Nephrotic Syndrome What Is Nephrotic Syndrome? Cause
Nephrotic Syndrome What Is Nephrotic Syndrome? Cause
Nephrotic Syndrome What Is Nephrotic Syndrome? Cause
Nephrotic syndrome is not a disease. It is a warning that Many conditions and diseases can cause nephrotic syndrome,
something is damaging your kidneys. Without treatment, that including:
problem could cause kidney failure, so it’s important to get
treatment right away.
• Minimal change disease (also called nil disease), a type
Nephrotic syndrome can occur at any age, but it is most common of kidney disease. The cause of minimal change disease
in children between the ages of 18 months and 8 years. is unknown, but it causes most of the cases of nephrotic
syndrome in children.1
• Membranous glomerulopathy and focal segmental
What causes nephrotic syndrome?
glomerulosclerosis, which are two other diseases of the
kidney. Both of these diseases affect the glomeruli. The
There are tiny blood vessels in the kidneys that filter waste and glomeruli help filter waste out of your blood. In focal
extra water from the blood. When these filters are damaged, you segmental glomerulosclerosis, some of the parts of the
get nephrotic syndrome. Protein helps move water from the glomeruli have scar tissue. This can affect how they
tissues into the blood. Healthy kidneys keep the right amount of filter the blood. In membranous glomerulopathy, the
protein in the blood. Damaged kidneys let protein slip from the tissue wall (membrane) that separates blood and urine
blood into the urine. Without enough protein in the blood, fluid and acts as a filter in your kidney becomes thickened
builds up in the tissues. This can cause swelling. and damaged.
• Diabetes and lupus. Diabetes is the most common cause
Many things can cause this blood vessel damage, including of nephrotic syndrome in adults in the United States.2
diabetes, lupus, infection, certain cancers, and some medicines. • Infections, such as HIV, hepatitis B, hepatitis C,
Sometimes doctors don't know what causes it. syphilis, malaria, tuberculosis, or post-streptococcal
glomerulonephritis.
A type of kidney disease called minimal change disease (also • Cancer, such as breast cancer, lung cancer, Hodgkin's
called nil disease) causes most of the cases of nephrotic syndrome lymphoma, or multiple myeloma.
in children. Doctors don't know what causes minimal change • Medicines, such as nonsteroidal anti-inflammatory
disease. drugs, penicillamine, gold therapy, or captopril.
• Illegal drugs, such as heroin.
What are the symptoms?
• Conditions such as preeclampsia, chronic graft rejection
following an organ transplant, and allergic reactions to
The most common early symptom of nephrotic syndrome, in both bee stings.
children and adults, is swelling in the tissues around the eyes or in • Unknown (idiopathic) factors.
the feet or ankles. They may also have swelling in the lungs that
can make it hard to breathe.
Symptoms
But many people who have nephrotic syndrome don't have
symptoms. Many people who have nephrotic syndrome do not have any
noticeable physical symptoms. When symptoms are present, they
can include:
How is nephrotic syndrome diagnosed?
******************************************
Unless nephrotic syndrome is clearly caused by diabetes, a kidney
biopsy is usually done to find the cause. Children do not usually
Being diagnosed: have a biopsy.
Call 911 or other emergency services if you have nephrotic • Keeping your blood pressure at less than 125/75 mm Hg
syndrome and you develop: with medication, diet, and exercise. For more
information, see the topic High Blood Pressure
• Chest pain. (Hypertension).
• Severe shortness of breath or difficulty breathing. • Keeping strict control over your blood glucose level if
• Sudden and severe abdominal or one-sided (flank) pain. you have diabetes. For more information, see the topic
Type 1 Diabetes or Type 2 Diabetes.
• Maintaining healthy levels of fats (lipids), such as
Call your doctor immediately if you have:
cholesterol and triglycerides. For more information, see
the topic High Cholesterol.
• Bloody urine (gross hematuria). • Not smoking or using other tobacco products. For more
• Sudden, severe swelling in your legs, and pain when information, see the topic Quitting Tobacco Use.
you flex your feet.
