Nephrotic syndrome, renal vein thrombosis and renal failure. Report of case with recovery of renal function, loss of proteinuria and dissolution of thrombus after anticoagulant therapy

A case of nephrotic syndrome, renal vein thrombosis and advanced renal failure in a 52 year old woman with adult onset diabetes mellitus is reported. The diagnosis of renal vein thrombosis was established by selective renal venography. Following 15 months of anticoagulant therapy creatinine clearance increased to the level noted prior to renal vein thrombosis, and proteinuria decreased to less than 500 mg/day. Repeat renal venography demonstrated clearing of the renal vein thrombosis. Renal biopsy performed at this time revealed changes compatible with arterial nephrosclerosis on light microscopy, but there were no findings typical of diabetic intercapillary glomerulosclerosis. No immune deposits were seen on immunofluorescent microscopy. Electron microscopy revealed fusion of the foot processes, but there were no electron dense deposits in or on the basement membrane to suggest membranous glomerulonephritis. Analysis of the sequence of events suggests that the renal vein thrombosis in this patient was probably a complication of the nephrotic syndrome, the etiology of which is not clear although the biopsy findings are compatible with lipoid nephrosis.     

Membranous Glomerulonephritis Associated with Sarcoidosis*

Summary: Two patients with sarcoidosis involving pulmonary hilar lymph nodes developed the nephrotic syndrome. Renal biopsy in both cases showed membranous glomerulonephritis. In one patient, there was an associated renal vein thrombosis.     

Membranous Glomerulonephritis Associated with Sarcoidosis*

Summary: Two patients with sarcoidosis involving pulmonary hilar lymph nodes developed the nephrotic syndrome. Renal biopsy in both cases showed membranous glomerulonephritis. In one patient, there was an associated renal vein thrombosis.     

Association of liver cirrhosis related IgA nephropathy with portal hypertension

A high incidence of IgA nephropathy has been reported in patients with liver cirrhosis, though, clinically evident nephrotic syndrome is very uncommon. Impaired hepatic clearance of circulating IgA immune complexes and subsequent deposition in renal glomeruli has been considered principally in the pathogenesis of liver cirrhosis associated IgA nephropathy. Here we report on a patient with cryptogenic liver cirrhosis and splenic vein thrombosis, who presented with nephrotic syndrome. Renal biopsy showed findings consistent with IgA nephropathy. Lower endoscopy showed features of portal hypertensive colopathy. Following initiation of propranolol and anticoagulant treatment to reduce portal pressure, a gradual decrease of proteinuria and hematuria to normal range was noted. The potential pathogenetic role of portal hypertension in the development of IgA nephropathy in cirrhotic patients is discussed.     

Immunopathology of the nephrotic syndrome associated with renal vein thrombosis. Report of two cases and brief review of the literature

Renal tissue from two patients with the nephrotic syndrome and renal vein thrombosis was studied by immunofluorescence microscopy in addition to conventional histologic and electron microscopic technics. Granular deposits of immunoglobulins G (IgG), M (IgM) and beta₁ C/beta₁ A globulin (one case) were seen by fluorescence microscopy along the basement membranes in a pattern similar to that observed in patients with chronic membranous nephropathy and in the experimental model of chronic serum sickness. Renal vein thrombosis, associated with the nephrotic syndrome, is not clearly separated clinically or pathologically from primary glomerular disease with the nephrotic syndrome. In view of the lack of experimental evidence to show that either the glomerular lesion or the proteinuria is the result of elevated venous pressure alone, the pathogenesis of the lesion must remain in doubt.     

A fulminant case of renal vein thrombosis in a patient with autoimmune disorder and membranous nephropathy

A previously healthy middle-aged woman noted a rapid onset of flank pain with gross hematuria. Enhanced CT scan showed thrombosis of the inferior vena cava and right renal vein. Laboratory findings revealed nephrotic proteinuria, Sjogren's syndrome (SjS), and Graves' disease (GD). A right nephrectomy was performed because of progressive and refractory renal necrosis. Renal specimens showed venous infarction with diffuse hemorrhagic and severe congestive renal necrosis, and membranous nephropathy (MN). The present case was diagnosed as acute renal necrosis due to catastrophic thrombosis in a patient with SjS, GD, and MN. It was thought that sudden development of thrombosis may have been caused by the status of the autoimmune disorders, and the associated MN.     

