Transcatheter Arterial Embolization Therapy for a Massive Polycystic Liver in Autosomal Dominant Polycystic Kidney Disease Patients

Polycystic liver is the most common extra-renal manifestation associated with autosomal dominant polycystic kidney disease (ADPKD), comprising up to 80% of all features. Patients with polycystic liver often suffer from abdominal discomfort, dyspepsia, or dyspnea; however, there have been few ways to relieve their symptoms effectively and safely. Therefore, we tried transcatheter arterial embolization (TAE), which has been used in treating hepatocellular carcinoma. We enrolled four patients with ADPKD in Seoul National University Hospital, suffering from enlarged polycystic liver. We embolized the hepatic arteries supplying the dominant hepatic segments replaced by cysts using polyvinyl alcohol particles and micro-coils. The patients were evaluated 12 months after embolization for the change in both liver and cyst volumes. Among four patients, one patient was lost in follow up and 3 patients were included in the analysis. Both liver (33%; 10%) and cyst volume (47.7%; 11.4%) substantially decreased in two patients. Common adverse events were fever, epigastric pain, nausea, and vomiting. We suggest that TAE is effective and safe in treating symptomatic polycystic liver in selected ADPKD patients.

The Association between Kidney Function, Coronary Artery Disease, and Clinical Outcome in Patients Undergoing Coronary Angiography

To characterize the association between chronic kidney disease (CKD), mortality, severity of coronary artery disease (CAD), treatment modality of CAD, and type of coronary stents among patients undergoing coronary angiography (CAG), we retrospectively reviewed the electronic medical records of the patients who underwent CAG at Seoul National University Bundang Hospital in Korea between May 2003 and January 2006. CKD was staged using an estimated glomerular filtration rate (eGFR) from the creatinine value prior to CAG. There were 3,637 patients included. The presence of CAD was 48% in CKD stage 1, 61% in stage 2, 73% in stage 3, 87% in stage 4, and 81% in stage 5. Survival rate gradually diminished for patients with decreasing renal function. No significant differences in all-cause and cardiac mortality were observed by medical treatment, PCI or CABG, in CKD patients with an eGFR less than 60 mL/min/1.73 m(2). CKD patients with drug-eluting stents showed significantly lower all-cause mortality (5.4% vs. 13.3%) and incidence of myocardial infarction (1.7% vs. 10%) than those with bare metal stents. In conclusion, an eGFR is a strong independent prognostic marker among patients undergoing CAG and the severity of CAD increases progressively with worsening renal function.

Nephrotic Syndrome Associated with Thymic Neuroendocrine Tumor; Case Report and Review of Literature / 대한신장학회지

We report a case of nephrotic syndrome with thymic neuroendocrine tumor. A 50-year-old woman was admitted with generalized edema. 24-hour urine collection revealed proteinuria of 20 gram daily and creatinine clearance of 28 mL/min. During examination, a thymic mass was found, which is unresectable due to metastasis to the lung. Biopsy of the thymus and the kidney revealed large cell neuroendocrine carcinoma of thymus origin and minimal change disease, respectively. Despite high dose steroid therapy, massive proteinuria continued for about 30 days. However, when the patient was given a chemotherapy (cyclophosphamide, adriamycin, vincristine), proteinuria began to decrease dramatically. The carcinoma showed the partial response after six cycles of chemotherapy. This is the first report on remission of malignancy-related nephrotic syndrome after chemotherapy, especially for thymic neuroendocrine tumor.

A case of primary renal angiosarcoma in a patient with end-stage renal disease / 대한내과학회지

Primary renal angiosarcoma is a rare and fatal malignant tumor. About 20 cases have been reported in the literature, but there has been no report of renal angiosarcoma in Korea. A 67-year old man with end-stage renal disease was admitted to the hospital for left flank pain. A computed tomography examination revealed a subcapsular hemorrhage in the left kidney. The patient was discharged after 2 weeks of observation. However, at 6 weeks after the discharge, the patient revisited the outpatient clinic due to aggravating pain, and a left nephrectomy was performed. The histopathological diagnosis was angiosarcoma. Three weeks after the nephrectomy, a re-operation was performed because of uncontrolled intra-abdominal bleeding. The surgical findings revealed massive metastasis. The patient died of an uncontrolled hemorrhage about 1 week after the second surgery. To our knowledge, this is the first report of a renal angiosarcoma in a patient with end-stage renal disease. It should be noted that a renal angiosarcoma may be a cause of a retroperitoneal hematoma.

