1/18. budd-chiari syndrome and inferior vena cava thrombosis in a nephrotic child.We observed budd-chiari syndrome in a boy aged 2 years 6 months with nephrotic syndrome due to hepatic vein and inferior vena cava thrombosis, confirmed by Doppler imaging. Normal values of the routine hemostatic parameters proved that they are of little predictive value for the thrombotic state. Immediate heparin infusion was initiated. High doses of heparin up to 59 IU/kg per hour were required for efficient anticoagulation. A remission of the nephrotic syndrome was achieved with vincristine. Oral anticoagulation with a vitamin k antagonist was continued for 6 months. Doppler imaging then indicated full re-establishment of the blood flow through the affected vessels. The clinical and Doppler data in this case are compatible with acute budd-chiari syndrome due to incomplete outflow obstruction of the hepatic veins and inferior vena cava. The favorable outcome was due to the immediate heparin infusion and prompt remission of the nephrotic syndrome. Doppler imaging was an important tool for non-invasive diagnosis and follow-up.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/18. role of lipids in the progression of renal disease in systemic lupus erythematosus patients.Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease marked by immune-complex mediated lesions in small blood vessels of various organs, especially the kidneys, although other factors may also be implicated in the pathogenesis of the disease. This article focuses on the role of lipids in the progression of glomerular, vascular and tubulo-interstitial lesions in two patients with lupus nephritis associated with pronounced hyper- and dyslipidemia. The pathogenesis of progressive glomerulosclerosis in both patients appears to be multifactorial. In addition to immune complex mediated lupus glomerulonephritis, progressively active in the first patient, severe nephrotic-range persistent proteinuria, arterial hypertension associated with hyperfiltration and hyperperfusion injuries and, to a minor extent, hyper- and dyslipidemia were observed. Immunological and non-immunological factors were shown to contribute to the development of tubulo-interstitial lesions. In both patients, in addition to local immune deposits, prominent tubulo-interstitial lipid deposits were probably causally related to both hyperlipidemia and the increased permeability of the glomerular filtration barrier. Tubular lesions were highlighted by intracytoplasmic lipid droplets as well as small cleft-like spaces found to be impacted in the tubular lumina. They were seen to penetrate tubular epithelial cells and eventually lodge in the interstitium, surrounded by mononuclear cell infiltrates and foam cells. In both patients, hypertensive angiopathy and extraglomerular vascular immune deposits were demonstrated. In addition, in the second patient, arteriolar and small arterial hyaline was found at the age of 28 years to be full of lipids and calcium precipitates, suggesting a peripheral atherosclerosis-like process which never occurs as a natural age-related condition. In conclusion, all parts of the nephron may be involved in the pathogenetic process causally related or influenced by hyper- or dyslipidemia. Associated either with endothelial cell injury and consequent insudation of lipids in the vascular walls, glomerular filtration barrier injury with hyperfiltration, or tubulo-interstitial lipid deposition, the mechanism of tissue damage by lipids in all parts of the nephron shares similarities with the pathogenesis of systemic atherosclerosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/18. Persistent hypotension and intestinal infarction in a patient with primary amyloidosis.A patient with primary amyloidosis and the nephrotic syndrome had diarrhea and gastrointestinal bleeding probably due to intestinal ischemia. He died with extensive intestinal infarction. The infarction was most likely caused by decreased splanchnic perfusion secondary to the chronic hypotension of the nephrotic syndrome and to amyloid deposition within the walls of the small blood vessels supplying the gut. Although amyloidosis was suspected prior to death, a fixation artifact probably prevented the correct antemortem biopsy diagnosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/18. A case of familial mediterranean fever with amyloidosis as the first manifestation.We describe a 22-year-old Turkish woman with nephrotic syndrome who had a history of acute myelocytic leukemia. After careful clinical evaluation, the patient underwent a renal biopsy. light microscopic examination showed deposition of congo-positive material both in the mesangium and around the small vessels. By histochemical analyses, the deposited material was proved to be amyloid A (AA). Because the patient's history did not reveal any event that might explain the development of secondary amyloidosis, she was screened for mutations causing familial mediterranean fever (FMF) and was found to be homozygous for the M694V mutation by denaturing gradient gel electrophoresis. We recommend that FMF-phenotype II and the development of amyloid nephropathy, before or without other symptoms of FMF, should be kept in mind in the face of unexplained proteinuria/amyloidosis, especially in high-risk ethnic groups.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/18. Mesenteric thrombosis causing short bowel syndrome in nephrotic syndrome.Nephrotic patients are at risk of developing venous and arterial thrombotic complications. pulmonary embolism due to affected deep leg veins is by far the most common event. Renal or cerebral vein thromboses have been described. thrombosis of arterial vessels is less frequent. Mesenteric infarction is a rare but severe complication in patients with nephrotic syndrome (NS). We report a 7-year-old boy with a steroid-dependent (SD) NS and a homozygous mutation of methylenetetrahydrofolate reductase, increasing the risk of thromboembolic events. He developed a thrombosis of his superior mesenteric artery during his ninth relapse, which was responsible for a necrosis of 240 cm of his small bowel, necessitating resection of necrotic parts and double external ostomy diversion. Remission was achieved with pulse prednisolone therapy. Corticoids were reduced over 4 months progressively. Oral cyclosporin A (CyA) was initiated for long-term treatment. Due to a short bowel syndrome with severe malabsorption, even oral administration of 22.5 mg/kg per day CyA did not lead to sufficient plasma levels. Intravenous cyclophosphamide pulse therapy over 6 months led to a complete remission. No relapse occurred over a period of more than 5 months after the last cyclophosphamide pulse. Anticoagulation and screening for increased susceptibility for thrombotic events are necessary in every nephrotic patient. Intravenous cyclophosphamide pulse therapy is a useful alternative in SDNS with impaired intestinal absorption of applied immunosuppressive drugs.