1. NEPHROTIC
Nephrotic-range proteinuria is the loss of 3 grams or more per day of protein into the
urine or on a single spot urine collection, the presence of 2 g of protein per gram of urine
creatinine. Nephrotic syndrome is the combination of nephrotic-range proteinuria with
a low serum albumin level and edema. Nephrotic syndrome has many causes,
including primary kidney diseases such as minimal change nephropathy, focal
glomerulosclerosis, and membranous nephropathy. Nephrotic syndrome can also result
from systemic diseases that affect other organs in addition to the kidneys, such as
diabetes, amyloidosis, and lupus erythematosus. Nephrotic syndrome may affect adults
and children of both sexes and of any race. It may occur in typical form, or in association
with nephritic syndrome. The latter connotes glomerular inflammation, with hematuria
and impaired kidney function.
Classification
Nephrotic syndrome can be primary, being a disease specific to the kidneys, or it can be
secondary, being a renal manifestation of a systemic general illness. In all cases, injury
to glomeruli is an essential feature. Kidney diseases that affect tubules and interstitium,
such as interstitial nephritis, will not cause nephrotic syndrome.
Primary causes of nephrotic syndrome include the following, in approximate order of
frequency:
Minimal-change nephropathy
Focal glomerulosclerosis
Membranous nephropathy
Hereditary nephropathies
Secondary causes include the following, again in order of approximate frequency:
Diabetes mellitus
Lupus erythematosus
Viral infections (e.g., hepatitis B, hepatitis C, human immunodeficiency virus
[HIV] )
Amyloidosis and paraproteinemias
Preeclampsia
Allo-antibodies from enzyme replacement therapy
Nephrotic range proteinuria may occur in other kidney diseases, such as IgA
nephropathy. In that common glomerular disease, one third of patients may have
2. nephrotic-range proteinuria. Nephrotic syndrome may occur in persons with sickle cell
disease and evolve torenal failure. Membranous nephropathy may complicate bone
marrow transplantation, in association with graft versus host disease. From a
therapeutic perspective, nephrotic syndrome may be classified as steroid sensitive,
steroid resistant, steroid dependent, or frequently relapsing.
The above causes of nephrotic syndrome are largely those for adults, and this article will
concentrate primarily on adult nephrotic syndrome. However, nephrotic syndrome in
infancy and childhood is an important entity. For discussion of this topic, see Pediatric
Nephrotic Syndrome.
Next Section: Pathophysiology
Read more about Nephrotic Syndrome on Medscape
Related Reference Topics
Pediatric Nephrotic
Syndrome
Membranous
Glomerulonephritis
Minimal-Change Disease
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Nephrotic syndrome is a group of symptoms that include protein in the urine, low blood
protein levels in the blood, high cholesterol levels, high triglyceride levels, and swelling
Causes
Nephrotic syndrome is caused by different disorders that damage the kidneys. This
damage leads to the release of too much protein in the urine. The most common cause in
children is minimal change disease. Membranous glomerulonephritis is the most
common cause in adults. In both diseases, the glomeruli in the kidneys are damaged.
Glomeruli are the structures that help filter wastes and fluids.
This condition can also occur from
Cancer
Diseases such as diabetes, systemic lupus erythematosus, multiple myeloma, and
amyloidosis
Genetic disorders
Immune disorders
Infections (such as strep throat, hepatitis, or mononucleosis)
Use of certain drugs
3. It can occur with kidney disorderssuch as:
Focal and segmental glomerulosclerosis
Glomerulonephritis
Mesangiocapillary glomerulonephritis
Nephrotic syndrome can affect all age groups. In children, it is most common between
ages 2 and 6. This disorder occurs slightly more often in males than females.
Symptoms
Swelling (edema) is the most common symptom. It may occur:
In the face and around the eyes (facial swelling)
In the arms and legs, especially in the feet and ankles
In the belly area (swollen abdomen)
Other symptomsinclude:
Skin rash or sores
Foamy appearance of the urine
Poor appetite
Weight gain (unintentional) from fluid retention
Seizures
Exams and Tests
The doctor will perform a physical exam. Laboratory tests will be done to see how well
the kidneys are working. They include:
Albumin blood test
Blood chemistry tests such as basic metabolic panel or comprehensive metabolic panel
Blood urea nitrogen (BUN)
Creatinine - blood test
Creatinine clearance - urine test
Urinalysis
Fats are often also present in the urine. Blood cholesterol and triglyceride levels may be
high.
A kidney biopsy may be needed to find the cause of the disorder.
Tests to rule out various causes may include the following:
Antinuclear antibody
Cryoglobulins
Complement levels
4. Glucose tolerance test
Hepatitis B and C antibodies
HIV test
Rheumatoid factor
Serum protein electrophoresis (SPEP)
Syphilis serology
Urine protein electrophoresis (UPEP)
This disease may also change the results of the following tests:
Vitamin D level
Serum iron
Urinary casts
Treatment
The goals of treatment are torelieve symptoms, prevent complications, and delay kidney
damage. To control nephrotic syndrome, the disorder that is causing it must be treated.
You may need treatment for life.
Treatments may include any of the following:
Keeping blood pressure at or below 130/80 mm Hg to delay kidney damage.
Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor
blockers (ARBs) are the medicines most often used. ACE inhibitors and ARBs
may also help decrease the amount of protein lost in the urine.
Corticosteroids and other drugs that suppress or quiet the immune system.
Treating high cholesterol to reduce the risk of heart and blood vessel problems. A
low-fat, low-cholesterol diet is usually not enough for people with nephrotic
syndrome. Medicines to reduce cholesterol and triglycerides (usually statins) may
be needed.
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 be helpful. Your health care provider may suggest a
moderate-protein diet (1 gram [gm] of protein per kilogram [kg] of body weight
per day).
Taking vitamin D supplements if nephrotic syndrome is long-term and is not
responding to treatment.
Taking blood thinner drugs to treat or prevent blood clots..
Possible Complications
Health problems that may result from nephrotic syndrome include:
Acute kidney failure
Atherosclerosis and related heart diseases
Chronic kidney disease