Prevalence of Staphylococcus aureus Nasal Carriage and CAPD-associated Infection / 대한신장학회잡지

BACKGROUND:Staphylococcu S. aureus (S. aureus) is one of the most important etiologic agents of CAPD-associated infection and the nasal carriage of S. aureus increases the risk of CAPD-associated infection. We evaluated the nasal carriage status of S. aureus in CAPD patients and the association between nasal carriage of S. aureus and CAPD-associated infection. METHODS:We did a retrospective study about 167 patients on CAPD who regularly visited outpatient department at Seoul National University Hospital, Seoul National University Boramae Hospital, Seoul National University Bundang Hospital. Nasal swab cultures for S. aureus were taken once between September of 2005 and February of 2006. RESULTS:Nasal swab culture showed that S. aureus nasal carriage rate was 22.2%. S. aureus nasal carrier group showed that increased incidence of exit site infection and peritonitis caused by S. aureus and all other causes of exit site infection, but these were statistically insignificant. In diabetic patients, S. aureus nasal carriage rate was 21.6%. The observation of these patients also showed that S. aureus nasal carriage insignificantly increased the incidence of exit site infection and peritonitis caused by S. aureus and all oth er causes of exit site infection. CONCLUSION:In our study, the S. aureus nasal carriers did not show significantly higher risk for development of exit site infection and peritonitis by S. aureus or all other causes of exit site infection.

A Case Report of Successful Treatment with Plasmapheresis and Intravenous Immunoglobulin in a Renal Transplant Recipient with Acute Humoral Rejection / 대한신장학회잡지

Acute humoral rejection after renal transplantation is associated with a higher frequency of allograft dysfunction and graft loss. We report a case of acute humoral rejection which was treated successfully with plasmapheresis and intravenous immunoglobulin. A 31- year-old man developed azotemia after kidney transplantation. Kidney biopsy finding was compatible with antibody-mediated rejection, demonstrated by the infiltration of monocytes and neutrophils and the deposition of C4d on glomerulus and peritubular capillaries. We performed five plasmapheresis with concomitant treatment of intravenous immunoglobulin after each session. With aggressive treatment, there was improvement of oliguric acute renal failure, accompanied by decrease in the percentage of PRA and the titer of donor specific antibodies. Repeated kidney biopsy revealed persistent C4d staining on peritubular capillaries despite disappearance of donor specific antibodies. In conclusion, plasmapheresis and intravenous immunoglobulin are effective in treating acute humoral rejection.

A Case of Collagenofibrotic Glomerulopathy with Characteristic Electron Microscopic and Immunohistochemistry Findings / 대한신장학회잡지

Collagenofibrotic glomerulopathy is a relatively rare glomerular disease characterized by the accumulation of spiraled frayed collagen fibrils in mesangial and subendothelial areas. Clinically, patients with the disease usually present with moderate proteinuria and edema. They tend to have hypertension and their renal function deteriorates slowly. We report the case of a patient with collagenofibrotic glomerulopathy who also had Takayasus arteritis and presented with hypertension, proteinuria and dyspnea on exertion. Electron microscopy of the renal biopsy revealed massive accumulation of peculiar collagen fibers and immunohistology using monoclonal antibodies to collagen type III revealed positive stain in glomerular tufts.

A Case of Collagenofibrotic Glomerulopathy with Characteristic Electron Microscopic and Immunohistochemistry Findings / 대한신장학회잡지

Collagenofibrotic glomerulopathy is a relatively rare glomerular disease characterized by the accumulation of spiraled frayed collagen fibrils in mesangial and subendothelial areas. Clinically, patients with the disease usually present with moderate proteinuria and edema. They tend to have hypertension and their renal function deteriorates slowly. We report the case of a patient with collagenofibrotic glomerulopathy who also had Takayasus arteritis and presented with hypertension, proteinuria and dyspnea on exertion. Electron microscopy of the renal biopsy revealed massive accumulation of peculiar collagen fibers and immunohistology using monoclonal antibodies to collagen type III revealed positive stain in glomerular tufts.