Nephrotic Syndrome Facts

What is Nephrotic Syndrome?
Nephrotic Syndrome is a collection of signs and symptoms that occur when glomeruli are damaged. 

What Causes Nephrotic Syndrome?
Most often, Nephrotic Syndrome is defined by its primary diseases that attack the kidney’s filtering system. Doctors often call these diseases "idiopathic," which means that they have arisen without a known cause. These primary diseases include Minimal Change Disease (MCD), Focal Segmental Glomerulosclerosis (FSGS), and Membranous Nephropathy (MN) and are identified by kidney biopsy. (There are many other “secondary” causes of NS, including diabetes, cancer, amyloidosis, lupus, vasculitic disorders, viral infections, drugs and allergies, among others.) MCD is the most common cause of Nephrotic Syndrome in children - almost 85% of children with Nephrotic Syndrome symptoms have MCD. As a result, the term “Nephrotic Syndrome” is often used interchangeably with MCD. FSGS is the second most common cause of nephrotic syndrome in children, at 9.5%, and research is indicating that FSGS is on the rise much more than the other two causes, and is more difficult to treat than MCD. 3.5% of children with nephrotic syndrome is caused by Membranous Nephropathy or another cause.(There are many other “secondary” causes of NS, including diabetes, cancer, amyloidosis, lupus, vasculitic disorders, viral infections, drugs and allergies, among others.) MCD is the most common cause of Nephrotic Syndrome in children - almost 85% of children with Nephrotic Syndrome symptoms have MCD. As a result, the term “Nephrotic Syndrome” is often used interchangeably.

  1. Each person has two kidneys in their lower back.
 

2. The kidneys continuously filter blood and produce urine to remove waste products, salts, and excess fluid.

3. Each kidney is made up of approximately one million tiny filters called "glomeruli.” Much as a coffee filter keeps coffee grounds in, glomeruli keep valuable cells and protein in the blood.
 

4. When glomeruli become damaged, proteins begin leaking into the urine (proteinuria). Proteinuria causes fluid to accumulate in the body, and prolonged leakage can lead to kidney damage and even failure.
  

“IDIOPATHIC” DISEASE [arrow] MCD, FSGS, or MN [arrow] NEPHROTIC SYNDROME

 

What are the symptoms of NS?
• Proteinuria - Large amounts of protein lost by “spilling” into the urine - more than 3.5 grams of protein into urine during a 24-hour period, or 25 times the normal amount. (This is the primary indicator of Nephrotic Syndrome).
• Edema - Pronounced swelling in parts of the body, most visible in the head, hands and feet, can become painful
• Hypertension – Increased blood pressure
• Hypoproteinemia - Low blood protein
• Hypercholesterolemia - Often a high level of cholesterol in the blood 

How is NS diagnosed?
• Urinalysis: determine the amount of protein in the urine
• Blood: determine levels of Creatinine, albumin, cholesterol, and many other factors examined to rule out other causes
• “Glomerular Filtration Rate” (GFR): http://www.nkdep.nih.gov/resources/ExplainingGFR.htm is an estimator of kidney function by calculating blood creatinine levels with urine protein levels
• Sometimes a Renal (kidney) ultrasound or CT scan is performed
• Sometimes a Renal(kidney) Biopsy: Removal of a small portion of the kidney for examination under the microscope

What are the facts of Nephrotic Syndrome?
Nephrotic syndrome may affect adults and children, of both sexes and of any race. In total, 26 million Americans suffer from Chronic Kidney Disease, with Nephrotic Syndrome as one of the most common forms.1 “Idiopathic” Nephrotic syndrome (NS), or Nephrotic Syndrome that arises seemingly spontaneously, is a rare disease syndrome and yet responsible for approximately 12% of all causes of end-stage kidney disease (ESRD) and up to 20% of ESRD in children.2 Approximately 5 out of every 100,000 children are diagnosed with Nephrotic Syndrome every year (incidence) and 15 out of every 100,000 children are living with it today (prevalence).

How is NS treated?
Your nephrologist may recommend:
• Medications that suppress your immune system
• Diuretics and low salt diet help to control