Wegener's granulomatosis is a necrotizing and granulomatous vasculitis which involves upper and lower respiratory tract, kidney and skin. The patient who have the protracted Wegener's granulomatosis live a long peroid without major organ imvolvement, In mild cases, the progression is slow and may not need systemic cyclophosphamide treatment. Some cases, however, demonstrate renal involvement and may result in renal failure and death if adequate treatment is not provided. We experienced a case of protracted Wegener's granulomatosis that was a new concept and has not been reported !n Korea. The diagnosis was confirmed by clinical finding and histopathologic features of tiasue biopsy. This case represents a protracted Wegener's granulomatosis with paranasal sinusitis, rhinitis and skin purpura and progress to a gener alized form with kidney involvement after 6years. Treatment with oral cyclophosphamide, steroid and sulfamethoxazole-trimethoprime result improvement of skin, nasal symptom and labratory parameters.
Biopsy ; Cyclophosphamide ; Diagnosis ; Humans ; Kidney ; Korea ; Purpura ; Renal Insufficiency ; Respiratory System ; Rhinitis ; Sinusitis ; Skin ; Vasculitis ; Wegener Granulomatosis

BACKGROUND: The number of patients suffering from acute myocardial infarction is on the increase in Korea due to the westernization of life style. Recent improvement of therapeutic stratigies have shown early mortality benefits in acute myocardial infarction. But we don't have data how many patients died and what's the cause of death in these patients. This study aimed to find out the mortality rate, cause of death and it's relevant prognostic factors of myocardial infarction (MI) patients who admitted alive, and to construct a database which will be used to develop a risk stratification strategy for the implementation of new preventive therapeutic modalities, such as implantable cardioverter-defibrillator (ICD). METHODS: Seven hundred and forty two MI patients admitted to our hospital from March, 1999 to August, 2002 were included in this study. The risk factors and survivals were evaluated by medical record searching and telephone survey in these patients. RESULTS: The average age was 64 years-old and 67% was male. During the mean follow up 20.7+/-15.4 months, total 105 cardiac death (14.2%) was occurred and cumulative mortality rate at 1 year and 2 year was 5.69% and 10.80%, respectively. Of the total 129 death, in-hospital death was 68 (cardiac death 48, non-cardiac death 20) and out of hospital death was 60 (cardiac death 57, non-cardiac death 4). When it comes to cause of death, most common cause of cardiac death was malignant arrhythmia. The proportion of malignant arrhythmia in cardiac death was 81.3% and 72% of in-hospital and out of hospital death, respectively. Multivariate analysis showed that old age, low LV ejection fraction and no percutaneous coronary intervention (PCI) treatment were independent risk factors for cardiac mortality. CONCLUSIONS: Myocardial infarction shows still high mortality rate despite the recent development of therapeutic strategy. As post-MI patients with low LVEF or no PCI shows high mortality, the early reperfusion therapy should be encouraged. Additionally, because malignant arrhythmia was one of the most in cause of cardiac death, ICD therapy to prevent sudden cardiac death should be considered in an active manner.
Arrhythmias, Cardiac ; Cause of Death* ; Death ; Death, Sudden, Cardiac ; Defibrillators, Implantable ; Follow-Up Studies ; Humans ; Korea ; Life Style ; Male ; Medical Records ; Middle Aged ; Mortality* ; Multivariate Analysis ; Myocardial Infarction* ; Percutaneous Coronary Intervention ; Reperfusion ; Risk Factors ; Telephone

We report an unusual case of adult minimal change nephrotic syndrome relapsed after 15-year of complete remission. In this case, the disease had occurred to the patient for the first time when he was 52 years old; relatively high age, and had been remitted with steroid therapy. After 15 years of complete remission, he visited our hospital with the symptoms of the generalized edema and the pitting edema of both lower extremities that occurred 15 days ago. Massive proteinuria(15, 865 mg/day) and hypoalbuminemia(1.7 g/dL) were detected. The pathologic evaluation of the biopsied specimen of the kidney showed the global sclerosis in 19% of glomeruli in light microscopic finding and the fusion of epithelial foot processes in electron microscopic finding. He was treated with pulse steroid therapy (methylprednisolone 500 mg/day iv for 3 days) and then, with oral prednisolone (60 mg/day). Generalized edema and proteinuria disappeared after 14 days of treatment, and there has been no relapse ever since. Adult-onset minimal change nephrotic syndrome relapses within 4 years after complete remission in 90 % of relapsed patients. The relapse after more than 5 years of complete remission, like this case, is very rare, especially in the case of late-onset disease. However, the possibility of relapse of the minimal change nephrotic syndrome after several years of its remission should be considered constantly and the long-term follow-up more than 10 years may be needed.
Adult ; Edema ; Follow-Up Studies ; Foot ; Humans ; Kidney ; Lower Extremity ; Middle Aged ; Nephrosis, Lipoid* ; Prednisolone ; Proteinuria ; Recurrence ; Sclerosis

PURPOSE: Reactive oxygen species modulator 1 (Romo1) is a key mediator of intracellular reactive oxygen species production. However, examination of the clinical usefulness of Romo1 in cancers has been limited. We evaluated the association of Romo1 expression with clinical outcomes in advanced non-small cell lung cancer (NSCLC) patients treated with platinum-based chemotherapy. MATERIALS AND METHODS: Romo1 expression in tumor tissue was examined by immunohistochemistry and evaluated by histological score. Survival analyses were performed according to Romo1 expression and the association between Romo1 expression and clinical parameters was evaluated. RESULTS: A total of 88 tumor specimens were analyzed. Significantly shorter median progression-free survival (PFS) was observed in the high Romo1 group compared with the low Romo1 group (4.5 months vs. 9.8 months, p < 0.001), and the median overall survival (OS) of the high Romo1 group was also significantly shorter than that of the low Romo1 group (8.4 months vs. 15.5 months, p < 0.001). Results of multivariate analyses showed significant association of high Romo1 expression with both poor PFS (hazard ratio [HR], 2.75; 95% confidence interval [CI], 1.71 to 4.44) and poor OS (HR, 3.99; 95% CI, 2.36 to 6.74). Results of the subgroup analysis showed a similar association regardless of tumor histology. Romo1 expression showed no association with any clinical parameter including age, sex, smoking status, stage, differentiation, or tumor histology. CONCLUSION: Romo1 overexpression was associated with poor response to treatment and shorter survival in advanced NSCLC patients treated with platinum-based chemotherapy. Romo1 could be a potential adverse predictive marker in this setting.
Biomarkers ; Carcinoma, Non-Small-Cell Lung* ; Disease-Free Survival ; Drug Therapy* ; Humans ; Immunohistochemistry ; Lung Neoplasms ; Multivariate Analysis ; Platinum ; Prognosis ; Reactive Oxygen Species* ; Smoke ; Smoking