The coagulogram in patients with nephrotic syndrome

The coagulogram done in half of the 100 cases of nephrotic patients admitted into our hospital from 1986 to 1988 was studied. It is shown that the patients of either the renal vein thrombosis (RVT) positive or negative group were in hypercoagulability state. 46 patients in this series had RVT proved by renography. The mechanism of RVT and the clinical significance of the changes of these hemostatic data are discussed.     

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.     

Acute kidney injury as the first sign of spontaneous renal vein thrombosis: report of 2 cases

Spontaneous renal vein thrombosis (RVT) is very rare in the absence of nephrotic syndrome. It is more common in newborns and infants. RVT should always be included in the differential diagnosis of flank pain and hematuria, and because RVT can induce acute renal injury. A 19-year-old man was admitted to our hospital because he complained of right flank pain and oliguria for 3 days. Another patient, a 24-year-old man, complained of a severe and sudden onset of bilateral flank pain and anuria for a day. They were both healthy before they developed the described symptoms and had different levels of decrease in renal function when they visited the hospital. Color Doppler ultrasonography revealed RVT in both the patients. The patients received therapy, including anticoagulation and thrombolysis, following their diagnoses, and they recovered in a few days.     

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.     

Renal Vein Thrombosis in a Newborn With Abnormal Factor VIII Level: Clinical Case Report

Renal vein thrombosis (RVT) in neonates is a rare condition of low mortality but significant morbidity due to renal impairment.We report the case of a male term newborn with left RVT and elevated serum factor VIII (FVIII).The main symptoms of the patient and the important clinical findings: prompt diagnosis of RVT was possible because the classic clinical presentation of macroscopic hematuria, thrombocytopenia, and palpable flank mass were present in this newborn infant.The main diagnoses: finally, the reason of RVT was established when the infant was 3 months of age: the increased level of FVIII was confirmed. We discuss the diagnosis, therapy, and outcome of the patient and compare with the literature.Therapeutics interventions: however, despite anticoagulant therapy the left kidney developed areas of scarring and then atrophy.Conclusions and outcomes: Prothrombotic defects should be considered in all patients with perinatal RVT. Elevated factor VIII as a reason of RVT in neonatal period is particularly rare. Given a poor renal outcome in children associated with elevated levels of factor VIII, consideration could be given to more aggressive antithrombotic therapy in such cases.     

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.     

Follow-up study of patients with clinically suspected deep venous thrombosis and a normal venogram

Abstract. Objectives. To evaluate the clinical course in patients with clinically suspected deep venous thrombosis (DVT) of the leg and a normal venogram. Design. Prospective study over 15 months with a follow-up of 4–12 (median 8.6) months after a normal venogram. A questionnaire survey was performed at follow-up. Information from general practitioners and medical records was reviewed. An alternative diagnosis was established at presentation and at the time of follow-up. Setting. The Department of Internal Medicine in a Danish university hospital. Subjects. A total of 133 consecutive out-patients referred with clinical suspicion of DVT and a normal venogram. Main outcome measures. The state of symptoms at follow-up. The frequency of referrals to hospitals and contacts with general practitioners or medical specialists in the follow-up period. Clinical diagnoses provided at presentation and at follow-up. Results. The follow-up response rate was 78% (n = 104). The symptoms were still present at follow-up in 53 (51%) patients. More than half of the patients had been referred to medical facilities for the same disorder. Diagnoses could be established in 93 (70%) of the 133 patients at presentation and in 119 (89%) at follow-up. Conclusions. The majority of patients with clinical signs and symptoms of a DVT and a normal venogram may require a follow-up surveillance programme to ensure correct diagnosis and adequate treatment. Further studies are recommended to confirm our results and to assess the cost-effectiveness.     

Renal vein thrombosis: a case report

The presenting symptoms of acute renal vein thrombosis (RVT) can often be confused with those of nephrolithiasis. Delayed diagnosis and treatment of RVT can result in catastrophic complications, including loss of renal function and pulmonary embolism. A high clinical suspicion and early imaging with computed tomography or magnetic resonance imaging will allow early initiation of therapy and prevention of thrombus extension in patients with RVT.     

