Idiopathic nephrotic syndrome of the adult with asymptomatic thrombosis of the renal vein

Twenty-six adult patients with idiopathic nephrotic syndrome, ages ranging from 16 to 62 years, were prospectively evaluated with selective renal venogram for the presence of renal vein thrombosis (RVT). Ten patients had membranoproliferative glomerulonephritis (MPGN); 5, membranous nephropathy (MGN); 3, diffuse proliferative glomerulonephritis (DGPG), and 1 focal glomerulonephritis (FPG). Renal vein thrombosis was observed in 11 patients. The primary nephropathies in these patients were: MPGN in 4, MGN in 3, FGS in 2, FPG in 1, and DGPG in 1. All patients were asymptomatic. The clinical and renal pathology features were similar in patients with and without RVT. Other thromboembolic complications were observed in 4 patients. In conclusion, renal vein thrombosis was observed in 42% of our patients and MPGN was the most frequent nephropathy associated with RVT.     

Proteinuria following transplantation. Correlation with histopathology and outcome

A review of 693 renal transplant recipients revealed 77 (11%) in whom persistent, heavy proteinuria (greater than 2 g/24 hr) developed. Renal histology was available in all 77 patients. Twenty-one patients had received kidneys from living-related donors, the remaining 56 from cadaveric donors. The cause of proteinuria in these 77 patients was as follows: transplant glomerulopathy (30), allograft glomerulonephritis (22), chronic rejection (21), renal vein thrombosis (2), diabetic glomerulosclerosis (1), and hypertensive nephrosclerosis (1). Of the 22 patients who developed glomerulonephritis in the transplanted kidney, 6 had recurrent disease (3--membranous glomerulopathy, 2--focal sclerosis and hyalinosis, 1--membranoproliferative glomerulonephritis); 6 developed de novo glomerulonephritis; and in 10 the type of glomerulonephritis could not be classified as recurrent or as de novo because of lack of characterization of the original kidney disease. Renal vein thrombosis occurred in association with other lesions in an additional 5 cases (3--chronic rejection; 2--membranous glomerulopathy). In follow-up only 23.4% (18 of 77) of the patients maintained prolonged graft function; the majority of grafts being lost within one year of the development of persistent, heavy proteinuria. Of the 18 patients who retained their grafts, 8 had glomerulonephritis, 5 transplant glomerulopathy, and 5 chronic rejection. This study confirms the poor prognosis that has been reported with the development of nephrotic-range proteinuria in renal allograft recipients.     

The Prognosis of Renal Vein Thrombosis: A Re-evaluation of 27 Cases

Twenty-seven patients with renal vein thrombosis were retrospectivelystudied to evaluate their long-term prognosis and relevant prognosticfactors. Twenty-four patients presented with a nephrotic syndrome, and15 had renal impairment (8 acute; 7 moderate). Ten patientshad a previous history of proteinuria, and 14 of nephrotic syndrome.Renal biopsy performed in 20 patients, of whom 19 were nephrotic,showed membranous glomerulonephritis in 14, focal segmentalglomerulosclerosis in three, minimal change glomerulonephritisin two, and periarteritis nodosa in one. Renal vein thrombosiswas angiographically proven in all patients and was bilateralin 18, localised to the left renal vein in seven, and to theright in two. Thrombosis of the inferior vena cava was associatedin seven patients. Ten patients were treated by anticoagulants alone, nine by surgicalthrombectomy, seven by thrombolysis, and two did not receiveany specific treatment. One patient underwent successively thrombectomyand then thrombolysis. Eleven patients died within the first6 months, mainly from haemorrhagic complications (n = 5) orsevere sepsis (n = 2). Survivors were followed up from 6 monthsto 19 years. Nephrotic syndrome improved or even disappearedin 12 patients, and renal function did not worsen throughoutthe follow-up in any patients. The main prognostic factors were initial renal function andtype of nephropathy: patients with membranous glomerulonephritishad a significantly better renal function and a lower mortalityrate than patients with other nephropathies. Initial renal insufficiencywas significantly associated with a poor prognosis. There wasno advantage, in terms of survival, kidney function and nephroticsyndrome, of either thrombectomy or thrombolysis over anticoagulantsalone, despite two complete venous recanalisations after thrombolysis. Accordingly, patients with renal vein thrombosis from membranousglomerulonephritis should be treated by anticoagulants alone,since the long-term prognosis of this disease seems unaffectedby intercurrent renal vein thrombosis. With respects to therisk-to-benefit ratio, thrombectomy should be avoided and thrombolysisconsidered only in patients with initial acute renal failurefrom acute renal vein thrombosis.     