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/18. A male with angioimmunoblastic T-cell lymphoma and proliferative glomerulonephritis.In this article we present the case report of a 67-year-old male with a nephrotic syndrome due to a proliferative glomerulonephritis, associated with an angioimmunoblastic T-cell lymphoma. diagnosis was made on an axillary lymph node biopsy and showed expanded T-cell areas with multiple blood vessels, small mature lymphocytes, eosinophils, and plasma cells. A kidney biopsy was suggestive for a proliferative glomerulonephritis with intra- and extracapillary proliferation. Hypercellular glomeruli were seen, as well as multiple floride crescents. Interstitial edema and fibrosis were absent. Immunohistochemical reactions were negative; there was some mesangial reaction with IgM in the glomeruli. Treatment with high-dose corticosteroids was initiated, with clinical improvement, and was immediately followed by therapy with cyclophosphamide, hydroxydaunomycin, vincristine, and prednisone (CHOP), which induced complete remission with a follow-up of 1 year. To our knowledge, the association of angioimmunoblastic T-cell lymphoma and proliferative glomerulonephritis has only been described twice. It concerned elderly men who developed acute renal failure a couple of months after the diagnosis of an angioimmunoblastic T-cell lymphoma. In both, immunoglobulin-containing dense deposits within glomeruli were observed, which was not the case in our patient, where only some mesangial colorization of the IgM in the glomeruli was seen.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/18. breast cancer with nephrotic syndrome: report of two cases.We report two cases of women found to have breast cancers within a few months of being diagnosed with nephrotic syndrome. Case 1 was a 53-year-old Japanese woman in whom breast cancer was diagnosed 14 months after the onset of nephrotic syndrome. The histological diagnosis was invasive ductal carcinoma with no lymph node metastasis. We performed a modified radical mastectomy, after which the proteinuria and hypoproteinemia resolved almost completely, and the patient has been disease-free for 5 years since. Case 2 was a 61-year-old Japanese woman in whom breast cancer was diagnosed 2 months after the onset of membranous nephropathy. We performed a modified radical mastectomy and the histological diagnosis was invasive ductal carcinoma with marked lymphatic vessel permeation and involvement of five axillary lymph nodes. proteinuria and hypoproteinemia did not resolve postoperatively and there is a high possibility of remnant or recurrent cancer. To our knowledge, there are only four other reported cases of paraneoplastic membranous nephropathy complicating breast cancer. However, we speculate that the postoperative resolution of nephrotic syndrome might be a measure of cancer control.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/18. nephrotic syndrome and angiotropic lymphoma report of a case.A case of angiotropic lymphoma involving renal glomeruli and interstitial vessels associated with nephrotic syndrome and with minor lesions in the glomerular basal membrane is reported. A 56-year-old woman had fever, weakness and clinical findings of a nephrotic syndrome with normal renal function. Renal biopsy revealed that the glomeruli were infiltrated by neoplastic lymphoid cells positive for CD20 and CD45; the glomerular basement membranes showed a pattern of minimal change disease. This case and our review of the literature suggest that the rare association of intravascular lymphoma and glomerular disease is more than coincidental.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/18. Secondary amyloidosis causing nephrotic syndrome in a patient with non-Hodgkin's lymphoma: quite a rare diagnosis.Secondary amyloidosis is usually a complication of chronic inflammation. amyloidosis cases during the course of non-Hodgkin's lymphoma (NHL) are usually of AL-type, only one NHL patient with secondary amyloidosis has been reported. Our 79-year-old male patient visited us with multiple lymphadenopathies, and he was diagnosed with nodal marginal zone B-cell lymphoma. After four cycles of combined chemotherapy; his urea, creatinine levels started to increase and he developed nephrotic-range proteinuria. His rectal biopsy demonstrated amyloid deposition in submucosal vessel walls. The patient has been under hemodialysis for 10 months and his lymphoma is still in partial remission. We presented this case because it is the second NHL patient who developed secondary amyloidosis during his disease course.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/18. High concentrations of free kinins and kinin system components in abdominal transudate of a patient with nephrotic syndrome.High levels of bradykinin (60--80 ng/ml) were found in abdominal transudate from a patient with nephrotic syndrome caused by chronic glomerulonephritis. The abdominal transudate contained neutral kininogenase and its precursor, identified with plasma kallikrein and prekallikrein, respectively, as well as both forms of kininogen, the low-molecular-weight form predominating. The abdominal transudate was characterized also by very low kininase activity and low levels of alpha 1-antitrypsin (0.46 g/l) and alpha 2-macroglobulin. Large amounts of very low density lipoproteins were present in the transudate. Despite the difference in total protein content between the abdominal transudate and the patient's serum (4.3 g/l and 48 g/l, respectively) their protein fraction composition was similar. The data obtained suggest that bradykinin is important in maintenance of long-lasting blood vessel hyperpermeability, which, in turn, is a driving force in the pathogenesis of refractory nephrotic edema.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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