No abstract available.
Encephalomyelitis, Acute Disseminated* ; Magnetic Resonance Imaging

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

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

We studied 600 normal lower extremities of Korean students of elementary school in order to establish normal values for the rotational profile and to define any differences between ethnic groups. Rotational profile examined at this study included medial and lateral rotation of the hip and thighfoot angle. The mean value of medial rotation of the hip was 48 degrees with a range of 35 to 64 degrees in male subjects, and 50 degrees with a range of 35 to 60 degrees in female subjects. The mean value of lateral rotation of the hip was 57 degrees with a range of 43 to 75 degrees, and 53 degrees with a range of 40 to 72 degrees in female subjects. Unlike medial rotation, lateral rotation of the hip was greater in male than in female subjects by a mean difference of 4 degrees(T-test, p<0.05). The mean value of thigh-foot angle was 10 degrees with a range of -2 to 23 degrees in male subjects, and 12 degrees with a range of 0 to 21 degrees in female subjects. The total range of hip rotation was slightly greater in Korean than in Caucasians or in Chinese, but, to conclude this, more large study by the same methods will be needed in other areas of Korea.
Asian Continental Ancestry Group ; Ethnic Groups ; Female ; Hip ; Humans ; Korea ; Lower Extremity* ; Male ; Reference Values

No abstract available.
Arthrogryposis*

No abstract available.
Sinusitis*

No abstract available.
Animals ; Macrophages, Peritoneal* ; Mice* ; Mycobacterium bovis*

The purpose of this study is to evaluate the morphologic findings of the labrum and capsuloligamentous structures in the normal and unstable shoulders and to compare the effectiveness of double contrast arthrographic findings which were taken with supine and prone position. We reviewed the computerized tomographic arthrogram in 9 cases of normal shoulder and l3 cases of shoulder instability. We evaluated the shape of the labrum, the attachment of anterior capsule and the amount of capsular laxity. Capsular laxity was compared each others in normal, the unidirectional instability and the multidirectional instability. Among these materials, we simultaneously performed CT arthrogram with supine and prone position and evaluated which is more reliable and accurate. Scapular inclination was ahout 25 degrees in the supine position and ahout 60 degrees in the prone position. Normal glenoid labrum varied in size and shape, so its lack of a classic appearance might not indicate a labral tear. Variations existed in the anterior capsular attachment onto the glenoid. The anterior capsule was used to attach far away from the glenoid rim. but it might not indicate an anterior instability. With the prone position, the labral lesion took more excellent visualization due to the contrast coating on the glenoid labrum in both anterior and posterior. Anterior capsular expansion was seen well with prone position in anterior instability. In summary, for proper interpretation of the instability, awareness of the morphologic variations of the labrum and capsuloligamentous structure is important. CT double contrast arthrography with prone position has made a significant contribution to improving and easing the documentation of variety of anatomic abnormalities. Now we recommend the prone position in CT arthrogram for imaging glenoid labrum pathology in shoulder instability.
Arthrography ; Pathology ; Prone Position ; Shoulder* ; Supine Position

We performed clinical and virological studies on 79 children with hand, foot and mouth disease (HFMD) who had been admitted or visited to the department of Pediatrics, Asan Medical Center from February to August, 1990. The results were as follows; 1) There were total of 79 cases with hand, foot and mouth disease during this period. The aseptic meningitis was combined in 18 cases (22.8%). 2) The male-to-female ratio of HFMD was 2.03 to 1, but that of aseptic meningitis was 8 to 1. 3) Hand, foot and mouth disease occurred more frequently in under the age of 5 years, and the aseptic meningitis in under the age of 1 year. 4) Hand, foot and mouth disease and the aseptic meningitis occurred mostly in May and June. 5) In addition to the skin manifestations characteristic in hand, foot and mouth disease, some of the patients with aseptic meningitis had the following symptoms: change of consiousness, seizure, monoparesis in the upper extremity, paraparesis in the lower extremity. 6) The causative agent of hand, foot and mouth disease associated with the aseptic meningitis in Seoul in 1990 seemed to be enterovirus 71 according to the serologic study.
Animals ; Child ; Chungcheongnam-do ; Enterovirus ; Foot-and-Mouth Disease ; Hand* ; Hand, Foot and Mouth Disease* ; Humans ; Lower Extremity ; Meningitis, Aseptic* ; Paraparesis ; Paresis ; Pediatrics ; Seizures ; Seoul* ; Skin Manifestations ; Upper Extremity

MELAS syndrome is a rare but distinct clinical entity belonging to a group of mitochondrial encephalomyopathies characterized by the tetrad of mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes. We experienced a case of MELAS syndrome in an 8 year-old boy who showed headache, pain of the eyeball, vomiting, stroke-like episodes such as visual disturbance and dysarthria, myoclonic seizure, confusion, and walking disturbance. His serum lactate level was elevated up to 48 mg/dl. MRI findings showed high signal intensities T2-weighted image and low signal intensities in T1-weighted image in the right thalamus and parietooccipital lobe and bilateral symmetric high signal intensity in T1-dweighted image in the basal ganglia. We have seen the dispersed ragged-red fibers with modified Gomori trichrome staining on light microscope, and abundant and dysmorphic mitochondria on electon microscope in the specimen of muscle biopsy. esis of SLE.
Basal Ganglia ; Biopsy ; Child ; Dysarthria ; Headache ; Humans ; Lactic Acid ; Magnetic Resonance Imaging ; Male ; MELAS Syndrome* ; Mitochondria ; Mitochondrial Encephalomyopathies ; Seizures ; Thalamus ; Vomiting ; Walking