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.     

Renal vein thrombosis and the nephrotic syndrome. Report of two cases with successful treatment of one

A nephrotic syndrome and bilateral renal vein thrombosis developed in two women. One suffered low back pain, the other oliguric renal failure. Both suffered pulmonary emboli and had exhibited edema, proteinuria and hypertension during pregnancies several years before. Results of thrombectomy and anticoagulant therapy were successful in one patient, although she suffered serious hemorrhage at the biopsy site during anticoagulant therapy. The other patient died of pulmonary embolism. Renal interstitial changes usually associated with renal vein thrombosis were found in both, but neither showed membranous nephropathy (membranous glomerulonephritis). This study suggests that (1) although membranous nephropathy is frequently associated with renal vein thrombosis, it is probably not the result of that condition nor invariably associated with it. (2) Prognosis in renal vein thrombosis with nephrotic syndrome may be partially dependent upon underlying renal disease. (3) Renal biopsy is useful for diagnosis and assaying prognosis. (4) Treatment should consist of thrombectomy and anticoagulants. However, serious hemorrhage may occur in patients recently subjected to biopsy.     

Atteinte coronarienne et syndrome néphrotique au cours du lupus systémique : à propos d’une observation

IntroductionHeart failure during systemic lupus erythematosus has various causes.Case reportA 29-year-old female presented with a systemic lupus flare and a nephrotic syndrome, followed by cardiogenic shock requiring extra-corporeal membranous oxygenation. Ventricular dysfunction was related to massive myocardial infarction due to an anterior interventricular artery thrombosis and an underlying atheroma. The young age and the absence of chest pain were not suggestive of coronary artery disease initially. Coronary thrombosis was probably favored by the nephrotic syndrome, in which the arterial thrombotic risk is increased.ConclusionCoronary artery disease should be systematically evoked in the presence of ventricular dysfunction in patients with systemic lupus, including when they are young and in the absence of chest pain. Nephrotic syndrome should be identified as a risk factor for arterial thrombosis.     

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.     

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.     

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.     

Collapsing focal segmental glomerulosclerosis in a patient with oral cavity cancer: A case report

Rationale:Focal segmental glomerulosclerosis (FSGS) is one of the most common glomerular diseases, leading to end-stage renal disease. Among the 5 variants of FSGS, the collapsing variant is rare and has the worst prognosis. Solid and hematologic malignancies are associated with glomerular diseases, such as membranous nephropathy, minimal change disease, and FSGS. However, squamous cell carcinoma of the oral cavity is rarely associated with nephrotic syndrome, especially FSGS.Patient concerns:A 55-year-old woman diagnosed with oral cavity cancer presented with generalized edema with heavy proteinuria and renal dysfunction after neoadjuvant chemotherapy and wide surgical excision.Diagnosis:Renal biopsy shows segmental or global collapse of glomerular capillaries with marked hyperplasia and swelling of overlying epithelial cells, suggesting a collapsing variant of FSGS.Interventions:After the renal biopsy, we prescribed oral prednisolone at a dose of 1 mg/kg/day. Despite immunosuppressive treatment, renal function deteriorated, and hemodialysis was started.Outcomes:After 23 sessions of hemodialysis and high-dose oral glucocorticoid treatment, renal function gradually improved, and oral glucocorticoid therapy was discontinued after 8 months. Currently, this patient is in a cancer-free state and has normal renal function without proteinuria.Lessons:Unusual collapsing FSGS might be associated with neoadjuvant chemotherapy and wide surgical excision in patients with oral cavity cancer. Proper diagnostic workup, such as renal biopsy and high-dose glucocorticoid therapy, might have helped recover from nephrotic syndrome and acute renal injury in cancer patients.     

Membranous nephropathy and crescentic glomerulonephritis

The most frequent causes of glomerular diseases whose main clinical syndrome are nephrotic syndrome and acute renal failure may have several causes: acute tubular necrosis, thrombosis of renal veins, acute tubulointerstitial nephritis. Infrequently, the association between primary glomerular disease (membranous nephropathy and others) and crescentic glomerulonephritis can cause this clinical picture. We describe a young woman without systemic disease with nephrotic syndrome and acute renal failure secondary to membranous nephropathy and superimposed crescentic glomerulonephritis. She received steroids and cyclophosphamide with stabilization of renal function after two months of follow-up.     

