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.
Pregnancy*

When a soft tissue mass in the bony wall of the paranasal sinus is present, it is difficult to make a distinction between tumor and inflammatory mass on CT. Fungal sinusitis may have soft tissue attenuation on the bony wall of the sinus, bony sclerosis, focal bony destruction, and calcific area on CT. This is a report of four proven cases of fungal sinusitis, asperogillosis in 3 cases and mucormycosis in 1 case, All 4 patients had CT and one patient had MRI, On CT, bony sclerosis and destruction were well visualized in all cases. On MRI, mycetoma in the maxillary sinus was hypointense on T1 weighted images and more hypointense on T2 weighted images. Although CT appears to be the best modality for initial examination of the patient with sinusitis, the differentiation of fungal sinusitis from tumor mass or other entity may be better accomplished with MRI.
Humans ; Magnetic Resonance Imaging ; Maxillary Sinus ; Mucormycosis ; Mycetoma ; Sclerosis ; Sinusitis

Nonunion is one of the important complication during treatment of forearm fracture. Nonunion of the forearm fracture inevitably prolongs the treatment course and markedly impairs the function of the forearm. We analyzed 34 cases of nonunion of forearm fracture in 21 persons from Jan. 1979 to Dec. 1985. The results are as follows. 1. The greater force of traffic accident of machine injury produced nonunion more frequently. 2. The predilection site of the nonunion was coincidental to the fracture site of the forearm. 3. Unstable fracture such as comminuted, segmental, spiral or oblique fracture was more closely related to the nonunion than the stable fracture. Hypervascular type nonunion was more prevalent than the avascular type. 4. The probable causes of nonunion were poor external fixation, severe soft tissue injury, displaced or comminuted fracture and inadequate or inappropriate surgery. 5. Obtained bone union in all cases, but the functional result was not improved compared to the preoperative state.
Accidents, Traffic ; Clinical Study ; Forearm ; Fractures, Comminuted ; Humans ; Soft Tissue Injuries

No abstract available.
Varicose Veins

Angiographic findings were analysed in 22 cases of coronary occlusive disease which were selected from 50 cases of coronary arteriographies done in Department of Radiology, Seoul National Un iversity Hospital from September, 1981 to August, 1984. There were 16 cases of single vessel disease, in which 12 were LAD involvement and 4 were RCA involvement, 2 cases of two vessel and 4 cases of three vessel disease. Site, degree and extent of stenotic involvement in those occlusive coronary diseases were anal ysed with review of literature for selective coronaryarteriography. Since coronary arteriography is the final and accurate examination for the evaluation of occlusive coronary arterial disease, it is expected to be widely used for the diagnosis of occlusive coronary arterial disease which is rapidly increasing recently in this country.
Angiography ; Coronary Disease ; Diagnosis ; Seoul ; United Nations

PURPOSE: We report a case of cortical blindness due to transient cerebral ischemia associated with general anesthesia. METHODS: A 45 year-old woman complained of bilateral visual loss after emergent incision and drainage for the treatment of extensive pyogenic abscess around the left mandibular space causing severe respiratory distress, and transient cerebral ischemia due to difficult tracheal intubation. At first ophthalmologic examination, 8 days after the operation, visual acuity was hand movement. Functions of external ocular muscles, blinking reflex, accommodation, pupillary light reflex, color vision test, intraocular pressure, and the findings of anterior segment, optic nerve and fundus were normal. Humphrey visual field examination showed nonspecific visual field defect, and pattern visual evoked potential examiantion showed flattening of waveforms. Both brain magnetic resonance imaging and electroencephalography were normal. However, brain single positron emission computerized tomography demonstrated general decrease of blood perfusion in the cerebrum including the occipital lobes. RESULTS: Without any specific treatment, her vision improved gradually, and 6 weeks after operation, it was 0.4 in the right and 0.7 in the left eye. After 3 months, it was 0.9, and no other abnormal ocular findings were noted on both eyes. CONCLUSIONS: Cortical blindness can occur by transient cerebral ischemia associated with general anesthesia, and its visual prognosis may be good.
Abscess ; Anesthesia, General* ; Blindness, Cortical* ; Blinking ; Brain ; Cerebrum ; Color Vision ; Drainage ; Electroencephalography ; Electrons ; Evoked Potentials, Visual ; Female ; Hand ; Humans ; Intraocular Pressure ; Intubation ; Ischemic Attack, Transient ; Magnetic Resonance Imaging ; Middle Aged ; Muscles ; Occipital Lobe ; Optic Nerve ; Perfusion ; Prognosis ; Reflex ; Visual Acuity ; Visual Fields