Membranous glomerulopathy following kidney transplantation. Association with renal vein thrombosis in two of nine cases

The association of renal vein thrombosis and membranous glomerulopathy in native kidneys has been well documented. However, this association has only occasionally been described following renal transplantation. A review of 693 renal transplant recipients revealed 77 (11%) in whom persistent, heavy proteinuria (greater than 2 g/24 hr) developed. Renal histology was available in all 77 patients. A diagnosis of membranous glomerulopathy was made in 7 patients. Two further cases were added on the basis of biopsy findings--1 patient had a protein excretion of 1.7 g/24 hr, the other had microscopic hematuria and red cell casts in the urine. Renal venography was performed in 4 cases. The decision to perform venography was based on clinical criteria (thrombophlebitis or marked edema of the leg on the side of the allograft), and/or histological criteria (associated interstitial edema and venous congestion). Renal vein thrombosis was present in 2 patients. Three of the 9 patients had membranous glomerulopathy as the cause of their end-stage renal disease; 2 of these patients had received kidneys from living-related donors. Four of the patients were classified as having de novo membranous glomerulopathy on the basis of having a different cause for their end-stage renal disease. Two patients were classified as having unspecified membranous glomerulopathy; both of these patients had had chronic glomerulonephritis, but there was lack of characterization of the original glomerular disease. Seven of the 9 patients continued to have stable allograft function 1-12 months after the diagnosis of membranous glomerulopathy was made in the renal allograft. The remaining 2 patients both had associated renal vein thrombosis; 1 had progressive deterioration of renal function and returned to dialysis 24 months after the diagnosis of membranous glomerulopathy had been made, while the other died of unrelated causes 27 months after the diagnosis had been made.     

Behcet's syndrome associated with minimal change glomerulonephritis and renal vein thrombosis

A 35-year-old Kashmiri male with a 12-year history of recurrent aphthous ulcers of the mouth and scrotal ulcers was admitted with pedal edema, facial puffiness and proteinuria. Kidney biopsy showed minimal change glomerulonephritis. During hospitalization he developed erythema nodosum and left renal vein thrombosis and features of right cerebral thrombosis. He improved with a course of heparin and prednisolone. In this report a case of Behcet's syndrome with minimal change glomerulonephritis and left renal vein thrombosis is presented.     

A case of nephrotic syndrome associated with pulmonary infarction and renal vein thrombosis. (A review of literature)

A case of nephrotic syndrome found by pulmonary infarction associated with renal vein thrombosis was reported. The renal biopsy showed the presence of membranous glomerulonephritis. The patient had the increase of serum level of the coagulation factor II, V, fibrinogen and FDP, and the level of urine FDP. These laboratory data suggested that the hyper coagulation state of his blood caused by nephrotic state, induced his multiple thromboembolism. We surveyed 47 case report abstracts of nephrotic syndrome patients having thromboembolism on the Japanese Journal of Nephrology published from 1974 to 1986. The histopathology of their renal biopsies showed the high incidence of membranous glomerulonephritis, followed by minimal change nephrotic syndrome and focal glomerular sclerosis. Renal vein thrombosis was the most common thromboembolism in the reports, followed by thrombosis of cerebral vessels, peripheral vessels and pulmonary artery. Some patients died of pulmonary infarction or myocardial infarction. These data showed that we must keep in mind of the possibility of thromboembolism in the treatment of nephrotic syndrome.     

Spontaneous thrombosis of the renal vessels. Rare entities to be considered in differential diagnosis of patients presenting with lumbar flank pain and hematuria.