The nephrotic syndrome associated with neoplasia: An unusual paraneoplastic syndrome: Report of a case and review of the literature

The nephrotic syndrome complicating malignancy in the absence of renal vein thrombosis, amyloid or neoplastic infiltration of the kidney is an unusual occurrence. A case of diffuse, well differentiated, lymphocytic lymphoma and lipoid nephrosis documented by light microscopy, electron microscopy and immunofluorescent studies is reported. A review of the literature revealed 76 case reports in which the nephrotic syndrome was associated with neoplasia. The most frequently associated neoplasms are Hodgkin's disease, various carcinomas, nonHodgkin's lymphoma and leukemia in descending order. The most frequent renal lesion in patients with the nephrotic syndrome associated with various carcinomas is membranous glomerulonephritis (81 per cent) as opposed to patients with lymphomas or leukemias who have predominantly lipoid nephrosis (60 per cent). The evidence is reviewed suggesting that the lesions in membranous nephropathy are immunologically mediated by tumor or viral antigen-antibody complexes and in lipoid nephrosis perhaps by a defect in T-lymphocyte function.     

Flankenschmerz und Proteinurie bei einem 44-j?hrigen Mann

The case of a patient with renal vein thrombosis manifesting in nonspecific symptoms such as side pain, nausea and hematuria is presented. A definitive diagnosis was established using computed tomography. Concomitant nephrotic syndrome, a common accompanying disease of renal vein thrombosis, was caused by glomerulonephritis in our patient. This could be clarified, after 8 months of coagulation therapy for the thrombosis, by means of renal biopsy and was treated with ciclosporine. When treated appropriately (anticoagulation therapy) renal vein thrombosis with nephrotic syndrome does not carry an increased risk of mortality.     

Fibrinolytic therapy for bilateral renal vein thrombosis

A patient with nephrotic syndrome due to membranous glomerulonephritis experienced painful bilateral renal vein thrombosis (RVT) complicated by pulmonary emboli. He was treated with systemic fibrinolysis with streptokinase for 72 hours. Rapid clinical improvement of his condition, a fall in his serum creatinine level, and a sharp rise in his platelet count were observed. Venography verified complete lysis of the thrombus within the vena cava and reperfusion of the right renal vein. The left renal vein remained chronically occluded. Thrombolytic therapy should be further investigated as initial therapy for patients with clinical evidence of RVT.     

Glomerular deposition of tumor antigen in membranous nephropathy associated with colonic carcinoma

Renal biopsy in a 60 year old man with idiopathic nephrotic syndrome revealed the characteristic light, immunofluorescent and electron microscopic features of membranous nephropathy. Elevated serum levels of carcinoembryonic antigen (CEA) were present, and a colonic carcinoma was found and resected. CEA could not be demonstrated in the glomerular immune deposits. An antibody was demonstrated in the patient's serum 1 week after resection of the tumor which was reactive with an antigen deposited on the glomerular basement membrane. This reactivity was specifically abolished by absorption of the serum with homogenates of the patient's tumor, but it was not altered by absorption with normal colon, colonic polyps, liver or spleen. The nephrotic syndrome persisted after resection of the tumor. A renal biopsy 4 months later showed evidence of complex resolution, and the tumor-associated antigen was no longer detectable in glomeruli.The nephrotic syndrome associated with colonic carcinoma in this patient appeared to be mediated by glomerular deposition of immune complexes containing a tumor antigen. Apparent complete removal of the source of antigen was followed by evidence of complex resolution but had no effect on the nephrotic syndrome.     

Unilateral renal vein thrombosis and nephrotic syndrome: Report of a case with protein selectivity and antithrombin III clearance studies

A case of the nephrotic syndrome with unilateral renal vein thrombosis is reported. The patient, an 18 year old man, presented with a six month history of edema and the recent development of a left-sided varicocele. An enlarged left kidney and a thrombus in the left renal vein were demonstrated roentgenographically. A biopsy specimen of the right kidney was interpreted as membranous glomerulonephritis. Selective renal function studies showed nearly identical creatinine excretion, and similar total protein excretion and protein selectivity from each kidney. Thus, the thrombus in the left renal vein did not influence glomerular filtration rate or quantitative or qualitative protein excretion. A high urinary output and a decreased serum level of antithrombin III were measured. These findings suggest a mechanism to explain the increased thrombotic tendency seen in this and other patients with the nephrotic syndrome.     

Membranous Nephropathy Associated with Donor Lymphocyte Infusion following Allogeneic Bone Marrow Transplantation

Nephrotic syndrome after hematopoietic stem cell transplantation (HSCT) followed by donor lymphocyte infusion (DLI) has never been described. We report the case of a myelodysplastic syndrome (MDS) patient who developed nephrotic syndrome with membranous nephropathy 18 months after allogeneic HSCT and 4 months after DLI. A 50-year-old woman with MDS underwent allogeneic bone marrow transplantation from her HLA-matched brother. MDS relapsed 55 days after transplantation, donor lymphocytes were infused as adoptive immunotherapy, and complete remission was achieved. Four months after the third DLI, the patient developed nephrotic syndrome with proteinuria up to 9 g/day. Renal biopsy revealed granular deposits of immunoglobulin G along the glomerular basement membrane, and subepithelial electron-dense deposits. A diagnosis of membranous nephropathy was made. For maintenance of the immunotherapeutic effect of DLI, minimum doses of immunosuppressive therapy for decreasing proteinuria were administered, and improvement of nephrotic syndrome and persistent complete remission of MDS were achieved.     