Antithrombin III and the Nephrotic Syndrome

Plasma and urinary antithrombin III (AT-III) was measured in 15 cases of nephrotic syndrome. Plasma AT-III correlated well with serum albumin, but poorly with pro-teinuria, whereas urinary AT-III correlated well to proteinuria. The plasma AT-III level had a mean similar to 25 healthy controls, but the range was significantly wider. A case with nephrotic syndrome and left renal vein thrombosis is reported. The urinary output of AT-III rose and the plasma level fell with the activity of the disease. Although AT-III and albumin have similar molecule weight, their renal clearance was found to be different. It is suggested that urinary loss of AT-III plays a role in the hypercoagulable state sometimes found in the nephrotic syndrome.     

Crohn’s disease associated with renal amyloidosis successfully treated with an elemental diet

We report a case of Crohn’s disease associated with nephrotic syndrome due to renal amyloidosis in a 21-year-old man in whom remission of both Crohn’s disease and the nephrotic syndrome has been maintained with an elemental diet. The patient developed toxic megacolon and nephrotic syndrome due to renal amyloidosis. Intensive intravenous prednisolone therapy with total parenteral nutrition was dramatically effective in treating the toxic megacolon and inducing remission in Crohn’s disease and afterward, remission of the nephrotic syndrome. Remission of both conditions has been maintained for more than 2 years with the elemental diet. To our knowledge, this is the first confirmed case of Crohn’s disease complicated with renal amyloidosis in which only slight proteinuria (below 0.3 g/day) was shown with an elemental diet used for a long period.     

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.     

Steroid-resistant nephrotic syndrome in infants caused by a novel compound heterozygous mutation of the NUP93: A CARE case report

Rationale:Steroid-resistant nephrotic syndrome (SRNS) is a special kidney disease. SRNS is characterized by steroid-resistant, clinical variability, and genetic heterogeneity. Patients with SRNS often may eventually need renal transplantation.Patient concerns:A 10-month-old Chinese male infant presented with oliguria, renal dysfunction, hypertension, and anemia.Diagnoses:Combined with clinical manifestations, laboratory testing and sequencing results, the patient was diagnosed as SRNS.Interventions:Combined intravenous methylprednisolone and cefoperazone sulbactam did not improve the patient''s condition. Thus, SRNS associated with hereditary nephrotic syndrome was strongly suspected. Genetic testing for hereditary renal disease of the patient revealed 2 novel heterozygous mutations in the Nucleoporin 93 (NUP93) gene, which were predicted pathogenic and harmful by bioinformatic softwares of SIFT, PolyPhen_2 and REVEL.Outcomes:As general physical health deterioration and renal dysfunction, the patient died of a severe infection.Lessons:The novel NUP93 heterozygous mutations identified in the current study broadened the genetic spectrum of SRNS and further deepened our insight into pathogenic mutations of NUP93 to improve disease diagnosis.     

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.     

Nephrotic syndrome in childhood

Childhood nephrotic syndromes are most commonly caused by one of two idiopathic diseases: minimal-change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS). A third distinct type, membranous nephropathy, is rare in children. Other causes of isolated nephrotic syndrome can be subdivided into two major categories: rare genetic disorders, and secondary diseases associated with drugs, infections, or neoplasia. The cause of idiopathic nephrotic syndrome remains unknown, but evidence suggests it may be a primary T-cell disorder that leads to glomerular podocyte dysfunction. Genetic studies in children with familial nephrotic syndrome have identified mutations in genes that encode important podocyte proteins. Patients with idiopathic nephrotic syndrome are initially treated with corticosteroids. Steroid-responsiveness is of greater prognostic use than renal histology. Several second-line drugs, including alkylating agents, ciclosporin, and levamisole, may be effective for complicated and steroid-unresponsive MCNS and FSGS patients. Nephrotic syndrome is associated with several medical complications, the most severe and potentially fatal being bacterial infections and thromboembolism. Idiopathic nephrotic syndrome is a chronic relapsing disease for most steroid-responsive patients, whereas most children with refractory FSGS ultimately develop end-stage renal disease. Research is being done to further elucidate the disorder's molecular pathogenesis, identify new prognostic indicators, and to develop better approaches to treatment.     

