Nephrotic syndrome (patient information)
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Nephrotic syndrome is a group of signs and symptoms of a kidney filtering system disease (glomerular disease) which is associated with massive loss of protein in urine (called proteinuria), body or leg swelling (called edema), excessive or abnormal blood clotting (hypercoagulability) and high blood fat content (called hyperlipidemia). The massive loss of protein in the urine results in low level of essential proteins (primarily the essential protein called albumin) in blood leading to hypoalbuminemia.
Key Words and their meanings
- Hyper: excess
- Hypo: little or low level
- Albumin: essential amino acid.
- Amino Acids: building blocks for proteins
- Lipids: Fat
- Glomeruli: basic unit of the kidney filtration system
Nephrotic syndrome is a group of symptoms including protein in the urine (more than 3.5 grams per day), low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling.
What are the symptoms of Nephrotic syndrome?
Swelling is the most common symptom. It may occur:
- In the face and around the eyes.
- In the arms and legs, especially in the feet and ankles
- In the belly area (swollen abdomen)
Other symptoms include:
- Foamy appearance of the urine
- Weight gain (unintentional) from fluid retention
- Poor appetite
- High blood pressure
Patients that experience any of the above signs and symptoms should see their primary care physician immediately.
What causes Nephrotic syndrome?
Causes of Nephrotic Syndrome can be primary (that is; from the kidney glomeruli) or secondary (from an external insult to the kidney). Primary cause of Nephrotic Syndrome is often referred to as Idiopathic i.e. there are no known causes for the kidney abnormality. Such is the case in Minimal change kidney disease seen in children. Primary causes of nephrotic syndrome are usually described by the histology, e.g. minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS) and membranous nephropathy (MN). These diseases are considered to be "diagnoses of exclusion," meaning they are diagnosed only after secondary causes have been excluded.
Secondary Nephrotic Syndrome is nephrotic syndrome that is associated with well-known kidney diseases like autoimmune disease like Henoin Scholein purpura, IgA nephropathy, lupus nephritis etc. Secondary Nephrotic syndrome is also associated with post streptococcal glomerulonephritis, Alport's syndrome. It has also been associated with allergic reactions, drugs like NSAIDs, vaccination like the influenza and pneumococcal vaccines, tumors and viral infections.
A complete list of possible causes of nephrotic syndrome can be found here.
The doctor will perform a physical exam. Laboratory tests will be done to see how well the kidneys are working. They include:
- Creatine - blood test
- Blood urea nitrogen (BUN)
- Creatinine clearance
- Albumin blood test - may be low
- Urinalysis - reveals large amounts of urine protein
- Fats are often also present in the urine. Blood cholesterol and triglyceride levels may increase.
Kidney biopsy may be needed.
Tests to rule out various causes may include the following:
- Glucose tolerance test
- Antinuclear antibody
- Rheumatoid factor
- Complement levels
- Hepatitis B and C antibodies
- VDRL serology
- Serum protein electrophoresis
This disease may also alter the results of the following tests:
- Urinary casts
- Protein electrophoresis - urine
- Serum iron
When to seek urgent medical care?
Call your health care provider if symptoms which may indicate nephrotic syndrome occur.
Call your health care provider if nephrotic syndrome persists or if new symptoms develop, including severe headache, fever, sores on the skin, cough, discomfort with urination, or decreased urine output.
Go to the emergency room or call the local emergency number (such as 911) if convulsions occur.
The goals of treatment are to relieve symptoms, prevent complications and delay progressive kidney damage. Treatment of the disorder that causes the condition is necessary to control nephrotic syndrome. Treatment may be needed for life.
Controlling blood pressure is the most important measure to delay kidney damage. The goal is to keep blood pressure at or below 130/80 mmHg. Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) are the medicines most often used in this case. ACE inhibitors may also help decrease the amount of protein loss in the urine.
Corticosteroids and other drugs that suppress or quiet the immune system may be used.
High cholesterol and levels should be treated to reduce the risk of heart and blood vessel problems. However, a low-fat, low-cholesterol diet is usually not as helpful for people with nephrotic syndrome. Medications to reduce cholesterol and triglycerides may be needed, most commonly statins.
A low salt diet may help with swelling in the hands and legs. Water pills (diuretics) may also help with this problem.
Low protein diets may or may not be helpful. A moderate-protein diet (1 gram of protein per kilogram of body weight per day) may be suggested.
Vitamin D may need to be replaced if nephrotic syndrome is chronic and unresponsive to therapy.
Blood thinners may be required to treat or prevent clot formation.
Medications to avoid
Patients diagnosed with nephrosis should avoid using the following medications:
If you have been diagnosed with nephrosis, consult your physician before starting or stopping any of these medications.
Where to find medical care for Nephrotic syndrome?
Appropriate treatment of conditions that can cause nephrotic syndrome may help prevent the syndrome. It is advised that Nephrotic Syndrome patients should receive the influenza vaccine annually and the pneumococcal vaccine when due.
What to expect (Outlook/Prognosis)?
The outcome varies; the syndrome may be acute and short-term or chronic and unresponsive to therapy. The cause and development of complications also affects the outcome.
- Atherosclerosis and related heart diseases
- Renal vein thrombosis
- Acute kidney failure
- Chronic kidney disease
- Infections, including pneumococcal pneumonia
- Fluid overload, congestive heart failure, pulmonary edema