Pulmonary thrombosis in steroid-sensitive nephrotic syndrome

Deshpande, Pankaj; Griffiths, Mark

doi:10.1007/s00467-004-1741-4

Cited by 13 publications

(4 citation statements)

References 19 publications

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“…Thromboembolic complications may be associated with significant mobility including pulmonary embolism and renal vein thrombosis [60][61][62]. Pulmonary embolic episodes are silent [63].…”

Section: (Ii) Thromboembolismmentioning

confidence: 99%

Steroid Sensitive Nephrotic Syndrome in Children

Bhimma¹

2014

J Nephrol Ther

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Nephrotic Syndrome (NS) is one of the most frequent glomerular diseases seen in children. Children who go into complete remissin following treatment with corticosteroids are classified as having "steroid sensitive" NS. In developed countries over 80% of children with idiopathic NS have steroid sensitive disease although response to steroids is somewhat tempered in developing countries, especially in black children, the majority of whom have steroid resistant disease.The exact pathogenesis of this condition remains elusive. Podocyte injury and proteinuria are the two main issues in the pathogenesis. Recent studies suggest alterations in both innate and adaptive immune responses. There is release of cytokines by T-cells as well as a strong contribution of B-cell immunity. Genetic studies have reported human leucocyte antigen (HLA) class II antigens DR and DQ associations linked to steroid sensitive NS and in small case studies, single gene mutations e.g. PLCE1 although to date no homozygous mutations in NPHS1 or NPHS2 and WT-1 genes have been reported.Most children with steroid sensitive NS have multiple relapses and a significant percentage also develop steroid dependent NS. These children receive multiples courses of steroids and are at high risk of developing steroid toxicity. Patient with frequent relapses who develop steroid dependency thus require alternative treatment. Steroids sparing agents used include levamisole, cyclophosphamide, mycophenolate mofetil (MMF), calcineurin inhibitors (cyclosporine and tacrolimus), rituximab and vincristine. The steroid-sparing effects of these agents have greatly reduced the adverse effects seen with long-term use of steroids.Despite the wide arsenal of agents available, therapy needs to be individualised to achieve optimal care of each child. More randomised controlled trials are required to evaluate the therapeutic and economic efficacy of this agent, define criteria for selection of patients for use of particular agents and to determine the safety profile of these drugs in children.This article reviews the epidemiology, pathogenesis, clinical presentation, diagnosis, complications, management and long term outcome of children with steroid sensitive NS.

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“…Thromboembolic complications may be associated with significant mobility including pulmonary embolism and renal vein thrombosis [60][61][62]. Pulmonary embolic episodes are silent [63].…”

Section: (Ii) Thromboembolismmentioning

confidence: 99%

Steroid Sensitive Nephrotic Syndrome in Children

Bhimma¹

2014

J Nephrol Ther

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“…There have also been several reports of pulmonary thrombosis in NS patients. [4][5][6][7] Our patient experienced a fatal pulmonary thrombotic complication during anticoagulant therapy with intravenous heparin infusion. Intravenous heparin perfusion is widely performed, and it is a feasible therapy in patients with NS.…”

Section: Discussionmentioning

confidence: 94%

Fatal diffuse pulmonary arterial thrombosis as a complication of nephrotic syndrome

et al. 2007

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A 21-year-old man was admitted to our hospital because of leg edema. Because laboratory findings revealed massive proteinuria and hypoproteinemia, he was diagnosed as having nephritic syndrome caused by minimal change disease. He was given a continuous heparin infusion and intravenous steroid therapy, at a prednisolone dose of 1 mg/kg per day, and his condition gradually improved. Five months after discharge, the patient's proteinuria relapsed. He was readmitted to our hospital and we restarted anticoagulant treatment with intravenous heparin and 60 mg prednisolone. On the third hospital day, he complained of chest pain with sudden onset and dyspnea. He quickly developed shock and died. The findings of an autopsy confirmed the presence of diffuse fibrin thrombi in bilateral pulmonary arteries, and we diagnosed the cause of death as diffuse pulmonary artery thrombosis. A coagulation test for activated partial thromboplastin time (aPTT) had already shown that aPTT was prolonged before the initiation of treatment. There may have been a deficit of antithrombin III (ATIII) - a cofactor of heparin - because of the proteinuria; thus, the continuous heparin treatment might not have been effective for the prevention of thrombosis. Alternatives to heparin treatment that do not suppress AT III, such as nafamostat mesilate or argatroban, which do not require the presence of AT III for their anticoagulant action, should be considered in cases similar to the that in the patient reported here. In patients with nephrotic syndrome who exhibit altered coagulation test results, the choice of anticoagulation therapy for treatment of the hypercoagulabilty status associated with nephrotic syndrome should be carefully considered.

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“…The therapeutic approach to thrombosis in NS may be the use of anticoagulants as a preventive measure or an attempt at thrombolysis with streptokinase, urokinase, and stanozolol [1] or simply with longterm intravenous heparin infusions following a loading dose in childhood pulmonary embolism [14]. In general, antithrombotic management during NS is done when thromboembolic events occur, corticosteroid treatment is discontinued, and patients have received AT III concentrate and heparin infusion [6].…”

Section: Discussionmentioning

confidence: 99%

Prophylaxis with AT III for thromboembolism in nephrotic syndrome: why should it be done?

et al. 2008

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Children with nephrotic syndrome (NS) are at risk for sinovenous and arterial thrombosis, uncommon but serious complications of the nephrotic syndrome. Multiple factors are involved in the hypercoagulable state of patients with NS, for instance, enhanced platelet reactivity and deficiency of antithrombin III due to urinary loss of this protein. We report the case of a 7-year-old girl with relapse of nephrotic syndrome and with a clinical risk for thromboembolic complications, identified by very low AT III and albumin serum levels and high fibrinogen and cholesterol serum levels. However, having symptoms of hypovolemia, she was treated with albumin and diuretics, known risk factors for thrombotic incidents, although these drugs were both administered after prophylactic intravenous antithrombin. There are no randomized controlled clinical trials supporting prophylactic anticoagulation in the management of nephrotic syndrome. Arterial thromboses during nephrotic syndrome has been associated with thrombophilic states and the use of diuretics. It might be advisable to do laboratory monitoring for coagulation profiling and, in children at risk, prophylactic treatment with AT III before administering albumin infusion and diuretics.

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Pulmonary thrombosis in steroid-sensitive nephrotic syndrome

Cited by 13 publications

References 19 publications

Steroid Sensitive Nephrotic Syndrome in Children

Steroid Sensitive Nephrotic Syndrome in Children

Fatal diffuse pulmonary arterial thrombosis as a complication of nephrotic syndrome

Prophylaxis with AT III for thromboembolism in nephrotic syndrome: why should it be done?

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