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Nephrotic syndrome
Nephrotic syndrome is a disorder where the kidneyshave been damaged, causing them to leak proteinfrom the bloodinto the urine. It is a fairly benign disease when it occurs in childhood, but may lead on to chronic renal failure, especially in adults, or be a sign of an underlying serious disease such as systemic lupus erythematosusor a malignancy.
Signs and symptoms
Laboratory Findings
DiagnosisHigh urine levels of proteincan readily be detected with a dipstick. The best way to make a diagnosis is to quantify the amount of protein in a 24-hour urine sample or a random albumin to creatinine ratio (ACR). A diagnosis of nephrotic syndrome requires more than 3.5 grams of proteinuria per 1.73 square meter surface area in adults. It is important to note, however, that nephrotic syndrome can be associated with lesser degrees of proteinuria, and many of the complications of nephrotic syndrome are due to hypoalbuminemia and the resultant decreased plasma oncotic pressure. Therefore, the same consequences can result independently of the level of proteinuria, as long as the same degree of hypoalbuminemia is achieved. Once the diagnosis of nephrotic syndrome is reached, further investigations must focus on the underlying disease process. PathogenesisThe glomeruliof the kidneys are the parts that normally filter the blood. They consist of capillariesthat are fenestrated (leaky, due to little holes called fenestrae or windows) and that allow fluid, salts, and other small solutes to flow through, but normally not proteins. In nephrotic syndrome, the glomeruli become damaged due to diabetes, glomerulonephritis, or even prolonged hypertension(high blood pressure) so that small proteins, such as albumincan pass through the kidneys into urine. Nephrotic syndrome is characterised by proteinuria (detectable protein in the urine), and low albumin levels in blood plasma. As a compensation, the liver begins to make more of all its proteins, and levels of large proteins (such as alpha 2-macroglobulin) increase. Edema usually occurs due to salt and water retention by the diseased kidneys as well as sometimes due to the reduced colloid oncotic pressure(because of reduced albumin in the plasma). Cholesterollevels are also increased, and though the mechanism isn't fully understood, it is thought to be due to the increased synthesis of lipoproteinsin the liver. There is an increased tendency for thrombosis(up to 25%), perhaps due to urinary loss of inhibitors of clotting such as antithrombin III. Similar loss of immunoglobulinsincreases the risks of infections and relevant immunisation is recommended against pneumococcus, Haemophilus influenzae, and meningococcus. Differential diagnosisPrimary renal diseases
Secondary renal diseases
Others
TreatmentWhen treating nephrotic syndrome, if the underlying problem is apparent, (e.g. hypertension, diabetes) then this should be addressed. Some types of nephrotic syndrome respond to therapy with steroids(especially minimal change disease) and/or other immunosuppressive therapy. Others are followed up in clinic with management of blood pressure, cholesterollevels, coagulationproblems and renal failure. In most types of nephrotic syndrome, the protein excretion improves with the use of ACE inhibitormedication. This is generally used for the treatment of hypertension, but can also improve proteinloss, even if the blood pressure is normal. PrognosisThe prognosis depends on the cause of nephrotic syndrome. It is usually good in children, because minimal change diseaseresponds very well to steroidsand does not cause chronic renal failure. However other causes such as focal segmental glomerulosclerosisfrequently lead to end stage renal disease. Factors associated with a poorer prognosis in these cases include level of proteinuria, blood pressurecontrol and kidney function (GFR). Reference
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sv:Nefrotiskt syndrom
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