Authors report herein a case of a 27-year-old male patient who had been suffering from chronic ulcerative dermatitis with scar-like changes and successive involvement of the ankles, limb folds, nape and abdomen, and no tendency to heal from age 4. At the age 14, an immunologic study showed a selective IgA deficiency with partial T-lymphocyte inactivation. Ten years later, at age 24, he showed a severe form of morphea over a generalized area and disabling joint contractures, and was diagnosed as disabling pansclerotic morphea with an IgA value that returned to a near normal level. At the age 27, an adult-fist, 7×6×4cm sized, squamous cell carcinoma with an easy bleeding tendency like an overgrowing granulation tissue vascular tumor had developed on the chronic ulcerative lesion on the posterior aspect of the right ankle for 2 months. Aggressive metastatic lesion occurred on the right popliteal area 3 months later. He died 1 month thereafter.
Abdomen ; Adult ; Ankle ; Carcinoma, Squamous Cell* ; Contracture ; Dermatitis ; Epithelial Cells* ; Extremities ; Granulation Tissue ; Hemorrhage ; Humans ; IgA Deficiency ; Immunoglobulin A ; Joints ; Male ; Scleroderma, Localized* ; T-Lymphocytes ; Ulcer*

The vagina is an infrequent site of primary sarcomas, which are less than 2% of all malignant vaginal neoplasm. The most common malignant mesenchymal tumor of the vagina is rhabdomyosarcoma, but leiomyosarcoma is rare type tumor of the vagina. Microscopically, spinle cells with pleomorphic nuclei and mitotic figures were frequently noted, but cross-striations were not found in the tissue. Alpha-smooth muscle actin and desmin were positive, but sarcomeric actin was negative in the tumor cells. The authors report a case of primary leiomyosarcoma of the vagina with brief literature review.
Actins ; Desmin ; Leiomyosarcoma* ; Rhabdomyosarcoma ; Sarcoma ; Vagina* ; Vaginal Neoplasms

p53 is a tumor suppressor gene product identified in a wide range of tumor including colorectal carcinoma. Genetic alterations in the p53 tumor suppressor gene are common in human colorectal carcinoma. bcl-2 is a protooncogene that inhibits apoptosis. The products of mutant p53 gene and bcl-2 have been associated with prognosis in several malignancies including colorectal carcinomas. This study was undertaken to evaluate values of p53 and bcl-2 oncoproteins as prognostic factors relative to clinicopathological factors and correlation of their expression. Analyses were made on achieval pathologic tissues of 80 patients with colorectal carcinomas including 34 patients able to follow-up over 5 years. The oncoproteins were localized using commercially available monoclonal antibodies:DO-7 for p53, clone124 for bcl-2. Expression of bcl-2 was cytoplasmic, whereas nuclear p53 expression was localized in carcinoma cells. The patients were 17 to 83 years of age. The expression of p53 and bcl-2 was determined respectively in 30 (37.5%) and 21 (26.3%)cases. The expression of p53 nuclear expression was not correlated with tumor location, size, histologic grade, Duke's classification, regional lymph node metastasis and tumor recurrence. The cytoplasmic expression of bcl-2 was not correlated with tumor location, size, histologic grade, Duke's classification, regional lymph node metastasis and recurrence. Univariate analysis showed that patients with p53 nuclear expression were not associated with poorer overall survival than patients with p53 negative, and also showed in patients with bcl-2 expression. The expression of p53 did not affect to that of bcl-2. We concluded that the p53 nuclear and bcl-2 cytoplasmic expression were not independent prognostic factors in colorectal carcinomas. Evidence of reciprocity of bcl-2 and p53 expression was not found.
Apoptosis ; Classification ; Colorectal Neoplasms* ; Cytoplasm ; Follow-Up Studies ; Genes, p53 ; Genes, Tumor Suppressor ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Oncogene Proteins ; Prognosis ; Recurrence

Hepatitis C was known to be associated with many autoimmune disease, but the pathophysiology was not well understood. Antiphospholipid antibodies, which are autoantibodies detected to negatively charged phospholipids, are sometimes detected in infectious diseases, including sypilis and autoimmun diseases, such as systemic lupus erythematosus and unknown etiology. A few reports suggested that there is a relation between primary antiphospholipid syndrome and hepatitis C. We recently experienced a case of primary antiphospholipid syndrome with chronic hepatitis C in 20 years old womam who developed cerebral infartion. A brief review of related literature is presented.
Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Autoantibodies ; Autoimmune Diseases ; Communicable Diseases ; Hepatitis C ; Hepatitis C, Chronic* ; Hepatitis, Chronic* ; Humans ; Lupus Erythematosus, Systemic ; Phospholipids ; Young Adult