• Swelling of the scrotum. If you were diagnosed with nephrotic syndrome in the past:
• Sudden, severe increase in swelling around the eyes or
in the hands, legs, or feet. • Avoid dehydration. For more information, see the topic
• Sudden weight gain, such as 2 lb (0.9 kg) in 24 hours or Dehydration.
5 lb (2.3 kg) in a week. o Promptly treat illnesses that cause dehydration,
such as diarrhea, vomiting, or fever.
Watchful Waiting o Prevent dehydration during hot weather and
when you exercise. Drink 8 to 10 glasses of
If you think your child or you may have nephrotic syndrome, or if fluids (water or rehydration drinks) each day.
your child or you have the condition and the symptoms are Drink extra water before, during, and after
getting worse, don't wait to get treatment. Call your doctor right exercise. Take a container of water or sports
away. drink with you when you exercise, and try to
drink at least every 15 to 20 minutes. Use a
Exams and Tests sports drink if you will be exercising for longer
than 1 hour.
o Avoid caffeine drinks, such as coffee and
In addition to a medical history and physical exam, other tests to colas. They increase urine output, which
diagnose nephrotic syndrome include: increases dehydration.
o Avoid alcoholic beverages. They increase
• A 24-hour urine collection, which measures the total urine output, which increases dehydration.
amount of protein in the urine collected over 24 hours. o Do not take salt tablets. Most people get plenty
You will be diagnosed with nephrotic syndrome if you of salt in their diets. Use a sports drink if you
have more than 3500 mg of protein in your urine. are worried about replacing minerals lost
• Urine test for albumin. Lower levels of albumin in the through sweating. Do not use a sports drink if
blood can cause fluid to collect in the ankles, lungs, or you have heart failure unless you are instructed
abdomen. to do so by your doctor.
• Creatinine and creatinine clearance. Results of these o Stop working outdoors or exercising at the first
tests give information on how well your kidneys are sign of dizziness, lightheadedness, or fatigue.
working. o Wear one layer of lightweight, light-colored
• Blood profile tests to measure the amount of protein, clothing when you are working or exercising
cholesterol, and sugar (glucose) in the blood. outdoors. Replace sweaty clothing with dry
clothing as soon as you can.
• Kidney ultrasound to look at the kidneys. This exam can
rule out other causes of your symptoms. • Avoid medications that may harm the kidney.
• Avoid X-ray tests that use contrast material.
You may need other tests before treatment for nephrotic • Prevent heart disease. Lifestyle changes—eating a low-
syndrome begins, including: fat diet, quitting smoking, and getting regular exercise—
can help reduce your overall risk of developing heart
disease and stroke. For more information, see the topic
• A test for varicella (chickenpox) antibodies. Coronary Artery Disease.
• A bone density scan.
******************************* • Changes in diet to replace nutrients lost through the
urine, reduce fluid buildup in the body, and reduce the
Getting treatment: risk of complications. Some doctors prescribe a diet that
limits protein, salt (sodium), and fats but is high in
carbohydrates. The amount of protein allowed may vary,
• How is nephrotic syndrome treated?
depending on your condition.
• Will I need to take medicines?
• Anticoagulants, such as warfarin (Coumadin) or
• Is surgery used to treat nephrotic syndrome? heparin, to treat blood clots if they form.
• Early treatment of infections with antibiotics.
Treatment Overview • Vaccinations with a pneumococcal vaccine (What is
a PDF document?), such as Pneumovax, to prevent
Treatment for nephrotic syndrome depends on the cause and the infections; chickenpox (varicella) vaccines; and a yearly
age of the person who has the condition. Medicines, changes in flu shot. Vaccination is not recommended until nephrotic
diet, and care for other conditions, such as diabetes or high blood syndrome has responded to treatment with
pressure, are all possible treatments for this syndrome. These corticosteroids.5, 6
treatments may reverse, slow or prevent further kidney damage. • Calcium and vitamin D supplements to protect your
bones and help prevent osteoporosis during long-term
Most children who have nephrotic syndrome do well with corticosteroid treatment (for example, prednisone).
treatment and have a normal life expectancy. Children older than
age 12 at the time of diagnosis and adults who also have diabetes You can help slow the progression of kidney damage caused by
or high blood pressure do not respond as well to treatment as do nephrotic syndrome by:
children younger than 12.
