Dizziness ; Fentanyl* ; Heart Rate ; Hemodynamics ; Humans ; Hypnotics and Sedatives ; Nausea ; Pain, Postoperative ; Postoperative Period ; Propofol* ; Recovery Room ; Respiratory Insufficiency ; Urinary Retention ; Vomiting