To clarify whether a protein restriction diet in the pre-dialysis period affects the overall prognosis after initiating hemodialysis therapy, we compared the survival between patients with and without a protein restriction diet. Among 310 patients in whom hemodialysis was introduced between 1997 and 2000 at Toride Kyodo General Hospital, two hundred and ten patients were excluded due to an insufficient observation period ( < 6 months) or the lack of records estimating their protein intake. One hundred patients were finally included in this study. All of these patients were followed at the hospital with their estimated protein intake using repeated (three times or more) urea nitrogen appearance in 24-hour collected urine samples over 6 months prior to the initiation of dialysis therapy. The patients were divided into a protein restriction diet group (PRD 61 cases) and non restriction diet group (NRD 39 cases), according to their estimated protein intake less or above 0.7 g/kg/day. Among the patient profile items, gender, cause of renal failure, and serum albumin did not differ between the two groups, but the age was higher in NRD (p < 0.01) . Nineteen patients (PRD 8, NRD 11 cases) died during the observation period (0-65 months) . On the analysis of their survival, PRD showed a better survival ratio by the Kaplan-Meier method (p < 0.01) . Among the variables examined by Cox's proportional hazard test, age, PRD, and their combination showed significant risk ratios (1.06, 0.30, and 0.39 respectively) on survival after the initiation of hemodialysis. Hence a protein restriction diet in the pre-dialysis period does not deteriorate the prognosis even after the initiation of hemodialysis therapy.

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Eight courses of LDL-apheresis (LDL-A) with the liposorber LA-15 system (Kaneka, Osaka, Japan) were analyzed in 6 patients with steroid-resistant nephrotic syndrome. Of the 8 courses of LDL-A, 5 were administered to treat focal glomerular sclerosis and 3 for minimal-change type nephrotic syndrome in 4 male and 4 female patients. The patients averaged 46.1 ± 12.4 years in age at the time of LDL-A. LDL-A treatment consisted of about 4, 000 ml of blood plasma over 2-3 hours, and was performed l-3 times per week and 9-12 times (average : 11.6) per course. Before and after a course of LDL-A, 24 hour urine protein, creatinine clearance (CCr), biochemistry tests, and coagulation tests (thrombin-anti thrombin III complex (TAT), plasmin-α2 plasmin inhibitor complex (PIC), and β-thromboglobulin (β-TG) ) were performed. Of the 8 courses, 4 achieved a complete remission, and one achieved a type I incomplete remission (response group) . Two patients receiving the other three courses eventually required hemodialysis (nonresponse group) . In the response group, LDL-A was administered for an average of 3.8±2.0 months after the disease onset. This interval was significantly shorter than that of 23.3 ± 10.3 months in the non response group(p = 0.005) . Before LDL-A, TAT was 38.0 ± 19.1 and 7.6±2.1ng/ml in the response and non-response groups, respectively, showing a significant difference (p = 0.037) . In the response group, CCr was 37.0±5.0 ml/min before LDL-A, and increased significantly to 55.7 ± 12.0ml/ min after LDL-A (p = 0.038) . The disease did not recur in the response group after an average of 37 months of follow-up. These results indicate that LDL-A should be performed as early as possible after the onset of nephrotic syndrome, and that before LDL-A, TAT was high in the response group.

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A 26-year-old woman with neurofibromatosis 1 was admitted to our hospital for investigation of prolonged hypertension after toxemia of pregnancy. Plasma renin activity was elevated. From the renogram, stenosis of right renal artery was suspected and we performed renal arteriography, which revealed proximal right renal artery stenosis. The intravascular ultrasound (IVUS) image showed concentric stenosis with intimal and medial hypertrophy, which was iso-echoic and partly high echoic. Percutaneous transluminal renal angioplasty was performed and the lesion was well dilated. After angioplasty, blood pressure normalized in a week. From the clinical course and the IVUS image, we suspected that renal artery stenosis was due to neurofibromatosis 1.

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We report a case of methicillin-resistant Staphylococcus aureus (MRSA) -associated glomerulonephritis treated with antibiotic therapy. A 67-year-old man was admitted to our hospital because of proteinuria, hematuria, purpura, and high fever one month after a graft replacement of an abdominal aortic aneurysm. MRSA was detected in specimens of his blood, sputum, and joint fluid. Before his operation, he had shown no renal abnormalities. He presented with a rapid deterioration of renal function following MRSA infection. Maximum level of proteinuria was 1.5 g/day, serum creatinine(Cr) was 3.5 mg/dl, and blood urea nitrogen was 57 mg/dl. Renal biopsy revealed necrotizing crescentic glomerulonephritis. Immunofluorescence examination showed 1gA and C3 deposits. Clinical and pathological examinations showed the typical features of MRSA-associated glomerulonephritis. Vancomycin(R) and fosfomycin were administered intravenously. The serum level of C-reactive protein fell from 22.0 mg/dl to 0.1 mg/dl. Proteinuria also decreased and the patient's renal function improved in parallel with the decreased activity of MRSA infection. After three months of antibiotic treatment, proteinuria was negative and the level of serum Cr had dropped to 0.9 mg/dl. These findings suggest that antibiotic treatment can lead to complete remission of MRSA-associated glomerulonephritis.

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Denys-Drash syndrome is a rare disorder consisting of pseudohermaphrodism, Wilms' tumor and nephropathy. We describe here a boy with severe hypospadias and undescended testes, who presented with end stage renal failure at the age of 1 year and 8 months when he was referred to our hospital. Emergency hemodialysis was performed because of oliguria, edema and severe hypertension, and then peritoneal dialysis was started. The findings of the renal biopsy showed diffuse mesangial sclerosis, consistent with the characteristic change in Denys-Drash syndrome. The analysis of WT 1 gene revealed a G-to-A point mutation at 1, 186 resulting in a change from Asp to Asn at 396 in exon 9. Since he had no urine output and his kidneys were not functional and in addition, patients with this mutation have been reported to have a high risk of Wilms' tumor, bilateral nephrectomy was performed. The removed kidneys showed no malignancies. Since Denys-Drash syndrome is frequently associated with Wilms' tumor, renal biopsy and gene analysis should be performed on male patients with gonadal anomaly, such as hypospadias and/or undescended testes, and proteinuria.

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