PURPOSE: An increased expression of cyclin D1 has been observed in several tumors. Vimentin is known to be present in undifferentiated thyroid cancer. The serum thyroglobulin level can be a useful laboratory parameter to detect tumor recurrence in patients suffering with treated, well-differentiated thyroid carcinoma. We performed immunohistochemical assay for the cyclin D1, vimentin and thyroglobulin (Tg) expression in 97 papillary thyroid cancer tissue specimens to investigate their clinical implication for the prognosis of papillary thyroid carcinoma patients. METHODS: Immunohistochemical assays for cyclin D1, vimentin and Tg were performed on 97 consecutive thyroid carcinoma specimens. The correlation between these factors and the clinicobiological parameters weas analyzed. RESULTS: In 97 papillary thyroid carcinoma specimens, the expression of cyclin D1, vimentin and Tg was seen in 64 cases (66.0%), 36 cases (37.1%) and 55 cases (56.7%), respectively. Only the cyclin D1 expression was significantly increased in the larger tumors (P=0.01). However, there was no evident correlation between these factors and gender, age, tumor size, the lymph node status, the TNM stage and the number of tumors. CONCLUSION: An increased expression of cyclin D1 was present in the larger tumors. Overexpression of cyclin D1 seemed to play a role in carcinogenesis and tumor growth. However, the clinical utility of cyclin D1, vimentin and Tg in thyroid cancer patients has to be further defined by prospective studies with larger sample sizes.
Carcinogenesis ; Cyclin D1* ; Cyclins* ; Humans ; Lymph Nodes ; Prognosis ; Recurrence ; Sample Size ; Thyroglobulin* ; Thyroid Gland* ; Thyroid Neoplasms* ; Vimentin*

Gaucher's disease (GD) is the most common inherited lysosomal storage disease, manifested by generalized accumulation of glucocerebroside in macrophages of the reticuloendothelial system due to a deficient lysosomal beta-glucocerebrosidase (GC). It is inherited by an autosomal recessive pattern in which three clinical phenotypes have been described based on the presence and severity of neurologic involvement. GD is treated possible by GC enzyme replacement therapy, allogeneic bone marrow transplantation (BMT), and gene therapy. We here report the exprience of successful allogeneic BMT in a 16-year-old female patient with GD type III which was demostrated markedly increased Gaucher cells in bone marrow and absence of GC activity in peripheral blood monocytes by FACS using 5'- pentafluorobenzoylaminofluorescein-di-beta-D-glucoside (PFBFDGlu) as substrate. Donor marrow engraftment was confirmed by chromosome analysis using microsatellite and by bone marrow examination. Assay of GC activity using FACS revealed normal level of enzyme activity. She remains alive and well after 12 months of BMT.
Adolescent ; Bone Marrow Examination ; Bone Marrow Transplantation* ; Bone Marrow* ; Enzyme Replacement Therapy ; Female ; Gaucher Disease* ; Genetic Therapy ; Glucosylceramidase ; Humans ; Lysosomal Storage Diseases ; Macrophages ; Microsatellite Repeats ; Monocytes ; Mononuclear Phagocyte System ; Phenotype ; Tissue Donors

Recently the percutaneous aspiration and sclerotherapy has become the most advocated procedure for the symptomatic, large, and simple renal cyst because of its minimal invasiveness and high effectiveness. But the recurrence rate of the simple aspiration and sclerotherapy was reported highly (30-70%). We analysed the effect of percutaneous aspiration and repeated sclerotherapy of the renal cyst in 42 patients from Nov. 1989 to Apr. 1995. Sclerosing agent were 99% ethanol in 33 cases, minocycline in 4 cases, and only aspiration was done in 6 cases. The method of repeated sclerotherapy was achieved about 2.2 times as which aspiration and instillation of sclerosing agent in standing of 8.3 Fr. pigtail catheter during average 2.5 days. After follow up for 17.4 (6-59) months, the complete collapse rate of the renal cyst was 78.7% in the using group of 99% ethanol. 25% in minocycline, 16.696 in the aspiration only. The complete collapse rate according to the times of sclerotherapy was 45.5% in one times, 78.6% in two times, 92% in three times. In conclusion, the sclerotherapy of the simple renal cyst with 99% ethanol was most effective, and we can be expect that the rate of complete collapse has been increased in the repeated sclerotherapy.
Catheters ; Ethanol ; Follow-Up Studies ; Humans ; Minocycline ; Recurrence ; Sclerotherapy*

