Nephrotic idiopathic

Loop diuretics are used in the treatment of edema associated with CHF, cirrhosis of the liver, and renal disease, including the nephrotic syndrome. These drug s are particularly useful when a greater diuretic effect is desired. Furosemide is the drug of choice when a rapid diuresis is needed or if the patient has renal insufficiency. Furosemide and torsemide are also used to treat hypertension. Ethacrynic acid is also used for the short-term management of ascites caused by a malignancy, idiopathic edema, or lymphedema. 

Edematous states To induce diuresis or remission of proteinuria in the nephrotic syndrome (without uremia) of the idiopathic type or that caused by lupus erythematosus. 

Edema Edema associated with CHF, hepatic cirrhosis, and the nephrotic syndrome steroid-induced edema, idiopathic edema, and edema due to secondary hyperaldosteronism. 

Schieppati A, Perna A, Zamora J, Giuhano GA, Braun N, Remuzzi G. Immunosuppressive treatment for idiopathic membranous nephropathy in adults with nephrotic syndrome. Cochrane Database Syst Rev 2004. 

In the following we concentrate mainly on the pathogenesis of minimal change disease, focal and segmental glomerulosclerosis, and idiopathic membranous nephropathy. These three diseases are responsible for about 60-95% of nephrotic syndromes and their prevalence depends on age. 

Idiopathic membranous nephropathy is the commonest form of nephrotic syndrome in middle-aged and elderly patients. The glomerular capillary wall is thickened due to immune deposits (containing mosty immunoglobulin G, IgG)... 

G3. Garin, E. H., Circulating mediators of proteinuriain idiopathic minimal lesion nephrotic syndrom. Pediatr. Nephrol. 14, 872-878 (2000). 

Neuhaus TJ, Fay J, Dillon MJ, Trompeter RS, Barratt TM (1994) Alternative treatment to corticosteroids in steroid sensitive idiopathic nephrotic syndrome. Arch Dis Child 71 522-526. 

Levamisole 2 mg/kg on alternate days was given to 25 glucocorticoid-dependent children with frequent relapses of idiopathic nephrotic syndrome (8). The steroid was tapered, and continued for 3-14 months. During treatment with levamisole the relapse frequency was reduced by 40%. Two patients developed mild transient leukopenia, which disappeared 2 weeks after withdrawal. One had a slight rash that disappeared while treatment was continued and one complained of epigastric pain, which led to drug withdrawal. 

There was a significant reduction in proteinuria in ten children with idiopathic nephrotic syndrome after pefloxacin therapy (mean dose 2-4.6 mg/kg/day for 4—8 weeks) (1). AH had received a conrse of cyclophosphamide at least 6 months before. One patient discontinned pefloxacin within 2 weeks becanse of nansea and vomiting, one complained of arthralgia, and one developed nail discoloration. 

Sharma RK, Sahu KM, Gulati S, Gupta A. Pefloxacin in steroid dependent and resistant idiopathic nephrotic syndrome. J Nephrol 2000 13(4) 271. ... 

Th2 cells are pathogenic in gold salt-induced glomerulopathy in Brown-Norway (BN) rats (this part will be developed below). The analysis of mRNA obtained from kidneys of Buffalo/Mna rats that spontaneously develop a nephrotic syndrome associated with focal segmental glomerular sclerosis revealed an early Th2 biased profile of cytokine expression, consistent with the involvement of Th2 cells in this model of human idiopathic nephrotic syndrome [19]. In minimal change glomerulopathy, an association... 

Antignac C Genetic models clues for understanding the pathogenesis of idiopathic nephrotic syndrome. J Clin Invest 2002 109 447-9. 

Meyrier A, Noel LH, Auriche P, Callard P. Long-term renal tolerance of cyclosporin A treatment in adult idiopathic nephrotic syndrome. Collaborative Group of the Societe de Nephrologie. Kidney Int 1994 45 1446-1456. 

El-Husseini A, El-Basuony F, Mahmoud I, Sheashaa FI, Sabry A, Hassan R,Taha N, Flassan N, Sayed-Ahmad N, Sobh M. Long-term effects of cyclosporine in children with idiopathic nephrotic syndrome a single-centre experience. Nephrol Dial Transplant 2005 20 2433-2438. 

FSGS accounts for less than 15% of the cases of idiopathic nephrotic syndrome in children and about 15% to 20% in adults however, it may account for 36% to 80% of the cases in African-Americans. Almost all the patients present with proteinuria, and many of them have all the features of nephrotic syndrome. The proteinuria is nonselective, containing albumin and other higher-molecular-weight proteins, and is usually less severe when compared to patients who have minimal-change disease. Hypertension, microscopic hematuria, and renal dysfunction may be seen in up to half of the patients. Reduced renal function becomes more prevalent as the disease progresses. 

Sarasin FP, Schifferli JA. Prophylactic oral anticoagulation in nephrotic patients with idiopathic membranous nephropathy. Kidney Int 1994 45 578-585. 

Tune BM, Mendoza SA. Treatment of the idiopathic nephrotic syndrome Regimens and outcomes in children and adults. J Am Soc Nephrol 1997 8 824-832. 

Day CJ, Cockwell P, Lipkin GW, et al. Mycophenolate mofetil in the treatment of resistant idiopathic nephrotic syndrome. Nephrol Dial Transplant 2002 17 2011-2013. 

At the present time, the efficacy and the clinical benefit of cyclosporin therapy has been conclusively demonstrated for severely affected patients in four diseases autoimmune uveitis, psoriasis, idiopathic nephrotic syndrome and rheumatoid arthritis, as well as in transplant patients. 

Lyons H, Pinn VW, Cortell S, Cohen JJ, Harrington JT. Allergic interstitial nephritis causing reversible renal failure in four patients with idiopathic nephrotic syndrome. N Engl J Med 1973 288 124-128. 

Banfi G, Moriggi M, Sabadini E, Felling G, D Amico G, Ponticelli G. The impact of prolonged immunosuppression of the outcome of idiopathic focal segmental glomerulosclerosis with nephrotic syndrome in adults a collaborative retrospective study. Clin Nephrol 1991 36 53-59. 

Additional indications for GCs include certain types of shock, hepatic diseases (e.g., hepatic necrosis), renal diseases (e.g., idiopathic nephrotic syndrome), and certain respiratory disorders (e.g., pulmonary sarcoidosis). It should be understood that in many of the conditions GCs are palliative at best and that in some instances their use may be controversial. 

Ethacrynic acid is normally used in the treatment of fluid retensive conditions due to congestive heart failure, cirrhosis of the liver, renal disease, and the nephrotic syndrome. It is invariably employed for the control and management of ascites due to lymphoedema, idiopathic oedema and malignancy. It is also recommended through i.v in an emergency situation of acute pulmonary oedema. 

Triamterene is usually recommended in the treatment of oedema associated with nephrotic syndrome, cirrhosis of liver, and congestive heart failure. It has also been used for the control and management of idiopathic oedema, steroid-induced oedema, oedema caused by hyperaldosteronism and in such oedematus patients who fail to respond to other therapy. It is usually used in conjunction with other diuretics like thiazides. 

Torres, V.E., Velosa, J.A., Holley, K.E., Frohnert, P.P., Zincke, H. and Sterioff, S. (1984). Meclofenamate treatment of recurrent idiopathic nephrotic syndrome with focal segmental glomerulosclerosis after renal transplantation. Mayo Clin. Proc., 59, 149—52... 

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