A Case of Anti-Glomerular Basement Membrane Disease Improved by Early Plasmapheresis and Immunosuppression Therapy / 대한신장학회지

Anti-glomerular basement membrane disease is a rare autoimmune disease characterized by rapidly progressive renal failure and/or pulmonary hemorrhage. The presence of severe crescentic glomerular inflammation with linear deposition of immunoglobulin G along the glomerular basement membrane is pathognomonic. Because renal function is rapidly and often irretrievably destroyed, many patients require hemodialysis all through their lifetime. We report a case of 33 year(s)-old man who was diagnosed as anti-glomerular basement membrane disease without pulmonary hemorrhage. The patient was treated with pulse methylprednisolone and plasmapheresis followed by oral corticosteroid and cyclophosphamide. His renal function was successfully recovered with early diagnosis and aggressive treatment.

Clinicopathological Correlation of Lupus Nephritis / 대한신장학회지

PURPOSE: Clinical treatment for lupus nephritis largely depends upon histological renal biopsy classification. But it has been reported that serologic biochemical markers are not strongly associated with pathologic classification. The aim of this study is to see whether serologic markers could predict pathologic class of lupus nephritis for appropriate treatment. METHODS: We investigated 67 patients, who underwent renal biopsy with lupus nephritis at Hanyang University Hospital between January, 2005 and August, 2007. Biological markers for this study are hematuria, proteinuria, serologic data of lupus activity and azotemia. They were retrospectively analyzed from patients grouped by ISN/RPS 2003 lupus nephritis classification. RESULTS: Total 67 patients (men 5, women 62) were enrolled and the mean age of the patients was 30.6+/-9 years. The number of patient group by pathologic classification was 4 cases for class II, 15 cases for class III, 30 cases for class IV and 15 cases for class V. Spot urine protein to creatinine ratio more than 3 increased in class IV group statistically (p=.007). C3 level decreased more in class IV group than class III, V groups. Ten patients showed azotemia, and 9 of them were class IV group (p=.048). CONCLUSION: The patients with more increased proteinuria, decreased C3 level and azotemia showed more frequently in class IV group. Hence those three biological markers may be a clinical clue to pathologic diagnosis.

Cerebral Infarction as a Complication of Nephrotic Syndrome: A Case Report with a Review of the Literature

Arterial thrombosis is relatively rare compared with venous thrombosis in nephrotic syndrome. However, the assessment of its pathogenesis and risk factors in individual patient with nephrotic syndrome is necessary to allow appropriate prophylactic management because it is a potentially serious problem. Hereby, with review of the literature, we report a case of a 53 yr-old man with cerebral infarction associated with nephrotic syndrome due to focal segmental glomerulosclerosis during the course of treatments with diuretics and steroid. It reveals that the hypercoagulable state in nephrotic syndrome can be associated with cerebral infarction in adults. Prophylactic anticoagulants can be considered to reduce the risk of serious cerebral infarction in nephrotic patients with risk factors such as severe hypoalbuminemia and on diuretics or steroid treatment, even in young patients regardless of types of underlying glomerular diseases.

Clinical Presentation of Diabetes Mellitus after Kidney Tansplantation / 대한신장학회잡지

BACKGROUND: Incidence rate of diabetes mellitus (DM) after renal transplantation has been reported variably, ranging from 2.5% to 45%. The new diagnostic criterion of diabetes presented by American Diabetes Association and use of new immunosuppressants may increase the incidence of post-transplant DM. METHODS: We investigated the incidence of post- transplant DM in 135 patients, who underwent kidney transplantation at Hanyang University Hospital between March, 1998 and February, 2003. The association of risk factors (age, sex, types of immunosuppressive agents, presence of acute rejection, and relationship with donor) with post-transplant DM was retrospectively evaluated using multivariate analysis. RESULTS: The incidence of post-transplant DM was 19.3%, and the significant risk factors were age over 40 years and male sex. Presence of acute rejection and relationship between donor and recipient were not significantly associated with the occurrence of post-transplant DM. Notably, the frequencies of post-transplant DM between cyclosporine and low- dose tacrolimus were not significantly different. CONCLUSION: The incidence of post-transplant DM is higher than that of DM in general population. Blood glucose should be frequently monitored in older male patients, considering their significant risk for post-transplant DM. Low-dose tacrolimus regimen dose not seem to increase the risk for post-transplant DM compared with cyclosporine.

A Case of Pulmonary Leiomyoma in a Renal Transplanted Patient / 대한신장학회잡지

The overall incidence of malignancy in a renal transplanted patient is 3 to 5 times higher compared with general population. We report a very rare case of multiple leiomyoma originated from lung after renal transplantation. 33-year-old male underwent renal transplantation in November, 1989. A 5 mm sized pulmonary nodule was found in the left lower lobe incidentally in March, 2001. The size of pulmonary nodule increased and same lesion was found on the opposite side of the lung on chest roentgenogram after one year, so we proceeded with computed tomography of the chest. Variable sized multiple nodules in the whole lung field were noted, which were thought be metastatic lesions, and for detection of the primary site of malignancy, further study including esophagogastroduodenoscope, colon study, abdominal ultrasound, abdominal CT, bone scan and tumor marker were checked, but there was no evidence of primary cancer. After open lung biopsy low malignant potential smooth muscle tumor was noted, and Epstein-barr virus (EBV) DNA was detected. The patient had been maintained on immunosuppressive therapy with cyclosporine and mycophenolate mofetil (MMF), and after the diagnosis of leiomyoma administration of MMF was stoped, and cyclosporine dosage was reduced. With the reduction of immunosuppressants, intravenous immunoglobulin trial was done for the first cycle, but the efficacy of treatment is not clear.

Urinary Transforming Growth Factor-beta Induced Gene-h3 (betaig-h3)as a Marker of Lupus Activity in SLE with Nephritis / 대한류마티스학회지

BACKGROUND: TGF-beta-induced gene-h3 (betaig-h3) is a novel gene induced by active TGF-beta and the association with other renal disease is reported. Lupus nephritis is characterized by excessive extracelluar matrix accumulation and the implication that TGF-beta is increased in lupus nephritis is known. We measured the urinary betaig-h3 in lupus nephritis and sought its association with the activity of lupus nephritis through renal biopsy. The objective of this study was to examine urinary betaig-h3 excretion in lupus nephritis and the association with activity of lupus nephritis. METHODS: Fifteen patients (median age 32.6 2.9 years, range 18~64) who developed lupus nephritis underwent renal biopsy. At the time of biopsy, they showed significant proteinuria. Total urinary betaig-h3 concentration was assayed by enzyme-linked immunoabsorbent assay and expressed as a ratio to urinary creatinine concentration. RESULTS: There were correlations between urinary betaig-h3 and the reduction of C3 (r= 0.566, p=0.028<0.05), the magnitude of proteinuria (r=0.531, p=0.042<0.05). The Activity Index, Chronicity Index in the renal biopsy, C4, anti-dsDNA Ab titer were not significantly correlated with urinary betaig-h3 excretion, but the patients with high Activity Index had the increased level of urinary betaig-h3. Five patients who had fibrinoid necrosis in renal biopsy showed higher level of urinary betaig-h3 than the others (107.78 43.02 vs. 50.21 10.12 ng/ ml, p=0.061) CONCLUSION: In this study, There is some correlation between urinary betaig-h3 and the activity of lupus nephritis. Urinary betaig-h3 may play a role in predicting the active lupus nephritis. A further study is needed in large population and in situ expression of betaig-h3.