• Keeping blood pressure at less than 125/75 mm Hg with
Doctors define complete recovery as living without symptoms or
medication, diet, and exercise. For more information,
treatment for more than 2 years.1
see the topic High Blood Pressure (Hypertension).
• Keeping strict blood glucose control if you have
Initial treatment diabetes. For more information, see the topics Type 1
Diabetes and Type 2 Diabetes.
Treatment of nephrotic syndrome depends on the cause of the • Maintaining healthy levels of fats (lipids), such as
disease and may include: cholesterol and triglycerides. For more information, see
the topic High Cholesterol.
• Corticosteroids, such as prednisone or prednisolone, to • Not smoking or using other tobacco products. For more
reduce swelling. information, see the topic Quitting Tobacco Use.
• Diuretics, such as bumetanide (Bumex) or furosemide • Avoiding medications that may harm the kidneys.
(Lasix), to reduce fluid buildup in the body (edema) and • Avoiding X-ray tests that use contrast material.
help with reducing sodium, potassium, and water. Fluid • Preventing coronary artery disease. Lifestyle changes
reduction should occur slowly to avoid further kidney such as eating a low-fat diet, quitting smoking, and
damage and low blood pressure. getting regular exercise can help reduce your overall
• Medications, such as angiotensin-converting enzyme risk of developing heart disease and stroke. For more
(ACE) inhibitors and angiotensin II receptor blockers information, see the topic Coronary Artery Disease.
(ARBs), to reduce the amount of protein lost in the • Weighing yourself daily so that you are aware of any
urine, lower blood pressure, and slow the progress of the rapid weight gain.
disease.
• In rare cases, salt-free albumin given through a vein
You may need emotional support during treatment for nephrotic
(IV). Albumin helps remove extra fluid from the tissues.
syndrome. If you or your child has nephrotic syndrome and you
are having a hard time handling treatment or the severity of your
You may need emotional support during treatment for nephrotic child's condition, talking with a doctor or seeking counseling may
syndrome. If you or your child has nephrotic syndrome and you help.
are having a hard time handling treatment or the severity of your
child's condition, talking with a doctor or seeking counseling may
Treatment if the condition gets worse
help.
Ongoing treatment
Clinical trials are ongoing to test more effective medicines for the
treatment of steroid-resistant (relapsing) nephrotic syndrome. Ask
Ongoing treatment for nephrotic syndrome and complications of your doctor about clinical trials if treatment has not successfully
the disease include: controlled your nephrotic syndrome. To participate in a clinical
trial, you may need to travel to a large treatment center.
• Daily or alternate-day prednisone, if nephrotic
syndrome returns. Medications
• Cyclophosphamide, cyclosporine, or CellCept, when
treatment with corticosteroids is not successful. Nephrotic syndrome is usually successfully treated with a
• Steps to lower blood pressure, including medication, a combination of medicines.
healthier diet, and exercise. Untreated high blood
pressure increases your risk for stroke or heart attack. Medication Choices
For more information, see the topics High Blood
Pressure (Hypertension), Coronary Artery Disease, and
Medicines to treat nephrotic syndrome include:
Stroke.
• Corticosteroids, such as prednisone or prednisolone, to serving size may increase your sodium beyond a healthy
reduce swelling. level.
• Albumin, to restore blood volume. • Avoid foods that contain monosodium glutamate (MSG)
• Diuretics, such as bumetanide or furosemide, to help and disodium phosphate.
maintain fluid balance. • Avoid canned foods.
