- Nephrotic syndrome (NS) is not a disease but a collection of symptoms as a consequence of damaged kidneys. In particular, there tends to be generalized swelling of the body, especially around the eyes, feet, hands, and abdomen.
- Even though the majority of cases of NS are caused by diseases within the kidneys (primary), systemic diseases (outside the kidney's) can also cause kidney disease and NS.
- Fever and symptoms of infection
- Loss of appetite
- Puffy eyelids
- Weight gain
- Scrotal swelling (fluid in the skin covering the testicles)
- Bloated or distended abdomen (excess fluids)
- Shortness of breath (fluid in the lungs)
- Low volume of urine
- Anemia or low blood pressure (weakness, dizziness, shortness of breath)
- Blood clots can block the blood flow to the kidneys (renal vein thrombosis)
- Glomeruli are the tiny blood vessels in the kidneys that filter waste and excess water from the blood and convert them to urine, which is then stored in a balloon-like sac called the bladder. The stored urine is excreted (i.e., peed out) as needed.
- Glomeruli reabsorb valuable proteins (albumin) and play a key role in the balance of other chemicals such as lipids (fats) and salt.
- When the glomeruli are damaged, albumin leaks out into the urine and the blood's albumin level falls.
- Albumin is needed to keep the water inside the blood vessels, and when the levels are low, water leaks out the blood vessels into the surrounding tissue, causing swelling or edema.
- Glomerular damage can be the result of the following renal conditions:
- Primary kidney diseases -- fibrillary glomerulopathy, focal glomerulonephritis, IgA Nephropathy, Membranoproliferative glomerulonephritis (i.e., MPGN, Minimal change disease and Rapidly progressive glomerulonephritis).
- Most primary causes have an association with immune system complexes (i.e., antibodies cause damage to the glomeruli).
- Systemic diseases -- Diabetes Mellitus, systemic Lupus erythromatosis, multiple myeloma, amyloidosis, chronic lymphocytic Leukemias.
- Hereditary diseases (e.g., passed on from parent to child), such as Alport's Syndrome and Fabry's disease can also cause NS.
- Infections such as streptococcal pharyngitis, syphilis, endocarditis (infection of heart valves) HIV, Hepatitis (B and C), Flu, and cancers (lymphomas, breast, colon, kidney, lung) can cause NS.
- Heavy metal poisoning (e.g., mercury and gold), snake venom, insect stings, poison ivy, drugs such as heroin, and medications (abuse of aspirin) can cause NS.
- Clinical signs and medical history are essential.
- A General medical doctor will often refer the NS patient to a kidney specialist (nephrologist).
- Tests and examination of patients may vary with different diseases.
- Tests include:
- Blood tests for low albumin levels, high lipids (fats like cholesterol and triglycerides), and possibly anemia (due to iron deficiency).
- Blood is also sent for a complete blood
count, and tests of the kidney and liver function. Glucose, calcium, potassium, sodium, and other electrolytes are also possibly abnormal.
- Depending on the disorder, special blood samples are collected and tested for diabetes (fasting glucose), antibodies (specific proteins), SLE (antinuclear antibody or ANA), hepatitis (B and C), HIV test, and syphilis (RPR or VDRL).
- If infection is suspected as the cause for NS, blood cultures are sent.
- Urine analysis (U/A) consists of measurements of the urine's contents and direct examination under a microscope.
- U/A reveals frothy (foamy) urine and casts (clumps) of red blood cells, fatty cells, and pieces of damaged glomeruli. There may be red colored urine due to bleeding (hematuria), and excess proteins, glucose, and amino acids.
- Pictures of the kidneys (X-rays, ultrasound, MRI, CAT scan)
- Intravenous pyelography (IVP) and renal venogram are
done after injection of dye. IVP and venogram are
done by a radiologist and are slightly uncomfortable. They should not be done if there are allergies to dyes or bleeding problems.
- In the hospital, a radiologist may also use a special needle and take a tiny piece of kidney tissue to be examined under a regular or even an electron microscope (very powerful microscope). This needle biopsy has risks (bleeding) but is very helpful.
- Same as causes
- In children, 80% of cases of NS are due to MCD (minimal change disease), often following a viral infection (Flu or cold).
- Treatment of the diseases that caused NS is essential.
Vaccines can prevent certain illnesses that cause NS (e.g.,
pneumococcal and influenza vaccines).
- Antibiotics, if there is strep throat, syphilis, or endocarditis
- Blood thinners (heparin or Coumadin) if there is blood clotting blocking the normal blood flow to the kidneys
- Erythropoietin is a hormone that can improve anemia.
- ACE inhibitors (e.g., Vasotec, Capoten) are high blood pressure medicines that help to protect the kidneys of diabetics.
- Diet low in salt (sodium) and saturated fats (animal fat, dairy, and solid oils, such as butter)
- Restriction of fluids (water, juice, etc.) -- amount is calculated by physician or a dietician.
- Adequate protein intake -- try fish, soy, and vegetable sources of protein
- Adequate potassium intake. Sometimes,
potassium has to be restricted.
- Multivitamins and multiminerals are good to take.
- Iron supplements may be prescribed.
- Weight loss is helpful if obese.
- Avoid excessive sunlight and drugs or medications that can further damage kidneys (read the side effects and look for renal toxicity).
- Diuretics such as Lasix can help remove excess water.
- Medications such as steroids (prednisone), Cyclosporin, and Cytoxan can help in some cases of NS.
- Cholestyramine and Zocar can lower cholesterol.
- If the kidneys have failed completely, dialysis (a machine that filters the blood) is administered until they improve, or a kidney is donated for transplant.
- The doctors will need to monitor blood and urine parameters for a period until NS resolves.
The earlier you seek treatment for your child, the better. If you have any questions, call the U.S. National Kidney Foundation at 1-800-622-9010.
- Renal failure due to long term
- Injury to kidneys