Nephrotic Syndrome

• ● Nephrosis is an altered glomerular permeability.

  ● You may confuse this with glomerulonephritis, remember that glomerulonephritis is the inflammation of the glomeruli

  ● This is due to fusion of the glomeruli membrane surfaces which leads to abnormal loss of protein in urine.

  ● Cause is an immunologic mechanism due to hypersensitivity to an antigen–antibody reaction or an autoimmune process, probably from a T-lymphocyte dysfunction.

  ● Common in boys, mostly 3 years old.
• 1. Congenital, which occurs as an autosomal recessive disorder.

  2. Secondary, as a progression of glomerulonephritis, sickle cell anemia, or systemic lupus erythematosus (SLE). - SLE is an autoimmune disease where the immune system of the body mistakenly attacks healthy tissues including skin, joints, kidneys, brain and other organs.

  3. Idiopathic, no known cause.

  ● In children, the congenital form is rare while the idiopathic form is the most common cause.
• 1. Proteinuria – the increase glomerular permeability leads to protein loss in the urine

  2. Hypoalbuminemia – the same increase glomerular permeability subsequently leads to the hypoalbuminemia or a low serum albumin level
• 3. Generalized edema – to include periorbital edema, ascites, and even scrotal edema in boys. This low level of protein in the blood causes osmotic pressure, fluid will shift from in to out of the cell. Also, as blood volume decreases, the kidneys begin to conserve sodium and water adding to the potential edema

  4. Hyperlipidemia or the increase blood lipid level – this occur because the liver increases production of lipoprotein. This is to compensate for protein loss. Lipids are too large to be lost in the urine so they rise in the blood instead.
• ● In nephrotic syndrome, laboratory studies will reveal marked proteinuria. A single test will show 1+ to 4+ protein. While a 24 hour total urine test will show 50 grams protein. Remember in acute poststreptococcal glomerulonephritis, the 24 hour total urine test is only 1 gram. So, it is larger in nephrotic syndrome.
• ● Again, normally there should be no protein in the urine. Also the protein loss in nephrotic syndrome is almost entirely albumin while in proteinuria of glomerulonephritis, it is non-specific.

  ● Some children with nephrotic syndrome also exhibit hematuria at the onset. But it is minimal in contrast to that seen in glomerulonephritis. Hematuria and glomerulonephritis is profuse.
• 1. Begins with a course of daily corticosteroids, such as IV methylprednisolone or oral prednisone which may be given at home, to reduce proteinuria and subsequently the edema.

  ● Corticosteroids are actually anti-inflammatory drugs, it will initially address the immune mechanism causing the altered glomerular permeability.

  ● An initial dose of prednisone is given until diuresis without protein loss is accomplished. So, every morning parents must test the urine using a chemical reagent strip and then record.
• ● Once a week it is also important to collect 24- hour urine specimen so total protein loss can be measured too. It should be decreasing because normally in a 24-hour urine specimen in nephrotic syndrome, there is about 15 grams of protein which normally should be none.

  2. Institute measures to keep child free of infection while the immune system is suppressed.

  ● because of prolonged use of corticosteroids.
• 3. In about 1 to 2 months (sometimes in 4 weeks), when protein loss is accomplished and edema subsides, prednisone may be given every other day instead ofeveryday.

  ● Prednisone has the potential to halt growth however growth is apparently not delayed if the drug is given on an alternate day or on a day after day basis.
• 4. Children who respond poorly to prednisone may need diuretic therapy with furosemide (Lasix).

  ● You might be wondering also why diuretics are not given for the edema. Again, the problem here is the actual nephron or glomeruli and diuretics mechanism of action are on the specific structure glomeruli. So, their action is not effective because the damage is there.

  ● Diuretics are not commonly used here as they tend to cause hypotension and hypokalemia as well. diuretics decrease blood pressure and furosemide loses potassium.
• ● Hypotension and hypokalemia in this case will further compound a problem. But if necessary especially with extensive edema, furo may be given but always check bp and blood potassium.

  5. IV albumin may be administered to temporarily correct hypoalbuminemia.

  ● To temporarily increase albumin and shift fluid into the bloodstream, that's the purpose.

  ● It is not recommended to do it on a long-term basis because albumin is loss in the urine.
• 6. There are no clear guidelines in treating hyperlipidemia but may try statins, bile acid sequestrants, and nicotinic acid to lower cholesterol.

  ● This is important because continuous hyperlipidemia may lead to cardiovascular diseases and thromboembolism.

  7. If all else fails, a course of cyclophosphamide (Cytoxan), cyclosporine (Sandimmune), or mycophenolate mofetil (CellCept), may be effective in reducing symptoms or preventing relapses

  ● These drugs are strong immunosuppressant agents which can again suppress the immune reaction happening in this syndrome
• ● Immunosuppressant drugs are also used as chemotherapy in cancer

  8. Reduce Na, eat high protein foods, avoid fats, monitor fluid intake.

  ● Reduce Na because of edema

  ● Eat high protein foods because protein is lost - Avoid fats because there is hyperlipidemia

  ● Monitor fluid intake in order to prevent further fluid retention