• Cyclophosphamide, cyclosporine, or CellCept, when • Avoid salted ham, potato chips, pretzels, salted nuts, and
treatment with corticosteroids is not successful. other salty snack foods.
Most children are successfully treated with corticosteroids, Skin care when you have nephrotic syndrome
though relapses are common.7
Nephrotic syndrome may cause your skin to become dry and
Clinical trials are ongoing to test more effective medicines for the fragile, break open easily, bleed, and become infected. You can
treatment of steroid-resistant (relapsing) nephrotic syndrome. Ask prevent or reduce additional skin problems by using these tips:
your doctor about information on clinical trials if treatment has
not successfully controlled your nephrotic syndrome. To • Check for areas that are red, warm to the touch, or
participate in a clinical trial, you may need to travel to a large bleeding. Use a mirror or ask someone else to look at
treatment center. your feet, back, or buttocks if you have trouble seeing
these areas.
What To Think About • Avoid excessive bathing. Soap and water deplete your
skin of the essential oils that hold in moisture.
Most children who have nephrotic syndrome do well with o Limit showers or baths to 10 minutes.
treatment and have a normal life expectancy. Children older than o Do not use hot water to bathe.
age 12 at the time of diagnosis and adults who also have diabetes o Use bath oils in the tub. (Be careful, because
or high blood pressure do not respond as well to treatment as do bath oil will make the tub slippery.)
children younger than 12. o Use mild soaps, such as Dove or Cetaphil,
especially under the arms and in the genital
Surgery area.
• Use a moisturizing lotion after you bathe, or more
Surgery is not used to treat nephrotic syndrome. But if treatment frequently if your skin tends to become dry easily. Avoid
is not controlling your condition and you have developed chronic products that contain alcohol, because they can dry your
kidney disease, your doctor may recommend a kidney transplant. skin.
• Avoid scratching your skin or rubbing it vigorously with
************************** towels. If itching is a problem, see relief from itching.
• Avoid chemicals that may irritate the skin, such as
Living with nephrotic syndrome: rubbing alcohol, soaps, detergents, or solvents.
• Use baking soda or cornstarch powders on areas of your
skin that become irritated. Avoid powders that contain
• Should I reduce my salt intake?
talc. Breathing in talc can irritate your lungs.
• What can I do about my dry skin?
• Avoid tight-fitting clothes and shoes. Wear natural-fiber
• What medicines should I avoid if I have nephrotic clothes that allow moisture to evaporate from your skin.
syndrome? Avoid wearing wool and acrylic fabrics next to your
skin.
Reducing your salt intake
Medications that can cause acute renal failure
It's often a good idea to reduce the amount of salt (sodium) in
your diet if you are diagnosed with certain conditions, such as Many medications can cause acute renal failure. Examples
nephrotic syndrome, Cushing's syndrome, or heart failure. include:
Exactly how much daily salt is needed varies from person to
person.
• Antibiotics, such as aminoglycosides, cephalosporins,
amphotericin B, bacitracin, acyclovir, and vancomycin.
Try some of these tips for lowering your salt intake:
• Blood pressure medicines called ACE inhibitors (such
as captopril and ramipril) and angiotensin receptor
• Flavor your foods with herbs and spices such as basil, blockers (ARBs).
tarragon, or mint, or use salt-free sauces or lemon juice. • Medicines used for cancer treatment (chemotherapy),
Try plain or flavored vinegar to flavor soups and stews. such as cisplatin, carboplatin, and methotrexate.
Use about 1 tsp (4.9 mL) of vinegar for every 2 qt (1.9
• Dyes (contrast media) used in medical imaging tests.
L) of soup or stew.
• Illegal drugs, such as heroin and methamphetamine.
• Choose fresh or frozen vegetables and fruits.
• Medicines used to treat HIV (protease inhibitors), such
• Include more grains and beans in your diet.
as indinavir and ritonavir.
• Choose foods marked “low-salt” or “low-sodium.”
• Nonsteroidal anti-inflammatory drugs, such as
Foods labeled this way must contain less than 140 mg of
ibuprofen, ketoprofen, or aspirin.
sodium per serving.