Simultaneous urokinase perfusion in renal artery and vein in a case of renal vein thrombosis]

We present he case of a young man with nephrotic syndrome, caused by membranous glomerulonephritis, who developed renal vein thrombosis with extension to the inferior vena cava is presented. Renal vein thrombosis was diagnosed by echo Doppler and confirmed by angio-CT scan. At the hospitalization the patient presented a severe left flank pain, edema of the lower limbs and painful left testicular tumefaction. The treatment consisted of: 1) systemic anticoagulation with sodic heparin, 2) placement of temporary vena cava filter through the right jugular vein, 3) direct thrombolysis into endocaval thrombus with early lysis of thrombus, and 4) renal thrombolysis with selective simultaneous renal artery and renal vein infusion of urokinase. Angiography performed after 24 hours of loco-regional thrombolysis showed complete lysis of renal thrombus; clinically there was a regression of left flank pain. We conclude that, face to renal vein thrombosis, thrombolytic treatment with simultaneous renal artery and renal vein perfusion is mandatory. Furthermore it is very important, in presence of caval extension of renal thrombus, to place a temporary vena cava filter before starting thrombolysis, considering the high risk of pulmonary embolism related to this pathology.     

Nephrotic syndrome in Hodgkin's disease. Evidence for pathogenesis alternative to immune complex deposition.

The nephrotic syndrome has been reported to occur in patients with Hodgkin's disease even in the absence of amyloidosis, tumor infiltration of renal vein thrombosis. Three patients are presented with Hodgkin's disease and the nephrotic syndrome whose renal biopsy specimens studied with light, immunofluorescence and electron microscopy were compatible with "lipoid nephrosis" (minimal change disease). A review of the literature reveals 35 patients with Hodgkin's disease and the nephrotic syndrome. Renal tissue was available for examination in only 27 patients. The majority of patients apparently had glomerular alterations consistent with lipoid nephrosis. The nephrotic syndrome in most of these patients remitted with a variety of methods of therapy (including excision, irradiation, prednisone and cyclophosphamide) and tended to relapse with a recurrence of Hodgkin's disease. In three-fourths of the patients with Hodgkin's disease and the nephrotic syndrome, the Hodgkin's disease was of a mixed cellularity type. The etiology of lipoid nephrosis, although unclear, may be a consequence of altered lymphocyte function. Hodgkin's disease is a malignancy involving T lymphocytes, and the nephrotic syndrome occurring in the course of Hodgkin's disease may be a result of an adverse effect of glomeruli by products of tumor lymphocytes rather than of glomerular deposition of immune complexes.     

Arterial thrombosis due to minimal change glomerulopathy secondary to nonsteroidal anti-inflammatory drugs.

The authors report a 49-year-old woman who had been treated with diclofenac for her back pain. Nephrotic syndrome followed by occlusion of the right profound femoral and popliteal arteries occurred. Successful thrombectomy was performed. Another nonsteroidal anti-inflammatory drug (NSAID) was administered by injection during hospitalization. Renal biopsy showed minimal change glomerulopathy (MCG). Remission was delayed despite large-dose steroid administration. The delayed remission in this patient may be caused by impaired renal function and the possible effect of a second period of NSAID administration. The authors present the first case of femoral arterial thrombosis associated with nephrotic syndrome secondary to NSAID-associated MCG. Careful history-taking and physical examination may detect early causes and complications of nephrotic syndrome. Any rechallenge of NSAID should be avoided.     

Nephrotic syndrome associated with adenocarcinoma of the breast

Although the nephrotic syndrome has been reported in association with a wide variety of neoplastic diseases, it has only rarely been noted as a complication of breast carcinoma. We describe a patient who presented with the nephrotic syndrome and who was subsequently found to have carcinoma of the breast. The findings on renal biopsy were indicative of membranous nephropathy with positive immunofluorescent staining for immunoglobulin G (IgG) and the third component of complement (C3). The nephrotic syndrome completely resolved following successful treatment of the cancer. We have now followed this patient for over two years and there has been no recurrence of tumor or the nephrotic syndrome.     

Venous thromboembolism in patients hospitalized with nephrotic syndrome

Purpose

Whether pulmonary embolism in patients with the nephrotic syndrome is caused by deep venous thrombosis or renal vein thrombosis is controversial. To determine which is the likely cause of pulmonary embolism in patients with the nephrotic syndrome, we investigated data from the National Hospital Discharge Survey.