Renal vein thrombosis and selective arterial or venous thrombolytic therapy

Summary Background: Renal vein thrombosis (RVT) complicating the nephrotic syndrome is associated with a poor prognosis. Methods/Results: RVT was diagnosed in 12 of 60 patients with a diagnosis of nephrotic syndrome suggested by computed tomography (CT) and subsequently confirmed by selective renal angiography. Fifty patients carried a diagnosis of primary glomerulonephritis with various pathological findings, and 10 patients had lupus nephritis. Renal vein and peripheral vein blood samples were collected in the 12 patients with RVT and were assayed for fibrin(ogen) degradation products (FDP), antithrombin III (AT III), VIIIR:AG, and fibrinogen. The results suggested a state of hypercoagulation. Of these 12 patients, 7 were given 200,000 units of urokinase (UK) over 60 minutes in divided doses selectively via the renal vein. Five patients were given 200,000 units UK selectively into the renal artery. All patients also received 2.5 mg/day warfarin and 75 mg/day persantine. Except for three patients with focal glomerulosclerosis, all patients received 40 mg/day prednisone. After 1 month, the CT scan and blood samples for FDP, AT III, VIIIR:AG, and fibrinogen were repeated. Patients receiving intra-arterial UK had complete resolution of their thrombi. Complete resolution was also suggested in 2 of the 7 patients receiving UK by renal vein, and there was partial resolution in the other five. The hypercoagulation state decreased in all patients. Conclusions: We conclude that RVT is not an uncommon event in patients with nephrotic syndrome. The diagnosis can be supported reliably using abdominal CT scanning. Although a small number of patients were included in this nonrandomized study, it appeared that intra-arterial thrombolytic therapy yielded better results. The patients with minimal change disease have a good prognosis.     

Catheter‐directed therapy for acute renal vein thrombosis in systemic lupus erythematosus: A case report

We report our experience using catheter‐directed thrombectomy/thrombolysis (CDT) to treat a patient with acute renal vein thrombosis (RVT) associated with systemic lupus erythematosus (SLE). A 34‐year‐old woman presented with persistent left flank pain, and a renal ultrasonography examination revealed an enlarged left kidney. Contrast‐enhanced computed tomography confirmed the presence of acute left RVT. Because medical treatment failed to relieve her pain and the renal function was deteriorating, we attempted to salvage the occluded left renal vein using an endovascular approach. The pain was completely relieved after a CDT and an overnight urokinase infusion. A follow‐up computed tomography examination revealed the complete resolution of the thrombus. The creatinine level returned to normal (1.7–0.4 mg/dL), along with contrast enhancement in the left kidney, and this suggested the preservation of renal function. To our knowledge, this is the first report utilizing CDT to treat SLE‐associated RVT. When the renal function is deteriorating, CDT is worth considering for RVT if conventional medical treatment has failed. © 2016 Wiley Periodicals, Inc.     

Residual vein thrombosis to establish duration of anticoagulation after a first episode of deep vein thrombosis: the Duration of Anticoagulation based on Compression UltraSonography (DACUS) study

Residual vein thrombosis (RVT) indicates a prothrombotic state and is useful for evaluating the optimal duration of oral anticoagulant treatment (OAT). Patients with a first episode of deep vein thrombosis, treated with OAT for 3 months, were managed according to RVT findings. Those with RVT were randomized to either stop or continue anticoagulants for 9 additional months, whereas in those without RVT, OAT was stopped. Outcomes were recurrent venous thromboembolism and/or major bleeding. Residual thrombosis was detected in 180 (69.8%) of 258 patients; recurrent events occurred in 27.2% of those who discontinued (25/92; 15.2% person-years) and 19.3% of those who continued OAT (17/88; 10.1% person-years). The relative adjusted hazard ratio (HR) was 1.58 (95% confidence interval [CI], 0.85-2.93; P = .145). Of the 78 (30.2%) patients without RVT, only 1 (1.3%; 0.63% person-years) had a recurrence. The adjusted HR of patients with RVT versus those without was 24.9 (95% CI, 3.4-183.6; P = .002). One major bleeding event (1.1%; 0.53% person-years) occurred in patients who stopped and 2 occurred (2.3%; 1.1% person-years) in those who continued OAT. Absence of RVT identifies a group of patients at very low risk for recurrent thrombosis who can safely stop OAT. This trial was registered at http://www.ClinicalTrials.gov as no. NCT00438230.     

