No abstract available.
Brain ; Humans ; Myotonic Dystrophy

To determine size and shape discrepancy between the bone of Korean women and the prostheses in total knee arthroplasty, the height(antero-posterior length) and width(medio-lateral length) of the distal femur and the proximal tibia were measured at the bone resection level for 104 knees of 62 female patients who underwent primary total knee arthroplasty and compared with the same parameters of the total knee prostheses designed by five different companies. On the condition that the height of the prostheses matched with each cases of the bone were optimal, the difference in the width was evaluated, and the criteria of the discrepancy were overhang if the width of the prosthesis was larger than bone, optimal if width of the prosthesis was same or smaller and difference was within 5 mm, and down-size if smaller and difference was over Smm. In femoral prostheses, 34.6% of all were optimal, 13.1%, overhang and 52.3%, down-size. In tibial prosthesis, 54.1% of all were optimal, 13.9%, overhang and 32%, down-size. In conclusion, although further investigation for clinical application is needed, there were discrepancies in size and shape between the femur and tibia of Korean women and total knee prostheses and improvement in design should be considered.
Arthroplasty* ; Female ; Femur ; Humans ; Knee Prosthesis ; Knee* ; Prostheses and Implants* ; Tibia

We report a monozygotic twins with temporal lobe epilepsy, almost concordant in the onset time of firt seizure, lateralization and localization of epileptiform discharges. Partial seizures with secondarily generalized seizure occurred 15 days apart. EEG showed frequent spike-slow wave complexes on the right temporal area but the findings of brain MRI were normal in both. Past medical, delivery, developmental and family history were unremarkable. They had no physical and mental handicaps, and any other underlying causes of epilepsy. They have been on monotherapy with seizure free, although follow up EEG showed frequent spike-slow wave complexes after medication. As seizures developed simultaneously, the localization and lateralization of epileptiform discharges were concordant in our monozygotic twins, we suggest that genetic factor might be the most probable etiology of epilepsy in our cases but needs to be elucidated.
Brain ; Electroencephalography ; Epilepsy ; Epilepsy, Temporal Lobe ; Follow-Up Studies ; Humans ; Seizures ; Temporal Lobe ; Twins, Monozygotic

PURPOSE: According to the staging system for breast cancer by the 2003 revised American Joint Committee on Cancer (AJCC), the patients with 10 or more positive axillary nodes are classified as N3 and also as a new stage, i.e., stage IIIC. The aim of this study was to investigate the prognosis of patients with 10 or more positive nodes. METHODS: The database of 125 patients with 10 or more positive axillary nodes who underwent surgery at Korea Cancer Center Hospital between 1997 and 2001 were reviewed. The age of the patients, the T stage, the number and site of the positive nodes, the hormone receptor status, the HER-2 over-expression, and the treatment modalities were examined in reference to the disease-free survival (DFS). RESULTS: At the median follow-up time of 40 months, 16 cases (13%) of locoregonal recurrence and 57 cases (46%) of systemic relapse had developed in 67 patients (54%). The DFS and overall survival rates at 5 years were 46% and 55%, respectively. On univariate analysis, the T stage (p<0.001), hormone receptor status (p=0.001), and neoadjuvant chemotherapy (p=0.014) were predictive factors of recurrence. On multivariate analysis, the T stage (p=0.002) and hormone receptor status (p=0.02) were independent predictors of recurrence. The patients with hormone receptor positive tumor had a 58%, 5-year DFS rate. On the contrary, in 9 of 10 patients with T4 tumor, recurrence developed within 2 years after the initial treatment. CONCLUSIONS: This study showed that stage IIIC according to the revised AJCC staging system was not a prognostically homogeneous group. Some notably high survival rates were observed in a subgroup of patients, and especially for those patients with hormone receptor positive tumor. In contrast, the prognosis of patients with T4 tumor was significantly worse than that of the patients with the other stage IIIC disease. Thus, we suggest that the stage IIIC group in the new AJCC staging system needs to be refined to provide more reliable prognostic information for the patients with advanced breast cancer.
Breast Neoplasms* ; Breast* ; Disease-Free Survival ; Drug Therapy ; Follow-Up Studies ; Humans ; Joints ; Korea ; Lymph Nodes* ; Multivariate Analysis ; Neoplasm Staging ; Prognosis* ; Recurrence ; Survival Rate

