Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder characterized by innumerable bilateral renal cysts. It has an prevalence rate of one in 200~1,000 individuals and is a relatively common cause of renal failure. As renal function deteriorates, overall renal size usually diminish in patients with chronic renal failure. However, renal size of patients with ADPKD usually continues to increase, even after the initiation of dialysis therapy, because numerous cysts replace renal mass. Attempted methods to reduce the size of enlarged kidneys have included needle aspiration and sclerotherapy, cyst decompression surgery, laparoscopic and surgical nephrectomy. The outcome of these therapy frequently has been suboptimal, and there is a need to develop a more effective therapy. We report a case of renal arterial embolization using 99% ethanol and lipiodol mixture for ADPKD in a hemodialysis pathient, which has not been previously reported.
Decompression ; Dialysis ; Ethanol* ; Ethiodized Oil* ; Humans ; Kidney ; Kidney Failure, Chronic ; Laparoscopy ; Needles ; Nephrectomy ; Polycystic Kidney, Autosomal Dominant* ; Prevalence ; Renal Dialysis* ; Renal Insufficiency ; Sclerotherapy

PURPOSE: Surgical removal for a mass in the pre-sacral space or mid rectum through a posterior approach is not frequent. We would like to present the technique of trans-sacral local resection as a posterior approach. We analyzed the follow up of patients who underwent surgery using the proposed technique. METHODS: A total of 21 patients who had undergone a trans-sacral local resection with lower sacrectomy between January 1997 and December 2006 were enrolled in this study. The diagnoses were large epidermal cyst, gastrointestinal stromal tumor, high grade adenoma, and early cancers in the mid rectum. We analyzed the surgical complications and disease recurrences. The mean follow up for tumors of the rectum was 53+/-35 mo. RESULTS: Epidural anesthesia was appropriate for all whole procedures. Among the 21 cases, there was one case of a rectocutaneous fistula as a postoperative complication (4.9%). In one case among the submucosal cancers, there was a systemic metastasis at 24 mo without local recurrence. CONCLUSION: In our experience, a trans-sacral resection with a lower sacrectomy is a good option and provides a wide and direct surgical exposure for the removal of a pre-sacral or a mid-rectal mass. Good bowel preparation is mandatory.
Adenoma ; Anesthesia, Epidural ; Epidermal Cyst ; Fistula ; Follow-Up Studies ; Gastrointestinal Stromal Tumors ; Humans ; Neoplasm Metastasis ; Postoperative Complications ; Rectal Neoplasms ; Rectum ; Recurrence

Behcet's disease is a multisystem disorder characterized by oral and genital ulcers, uveitis, and skin lesions. Renal involvement has rarely been observed in Behcet's disease. However, pathological reports have revealed various lesions, especially, amyloidosis, focal and segmental glomerulonephritis with crescents, and IgA nephropathy. A 49-year old female with Behcet's disease was admitted to evaluate proteinuria and microscopic hematuria, and renal biopsy showed IgA nephropathy (subclass V). Immunohistology revealed mesangial deposits of IgA and staining for C3. We report a case of IgA nephropathy which was diagnosed by renal biopsy in a patient with Behcet's disease.
Amyloidosis ; Biopsy ; Female ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Hematuria ; Humans ; Immunoglobulin A* ; Middle Aged ; Proteinuria ; Skin ; Ulcer ; Uveitis

Myxoma is the most common primary cardiac tumor. It usually develops on the interatrial septum, and occurs in the left atrium in more than 75% of cases. Myxoma in the ventricle is much less common and accunts for only 5% of cases. A myxoma arising from mitral valve is exceedingly rare and is normally located on the atrial side of valve, with an eqivalent distribution between the anterior and posterior leaflets. We report a case of myxoma that arose from both the interatrial septum and anterior mital leaflet, which has not been previously reported in the literature.
Heart Atria ; Heart Neoplasms ; Mitral Valve ; Myxoma*

PURPOSE: Anatomy of deep pelvis, narrow distal margin and tumor invasion into neighbor organ are obstacles for curative radical resection for advanced cancer of distal rectum. Technically, laparoscopic application after downstaging the tumor with preoperative concurrent chemotherapy (CCRT) may give a solution to overcome the anatomical difficulties. We compared the results of laparoscopic surgery in the patients who received CCRT with those of patients who had conventional surgery. MATERIALS AND METHODS: A continuous infusion of 5FU plus leucovorin and radiotherapy (50.4 Gy) in 28 fractions was given each patient as CCRT. They underwent D2 radical resection with TME and ANP for the rectal cancer in 4 weeks. RESULTS: Thirty three patients had laparoscopic resection such as LAR, colo-anal anastomosis and APR. The results were compared with 12 cases of the conventional resections. As a result of preoperative CCRT, the cancer was down-staged in 71%. Two year disease free survival was 75% and 74% in the group of conventional and laparoscopic resection, respectively (p=0.427). Ileus, voiding difficulty and leakage after surgery were not different between two groups. Weakness of ejaculation was noted in 9~11% of both groups. The DFS of the preoperative CCRT followed by radical resection in the groups with a response was more favorable than that in the group with progressive or stable disease. CONCLUSION: Radical resection of advanced distal rectal cancer could be done with performing a laparoscopic assisted operation after CCRT induced down-staging. We may suggest that laparoscopic assisted resection is a good treatment option as it doesn't increase the complications and it has a compatible survival rate to conventional surgery.
Atrial Natriuretic Factor ; Chemoradiotherapy* ; Disease-Free Survival ; Drug Therapy ; Ejaculation ; Fluorouracil ; Humans ; Ileus ; Laparoscopy ; Leucovorin ; Male ; Pelvis ; Radiotherapy ; Rectal Neoplasms* ; Rectum ; Survival Rate