Glomerulonephritis in Behçet’s Disease: Report of Seven Cases and Review of the Literature

Despite being recognised much more frequently than in the past, renal involvement has not previously been regarded as a feature of Behcet's disease (BD). In this study we aimed to assess the frequency of renal involvement in BD by performing urinalyses of 674 consecutive BD patients; we also retrospectively evaluated the charts of 4212 BD patients for the incidence of glomerulonephritis (GN). Urinary abnormalities (proteinuria and/or haematuria) were present in 10.8%; and during a period of 23 years GN was detected by renal biopsy in seven (0.16%) BD patients. Two patients with GN were lost to follow-up; end-stage renal failure developed in only one patient, and she underwent renal transplantation. We were unable to determine any pathognomonic feature that was predictive of renal involvement. Although males tend to have a more serious clinical course of BD the incidences of urinary abnormalities and GN were similar in both sexes in our series. According to our results, we can conclude that urinary abnormalities are more frequent in BD; however, serious renal lesions develop in only very few of these patients.     

The occurrence of malignant disease in adult nephrotic syndrome

The aim of the present study was to determine the incidence of malignant disease (MD) in adult patients with nephrotic syndrome (NS). Eighty-eight consecutive patients (38 females; 50 males; mean age 47 years; range 15-86) seen in a renal clinic in a 13-year period with NS were studied. Eight of the patients had or developed MD. One patient had MD (medullary thyroid carcinoma) and NS diagnosed concomitantly and two patients had MD (both malignant lymphoma) diagnosed 19 and 21 months after the diagnosis of NS, respectively. In conclusion, only few patients in a renal clinic at debut of NS do have a simultaneous MD. Furthermore, their risk of developing such a disease is at worst only slightly raised.     

肾病综合征合并特发性急性肾功能衰竭10例分析

１０例肾病综合征患者在病程中合并无明显诱因的急性肾功能衰竭，其临床特征均为大量蛋白尿，高度水肿，病程中突发少尿，尿渗透压降低，肾功能急骤性恶化，血肌酐及尿素氮升，高肾脏病理组织学显示肾小球无明显病变或轻微病变，但多数病人肾间质高度水肿及部分病人有肾小管上皮细胞呈灶状脱落坏死，经利尿、肾上腺皮质激素等治疗肾功能逐步恢复正常，提示肾病综合征合并特发性急性肾功能衰竭并不少见，发生率为肾功能逐步恢复正常。     

Residual vein thrombosis for assessing duration of anticoagulation after unprovoked deep vein thrombosis of the lower limbs: the extended DACUS study

The safest duration of anticoagulation after idiopathic deep vein thrombosis (DVT) is unknown. We conducted a prospective study to assess the optimal duration of vitamin K antagonist (VKA) therapy considering the risk of recurrence of thrombosis according to residual vein thrombosis (RVT). Patients with a first unprovoked DVT were evaluated for the presence of RVT after 3 months of VKA administration; those without RVT suspended VKA, while those with RVT continued oral anticoagulation for up to 2 years. Recurrent thrombosis and/or bleeding events were recorded during treatment (RVT group) and 1 year after VKA withdrawal (both groups). Among 409 patients evaluated for unprovoked DVT, 33.2% (136 of 409 patients) did not have RVT and VKA was stopped. The remaining 273 (66.8%) patients with RVT received anticoagulants for an additional 21 months; during this period of treatment, recurrent venous thromboembolism and major bleeding occurred in 4.7% and 1.1% of patients, respectively. After VKA suspension, the rates of recurrent thrombotic events were 1.4% and 10.4% in the no-RVT and RVT groups, respectively (relative risk = 7.4; 95% confidence interval = 4.9-9.9). These results indicate that in patients without RVT, a short period of treatment with a VKA is sufficient; in those with persistent RVT, treatment extended to 2 years substantially reduces, but does not eliminate, the risk of recurrent thrombosis.     

Abdominal venous thrombosis in neonates and infants: role of prothrombotic risk factors - a multicentre case-control study. For the Childhood Thrombophilia Study Group