We report 2 cases of spontaneous thrombotic occlusion of the main renal vessels presenting with acute lumbar flank pain and hematuria suspect of nephrolithiasis. Clinical and laboratory signs of blood hypercoagulability, generalized arterial embolism, nephrotic syndrome or glomerulonephritis were absent. Excretory urography, nephrosonography and retrograde ureteropyelography showed no evidence of upper urinary tract calculi or other causes of obstruction. Renal angiography and cavography demonstrated an acute renal vein thrombosis in 1 patient and a thrombotic occlusion of all but one of the segmental renal arteries in the other patient. These 2 cases demonstrate that thrombotic occlusion of the renal artery or renal vein has to be considered in patients who are presenting with lumbar flank pain and hematuria, in whom the excretory urogram shows severe malfunction of one of the kidneys, and stone disease can be excluded. Renal angiography and cavography as well as CT scan should be carried out in these patients. When the disease is diagnosed at an early stage, an intra-arterial thrombolysis can be attempted.     

Venous thrombectomy in patients presenting with iliofemoral vein thrombosis after renal transplantation

In this study 14 patients presented with 15 episodes of iliofemoral vein thrombosis after renal transplantation. Seven patients (group 1) had viable renal grafts and were treated with conventional anticoagulation. Eight patients (group 2) had non-viable renal grafts and were subjected to graft nephrectomy and simultaneous venous thrombectomy without anticoagulation. The patients in group 2 had rapid resolution of the signs and symptoms of the iliofemoral vein thrombosis, and noninvasive vascular investigation at follow-up revealed competent and patent deep veins in all patients. In contrast, only 50% of the patients in group 1 had normal venous studies at follow-up. We recommend that renal transplant recipients who develop iliofemoral vein thrombosis and nonviable allograft postoperatively should be subjected to venous thrombectomy at the time of graft nephrectomy.     

Unilateral renal vein occlusion in rats

To study the relationship of renal vein thrombosis to membranous glomerulonephritis with the nephrotic syndrome, we attempted to simulate the former by occluding to 0.5 mm one renal vein in rats. Although increased proteinuria did occur during the first 3 days after such occlusion, there was little difference from control animals in the amount of proteinuria thereafter, up to 46 days, and no evidence of membranous glomerulonephritis by light, immunofluorescent, or electron microscopy.     

Renal vein thrombosis.

The manifestations, clinical course and treatment of 14 patients with non-malignant renal vein thrombosis are described. Most patients (10 of 14) had generalized vague illness and nephrotic syndrome but 4 were initially seen with acute symptoms of flank pain, hematuria or hypertension. Renal vein thrombosis affected young men 2.5 times more often than women and occurred on the left side 2.6 times more commonly than on the right or both sides. Red blood cell casts in the urinary sediment, heavy proteinuria and hypoalbuminemia were useful indicators of the disease. Excretory urographic signs were suggestive of renal vein thrombosis in all patients and these were corroborated by angiographic studies. Systemic anticoagulation with or without a renal failure program and diuretics, or simply a combination of the last 2 modalities, was used in 9 patients. In 2 of the 9 patients who were unresponsive the adjuvant use of cyclophosphamide and steroids effected a cure. The remaining 5 patients underwent nephrectomy or thrombectomy. All 14 patients were followed for 1 to 7 years (mean 1.6 years). Ten patients were cured or improved, 1 patient was unchanged, and in the remaining 3 patients the condition deteriorated and they subsequently required a renal allograft. The rationale for various forms of treatment is discussed.     

Spontaneous rupture of renal allografts: the importance of renal vein thrombosis in the cyclosporin era

Spontaneous renal allograft rupture occurring within 14 days of transplantation occurred in 15 patients from 791 consecutive transplants. In each of eight patients treated with azathioprine and prednisolone there was pathological evidence of rejection and only two patients had thrombosis of the renal vein. Of the seven cases occurring in patients treated with triple therapy regimen (low dose cyclosporin, prednisolone and azathioprine), histological evidence of rejection was present in only three cases, but renal vein thrombosis was found in all seven. Spontaneous rupture of a transplanted kidney, a relatively uncommon complication, is more likely to be due to renal vein thrombosis than to rejection in the cyclosporin era.     

Temporary inferior vena cava filter applied to 4 patients with urological disease

A temporary inferior vena cava (IVC) filter was placed in 4 patients. Patient 1 had an advanced testicular germ cell tumor with IVC tumor thrombosis, patient 2 presented with a large adrenal tumor with IVC tumor thrombosis, patient 3 was found to have deep vein thrombosis following grade 3b renal injury, and patient 4 was suffering severe SLE with renal vein thrombosis. The temporary inferior vena cava filter prevented pulmonary thromboembolism in all cases, and no adverse reaction was observed. Temporary inferior vena cava filter is safe and useful to prevent pulmonary thromboembolism associated with urological disorders.     

