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

Solid pseudopapillary tumor of the pancreas is a rare tumor that affects young females with low malignant potential and good prognosis with more than 90% survival at 5 years. Metastasis is very rare. We report the case of a 74-year-old female who had pancreatic solid-pseudopapillary tumor and synchronous hepatic metastasis.
Aged ; Female ; Humans ; Neoplasm Metastasis ; Pancreas ; Pancreatic Neoplasms ; Prognosis

The purpose of this study was to investigate the impact of depression, discomfort, spirituality, physical care, and opioid use on pain with terminally ill cancer patients residing in hospice units. The convenient sample of this study consisted of 41 terminally ill cancer patients at three hospice units in university affiliated hospitals. Patients were interviewed with structured questionnaires three times at predetermined intervals: admission to the hospice unit (Time 1), one week later (Time 2), and two weeks later (Time 3). The data was collected from January 1998 to January 1999 and was analyzed using ANOVA, Pearson correlation coefficient, and multivariate multiple regression. The results of this study were as follows: 1. The mean age of the participants was approximately 55 years old. In terms of diagnosis, lung cancer showed the highest frequency (19.5%), followed by stomach cancer and rectal cancer (17.1%). The motive of seeking hospice unit admission was control (72. 2%), followed by spiritual care (50%), and symptom relief (38.9%). 2. Regarding the type of pain felt, the highest pain frequency the participants experienced was deep pain (55%), followed by multiple pain (25%), intestinal pain (10%), then superficial (5%) and neurogenic pain (5%). For the level of pain measured by VAS, there was no significant difference among the three time points; Time 1 (5.04 +/-2.21), Time 2 (4.82+/-2.58) and Time 3(4.73+/-2.51). 3. There was significant change seen in spirituality and physical care in each time interval. Namely, the longer the length of admission at the hospice unit, the higher the importance of spirituality (p=0.0001) and the more the physical care the participants received (p=0.01). The opioid use at the three time points showed the following frequencies : Time 1 (75.6%), Time 2 (85.4%) and Time 3 (75.6%). 4. Regarding factors influencing pain, the pain level was significantly affected by the depression level (p <0.01) and the opioid use (p <0.1). These results were the most significant at the two time points (Time 1 and Time 2). At Time 3 (two weeks later), the pain level was significantly affected by the depression level (p <0.05) and the amount of physical care the participants received (p <0.1). In conclusion, the terminally ill cancer patients had moderate pain, were generally depressed, and were treated with opioid analgesics. As approaching death, the patients received more physical care due to increased physical symptoms experienced and they had a higher perception of the importance of spirituality. Thus, health care professionals need to provide continuous care for each of them to die comfortably physically, psycho- logically, and spiritually.
Analgesics, Opioid ; Delivery of Health Care ; Depression ; Diagnosis ; Hospices* ; Humans ; Logic ; Lung Neoplasms ; Middle Aged ; Surveys and Questionnaires ; Rectal Neoplasms ; Spirituality ; Stomach Neoplasms ; Terminally Ill*

Purpose: This report presents a case of repeated toxic conjunctival necrosis caused by amikacin eye drops in a patient with a corneal ulcer induced by Mycobacterium abscessus (M. abscessus).Case summary: During treatment for herpetic keratitis with complaints of conjunctival hyperemia and eye pain in the right eye for 1 month. Slit-lamp microscopy revealed a circular corneal epithelial defect and stromal infiltration of her right eye. Gram staining and culture were performed, and eye drops were prescribed for empirical treatment. The bacterial culture grew M. abscessus. Antibiotic susceptibility tests showed resistance to quinolone. The patient was prescribed amikacin eye drops and the corneal epithelial defects improved. Two weeks later, circular conjunctival epithelial defects and hyperemia were observed on the inferior conjunctiva. Conjunctival necrosis caused by amikacin was suspected. The amikacin eye drops were discontinued and the conjunctival lesion improved. However, the patient’s corneal ulcer recurred. The amikacin eye drops were restarted, but conjunctival toxicity recurred, so she was treated with topical clarithromycin and moxifloxacin. The eye drops were gradually reduced and the lesion healed leaving mild corneal opacity. Conclusions M. abscessus-induced keratitis resistant to quinolone antibiotics requires long-term combined therapy with multiple drugs. Amikacin eye drops should be used carefully as conjunctival toxicity may occur.

No abstract available.
Transplants

OBJECTIVE: To compare the objective and subjective image quality indicators and radiation doses of computed tomography (CT) venography performed using model-based iterative reconstruction (MBIR) at 80 kVp and adaptive statistical iterative reconstruction (ASIR)-V at 70 kVp. MATERIALS AND METHODS: Eighty-three patients who had undergone CT venography of the lower extremities with MBIR at 80 kVp (Group A; 21 men and 20 women; mean age, 55.5 years) or ASIR-V at 70 kVp (Group B; 18 men and 24 women; mean age, 57.3 years) were enrolled. Two radiologists retrospectively evaluated the objective (vascular enhancement, image noise, signal-to-noise ratio [SNR], contrast-to-noise ratio [CNR]) and subjective (quantum mottle, delineation of contour, venous enhancement) image quality indicators at the inferior vena cava and femoral and popliteal veins. Clinical information, radiation dose, reconstruction time, and objective and subjective image quality indicators were compared between groups A and B. RESULTS: Vascular enhancement, SNR, and CNR were significantly greater in Group B than in Group A (p ≤ 0.015). Image noise was significantly lower in Group B (p ≤ 0.021), and all subjective image quality indicators, except for delineation of vein contours, were significantly better in Group B (p ≤ 0.021). Mean reconstruction time was significantly shorter in Group B than in Group A (1 min 43 s vs. 131 min 1 s; p < 0.001). Clinical information and radiation dose were not significantly different between the two groups. CONCLUSION: CT venography using ASIR-V at 70 kVp was better than MBIR at 80 kVp in terms of image quality and reconstruction time at similar radiation doses.
Female ; Humans ; Image Enhancement ; Image Processing, Computer-Assisted ; Lower Extremity ; Male ; Noise ; Phlebography ; Popliteal Vein ; Retrospective Studies ; Signal-To-Noise Ratio ; Veins ; Vena Cava, Inferior ; Venous Thrombosis

