Thrombotic complications in childhood-onset idiopathic membranous nephropathy

Abstract

While the most common clinical feature of nephrotic syndrome is generalized edema, patients are at risk of developing other problems, such as bacterial infections, electrolyte abnormalities, and venous thromboses. Adults with membranous nephropathy appear to be at the greatest risk for developing thromboses, especially renal vein thrombosis. However, the same is not true for children with membranous nephropathy. A review of pediatric membranous nephropathy stated that renal vein thrombosis is unrecorded in childhood-onset membranous nephropathy. We present our experience in managing two children with idiopathic membranous nephropathy who developed venous thromboses. To our knowledge, this is the first report of pediatric patients with membranous nephropathy to develop a thromboembolic complication without evidence of predisposing factors or coagulation abnormalities. This report emphasizes the need for appropriate evaluation of patients with membranous nephropathy who develop signs and symptoms suggestive of arterial or venous occlusion in order to avoid missing this potentially life-threatening medical complication.

Arterial thrombosis associated with factor V Leiden and methylenetetrahydrofolate reductase C677T mutation in childhood membranous glomerulonephritis

“…This risk has been attributed to a variety of intrinsic factors, including coagulation protein abnormalities, impaired fibrinolysis, and increased platelet aggregation, as well as dehydration, trauma, diuretic and corticosteroid use [2]. Coagulation abnormalities reported in NS include increased platelet aggregation and increased fibrinogen levels [1]. This, when coupled with a decrease in AT III, protein C, and protein S, further increases hypercoagulable state in nephrotic patients [1][2][3].…”

“…The numerous abnormalities of the coagulation and homeostasis system in nephrotic patients may arise through distinct mechanisms: urinary loss of antithrombin III, platelet hyperactivity, thrombocytosis, alterations in the fibrinolytic system, and increased synthesis of procoagulant proteins [3]. The use of diuretics and the consequent volume contraction may play a contributory role in the genesis of thrombotic complications in nephrotic patients [1]. Resistance to activated protein C, caused by a single-point mutation in the factor V gene (factor V Leiden), has been reported as the most common hereditary cause of venous thrombosis [4,5].…”

“…The incidence of thromboembolic phenomena, including deepvein thrombosis and arterial thrombosis, is high among nephrotic patients, particularly those with idiopathic membranous nephropathy (membranous glomerulonephritis; MGN) [1,2]. The numerous abnormalities of the coagulation and homeostasis system in nephrotic patients may arise through distinct mechanisms: urinary loss of antithrombin III, platelet hyperactivity, thrombocytosis, alterations in the fibrinolytic system, and increased synthesis of procoagulant proteins [3].…”

“…While the most common clinical feature of nephrotic syndrome (NS) is generalized edema, patients are at risk of developing other problems, such as bacterial infections, electrolyte abnormalities, and venous thrombosis [1]. The incidence of thromboembolic phenomena, including deepvein thrombosis and arterial thrombosis, is high among nephrotic patients, particularly those with idiopathic membranous nephropathy (membranous glomerulonephritis; MGN) [1,2].…”

Superior sagittal sinus thrombosis and bilateral sixth-nerve palsy in a child with nephrotic syndrome

“…The laboratory examinations must include the following prethrombotic factors: I, II, VII, VIII, XII, antithrombotic, protein C, plasminogen, tissue plasminogen activator, plasminogen activator inhibitor, FV Leiden, prothrombin gene mutation (G20210A), methylene-tetrahydrofolate reductase (MTHFR), lupus anticoagulant, anticardiolipin antibodies, and lipoprotein alpha, which are responsible for the development of thrombosis [7].…”