Simultaneous manifestation of acute myocardial infarction and nephrotic syndrome

This report describes the simultaneous manifestation of ischemic heart disease and nephrotic syndrome in a 37-year-old woman presenting with acute anterior myocardial infarction. Symptoms of nephrotic syndrome, such as facial and peripheral edema accompanied by proteinuria and hyperlipidemia, and onset of severe retrosternal pain developed within 24 h. Coronary angiography revealed a complete thrombotic occlusion of the proximal portion of the left anterior descending artery with no evidence of arteriosclerotic lesions. Histologic examination of renal biopsy, including electron microscopy, revealed evidence of minimal change glomerulonephritis. Ultrastructural studies demonstrated widespread effacement of epithelial foot processes. Elevated levels of circulating fibrinogen appeared to be an important factor for the hypercoagulable state in this patient, suggesting a causative relationship between coronary thrombosis and nephrotic syndrome.     

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.     

A review of nephrotic syndrome associated with chronic lymphocytic leukemia

Four patients had the nephrotic syndrome and chronic lymphocytic leukemia (CLL), which may be pathogenetically related. Membranoproliferative glomerulonephritis appears to be the most common glomerular lesion in patients with CLL. Available evidence suggests that nephrotic syndrome associated with CLL is often related to immune-complex disease. It is also possible that disorders of immunoglobulin production and cellular immunity contribute to renal disease in patients with CLL.     

Renal vein thrombosis and inferior vena cava thrombosis in systemic lupus erythematosus

Phlebography of the inferior vena cava with selective study of the renal veins was performed in 43 patients with systemic lupus erythematosus (SLE). Inferior vena cava thrombosis (IVCT) or renal vein thrombosis (RVT) was found in 3 of 11 patients (27%) with nephrotic syndrome, in 8 of 13 (61.5%) with previous thrombophlebitis, and in 3 of 4 (75%) with suggestive acute clinical picture. In contrast, none of the 20 control patients with SLE had IVCT or RVT. These results show that SLE patients with thrombophlebitis have a very high risk of developing IVCT or RVT; patients with nephrotic syndrome have a smaller risk. Neither IVCT nor RVT was found in SLE patients without antecedent thrombophlebitis or nephrotic syndrome.     

Paraneoplastic glomerular diseases and malignancies

Paraneoplastic glomerulopathies are rare manifestations of neoplastic disease to be distinguished from iatrogenic renal damage. Solid tumors are preferentially associated with membranous nephropathy, whereas Hodgkin's lymphomas are associated with minimal change disease. The most common neoplasia associated with paraneoplastic glomerular disease are carcinomas of the lung and of the gastrointestinal tract. Nephrotic syndrome is the most frequent presentation of paraneoplastic glomerulopathy and the most critical glomerular disease regarding prognosis and patient care. Renal biopsy is recommended in patients with glomerular proteinuria or nephrotic syndrome and cancer, depending on life expectancy and therapeutic options. The primary treatment must be directed at the cancer in all cases. Symptomatic treatment of the nephrotic syndrome with diuretics and ACE inhibitors is justified. Prevention of nephrotic syndrome complications, i.e. thromboses and infections, should also be addressed and systematic regular renal follow-up is warranted. All treatments should be regularly reviewed to avoid toxicity, associated renal function loss or low albumin levels for patients receiving albumin-binding drugs. Epidemiologic studies have low evidence-based value. There is no widely accepted experimental model of the association of glomerulopathy and cancer. Thus, epidemiologic and mechanistic studies are needed to determine the true prevalence of paraneoplastic glomerulopathies and investigate new pathophysiologic approaches.     

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.