• Ulcer medicines such as cimetidine (Tagamet).
• Do not use salt during cooking or at the table. Talk to
your health professional before using a salt substitute. It
may not be recommended, because most salt substitutes Other chemicals, such as insecticides, herbicides, and ethylene
contain potassium. Potassium can build up in the bodies glycol, can also cause acute renal failure.
of people with kidney disease and cause severe illnesses
and even death. http://health.yahoo.com/urinary-overview/nephrotic-syndrome-
• Avoid fast foods, prepackaged foods (such as TV topic-overview/healthwise--aa124985.html
dinners and frozen entrees), and processed foods (such
as lunch meats and cheeses). Always check the serving **************************************************
size on processed food. Eating more than the single
Childhood Nephrotic Syndrome
Childhood nephrotic syndrome can occur at any age but is most Children who relapse frequently, or who seem to be dependent on
common between the ages of 1-1/2 and 8 years. It seems to affect prednisone or have side effects from it, may be given a second
boys more often than girls. type of drug called a cytotoxic agent. The agents most frequently
used are cyclophosphamide, chlorambucil, and cyclosporine.
A child with the nephrotic syndrome has these signs: After reducing protein in the urine with prednisone, the doctor
may prescribe the cytotoxic agent for a while. Treatment with
cyclophosphamide and chlorambucil usually lasts for 8 to 12
• High levels of protein in the urine weeks, while treatment with cyclosporine frequently takes longer.
• Low levels of protein in the blood The good news is that most children "outgrow" this disease by
• Swelling resulting from buildup of salt and water. their late teens with no permanent damage to their kidneys.
The nephrotic syndrome is not itself a disease. But it can be the Other Conditions That Involve the Childhood Nephrotic
first sign of a disease that damages the tiny blood-filtering units Syndrome
(glomeruli) in the kidneys, where urine is made. In about 20 percent of children with the nephrotic syndrome, the
kidney biopsy reveals scarring or deposits in the glomeruli. The
The kidneys are two bean-shaped organs found in the lower back. two most common diseases that damage these tiny filtering units
They are about the size of a fist. They clean the blood by filtering are focal segmental glomerulosclerosis (FSGS) and
out excess water and salt and waste products from food. Healthy membranoproliferative glomerulonephritis (MPGN). Very rarely,
kidneys keep protein in the blood, which helps the blood soak up a child may be born with a condition that causes the nephrotic
water from tissues. But kidneys with damaged filters may let syndrome (congenital nephropathy).
protein leak into the urine. As a result, not enough protein is left
in the blood to soak up the water. The water then moves from the Since prednisone is less effective in treating these diseases than it
blood into body tissues and causes swelling. You may see is in treating minimal change disease, the doctor may use
swelling around your child's eyes, belly, and legs. Your child may additional therapies, including cytotoxic agents. Recent
urinate less often than usual and may gain weight from the excess experience with a class of drugs called ACE inhibitors (a type of
water. blood pressure drug) indicates that these drugs help prevent
protein from leaking into the urine and keep the kidneys from
To diagnose childhood nephrotic syndrome, the doctor may ask being damaged in children with the nephrotic syndrome.
for a urine sample to check for protein. The doctor will dip a strip
of chemically treated paper into the urine sample. Hope Through Research
The National Institute of Diabetes and Digestive and Kidney
Too much protein in the urine will make the paper change color. Diseases (NIDDK) conducts and supports research to help many
Or the doctor may ask for a 24-hour collection of urine for a more kinds of people with kidney disease, including children. NIDDK's
precise measurement of the protein and other substances in the Division of Kidney, Urologic, and Hematologic Diseases
urine. maintains the Pediatric Nephrology Program, which supports
research into the causes, treatment, and prevention of kidney
The doctor may take a blood sample to see how well the kidneys diseases in children, including minimal change disease,
are removing wastes. Healthy kidneys remove creatinine and urea congenital nephrotic syndrome, primary glomerular disease, and
nitrogen from the blood. If the blood contains high levels of these postinfection glomerulonephritis.
waste products, some kidney damage may have already occurred.