Renoportal anastomosis in liver transplantation and its impact on patient outcomes: a systematic literature review

Portal vein thrombosis (PVT) is commonly encountered during liver transplantation (LT). Depending on the grade of thrombosis, varied management strategies are indicated. The aims of this study are to clarify the contemporary role of renoportal anastomosis (RPA) in patients with splanchnic vein thrombosis (SVT) undergoing LT and to systematically analyze all reported cases of RPA. A systematic literature search was performed according to Preferred Reporting Items for Systematic Reviews and Meta‐ Analyses statement guidelines. The study was limited to studies reported in English between January 1997 and May 2017. Only retrospective single center studies were included in the analysis. A total of 66 patients with SVT were reported to have undergone RPA during LT. Transient renal dysfunction was reported in 12 patients (18.1%), variceal hemorrhage in 2 patients (3%), early portal vein (PV) re‐thrombosis in 2 patients (3%), chronic renal dysfunction in 2 patients (3%), and late PV re‐thrombosis in 1 patient (1.5%). The overall patient and graft survival were each 80%. This analysis illustrates the decades‐long evolution of a technique practiced across the field of transplantation. Postoperative complications and graft survival appear to be encouraging, even in the setting of SVT.     

Nephrotic syndrome with renal vein thrombosis: pathogenetic importance of a plasmin inhibitor (alpha 2-antiplasmin)

Tests of fibrinolysis were measured by fibrin plate methods in 44 patients with nephrotic syndrome, in 14 of whom renal vein thrombosis was demonstrated. In both groups the level of total fibrinolytic activity was normal, that of vascular plasminogen activator was decreased, and that of an inhibitor of plasminogen activation was elevated. The level of a plasmin inhibitor, measured by the fibrin plate method, was elevated in 13 of 14 patients with, but only in 12 of 30 without, renal vein thrombosis (p less than 0.005). The plasmin inhibitor was identical with alpha 2-antiplasmin. The data suggest that an increased level of alpha 2-antiplasmin may be a factor in determining susceptibility to the development and persistence of renal vein thrombosis in patients with nephrotic syndrome.     

Renal cocktail: too hard for a diabetic

We herein report a case of a 67-year-old diabetic woman who presented with a history of fatigue for 1 month. Her investigations showed proteinuria, active urine sediment, p-ANCA positivity and worsened renal functions. A diagnosis of rapidly progressive glomerulonephritis was made. Renal biopsy revealed class V (membranous) lupus nephritis with superimposed ANCA-associated crescentic glomerulonephritis. She was treated with steroids and cyclophosphamide. Two months later, she presented with cytomegalovirus colitis and deep vein thrombosis of right leg. The case reflects an interesting renal pathology, and complications of the disease per se and its treatment.     

Bilateral Renal Vein Thrombosis Secondary to Membraneous Glomerulonephritis: Successful Treatment with Thrombolytic Therapy

Renal vein thrombosis (RVT) is a rare phenomenon. Bilateral RVT is even less common. RVT has been reported as idiopathic or in association with puerperium, hypercoagulable states, membraneous glomerulonephritis (MGn), renal transplantation, malignancy, and renal vein instrumentation or trauma. Due to its rarity, the literature describing RVT consists mainly of isolated case reports and there are no consistent recommendations regarding diagnosis and treatment of this vascular emergency. We present a case of bilateral RVT associated with MGn. A 45-year-old man complaining of nonspecific abdominal pain of 2 days’ duration was found to have bilateral RVT upon computed tomographic evaluation. He was successfully treated with emergent endovascular mechanical thrombectomy in conjunction with chemical thrombolysis and subsequently therapeutically anticoagulated. A renal biopsy demonstrated MGn, and the patient was placed on an appropriate medical regimen. He is well approximately 18 months later, with preserved renal function. Despite a multitude of potential causative mechanisms, all RVT patients should undergo a thorough workup for underlying hypercoagulable disorder and underlying benign or malignant renal causes. Treatment is aimed at prompt relief of venous obstruction and preservation of renal parenchymal function. Interventional radiological procedures, including thrombolysis and thrombectomy, have proven to be valuable in the treatment of RVT. Surgical options include thrombectomy and nephrectomy.     