BACKGROUND: Even though there have been many case reports on nephrotic syndrome in patients with malignancy, an overall study on malignancy- associated nephrotic syndrome is rare in Korea. The purpose of this study was to explore the clinical and pathologic findings and clinical course of malignancy-associated nephrotic syndrome. METHODS: From January, 1986 to December, 2003, the medical records of patients with nephrotic syndrome and concomitant malignancy were retrospectively reviewed. RESULTS: Forty-eight patients (2.3%) out of 2, 085 patients with nephrotic syndrome had concomitant malignant disease during the study period. The mean age of patients was 57.9+-1.6 years with sex ratio of 2.4: 1. The most common primary origin of malignancy was liver (8 patients, 16.7%) and lungs (8 patients, 16.7%), and adenocarcinoma (17 patients, 35.4 %) was the leading histologic type of malignancy. There was no significant difference in 24-hour urinary protein excretion among patients grouped by TNM stage. Percutaneous renal biopsy was performed in 26 patients (54.2%), renal pathology revealed membranous nephropathy and minimal change disease in 9 patients (34.6%) each, membranoproliferative glomerulonephritis in 6 (23.1%), and IgA nephropathy in 2 patients (7.7%). When the patients were divided into progression and remission group based on the clinical course of underlying malignancy, there were significantly more patients with improved nephrotic syndrome in the remission group than the progression group (55.0% vs. 0%, p<0.05). CONCLUSION: Malignancy should be considered as a cause of nephrotic syndrome in adults, and the treatment of underlying malignancy may affect the outcome of nephrotic syndrome in patients with malignancy.
Adenocarcinoma ; Adult ; Biopsy ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Humans ; Korea ; Liver ; Lung ; Medical Records ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Pathology ; Retrospective Studies ; Sex Ratio

Following a report by Hultengren et al. (Acta Chir Scand, 1965), it has been suggested that analgesic abuse predisposes to urothelial neoplasia. Urinary tract malignancy is combined in 8-10% of patients with analgesic nephropathy. Microscopic or gross hematuria can be the first sign leading to the diagnosis of uroepithelial malignanacy in analgesic abusers. Since uroepithelial malignancies found in analgesic abusers tend to be multiple and have a worse prognosis, continued monitoring is essential, and new hematuria should be evaluated with urinary cytology, and cystoscopy with reterograde pyelography. Phenacetin found to be the chief cause of malignancies in analgesic abusers, it has been anticipated to be a human carcinogen and was banned as an OTC drug since 1987. But still there remains a debate whether acetaminophen and other compound analgesic components are carcinogenic. We report the case of a 58-year-old man with a history of analgesic abuse who was diagnosed with transitional cell carcinoma combined with analgesic nephropathy. We also review the literature.
Acetaminophen ; Analgesics ; Carcinoma, Transitional Cell* ; Cystoscopy ; Diagnosis ; Hematuria ; Humans ; Middle Aged ; Nephritis, Interstitial ; Phenacetin ; Prognosis ; Ureter* ; Urinary Tract ; Urography