But most children with the nephrotic syndrome do not have ************************
permanent kidney damage. http://www.patient.co.uk/showdoc/27000748/
In some cases, the doctor may want to examine a small piece of Nephrotic Syndrome
the child's kidney under a microscope to see if substances there
are causing the syndrome. The procedure of collecting a small
tissue sample from the kidney is called a biopsy, and it is usually
performed with a long needle passed through the skin. The child
will be awake during the procedure and receive calming drugs
Nephrotic syndrome is a condition where the 'filters' in the
and a local painkiller at the site of the needle entry. General
kidney become 'leaky' and large amounts of protein leak from
anesthesia is used in the very rare cases where open surgery is
your blood into your urine. The main symptom is oedema
required. The child will stay overnight in the hospital to rest and
(fluid retention) which is mainly due to the low protein level
allow the health care team to ensure that no problems occur.
in the blood. Various diseases can cause nephrotic syndrome,
some more serious than others. Treatment and outcome
Minimal Change Disease (prognosis) vary, depending on the cause. The common cause
The most common form of the nephrotic syndrome in children is in children (minimal change disease) usually responds very
called minimal change disease. Doctors do not know what causes well to treatment.
it. The condition is called minimal change disease because
children with this form of the nephrotic syndrome have normal or
nearly normal biopsies. If your child is diagnosed with minimal
change disease, the doctor will probably prescribe prednisone,
which belongs to a class of drugs called corticosteroids.
Prednisone stops the movement of protein from the blood into the Understanding kidneys and urine
urine, but it does have side effects that the doctor will explain.
Following the doctor's directions exactly is essential to protect The kidneys clear waste materials from the body and maintain a
your child's health. The doctor may also prescribe another type of normal balance of fluids and chemicals in the body. The two
drug called a diuretic, which reduces the swelling by helping the kidneys lie to the sides of the upper abdomen, behind the
child urinate. intestines, and either side of the spine. Each kidney is about the
size of a large orange, but bean-shaped.
When protein is no longer present in the urine, the doctor will A large renal artery takes blood to each kidney. The artery divides
begin to reduce the dosage of prednisone. This process takes into many tiny blood vessels (capillaries) throughout the kidney.
several weeks. Some children never get sick again, but most do In the outer part of the kidneys tiny blood vessels cluster together
develop swelling and protein in the urine again, usually following to form structures called glomeruli.
a viral illness. However, as long as the child continues to respond
to prednisone and the urine becomes protein free, he or she has an Each glomerulus is like a filter. The structure of the glomerulus
excellent long-term outlook without kidney damage.
allows waste products and some water and salt to pass from the proteins normally found in the bloodstream, the main
blood into a tiny channel called a tubule, while keeping blood protein that leaks from the blood into the urine is called
cells and protein in the bloodstream. Each glomerulus and tubule albumin. A low blood level of albumin is a main feature
is called a 'nephron'. There are about one million nephrons in each of nephrotic syndrome.
kidney. • Fluid retention (oedema). This is a consequence of the
low level of albumin in the bloodstream, and other
complex factors not fully understood.
• A high blood level of cholesterol and other lipids (fats).
This is due to the change in the balance of various
protein levels in the blood due to the protein leakage.
• Normal kidney function, at least initially. This means
that the 'waste clearing' function of the kidneys is not
affected - at least not at first. However, some of the
conditions that cause nephrotic syndrome can progress
to cause kidney failure.
The name 'minimal change' comes from the fact that there is
virtually no change detectable in the glomeruli if a sample of
kidney is looked at under the microscope. Although the glomeruli
look normal under the microscope, there seems to be some minor
change in the glomeruli that allows leakage of protein. The cause
of minimal change disease is not clear. It probably has something
to do with a slight change in the immune system, or perhaps a
'reaction' of parts of the immune system to some unidentified
factor.