Treatment of renal vein thrombosis associated with nephrotic syndrome

Renal vein thrombosis is a rare entity in which true incidence is unknown. The disease occurs most frequently in patients with nephrotic syndrome, but it also can occur in the presence of other hypercoagulable state. Two cases of renal vein thrombosis with nephrotic syndrome which were treated by thrombectomy are reported here. One patient was successfully treated by renal vein and inferior vena cava thrombectomy before developing severe pulmonary embolism. The other was treated by renal vein thrombectomy by which fatal shock was able to be prevented. In those cases, immediate operation was indicated, primarily to prevent additional, possibly fatal, pulmonary embolism and also to improve perfusion of the kidney. In the hope of salvaging the kidney, thrombectomy may be the treatment of choice for acute renal vein thrombosis, complication of pulmonary embolism and inferior vena cava thrombosis, right renal vein thrombosis without collateral flow and acute renal vein thrombosis with shock.     

Vascular complications of renal transplantation

From January, 1963, to May, 1979, 600 renal transplants were performed at the Cleveland Clinic. There were 21 vascular complications in this series (3.5 per cent). Arterial complications included renal artery thrombosis in 5 patients, renal artery stenosis in 9 patients, and anastomotic hemorrhage in 2 patients. Venous complications included renal vein thrombosis in 2 patients and hemorrhage in 3 patients. Although vascular complications led directly to graft loss in 10 patients, only 1 resulted in patient death.     

多层螺旋CT血管成像在肾静脉血栓诊断和治疗中的应用

目的探讨多层螺旋CT血管成像（multi-slice spiral CT angiography,MSCTA）在肾静脉血栓（renal vein thrombosis,RVT）诊断和治疗中的临床应用价值。方法收集首诊RVT患者8例,慢性期RVT并左肾静脉重度狭窄或闭塞患者5例,均行CT平扫、动脉期及静脉期增强扫描,采用容积再现（volume rendering,VR）血管生长技术（addvessel,AV）进行血管重建,分析RVT、肾静脉侧支循环表现。结果13例RVT患者中,首诊8例（双侧3例,左侧5例）,CT平扫呈均匀稍高密度影,增强扫描示肾静脉主干及分支内见充盈缺损影,4例血栓延伸至下腔静脉内,1例合并左腰静脉干、左睾丸静脉内血栓,1例左睾丸静脉侧支循环形成。慢性期5例,CT平扫左。肾静脉结构显示不清,左肾静脉主干4例不显影,1例细如线样,均显示左卵巢静脉曲张,右卵巢静脉、双侧髂内静脉侧支循环形成。结论MSCTA能准确诊断RVT及肾静脉侧支循环,指导临床制定治疗方案。     

Evaluation of the inferior vena cava in potential pediatric renal transplant recipients

Vascular anomalies due to occlusion or thrombosis of the inferior vena cava (IVC) may be hazardous to renal transplantation, and preoperative vascular evaluation is important for safe and successful surgery. The purpose of this study was to assess the utility and accuracy of two-dimensional time-of-flight (2D-TOF) magnetic resonance venography (MRV) as an alternative to conventional angiography for evaluating the IVC and iliac vein in potential pediatric renal transplant recipients. Four children with chronic renal failure were evaluated with 2D-TOF MRV by superior presaturation pulse and target maximum intensity projection. The whole MRV examination and filming required less than 30 min. All four patients had a normal IVC and iliac vein. Two of the patients underwent renal transplantation, and the MRV findings were in total agreement with the final anatomy revealed intraoperatively. MRV is accurate for evaluating the condition of the IVC and iliac vein. It is a reliable, noninvasive and rapid technique that can be considered as an alternative to invasive angiography for evaluation of children scheduled for transplantation. We recommend the use of this noninvasive procedure to ascertain the patency of the IVC in all infants and children with a history of indwelling catheters in the IVC or those with a propensity to thrombosis, i.e., all recipients with nephrotic syndrome. The insertion of catheters in the femoral vein in children who may become candidates for renal transplantation should be discouraged.