Nephrotic syndrome medical therapy
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There are currently no guidelines for the management of edema associated with nephrotic syndrome. The slow reversal of edema is important at a rate of 0.5-1 kg daily to prevent electrolyte disturbances, hypotension, ischemic acute tubular necrosis, and hemoconcentration associated with aggressive diuretic therapy. Since proteinuria is one of the most significant factors for progression of a disease and is associated with outcome, treatment of proteinuria in nephrotic syndrome must always be considered a priority. Angiotensin-converting enzyme inhibitors (ACE-I), with or without angiotensin-II receptor blockers (ARB) have been extensively studied and are well-known to decrease proteinuria and the risk of progression of renal disease in patients with nephrotic syndrome. Pneumococcal vaccines are recommended for all patients with nephrotic syndrome.
Treatment of Edema
- There are currently no guidelines for the management of edema associated with nephrotic syndrome.
- The slow reversal of edema is important at a rate of 0.5-1 kg daily to prevent electrolyte disturbances, hypotension, ischemic acute tubular necrosis, and hemoconcentration associated with aggressive diuretic therapy.
- Recommended sodium restriction: approximately 2 g/day.
- IV loop diuretics, like furosemide or bumetanide, are mostly used as first line diuretics.
- The use of oral medications is generally avoided due to poor absorption in cases of intestinal edema and due to presence of hypoalbuminemia.
- Addition of thiazide-type diuretics, metolazone, or potassium-sparing diuretics are also reasonable options.
- There are currently no guidelines to outline the appropriate dosages and drug selection.
- IV albumin, although generally not recommended for hypoalbuminemia due to its transient effects, has been shown to have synergistic effects with diuretics for an increased delivery of protein-bound diuretics to sites of action.
- Nonetheless, albumin is still not widely recommended, and its risks may at times outweigh the benefits because it is associated with anaphylaxis, hypertension, and pulmonary edema.
Treatment of Proteinuria
- Since proteinuria is one of the most significant factors for progression of disease and is associated with outcome, treatment of proteinuria in nephrotic syndrome must always be considered a priority.
- Angiotensin-converting enzyme inhibitors (ACE-I), with or without angiotensin-II receptor blockers (ARB) have been extensively studied and are well-known to decrease proteinuria and the risk of progression of renal disease in patients with nephrotic syndrome. In such cases, the indication of ACE-I is beyond blood pressure control. Patients must thus be started on ACE-I regardless of the presence of hypertension or not.
- Treatment with combined agents has been shown to effectively reduce proteinuria more than treatment with single agents.
- Follow-up with measurements of serum electrolytes is recommended periodically.
- In adults, an addition of corticosteroids has not been proven to be beneficial, except if the underlying etiology necessitates the use of steroids or if no improvement on conservative therapy takes place. In converse, most children with nephrotic syndrome are diagnosed with minimal change disease (MCD) and require corticosteroids for the resolution of proteinuria.
Treatment of Hyperlipidemia
- There are currently no reliable randomized clinical trials to recommend the use of lipid-lowering therapy in patients with nephrotic syndrome.
- It is however well-established that treatment of nephrotic syndrome itself often resolves the associated hyperlipidemia.
- One meta-analysis and post hoc subgroup analysis showed that statin might be effective in reducing cardiovascular disease in patients with nephrotic syndrome. As such, nephrotic syndrome should not change the indication for lipid-lowering treatment.
- There is currently no evidence to support or reject protein intake in patients with nephrotic syndrome. While a low protein diet may predispose to malnutrition, high protein diet has also been shown to exacerbate proteinuria.
- Pneumococcal vaccines are recommended for all patients with nephrotic syndrome.
- Prophylactic antibiotics are not recommended for patients with nephrotic syndrome.
- Prophylactic anticoagulation is not recommended. Anticoagulation may be initiated in thrombotic events, with special consideration to the decreased efficiency of heparin in patients with nephrotic syndrome due to the decreased serum antithrombin III levels needed for heparin anticoagulation.
Nephrosis or the nephrotic phase of nephritis is considered an absolute contraindication to the use of the following medications:
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