In severe cases of nephrotic syndrome, the oedema can become You may also have various complications from the underlying
extensive. Fluid may accumulate in the lower back, the arms, in condition which has caused the nephrotic syndrome. For example:
the abdominal cavity (ascites) or in the chest between the lungs
and the chest wall (pleural effusion).
• One of the most common complications of kidney
The main reason why fluid leaks out from the blood vessels and disorders is high blood pressure.
into the body's tissues with nephrotic syndrome is because of a • Some kidney disorders lead to kidney failure.
low level of protein in the blood. As protein is lost from the body • Some disorders (for example, diabetes, rheumatoid
in the urine, the body makes more protein in the liver which arthritis, etc) that cause nephrotic syndrome can have
passes into the bloodstream. However, in time the amount made various other symptoms and complications.
by the liver cannot keep up with the amount lost by the leaky
kidneys, and so the blood level of protein goes down. If the blood
Possible complications from treatment
level of protein is low then fluid tends to leak out of the blood
vessels into the body tissues. (Protein and other chemicals in the
blood exert an 'osmotic pressure' which tends to pull fluid into the Often, the treatment of nephrotic syndrome requires a course of
blood vessels. If the concentration of protein reduces, the osmotic steroid medicines or other medicines to suppress the immune
pressure reduces, and fluid leaks out.) system (see below). Some people develop side-effects and
complications from these treatments, especially if the treatment
Note: nephrotic syndrome is just one cause of oedema. There are requires high doses, or is for long periods.
other causes of oedema. For example, heart failure is the most
common cause of oedema, especially in older people. Do I need any tests?
Other symptoms that may develop include: A simple 'dipstick' test of your urine can confirm that it contains a
lot of protein. The amount of protein lost can be measured by
more detailed urine and blood tests. A low blood level of albumin
• Your urine may appear frothy.
combined with a lot of protein found in the urine usually confirms
• Tiredness, lethargy and being off your food. that you have nephrotic syndrome.
• If the nephrotic syndrome persists for a long time then
you may develop wasting of your muscles, and your To find the cause of the nephrotic syndrome
nails may become white.
• Depending on the cause of the nephrotic syndrome, you
A range of blood tests may be done to try and identify the cause
may also have other symptoms. For example, if you of the nephrotic syndrome. A kidney biopsy may also be done. A
have nephrotic syndrome as a complication of kidney biopsy is when a small sample of tissue is removed from a
rheumatoid arthritis you may have a range of other kidney. The sample is looked at under a microscope, or tested in
symptoms caused by the arthritis. Some conditions of other ways. This is often the most important test to clarify the
the kidney can cause high blood pressure and/or kidney cause of the nephrotic syndrome in adults. However, a biopsy is
failure. not usually done in children under the age of eight years. This is
because most cases in young children are due to minimal change
What are the possible complications? disease. A trial of treatment is usually advised first, and a biopsy
done only if treatment does not work. (There are some exceptions
Possible complications from nephrotic syndrome itself to this general rule.)
Complications can be caused by nephrotic syndrome itself due to To check on the function of the kidneys
the loss of normal proteins from the blood. These include:
Blood tests can check on the function of the kidneys. That is, how
well they are clearing waste products from the bloodstream.
What is the treatment for nephrotic syndrome? • Agraharkar M, Gala G, eMedicine, Nephrotic
Syndrome, 2007.
Treatment of oedema • Hodson E, Willis N, Craig J; Corticosteroid therapy for
nephrotic syndrome in children. Cochrane Database
Diuretics ('water tablets') help to clear the body of oedema. Syst Rev. 2007 Oct 17;(4):CD001533. [abstract]
Diuretics work by acting on the cells in the kidney tubules to
make them pass out more water rather than 'reabsorbing' water ***********************
back into the bloodstream. So, you pass out more urine. The http://www.patient.co.uk/showdoc/40000676/
excess fluid in the body's tissues then passes back into the
bloodstream to keep the blood volume up to normal. Your doctor
may also advise you to limit the amount of salt in your diet to try Nephrotic Syndrome
and limit oedema.