BACKGROUND: The concept of myocardial injury after coronary occlusion is changing in recent years. Brief episode of ischemial induces reversible myocardial injury and repeated brief ischemic insults might cause myocardial necrosis due to cummulative damages. Recent observations showed that brief episodes of ischemia have protective effects on the myocardium increasing the myocardial tolerance to a subsequent sustained ischemic insult. This phenomenon is termed ischemic preconditioning and can be noticed after a variety of protocols in multiple species of experimental animals. This study was planned to 1) measure the changes of hemodynamic parameters and the ischemic damage of insulted myocardium during ischemic preconditioning, and 2) compare the infarct sizes with or without preconditioning. METHODS: Using canine model of a single 90 minutes' occlusion of left anterior descending coronary artery and 240 minutes' reperfusion, 14 mongrel dogs were randomized to with(n=7) or without(n=7) ischemic preconditioning such as four 5 minutes' occlusion and 5 minutes' reperfusion, Changes of hemodynamic parameters and extents of the ischemic myocardial damages during preconditioning were observed. And using in vitro myocardial staining with monastral blue and triphenyl tetrazolium chloride, we compared the infarct sizes and risk areas in two groups of occlusion and reperfusion canine model with and without preconditioning. RESULTS: 1) Heart rate was significantly decreased after first 5 minutes' occlusion compared with those of basal control(151+/-27 VS 163+/-25 BPM, p<0.05) without further changes in subsequent ischemic insults. Left ventricular systolic pressure was significantly decreased after first 5 minutes' occlusion(109.0+/-19.9 VS 130.6+/-23.3mmHg, p<0.005), and after first 5 minutes' reperfusion and second 5 minutes' occlusion compared with those of basal control(111.3+/-29.8, 109.9+/-17.2 VS 130.6+/-23.3mmHg respectively, p<0.05), without further changes during remaining ischemia. Left ventricular end diastolic pressure and maximum +dp/dt were not changed. Peak -dp/dt was decreased significantly after first and second 5 min occlusion(943.7+/-294.4, and 962.1+/-281.5) from basal control level(1168.2+/-358.8mmHg, p<0.05). Thereafter no change was noted during remaining preconditioning. The changes in rate-pressure product were same as those of left ventricular systolic pressure(first 5 minutes occlusion ; 17.3+/-3.7 VS 21.2+/-3.5, p<0.005, second 5 minutes' occlusion ; 17.9+/-5.3, 18.1+/-3.4 VS 21.2+/-3.5, p<0.05). 2)Transmyocardial lactate extraction ratio was significantly decreased in early phase of ischemic preconditioning(17.5+/-11.3 VS 25.2+/-9.9%, p<0.05). 3) Hemodynamic parameters such as heart rate, left ventricular systolic pressure, left ventricular end-diastolic pressure, maximum +dp/dt, peak -dp/dt and rate-pressure product were changed similarly in both control and precontioned groups. 4) There was no significant difference of mean myocardial blood flows in infarct zones, which represent collateral blood flow, after 5 minutes' brief occlusion and 60 minutes of sustained occlusion in preconditioned group. 5) The infarct area/risk area ratio was significantly reduced in preconditioned group(27.0+/-9.6 VS 5.6+/-3.1%, p<0.005), but the risk area/left ventricular area ratio showed no difference in the two groups. CONCLUSIONS: These findings suggest that, in the early phase of brief repeated occlusion and reperfusion, myocardial ischemic damage accompaning systolic and diastolic myocardial dysfuctions develops and myocardial protective effect of ischemic preconditioning was obtained at the same time. Ischemic preconditioning group demonstrated reduced infarct sizes compared to those of control group after 90 minutes' sustained ischemia and reperfusion in canine acute myocardial infarction model.
Animals ; Blood Pressure ; Coronary Occlusion ; Coronary Vessels* ; Dogs ; Heart Rate ; Hemodynamics ; Ischemia ; Ischemic Preconditioning* ; Lactic Acid ; Myocardial Infarction ; Myocardial Reperfusion ; Myocardium ; Necrosis ; Reperfusion

Myxoma is the commonest intracardiac tumor and it is well known to present in bizarre ways. Calcification of the tumor is uncommon, though probably more frequent in right than in left heart tumors. Diagnosis can be followed by surgery with satisfactory results. A 36 years old man with partially calcified right ventricular myxoma and a 51 years old man with calcified left atrial myxoma, which were revealed different echogenic densities on 2-dimensional echocardiogram and gross calcification on fluoroscopy, were treated with surgical removal. We report above two cases of calcified myxoma with literature review.
Adult ; Diagnosis ; Fluoroscopy ; Heart Neoplasms ; Humans ; Middle Aged ; Myxoma*