Radiologically Guided Renal Artery Embolization with an Amplatzer Vascular Plug as a Rescue Therapy for Refractory Nephrotic Syndrome in AL-Amyloidosis.

Nephrotic syndrome is common in immunoglobulin light-chain (AL) amyloidosis and successful therapy may pose a challenge. We report the case of a 63-year-old patient with severe nephrotic syndrome due to primary renal AL-amyloidosis with well-preserved renal function at first presentation. Therapy with high dose steroids, loop diuretics, and ACE-inhibitors did not affect his proteinuria and he was seriously disabled because of symptomatic orthostatic hypotension and anasarca. With the patient's informed consent, medical nephrectomy was tried with nonsteroidal-anti-inflammatory drugs (NSAIDs), cyclosporine, and aminoglycosides, with significant deterioration of his renal function, but without relevant effect on his proteinuria. Despite adequate anticoagulation life threatening thrombotic and bleeding complications occurred. Total renal ablation was finally achieved using an Amplatzer vascular plug Typ IV (AVP 4) with a self-expanding Nitinol mesh design, which was placed in both main renal arteries in the same intervention. The patient became completely anuric, protein loss stopped, and serum albumin slowly rose to normal levels. The patient's clinical condition dramatically improved and he regained his full mobility at the price of a lifelong renal replacement therapy. To our knowledge, this is the first reported usage of such a vascular occluder in the setting of refractory nephrotic syndrome with normal kidney function at the time of first presentation.

Recurrent, sequential, bilateral deep cerebellar hemorrhages: a case report.

INTRODUCTION: Hypertensive intra-cerebral hemorrhage is usually a one-time event and recurrences are rare. Most recurrences develop as part of long-term failure of blood pressure control. The site of the re-bleed is usually limited to the basal ganglia and thalami. CASE PRESENTATION: We report the case of a 59-year-old hypertensive Caucasian woman who developed two sequential, right- and then left-sided, deep cerebellar hemorrhages. The second hemorrhage followed the first one by 57 days, at a time when her blood pressure was optimally controlled. In spite of these critical sites and short duration between the two bleeds, the patient achieved a relatively good functional recovery. Her brain magnetic resonance angiogram was unremarkable. CONCLUSION: The development of recurrent hypertensive hemorrhage is rare and usually occurs within two years of the first bleed. To the best of our knowledge, this is the first reported case of bilateral, sequential, right- and then left-sided deep cerebellar hemorrhages. These hemorrhages were separated by eight weeks and the patient had a relatively good functional recovery. We believe that hypertension was the etiology behind these hemorrhages.

T cell vaccination in multiple sclerosis relapsing-remitting nonresponders patients.

Myelin autoreactive T cells are involved in the pathogenesis of multiple sclerosis (MS) and lead to propagation of the disease. We evaluated the efficacy of T cell vaccination (TCV) therapy for patients with aggressive relapsing-remitting MS who failed to respond to immunomodulatory treatments. Twenty nonresponders relapsing-remitting MS patients were immunized with autologous attenuated T cell lines after activation with synthetic myelin basic protein (MBP) and myelin oligodendrocyte glycoprotein (MOG) encephalitogenic peptides. Each patient received three vaccinations in 6- to 8-week intervals. Annual relapse rate decreased from 2.6 to 1.1, P = 0.026. Neurological disability stabilized as compared with the 2- and 1-year pretreatment progression rates. Significant reduction in the number and volume of active lesions, as well as reduction in T2 lesion burden, was demonstrated by quantitative MRI analysis. No serious adverse events were observed. Our findings suggest that TCV has beneficial clinical effects in MS patients who, in spite of immunomodulatory treatments, continue to deteriorate. TCV could serve as a potential alternative therapy for this subgroup of nonresponders patients.

Adjuvant treatment of high-risk stage II breast cancer with doxorubicin followed by high-dose chemotherapy and autologous stem-cell transplantation: a single-institution experience with 132 consecutive patients.

