Nephrotic Syndrome

Nephrotic Syndrome: Overview

Nephrotic syndrome is a collection of symptoms which occur because the tiny blood vessels (the glomeruli) in the kidney become leaky.  This allows protein (normally never passed out in the urine) to leave the body in large amounts.

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Causes and Development

A number of different kidney disorders are associated with nephrotic syndrome, including:

  • Minimal change disease or MCD (responsible for about 80% of nephrotic syndrome in children, and about 20% in adults) MCD is a disorder of the glomeruli
  • Focal glomerulosclerosis
  • Membranous glomerulopathy
  • Membranoproliferative glomerulonephropathy.

Other types of diseases can also result in nephrotic syndrome.  These include diabetes, sickle-cell anemia, amyloidosis, systemic lupus erythematosus, sarcoidosis, leukemia, lymphoma, cancer of the breast, colon, and stomach, reactions to drugs (including nonsteroidal anti-inflammatory drugs, lithium, and street heroine), allergic reactions (to insect stings, snake venom, and poison ivy), infections (malaria, various bacteria, hepatitis B, herpes zoster, and the virus which causes AIDS), and severe high blood pressure.

Diagnosis and Tests

Diagnosis is based first on the laboratory examination of the urine and the blood.  While the urine will reveal significant quantities of protein, the blood will reveal abnormally low amounts of circulating proteins.  Blood tests will also reveal a high level of cholesterol.  In order to diagnose one of the kidney disorders which cause nephrotic syndrome, a small sample of the kidney (biopsy) will need to be removed for examination.  This biopsy can be done with a long, very thin needle which is inserted through the skin under the ribs.

Prognosis

Prognosis depends on the underlying disorder.  Minimal change disease has the best prognosis of all the kidney disorders, with 90% of all patients responding to treatment.  Other types of kidney diseases have less favorable outcomes, with high rates of progression to kidney failure.  When nephrotic syndrome is caused by another, treatable disorder (infection, allergic or drug reaction), the prognosis is very good.

Signs, symptoms & indicators of Nephrotic Syndrome:

Lab Values - Common

High/low systolic blood pressure

Because the kidneys are involved in blood pressure regulation, abnormally low or abnormally high blood pressure may develop from nephrotic syndrome.

Symptoms - Abdomen

Symptoms - Food - General

Weak/loss of appetite

As nephrotic syndrome progresses, appetite is greatly decreased.

Symptoms - Hair

(Very) brittle hair

Over time, the protein loss occurring in nephrotic syndrome will result in a generally malnourished state.  Hair and nails become brittle, and growth is stunted.  Bone becomes weak, and the body begins to lose other important nutrients (sugar, potassium, calcium).

Symptoms - Immune System

Blood clotting problems

Disorder of blood clotting is a serious and frequent complication.

Regular/frequent infections

Infection is a serious and frequent complication of nephrotic syndrome.

Symptoms - Metabolic

Edema of the eyelids

As nephrotic syndrome progresses, edema is noticed in the eyelids, hands, feet, knees, abdomen and (in males) scrotum.

Symptoms - Muscular

Some/great loss of muscle tone

Over time, the loss of protein due to nephrotic syndrome causes muscles to become weak and small (muscle wasting).

Symptoms - Nails

Brittle fingernails

Over time, the protein loss occurring in nephrotic syndrome will result in a generally malnourished state, causing hair and nails become brittle.

Symptoms - Urinary

Having foamy urine

The first symptom of nephrotic syndrome is often foamy urine.

Conditions that suggest Nephrotic Syndrome:

General

Excessive Fatigue

As nephrotic syndrome progresses, the patient feels increasingly weak and fatigued.

Lab Values

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Risk factors for Nephrotic Syndrome:

Organ Health

Glomerulonephritis

If proteinuria is heavy enough, patients will begin to show symptoms of nephrotic syndrome.  Nephrotic syndrome is not a disease in itself, but a group of symptoms which are caused by the heavy loss of protein in the urine.

The majority of IgAN patients do not develop nephrotic syndrome, but it is fairly common in pediatric IgAN.

Nephrotic Syndrome can lead to:

General

Excessive Fatigue

As nephrotic syndrome progresses, the patient feels increasingly weak and fatigued.

Lab Values

Recommendations for Nephrotic Syndrome:

Diet

Reduced Water Consumption

Occasionally, the quantity of fluid a patient is allowed to drink is restricted.

Drug

Conventional Drug Use

Treatment depends on the underlying disorder which has caused nephrotic syndrome.  Medications which dampen down the immune system are a mainstay of treatment.  The first choice is usually a steroid drug such as prednisone.  Some conditions may require even more potent medications, such as cyclophosphamide or cyclosporine.

Treating the underlying conditions (for example lymphoma, cancers, heroine use, infections) which have led to nephrotic syndrome will often improve the symptoms of nephrotic syndrome as well.  Some patients will require the use of specific medications to control high blood pressure.  Some patients benefit from the use of diuretics (which allow the kidney to produce more urine) to decrease swelling.

Nephrotic syndrome may require aggressive treatment with corticosteroids until the level of proteinuria goes down.  A person usually needs to lose more than 3gm per day of protein in the urine before this begins to happen.

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