A 31 year old Chinese man developed the nephrotic syndrome, and wasfound to have some of the clinical features of renal vein thrombosis such as a rapid deterioration in renal function and great variability in proteinuria. Radiological studies confirmed the diagnosis of bilateral renal vein thrombosis. The clinical features and pathogenesis of renal vein thrombosis are discussed.
A 31-year-old Malay female presented with nephrotic syndrome without renal impairment. Renal biopsy features were in keeping with immunotactoid glomerulopathy (ITG). Non-Congophilic deposits were seen causing thickening of the glomerular capillary basement membrane with segmental accentuation, and widening of the mesangium. Immunofluorescence examination showed moderate amounts of IgG and C3 in the glomerular capillary walls with some in the mesangium. Ultrastructurally, 20-nm thick fibrils with microtubular organisation were present predominantly in the subendothelial region with similar fibrils in the mesangium. Although immunotactoid glomerulopathy and fibrillary glomerulonephritis (FG) have been recognised as entities with extracellular fibrillary material in the kidney, to date much remains to be clarified regarding these 2 conditions. While the renal biopsy findings in this patient are consistent with ITG, her clinical presentation is unlike that of usual ITG in that she is of a much younger age and has no associated haemopoietic disorder. Response to initial treatment of 8 weeks of prednisolone therapy was poor.
We report a 14 year old Indian-Muslim girl who developed a fulminant, disseminated and fatal varicella infection while receiving steroids for nephrotic syndrome. The terminal phase of her illness was complicated by a bleeding dyscrasia and circulatory collapse. Varicella infection in healthy children is a benign disease. However in neonates and immunosuppressed patients it may be severe and often fatal. There are many reports of fatalities occurring in cancer patients receiving chemotherapy, patients on immunosuppressives for asthma, haemolytic anaemia, rheumatic fever, and renal and bone marrow transplantation. Patients with nephrotic syndrome receiving cyclophosphamide treatment are at particular risk of developing severe chickenpox infection. To our knowledge, there has been only one report of fatal chickenpox infection in a child who received steroids for nephrotic syndrome. We report here a case of fatal haemorrhagic chickenpox complicating nephrotic syndrome.
Nephrotic syndrome is often associated with a hypercoagulable state and thrombotic complications. Thrombosis may be due to a number of abnormalities in blood, including AT III deficiency, increased concentrations of fibrinogen, factors V and VIII, and platelet hyperaggregability. The therapeutic approach to thrombosis in nephrotic syndrome is the use of anticoagulants as a preventive measure or an attempt at thrombolysis with streptokinase, urokinase, or stanozolol.
Managing primary or even secondary glomerulonephritis remains a challenge to many nephrologists. In primary focal segmental glomerulosclerosis (FSGS) with heavy proteinuria, renin aldosterone system blockade and high dose of oral prednisolone is the mainstay of treatment. Other immunosuppressive medications like Cyclophosphamide, Cyclosporine A and Mycophenolate Mofetil (MMF) are warranted if a complete remission is not achieved. We illustrate a case of 21 year old gentleman with primary FSGS that was difficult to achieve remission despite on high dose steroid and oral Cyclophosphamide. He was also not responsive to a combination of MMF and Cyclosporine A (CSA) and even throughout the therapy he developed significant steroid and CSA toxicity. He presented to our center with severe nephrotic syndrome and acute kidney injury requiring acute haemodialysis. Despite re-challenged him again on high dose prednisolone, total of 2.4g of intravenous Cyclophosphamide, and MMF, he failed to achieve remission. He was subsequently given intravenous Rituximab 500mg/weekly for 4 doses and able to attained remission for 1 year. He relapsed again and a second course of Rituximab 500mg/weekly for 6 doses were given to attain remission. This case demonstrates the difficulty in managing refractory steroid dependent FSGS and we found that Rituximab is proven beneficial in this case to induce remission.