BACKGROUND: It is important that hospitals conduct disaster drills to ensure prompt response in case of a pandemic and thereby prevent a biological disaster. METHODS: In a university hospital of Seoul, a drill was arranged by the members of the drill preparation team who were a part of the response team for infection control of novel influenza A (H1N1). The drill preparation team designed the scenario for the drill, made plans to resolve the potential problems that could occur during that scenario, and organized a survey team and a survey methodology. The scenario consisted of 2 modules: (1) for an intensive care unit and (2) for an emergency care center. The surveyors and field participants were evaluated after the drill exercise. RESULTS: This drill was conducted to improve the response to outbreaks of new infectious diseases. The drill event showed that the communication among the members responsible for the infection control was effective. However, the drill revealed certain drawbacks in the process; this drawbacks involved availability of adequate quarantine space, education on using personal protective equipments, assignment of medical and nonmedical staff, management of visitors, and installment of air-conditioners, heaters, and ventilation units in the areas with H1N1 outbreak. CONCLUSION: This drill helped to improve the process of infection control and overcome the drawbacks in the current process, and thereby helped in achieving positive outcome during the actual pandemic situation when the number of hospital visits and admissions because of H1N1 pandemic had rapidly increased. Although disaster plans and drills are not actively performed, the drill for infection control is essential because the risk for an outbreak of a new infectious disease is increasing.
Communicable Diseases ; Surveys and Questionnaires ; Dietary Sucrose ; Disasters ; Disease Outbreaks ; Emergency Medical Services ; Humans ; Infection Control ; Influenza, Human ; Intensive Care Units ; Mandrillus ; Pandemics ; Quarantine ; Ventilation

BACKGROUND: Understanding the burden of disease is important to establish cost-effective treatment strategies and to allocate healthcare resources appropriately. However, little reliable information is available regarding the overall economic burden imposed by chronic obstructive pulmonary disease (COPD) in Korea. METHODS: This study is a multicenter observational research on the COPD burden in Korea. Total COPD costs were comprised of three categories: direct medical, direct non-medical, and indirect costs. For direct medical costs, institutional investigation was performed at 13 medical facilities mainly based on the claims data. For direct non-medical and indirect costs, site-based surveys were administered to the COPD patients during routine visits. Total costs were estimated using the COPD population defined in the recent report. RESULTS: The estimated total costs were approximately 1,245 million US dollar (1,408 billion Korean won). Direct medical costs comprised approximately 20% of the total estimated costs. Of these, formal medical costs held more than 80%. As direct non-medical costs, nursing costs made up the largest percentage (39%) of the total estimated costs. Costs for COPD-related loss of productivity formed four fifths of indirect costs, and accounted for up to 33% of the total costs. CONCLUSION: This study shows for the first time the direct and indirect costs of COPD in Korea. The total costs were enormous, and the costs of nursing and lost productivity comprised approximately 70% of total costs. The results provide insight for an effective allocation of healthcare resources and to inform establishment of strategies to reduce national burden of COPD.
Delivery of Health Care ; Efficiency ; Health Care Costs ; Humans ; Korea* ; Nursing ; Pulmonary Disease, Chronic Obstructive*

PURPOSE: To compare the variable inflammatory parameters of acute pyelonephritis patients treated with inpatient therapy at 13 hospitals, according to the age and gender distributions. MATERIALS AND MATHODS: A total of 3,544 medical records of patients with confirmed acute pyelonephritis, and admitted to hospital between January 2000 and December 2005, were retrospectively analyzed. RESULTS: The mean age of the patients was 43.2+/-16.2 years old, with a male:female ratio of 1 : 5.1. The average duration of hospital admission was 7.9+/-5.3 days. Underlying diseases were found in 23.0% (749/3,252 patient), largely due to diabetes (35.1%). Radiological abnormal findings were found in 13.7%. The leukocyte count, ratio of segmented form, erythrocyte sedimentation rate (ESR), c-reactive protein, pyuria, positive blood culture, positive urine culture were 11,014+/-5,778/mm(3), 74.8+/-14.5%, 44.0+/-32.0 mm/hr, 12.4+/-9.3mg/dl, 83.9%, 10.5% and 46.7%, respectively. E. coli grow in 79% of the urine culture positive patients. In a comparison of 3 age groups (<40 years, 40-60 years, >61 years), the elderly patients had a greater number of underlying diseases and more pathogens in cultured blood. When divided into males and females, the elderly male patients had more pathogen in cultured urine, but contrary to the male patients, the elderly female patients had elevated leukocyte count and erythrocyte sedimentation rate. Also, the old patient group had more resistance to ampicillin when they had E. coli as the uropathogen (p=0.021). Patients with higher ESR required longer hospital admission periods. CONCLUSIONS: It was found that variable clinical parameters of acute pyelonephritis patients treated with inpatient therapy differed according to both gender and age group in Korea. Therefore, these factors should be taken into account in the treatment plan.
Aged ; Ampicillin ; Blood Sedimentation ; C-Reactive Protein ; Female ; Humans ; Inpatients ; Korea ; Leukocyte Count ; Male ; Medical Records ; Pyelonephritis* ; Pyuria ; Retrospective Studies