OBJECTIVE: To investigate whether synovial fluid adenosine deaminase activity is useful in the differential diagnosis of joint swelling and in estimating the disease activity. METHOD: Adenosine deaminase activity was determined in the synovial fluid taken from patients with rheumatoid arthritis (n=21), osteoarthritis (n=ll), ankylosing spondylitis (n=3), and gouty arthritis (n=2). This enzyme activity was compared with the laboratory indices (ESR, CRP) in the blood and the other parameters in the synovial fluid. RESULT: More increased adenosine deaminase activity was found in the synovial fluid taken from patients with rheumatoid arthritis, ankylosing spondylitis, and gouty arthritis, as compared with that of osteoarthritis patients. Synovial fluid ADA activity was significantly corelated with the WBC count in the synovial fluid, but there was no statistical corelation between other synovial parameters and adenosine deaminase activity. CONCLUSION: Adenosine deaminase activity is useful in the differential diagnosis of joint swelling between inflammatory joint disease and osteoarthritis, but not useful in estimating the disease activity.
Adenosine Deaminase ; Adenosine* ; Arthritis, Gouty* ; Arthritis, Rheumatoid* ; Diagnosis, Differential ; Humans ; Joint Diseases ; Joints ; Osteoarthritis* ; Spondylitis, Ankylosing* ; Synovial Fluid*

PURPOSE: The aim of this study was to confirm the factors that affect the mortality associated with the open surgical repair of ruptured abdominal aortic aneurysm (rAAA) and to analyze the long-term survival rates. METHODS: A retrospective review was performed on a prospectively collected database that included 455 consecutive patients who underwent open surgical repair for AAA between January 2001 and December 2012. We divided our analysis into in-hospital and postdischarge periods and analyzed the risk factors that affected the long-term survival of rAAA patients. RESULTS: Of the 455 patients who were initially screened, 103 were rAAA patients, and 352 were non-rAAA (nAAA) patients. In the rAAA group, 25 patients (24.2%) died in the hospital and 78 were discharged. Long-term survival was significantly better in the nAAA group (P = 0.001). The 2-, 5-, and 10-year survival rates of the rAAA patients were 87%, 73.4%, and 54.1%, respectively. Age (hazard ratio [HR], 1.05; 95% confidence interval [CI], 1.02–.08; P < 0.001) and aneurysm rupture (HR, 1.96; 95% CI, 1.12–.44; P = 0.01) significantly affected long-term survival. CONCLUSION: Preoperative circulatory failure is the most common cause of death for in-hospital mortality of rAAA patients. After excluding patients who have died during the perioperative period, age is the only factor that affects the survival of rAAA patients.
Aneurysm ; Aortic Aneurysm ; Aortic Aneurysm, Abdominal* ; Aortic Rupture ; Cause of Death ; Hospital Mortality* ; Humans ; Mortality ; Perioperative Period ; Prospective Studies ; Retrospective Studies ; Risk Factors ; Rupture ; Shock ; Survival Rate

Macrophage activation syndrome (MAS) is a rare complication in systemic lupus erythematosus (SLE) that can be triggered by infections. Due to the fact that MAS may mimic clinical features of underlying rheumatic disease, or be confused with an infectious complication, its detection can prove challenging. This is particularly true when there is an unknown/undiagnosed disease; and could turn into an even greater challenge if MAS and SLE are combined with a viral infection. A-14-year-old female came to the hospital with an ongoing fever for 2 weeks and a painful facial skin rash. Hepatomegaly, pancytopenia, increased aspartate aminotransferase, elevated serum ferritin and lactate dehydrogenase were reported. No hemophagocytic infiltration of bone marrow was reported. The patient was suspected for hemophagocytic lymphohistiocytosis. Her skin rashes were eczema herpeticum, which is usually associated with immune compromised conditions. With the history of oral ulcers and malar rash, positive ANA and low C3, C4 and the evidence of hemolytic anemia, she was diagnosed as SLE. According to the diagnostic guideline for MAS in SLE, she was diagnosed MAS as well, activated by acute HSV infection. After administering steroids and antiviral agent, the fever and skin rash disappeared, and the abnormal laboratory findings normalized. Therefore, we are reporting a rare case of MAS triggered by acute HSV infection as the first manifestation of SLE.
Anemia, Hemolytic ; Aspartate Aminotransferases ; Bone Marrow ; Exanthema ; Female ; Ferritins ; Fever ; Hepatomegaly ; Humans ; Kaposi Varicelliform Eruption ; L-Lactate Dehydrogenase ; Lupus Erythematosus, Systemic* ; Lymphohistiocytosis, Hemophagocytic ; Macrophage Activation Syndrome* ; Macrophage Activation* ; Macrophages* ; Oral Ulcer ; Pancytopenia ; Rheumatic Diseases ; Steroids