Several studies have shown conflicting results with the use of intensive consolidation chemotherapy for breast cancer. The aim of the present study was to investigate the efficacy, feasibility and toxicity of high-dose chemotherapy with stem cell support in patients with high-risk stage II breast cancer. From February 1994 to November 1998, 132 consecutive patients with multinode positive breast cancer were entered to the study. In total, 86 patients had >or=10 positive axillary lymph nodes, and 46 had 4-9 positive axillary lymph nodes with at least two additional predetermined risk factors at diagnosis. All patients were offered adjuvant chemotherapy (doxorubicin, 75 mg/m(2) x 4) followed by high-dose chemotherapy (cyclophosphamide 6000 mg/m(2), carboplatin 800 mg/m(2) and thio-tepa 500 mg/m(2)) and autologous stem cell support with growth factor. In all, 131 patients also received local radiation therapy and tamoxifen based on receptor status. After a median follow-up of 51 months (range 27-87), the disease-free and overall survival rates were 72 and 81%, respectively. There was no difference in the outcome for high-risk patients with > or < than 10 positive axillary lymph nodes. On Cox regression analysis only progesterone receptor status was predictive of disease-free, but not overall survival. There were no treatment-related deaths; grades III-IV toxicity was relatively low. This combined approach of doxorubicin followed by high-dose chemotherapy and stem-cell support, followed by locoregional radiotherapy, was safe and seems to be effective in patients with multinode positive stage II breast cancer. In previous trials of adjuvant high-dose therapy in this patient population, treatment-related morbidity and mortality markedly influenced the outcome. For this high-risk patient population, further testing of intensive chemotherapy regimens with a lower toxicity profile is warranted.

[A patient with rapidly progressing renal failure, florid syphilis and positive HIV serology]. / Eine Patientin mit rasch progredienter Niereninsuffizienz, florider Lues und positiver HIV-Serologie.

We describe the frist reported case in Switzerland of HIV-associated nephropathy (HIVAN). HIVAN shows a typical combination of clinical findings: black race, proteinuria, large hyper-echogenic kidneys, normal blood pressure, positive HIV serology and no autoantibodies. The histologic findings are typical: focal segmental glomerulosclerosis of the collapsing variant, often with marked interstitial nephritis. The disease normally appears before AIDS symptoms develop and follows a very aggressive course to end-stage renal disease. Therapy consists of a combination of nucleoside reverse transcriptase and proteinase inhibitors, ACE inhibitors, and possibly steroids. In end-stage renal disease patients can be managed by haemodialysis, continuous ambulatory peritoneal dialysis (CAPD) or kidney transplantation.

[Acute kidney insufficiency in an alcoholic patient with liver cirrhosis]. / Akute niereninsuffizienz bei einem Athyliker mit leberzirrhose.

We report on a 67-year-old patient who was hospitalized because of worsening of general condition, generalized edema and ascites with decompensated ethylic cirrhosis of the liver. On hospital admission renal failure was present which had developed within 5 months. In spite of intensive treatment with diuretics and repeated ascites punctures, fluid repletion reappeared repeatedly. Renal function remained stable for the first 10 days but worsened thereafter rapidly.

[Angiosarcoma following teratocarcinoma: "mutation", successive malignancy or irradiation]. / Angiosarkom nach Teratokarzinom: "Mutation", sukzessives Malignom oder Bestrahlung.

Angiosarcoma is a rare malignant soft-tissue tumor and affects preferably skin and adjacent subcutaneous tissue. Very rarely the tumor is radiation-induced. We report the case of a 28-year-old Swiss who developed an angiosarcoma of the gut 5 years after the successful therapy of metastasising teratocarcinoma of his right testis by surgery and radiation therapy. We discuss three possible etiologies for the genesis of the angiosarcoma, which appeared rapidly after the primary tumor at an unusual site in an unusual patient.

Plaque inhibition by sustained release of chlorhexidine from removable appliances.

We studied the effectiveness of a sustained-release delivery system for chlorhexidine in plaque prevention. A clinical trial in a group of eight students wearing orthodontic appliances coated by ethyl cellulose polymer containing the drug demonstrated that plaque accumulation was decreased for a period of four days. All oral procedures had been withdrawn during the clinical study. No side-effects of chlorhexidine, such as tooth staining and unpleasant taste, were observed.