BACKGROUND: Cardiovascular disease is known as an important predictor of mortality, not only in patients undergoing dialysis treatment but also in those who are starting dialysis treatment. In addition, it is well known that cardiovascular morbidity is about twice higher in diabetic patients. In this study, MIBI and echocardiography were performed in patients starting dialysis treatment, and a comparison of these findings between diabetic (DM) and non-diabetic (Non-DM) patients was done. METHODS: Among the patients diagnosed as end- stage renal disease (ESRD) and started dialysis treatment at Severance Hospital, 77 patients underwent MIBI and echocardiography when they were clinically stable within 4 weeks after the initiation of dialysis. Clinical characteristics, laboratory findings, MIBI and echocardiographic findings of the 77 patients were analyzed. RESULTS: The mean age of the patients was 58.4+/-10.8 years with sex ratio of 1.1: 1. Of the 77 patients, 52 were DM and 25 were Non-DM. There were 30 patients (39.0%) with abnormal findings on MIBI scan, 26 with reVersible and 4 with fixed defects, and 69 patients (89.6%) with left ventricular hypertrophy (LVH) on echocardiography. DM group showed higher prevalence of myocardial perfusion defect than Non-DM group (48.1% vs. 20.0%, p< 0.05). There were no differences in the prevalence of LVH (92.3% vs. 84.0%) and in left ventricular ejection fraction (LVEF) (56.1+/-13.1% vs. 57.5+/-11.8%) between DM and Non-DM groups. LVEF was significantly lower in patients with abnormal findings on MIBI scan than those with normal MIBI finding. CONCLUSION: The majority of ESRD patients starting dialysis treatment accompanied LVH and myocardial perfusion defect was present in many cases especially in diabetic patients. Therefore, early evaluation and treatment of ischemic heart disease are mandatory in diabetic patients starting dialysis treatment for ESRD.
Cardiovascular Diseases ; Diabetes Mellitus ; Dialysis* ; Echocardiography* ; Humans ; Hypertrophy, Left Ventricular ; Kidney Failure, Chronic ; Mortality ; Myocardial Ischemia ; Perfusion ; Prevalence ; Sex Ratio ; Stroke Volume ; Technetium Tc 99m Sestamibi* ; Tomography, Emission-Computed, Single-Photon*

A 45-year-old woman was admitted with proteinuria, hematuria and intermittent generalized edema. On admission, hypertension, chronic B-viral hepatitis were evident together with a decreased renal function. Light and electron microscopy revealed diffuse and nodular glomerulosclerosis indistinguishable from diabetic nodular glomerulosclerosis. Diabetes mellitus, however, had been excluded both by extensive clinical and by laboratory investigation. The differential diagnosis also included primary and secondary membranoproliferative glomerulonephritis, thrombotic microangiopathy, amyloidosis, monoclonal immunoglobulin deposition disease, fibrillary glomerolonephritis, immunotactoid glomerulopathy, light chain deposition disease. However, these entities could be excluded by immunohistochemistry and ultrastructural investigations. We report a case of idiopathic nodular glomerulosclerosis, which has not been previously reported in the Korean literature.
Amyloidosis ; Diabetes Mellitus ; Diabetic Nephropathies* ; Diagnosis, Differential ; Edema ; Female ; Glomerulonephritis, Membranoproliferative ; Hematuria ; Hepatitis ; Humans ; Hypertension ; Immunoglobulins ; Immunohistochemistry ; Microscopy, Electron ; Middle Aged ; Proteinuria ; Thrombotic Microangiopathies

BACKGROUND: Cardiovascular mortality is increased in ESRD patients and is not completely explained by common cardiovascular risk factors. Therefore, more attention is being focused on non-traditional risk factors such as endothelial dysfunction, malnutrition, chronic inflammation. This study was performed to investigate whether chronic inflammation and malnutrition plays an important role on the endothelial dysfunction in ESRD patients. METHODS: Seventy-five patients undergoing CAPD for more than six months were enrolled in the study. To evaluate the extent of endothelial dysfunction, flow-mediated vasodilation (FMD) of brachial artery was measured using doppler ultrasonography. The degree of chronic inflammation was assessed by measuring inflammatory markers (IL-6, hsCRP) and SGA was used to assess the nutritional status. RESULTS: According to SGA grade, the patients were divided into 2 groups (group 1: normal nutritional status, group 2: malnourished status). In group 2, IL-6 was significantly higher compared to group 1. FMD and albumin level were significantly lower in group 2 (11.37+/-4.93 vs. 8.41+/-4.23%, 3.7+/-0.4 vs. 3.4+/-0.3 g/dL, p<0.05). When the patients were divided into groups according to hsCRP level (group 1: > or =3 mg/L, group 2: <3 mg/L), BMI and fasting glucose were significantly higher in group 1 compared to group 2 (25.9+/-3.5 vs. 23.9+/-2.8 kg/m2, 105.8+/-22.3 vs. 93.3+/-11.4 mg/dL). HDL-cholesterol was significantly lower in group 1 (37.4+/-9.3 vs. 45.3+/-12.9 mg/dL). FMD was decreased in group 1 compared to group 2 (7.35+/-4.23 vs. 11.57+/-4.76%). Multiple regression analysis showed that average hsCRP concentration was an independent factors affecting brachial FMD. CONCLUSION: These findings suggest that endothelial dysfunction is associated with markers of inflammation and malnutrition, and chronic inflammation and malnutrition can be a predisposing factors for atherosclerosis in CAPD patients.
Atherosclerosis ; Brachial Artery ; Causality ; Fasting ; Glucose ; Humans ; Inflammation* ; Interleukin-6 ; Kidney Failure, Chronic ; Malnutrition ; Mortality ; Nutritional Status* ; Peritoneal Dialysis, Continuous Ambulatory* ; Risk Factors ; Ultrasonography, Doppler ; Vasodilation