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 edema
• A medication that blocks a hormone system called the renin angiotensin system (ACE inhibitor or ARB) to control blood pressure or lower urine protein
• Anticoagulants to prevent blood clots
• Statins to lower the cholesterol level
• Maintaining a healthy diet: Correct amounts of protein and fluid intake according to your nephrologist’s recommendations. A healthy diet consists of low salt with emphasis on fruits and vegetables, low in saturated fat and cholesterol. A low salt diet may help with swelling in the hands and legs. For more guidance on suggested foods visit Children's Hospital web site.
• Exercising
• Not smoking
• Vitamins
Good news: Corticosteroids help approximately 90% of patients who have MCD
Bad News:
• 70% of children will experience a relapse.
• Corticosteroids have potentially serious adverse effects such as obesity, poor growth, hypertension, diabetes mellitus and osteoporosis.4
• Corticosteroids are sometimes ineffective, called “Steroid resistance” or a person can become “Steroid dependent.”
Alternatives (which all carry significant side effects) include:
• Immunosuppressants such as Cyclophosphamide (Brand names include Endoxan, Cytoxan, Neosar, Procytox, Revimmune) and Cyclosporine (Brand names include Neoral, Sandimmune, Gengraf)
• Tacrolimus (Brand name: Prograf)- another immunosuppressant
• Mycophenolate mofetil (Brand name: CellCept)- another immunosuppressant
• Antibiotics such as Levamisole
• More research is needed to identify new drug therapies
What are the complications of NS?
• Edema - swelling
• Renal vein thrombosis - blood clots
• Increased blood cholesterol, can lead to Atherosclerosis - hardening of the arteries
• Malnutrition
• Skin breakdown
• Infection such as pneumonia
• End Stage Renal Disease
• Congestive Heart Failure
• Pulmonary edema - fluid in the lungs

When should I call a doctor?
• Severe headache, fever, sores on the skin, cough, discomfort with urination, or decreased urine output

What is the societal cost of Nephrotic Syndrome and its disease group?
The Nephrotic Syndrome disease group (defined as Minimal Change Disease, FSGS and Membranous Nephropathy, among others), accounts for approximately 12% of prevalent ESRD costing the United States more than $3 billion each year. 4-5 

How does research help?
Research allows for understanding the causes, providing accurate diagnoses, developing effective treatment plans, and hopefully finding a cure.

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1. National Kidney Foundation of Michigan, Merck: http://www.merck.com/mmpe/sec17/ch233/ch233c.html
2. Fine JL, Grzybicki DM, Silowash R, Ho J, et al. Evaluation of whole slide image immunohistochemistryinterpretation in challenging prostate needle biopsies. Hum Pathol 2008; 39: 564-572.
3. Agraharkar M, Gala G, Gangakhedkar AK. Nephrotic Syndrome. eMedicine. February 1, 2007. Available at: eMedicine. Accessed July 16, 2008.
4. Maisonneuve, P., Agodoa, L., Gellert, R., Stewart, J. H., Buccianti, G., Lowenfels, A. B., Wolfe, R. A.,Jones, E., Disney, A. P., Briggs, D., McCredie, M. and Boyle, P. (2000) Distribution of primary renal diseases leading to end-stage renal failure in the United States, Europe, and Australia/New Zealand: results from an international comparative study. Am J Kidney Dis. 35, 157-165
5. USRDS. (2007) USRDS 2007 Annual Data Report: Atlas of Chronic Kidney Disease and End-Stage Renal Disease in the U.S. In NIH, National Institute of Diabetes and Digestive and Kidney Diseases ed.)^eds.)
6. Vivarelli M, Moscaritolo E, Tsalkidis A, et al. Time for Initial Response to Steroids Is a Major Prognostic Factor in Idiopathic Nephrotic Syndrome. J Pediatr. Mar 9 2010;[
7. The primary Nephrotic syndrome in children: Identification of patients with minimal change Nephrotic syndrome from initial response to prednisone: A report of the International Study of Kidney Disease in Children. J Pediatr 1981;98:461-464