It is not a common case for neurosurgery department and the other departments to perform joint operation at the same time. Patients with severe head injury are a condition in which vital signs are unstable due to severe brain swelling and increased intracranial pressure, and emergency surgery is required. A 44-year-old man visited the trauma center with a motorcycle accident. The Glasgow Coma Scale score at the time of emergency department was 3 points, and the pupil was fixed at 6 mm on both sides. His medical history was unknown. His vital signs including blood pressure (BP), heart rate, respiratory rate and oxygen saturation were stable. Associated injuries included multiple fractures of whole body. Brain computed tomography revealed subarachnoid hemorrhage, intraventricular hemorrhage and severe cerebral edema. During the preparation of the craniectomy, abdominal ultrasonography performed because of decreased BP resulted in a large amount of hemoperitoneum. The bi-coronal craniectomy and splenectomy were performed simultaneously for about 4 hours. After fifty days of treatment, he was discharged with Glasgow Outcome Scale-extended 4 points and is undergoing rehabilitation. In severe polytrauma patients, active concurrent surgery is a good method to save their lives.
Adult ; Blood Pressure ; Brain ; Brain Edema ; Craniocerebral Trauma* ; Emergencies ; Emergency Service, Hospital ; Fractures, Multiple ; Glasgow Coma Scale ; Head* ; Heart Rate ; Hemoperitoneum ; Hemorrhage ; Humans ; Intracranial Pressure ; Joints ; Methods ; Motorcycles ; Multiple Trauma ; Neurosurgery ; Oxygen ; Pupil ; Rehabilitation ; Respiratory Rate ; Splenectomy* ; Subarachnoid Hemorrhage ; Trauma Centers ; Ultrasonography ; Vital Signs

We report a case of spinal intradural abscess which shows serial changes on magnetic resonance imaging (MRI). Well-encapsulated, rim-enhancing lesion with mass effect was visualized at ventral side of lumbar spinal canal on 17 days after initial negative MRI, which was thought to be epidural abscess. It was revealed to be intradural in location on operation and successfully treated by drainage and antibiotics. Follow-up MRI showed resolution of abscess. Clinical significance and pathogenesis of this case was briefly discussed.
Abscess* ; Anti-Bacterial Agents ; Drainage ; Epidural Abscess ; Follow-Up Studies ; Magnetic Resonance Imaging ; Spinal Canal ; Spine*

PURPOSE: Breast cancer has been reported as the most common cancer of women in the United States, Western Europe and Korea and about 210,000 and 10,000 women in United States and Korea every year, respectively are diagnosed with it. Breast cancer is curable with an early diagnosis, and many researchers have made efforts to find a marker for this malady, heat shock protein (HSP) consists of 6 groups, it is highly preserved throughout both the prokaryotic and eukaryotic cells and it acts as a molecular chaperone that's involved in protein folding. HSPs have been recently reported to be related with breast cancer. In this study, we investigated the changes of expression of HSP60 in tissues and cell lines of breast cancer. METHODS: We obtained breast cancer tissues and normal tissues from breast cancer patients, and we purchased several cancer cell lines from American tissue culture correction. We treated the tissues and the cell lines of human breast cancer with heat shock protein. Proteins and mRNAs were isolated from the tissues and the cells and then we performed Western blotting, reverse transcriptase-Polymerase chain Reaction and fluorescence activated cell sorter analysis on them. RESULTS: On Western blot, HSP60 was more overexpressed in the tissues and the cell lines of breast cancer than in the normal breast tissues and cell lines. The expression of HSP60 showed 2 types of molecular weight differences in the tissues and cell lines of breast cancer, and specifically, low HSP60 was over-expressed in the cancer tissues. There was no difference between the breast cancer cell lines and the normal cell lines in the expressions of HSP60 mRNA, according to the treatment with heat shock. Also, there was no relationship between phosphorylation and the structural difference of HSP60 protein, according to HSP60 protein's molecular weight. The expression of HSP60 has been mostly reported at the mitochondria; however, in this study, it was more predominantly detected at the cytoplasm than at the mitochondria in the breast cancer cell lines. CONCLUSION: We conclude that HSP60 may be used as a diagnostic marker for breast cancer. Detailed investigation of the usefulness and significance of the HSP60 expression as a prognostic factor is required in further studies.
Blotting, Western ; Breast ; Breast Neoplasms ; Cell Line ; Chaperonin 60 ; Cytoplasm ; Early Diagnosis ; Eukaryotic Cells ; Europe ; Female ; Fibrinogen ; Fluorescence ; Heat-Shock Proteins ; Hot Temperature ; Humans ; Korea ; Mitochondria ; Molecular Chaperones ; Molecular Weight ; Phosphorylation ; Protein Folding ; Proteins ; RNA, Messenger ; Shock ; United States