The factor V (FV) G1691A mutation, the prothrombin (PT) G20210A variant, the methylenetetrahydrofolate reductase (MTHFR) T677T genotype, together with fasting homocysteine (HCY) concentration, lipoprotein (Lp)(a), anti-thrombin (AT), protein C (PC), protein S (PS) and anti-cardiolipin antibodies were investigated in 65 consecutively recruited infants (neonate to < 12 months) with renal venous thrombosis (RVT; n = 31), portal vein thrombosis (PVT; n = 24) or hepatic vein thrombosis (HVT n = 10), and 100 age- and sex-matched healthy controls. FV G1691A was found in 14 babies (heterozygous: RVT n = 9, PVT n = 4; homozygous HVT n = 1) and five controls, the MTHFR TT677 genotype together with increased HCY in four infants with thrombosis (RVT n = 2; PVT n = 1; HVT n = 1) compared with one control, and the PT G20210A variant was present in one control only. PC type I deficiency was diagnosed in three patients (RVT n = 2; PVT n = 1) and AT deficiency in two patients (RVT n = 1; PVT n = 1). Three neonates with spontaneous thrombosis showed FV G1691A combined with Lp(a) and the FV G1691A was combined with the PT G20210A genotype in two infants. Additional triggering factors were reported in 27 patients (41.5%). The overall odds ratios (ORs) and 95% confidence intervals (CIs) with respect to the different thrombosis locations were: RVT (OR/CI: 10.9/3.85-31.1; P < 0.0001), PVT (5.47/1.7-17.6; P < 0.0007) and HVT (3.3/0.58-18.7; P = 0.18). The data presented here suggest that genetic prothrombotic risk factors also play an important role in abdominal venous thrombosis during infancy.     

Treatment of retinal vein thrombosis with pentoxifylline: a controlled, randomized trial

The aim of this study was to evaluate PXF (pentoxifylline; 1600 mg daily vs placebo) in patients with retinal vein thrombosis (RVT) in a 4-week trial, evaluating clinical outcome and retinal flow. Inclusion criteria were sudden loss of vision (SLV); retinal vein thrombosis (RVT); decrease in retinal vein flow; asymmetry between retinal veins (>40%) documented by duplex scanning (retinal vein thrombosis flow = RVTF). All 18 included patients completed the study. The groups were comparable. No side effect was observed. An improvement in arterial flow (p<0.05) and a decrease in analogue score (p<0.05) were observed in both groups (due to the spontaneous evolution with partial thrombus lysis in 4 weeks). The increase in arterial flow (PSF and EDF) were greater (p<0.05) in the PXF group. The RVFV increase was better in the PXF group (350% increase vs 200% increase in the placebo group; p<0.05). There was a significant difference in the analogue score decrease (4 vs 7) in the PXF group (p<0.05). In conclusion, PXF improved retinal flow after RVT better than placebo. It should be considered as an important treatment option.     

New therapeutic option for autosomal dominant polycystic kidney disease patients with enlarged kidney and liver

The kidneys of patients with autosomal dominant polycystic kidney disease (ADPKD) usually continue to increase in size even after patients begin dialysis, and mass effects can lead to severe complications. Thus far, renal manifestations of this disorder have been discussed only from the viewpoint of cyst formation or cytogenesis, not from that of vascular abnormality. Because kidneys in ADPKD patients are usually supplied by well-developed arteries, we attempted renal contraction therapy in ADPKD patients with renal transcatheter arterial embolization (TAE) using intravascular coils. Mainly peripheral branches of renal arteries encircling the cysts were embolized. Our treatment has been confirmed to be effective in 266 patients until 2005. Renal size continued to decrease to 53% of the pre-TAE after 1 year. Almost all patients have had improved quality of life and nutritional status. We next tried TAE in 76 intractable patients with symptomatic polycystic liver. We tried to embolize only the hepatic segments replaced by cystic lesions in which the hepatic arteries were well-developed but the intrahepatic portal vein was obstructed. Sixty-four patients have had a good clinical course with this method. Based on our observation of ADPKD through treatment with TAE, we speculate that cyst growth in both the kidney and the liver progresses via the mechanism of 'arteriogenesis' of large vessels as well as 'angiogenesis' of small vessels.     

老年肾病综合征并发急性肾功能衰竭21例临床分析

目的　深入探讨老年肾病综合征 (NS)并发急性肾功能衰竭 (ARF)的临床、病理及预后。　 方法　对 2 1例老年及 3 8例非老年NS并发ARF患者的病因、临床特征、病理特点以及预后等进行回顾性分析。　 结果　老年组NS并发ARF发病率为 2 4 7% ;非老年组为 7 3 %。老年组与非老年组比较有以下特点 :(1)老年NS并发ARF发病率高 ,男性多见 (71 4% ) ,病死率高 ,治愈率低 ;(2 )蛋白尿程度极重、血浆蛋白极低 ;(3 )病理以膜性肾病、局灶节段性肾小球硬化多见 ;(4 )病因以原发性肾小球疾病、糖尿病肾病为主。　 结论 　老年NS并发ARF发病率高 ,病理以膜性肾病、局灶节段性肾小球硬化为主 ,蛋白尿及低蛋白血症严重 ,预后差。