Description
Treatment of high blood pressure
Nephrotic syndrome is a pattern of presentation of renal disease,
Many people with kidney disorders have high blood pressure. If rather than a single pathological entity or diagnosis. Nephrotic
you develop high blood pressure then treatment is usually advised syndrome is also known as nephrosis and is defined by the
to bring your blood pressure down. A medicine called an ACE presence of nephrotic-range proteinuria, oedema, hyperlipidemia,
inhibitor is commonly used for this. and hypoalbuminaemia. It has serious complications and must be
on the differential diagnosis for any patient presenting with new
onset oedema.1
Treatment of the underlying cause
Pathophysiology
As mentioned, there are many causes of nephrotic syndrome. The
treatment depends on the underlying cause. Some causes are more
serious than others, some causes can be treated more easily than It comprises the following elements:
others.
Features of the nephrotic syndrome:
For example, treatment for minimal change disease usually works
well to stop the leak of protein from the kidneys. The usual • Glomerular dysfunction leading to excessive urinary
treatment for minimal change disease is a course of steroid protein excretion (formerly defined as >3.5 g/day but
medication which may last several months. In some cases, this is there appears to be individual variation around this cut-
a 'one off' treatment and the disease does not return. In some off figure)
cases of minimal change disease, the disease recurs from time to
• Hypoalbuminaemia as a result of urinary protein loss
time which needs repeated courses of steroid medication.
(albumin levels usually in range <25–30 g/l)
Steroids or other medicines called 'immunosuppressants' may be • Peripheral oedema due to hypoalbuminaemia
used to reduce inflammation and abnormal immune responses in • Hypercholesterolaemia/dyslipidaemia
various diseases that cause nephrotic syndrome. Your doctor will
advise on the treatment options for each situation. • Normal detoxifying renal function, at least initially
The primary abnormality in nephrotic syndrome is thought to be
What is the outlook (prognosis)? loss of a layer of negatively-charged heparin sulphate within the
glomerular basement membrane, that allows the increased
The outlook depends on the cause. For example, the most passage of large amounts of low-molecular weight anionic
common cause of nephrotic syndrome in children (minimal proteins during ultrafiltration. However, recent research has
change disease) usually responds well to treatment, and generally shown that the loss of albumin in the urine may not be due to
has a good outlook. The outlook is less good with some other excessive filtration across the glomerular basement membrane as
conditions. Your doctor will be able to give an outlook for your was previously supposed, rather a failure to reabsorb albumin
particular condition. after its ultrafiltration. It appears that renal disease may cause an
impairment of the ability of cells in the proximal renal tubules to
Further help and information endocytose albumin that has been filtered across the glomeruli,
and deliver it back into the blood supply around the renal
Nephrotic Syndrome in Children Support Group tubules.2
Initial management should focus on investigating the cause, 1. Hull RP, Goldsmith DJ; Nephrotic syndrome in adults.
identifying complications, and managing the symptoms of the BMJ. 2008 May 24;336(7654):1185-9.
disease.1 All patients should be referred to a nephrologist for 2. Russo LM, Sandoval RM, McKee M, et al; The normal
further investigation, which often includes a renal biopsy.1 kidney filters nephrotic levels of albumin retrieved by
proximal tubule cells: Retrieval is disrupted in nephrotic
Indications for acute admission include: states. Kidney Int. 2007 Jan 17;. [abstract]
3. Agraharkar M, Gala G, Gangakhedkar AK; Nephrotic
• Severe generalised oedema, particularly if pleural Syndrome. eMedicine, February 2007.
effusion/oedema causing respiratory compromise 4. Lane J; Nephrotic Syndrome. eMedicine, Dec 2008;
• Tense scrotal/labial oedema Paediatric overview.
• Complications of the nephrotic state (e.g. sepsis,
pneumonia, MI, DVT, growth failure)
• Inability to comply with therapy/inability to cope with
condition in family/independently