OBJECTIVES: The prevalence of malaria is increasing in recent years and also multidrug resistant malaria is increasing around the world and there is an increasing concern about imported malaria in nonendemic areas. Now many drugs are tried to find out effect on multidrug resistant malaria. We performed this study to investigate the thrapeutic effect of halofantrine in the treatment of chloroquine resistant imported malaria. METHODS: From Feb. 1992 to May 1995, we experienced 35 patients infected with malaria and treated 14 patients among 35 patients with halofantrine. RESULTS: 1) All 14 patients were sailor with a mean age of 39.4 years and infected with malaria. 2) The majority of patients were infected with malaria at Africa. 3) 10 patients were infected with Plasmodium falciparum and the remainder were undetermined. 4) In the 11 cases of chloroquine resistant malaria treated with quinine plus tetracycline combination therapy or Fansidar, 4 cases could not be tolerable due to side effects and resistance to the therapy, we substituted halofantrine for above regimens. 5) In the 10 cases, treated after May 1994, halofantrine was the first choice of treatment because they were the cases of malaria infected in the mid-Africa where the prevalence of chloroquine resistant malaria is high. 6) With halofantrine, all 14 cases were treated with minimal side effects suc4 as nausea, vomiting, anorexia, abdominal pain and fatigue. CONCLUSION: We think halofantrine is a simple and effective regimen against chloroquine resistant malaria and consider this agent as an alternative therapeutic regimen on chloroquine resistant malaria.
Abdominal Pain ; Africa ; Anorexia ; Chloroquine* ; Fatigue ; Humans ; Malaria* ; Military Personnel ; Nausea ; Plasmodium falciparum ; Prevalence ; Quinine ; Tetracycline ; Vomiting

Mature cystic teratomas of the ovary are one of the most common ovarian neoplasms found in reproductive age women. Malignant transformation is an uncommon complication in a mature cystic teratoma of the ovary, usually being reported in about 1-3% of cases. Of malignant transformations, squamous cell carcimona is the most common type and sarcomatous transformation is rare, its prognosis is poor. Adenocarcinoma occurs with less frequency. We experienced a case of squamous cell carcinoma and adenocarcinoma arising in mature cystic teratoma of the ovary, so we present this case with brief review of the literature.
Adenocarcinoma* ; Carcinoma, Squamous Cell* ; Female ; Humans ; Ovarian Neoplasms ; Ovary* ; Prognosis ; Teratoma*

OBJECTIVE: This study was performed to identify the prognostic factor for survival of patients with recurrent cervical cancer. METHODS: Sixty-eight patients were diagnosed as recurrent cervical cancer at the Seoul National University Hospital from January, 1988 to December, 1998. Recurrence was defined as new evidence of tumor after 6 months of disease free survival. Retrospective analysis was done in terms of clinical features and the Cox proportional hazard model was used to identify independent variables associated with an improved survival rate. Histopathologic types were distributed as follows; squamous cell carcinoma in 70.6%, adenocarcinoma in 11.8%, adenosquamous cell carcinoma in 11.8%, and small cell carcinoma in 1.5%. Distribution of FIGO stage was as follows; stage I in 25.0%, stage II in 66.2%, and stage III in 4.4%. Sites of recurrence were as follows; central pelvic recurrence in 44.1%, pelvic side wall recurrence in 11.8%, and distant metastasis in 44.1% and the most common site of distant recurrence was extrapelvic lymph nodes (29.4%). 29.4% of recurrences were observed within the first 12 months after initial therapy, 50.0% within 2 years and 64.7% within 3 years. RESULTS: Positive rate of SCC-Ag at initial diagnosis was 45.2% with cutoff value of 2.0 ng/ml. Positive rate of SCC-Ag at the diagnosis of recurrence was 60.0%. Overall response rate to the treatment was 29.1%. Complete response rate was higher in central pelvic recurrrence than pelvic side wall recurrence and distant metastasis (P = 0.002) and also higher in normal SCC-Ag level (< or = 2.0 ng/ml) at the diagnosis of recurrence than elevated level (P = 0.032). Cumulative survival rates of 1 year after recurrence was 66.8%, 2 year 36.7%, and 5 year 18.7%. Central recurrence showed higher cumulative survival rate than pelvic side wall or distant recurrence (P = 0.029). The patients with elevated SCC-Ag level at the time of diagnosis of recurrence showed lower cumulative survival rate than those with normal SCC-Ag level (P < 0.001). Cox proportional hazard model showed that SCC-Ag elevation at the time of diagnosis of recurrence retained significant values in predicting survival(OR = 2.56; 95% CI = [1.22-5.39]; P = 0.01). CONCLUSION: SCC-Ag elevation at the diagnosis of the recurrence is a strong independent prognostic indicator for survival of patients with recurrent cervical cancer.
Adenocarcinoma ; Carcinoma, Small Cell ; Carcinoma, Squamous Cell ; Diagnosis ; Disease-Free Survival ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Proportional Hazards Models ; Recurrence ; Retrospective Studies ; Seoul ; Survival Rate ; Uterine Cervical Neoplasms*