Methods

The number of patients discharged from nonfederal short-stay hospitals in the United States with a diagnostic code of nephrotic syndrome, deep venous thrombosis, renal vein thrombosis, and pulmonary embolism was obtained using ICD-9-M (International Classification of Diseases, Ninth Revision, Clinical Modification) codes.

Results

From 1979 to 2005, 925,000 patients were discharged from hospitals with the nephrotic syndrome and 898,253,000 patients did not have the nephrotic syndrome. With the nephrotic syndrome, 5000 (0.5%) had pulmonary embolism, 14,000 (1.5%) had deep venous thrombosis, and fewer than 5000 had renal vein thrombosis. The relative risk of pulmonary embolism comparing patients with the nephrotic syndrome to those who did not have it was 1.39, and the relative risk of deep venous thrombosis was 1.72. Among patients aged 18-39 years, the relative risk of deep venous thrombosis was 6.81. From 1991-2005, after venous ultrasound was generally available, the relative risk of deep venous thrombosis (all ages) was 1.77.

Conclusion

The nephrotic syndrome is a risk factor for venous thromboembolism. This is strikingly apparent in young adults. Renal vein thrombosis was uncommon. Therefore, pulmonary embolism, if it occurs, is likely to be due to deep venous thrombosis and not renal vein thrombosis.     

Membranous glomerulonephritis in chronic lymphocytic leukemia

We present a case of chronic lymphocytic leukemia associated with nephrotic syndrome. Renal biopsy revealed membranous glomerulonephritis accompanied by interstitial monoclonal lymphocytic infiltration. Combination therapy with cyclophosphamide, vincristine, and prednisone (COP) was successful in inducing an effective response in chronic lymphocytic leukemia. Improvement of renal function and proteinuria was also observed.     

Symptomatic thrombotic events among Egyptian patients with systemic lupus erythematosus: special consideration for renal vein thrombosis

OBJECTIVE: To evaluate the prevalence of symptomatic thrombotic events among Egyptian patients with systemic lupus erythematosus (SLE), and to evaluate the frequency and the risk factors associated with renal vein thrombosis in those patients. METHODS: Fifty-four patients with SLE, 51 (94.4%) females, were involved in this study. All of them were submitted for abdominal sonography, chest X-ray, echocardiography, and Doppler of renal, abdominal and lower limb veins, with examination of data on clinical and laboratory profile. Abdominal CT, brain MRI, MRI both hips, CT chest and pulmonary scintigraphy were used when needed. RESULTS: Sixteen patients (29.6%) were diagnosed with symptomatic thrombotic events. Eight patients had more than one type of thrombosis. Two patients (3.7%) were diagnosed by Doppler as having renal vein thrombosis (RVT). This was confirmed by abdominal CT. One of them presented with nephrotic syndrome, graded by renal biopsy as World Health Organization (WHO) class V, and had positive anticardiolipin antibodies (ACL). The other patient had RVT and inferior vena cava (IVC) thrombosis, nephrotic syndrome, positive ACL, and died before renal biopsy was performed. Both of them were without history of peripheral thrombotic events. One patient was diagnosed with IVC thrombosis, lupus nephritis grade II, positive ACL, and diagnosed by abdominal CT. One patient was diagnosed with portal vein thrombosis and had positive ACL. One patient with retinal vessel thrombosis and positive ACL. Four patients had deep vein thrombosis (DVT). Recurrent miscarriages were reported in 4 patients (7.4%), skin ulcerations in 3 (5.6%), avascular necrosis of the hips in 4 (7.4%), stroke in 1 (1.9%), and pulmonary hypertension in 2 patients (3.7%). CONCLUSION: Sixteen SLE patients (29.6%) were diagnosed with symptomatic thrombotic events. RVT was detected in 2 patients representing 3.7% of all patients, and 12.5% of patients with thrombosis. Both patients with RVT presented with nephrotic syndrome.     

Chronic renal vein thrombosis

Twenty-eight patients with demonstrated chronic renal vein thrombosis were studied. In seven, only small venous channels were involved; in 21, both small and large veins were thrombosed. A constellation of findings occurred with such frequency in these patients that we believe it virtually diagnostic of renal venous obstruction. These findings include the nephrotic syndrome, great variability in proteinuria and glomerular filtration rate, pulmonary embolization, sterile pyuria, hematuria, hyperchloremic acidosis, decreased renal tubular threshold for glucose and increased fibrin degradation products. These findings are an indication for definitive angiographic and biopsy procedures. Prolonged anticoagulant therapy was generally very effective.