Nephrosis, Edema, Kidney Failure, Hypertension, Vein thrombosis, Arteriosclerosis, Albumin, Anasarco, NS, Amyloidosis, Congenital nephrosis, Glomerular sclerosis, FSGS, Glomerulonephritis, GN, IgA nephropathy, Berger's Disease, Minimal change disease, MDC, Pre-eclampsia, Osteoporosis, Multiple myeloma, Systemic lupus erythematosus, Mesangiocapillary glomerulonephritis, Diabetes, Hyperproteinemia, Hyperlipedemia, Anarsca, Hyperalbunemia, Membranous Nephropathy, Primary Nephrotic Syndrome, Lipoid Nephorsis, NIL, lymphedema, Pitting edema
Nephrotic syndrome is a kidney disorder in which damage has occurred to the kidneys in the kidney's filtering system called the glomeruli. The resulting central feature is loss of proteins, albumin and globulin in the urine with the subsequent low levels of protein in both the blood. This condition is called proteinuria. Nephrotic Syndrome is a rare condition affecting approximately two in every ten thousand individuals. In children, it is general diagnosed between two and three years old and has a high rate among males than females.
The condition can be caused by a multitude factors. These include infection, drug exposure, hereditary disorders, immune disorders, or diseases that affect multiple body systems including diabetes, systemic lupus erythematosus, multiple myeloma, and amyloidosis (the stiffening and subsequent malfunction of the kidney due to fibrous protein deposit in the tissue) . It can also accompany kidney disorders such as glomerulonephritis, focal and segmental glomerulosclerosis and mesangiocapillary glomerulonephritis.
The following diseases can cause specific damage to the glomeruli and often result in the development of heavy proteinuria and in many instances NS: (1)
Amyloidosis (the stiffening and subsequent malfunction of the kidney due to fibrous protein deposit in the tissue)
Focal segmental glomerular sclerosis (FSGS) (creates scar tissue in the glomerulus, damaging its protein-repellant membrane)
Diffuse mesangial proliferative GN (affecting the messangium)
Membranous (damages the protein-repellant membrane)
Postinfectious (occurs after an infection)
IgA nephropathy (Berger’s disease) (deposit of specific immunoglobulin A causing an inflammatory reaction and leading to glomerulonephritis)
Minimal change disease (Nil’s disease)
Pre-eclampsia (rarely associated with NS, more often associated with heavy proteinuria)
In children the most common cause is minimal change disease. A disease of unknown etiology, it cause loss of protein through the urine. It is also difficult to diagnosis because biopsy result show up normal of near normal.
Patients will present with four main considerations.
1. High levels of protein in the urine
2. Low levels of protein in the blood (hypoalbuminemia)
3. High levels of cholesterol in the blood (hypercholesterolemia)
4. Edema, often severe usually involving the lower legs and feet. This may be referred to as focal edema.
Other clinical symptoms may include frothy urine, anoxrexia, malaise, retinal sheen, abdominal pain and wasting of muscles.
Complications can also include renal (kidney) failure, hypertension, susceptibility to infections and blood clotting (thrombosis). Other complications include hypoproteinemia, hypoalbunemia, hyperlipedemia with elevated cholesterols, triglicerides, other lipids and edema.
Nephrotic related edema causes tissues to be soft, puffy and impressionable (pitting edema) to the touch. Initially the edema will be in the legs, but in later stages will progress to abdomen, hands and even around the eyes. In very late stages the entire body may experience swelling (anasarca).In situations of edema, especially with marked edema, the skin may ooze clear fluids and breakdown of tissue is common. While the weeping wound complication of nephrotic syndrome is similar to the weeping wounds of lymphedema, the two condition are distinctly different.
In addition to blood tests, and urinalysis, the most important diagnostic tool is a kidney biopsy. The kidney biopsy may reveal the underlying cause and extent or progression of the disease.
The course of treatment for Nephrotic Syndrome includes the various complications. This is referred to as "nonspecific treatment." These treatment include Corticosteroid, immunosuppressive, antihypertensive, and diuretic medications and antibiotics for infections.
Supportive treatment may also include diet, high in quality protein and fiber, but low in saturated fat and cholesterol.
Specific treatment focuses on the underlying causes of the condition.
Treatment for the edema may include diuretics and or decongestive massage therapy.
Treatment of minimal change disease in children usually involves the use of diuretics for the relief of edema and the use of a corticosteroid called prednisone. Minimal Change Disease may also be referred to as Lipoid Nephorsis or NIL Disease.
Other features of treatment may include hospitilization, a prolonged period of treatment, frequent monitoring by patients and doctors, administration of drug associated with significant adverse events. (2)
Patient prognosis depends on several factors. The most important one being the cause or underlying reason for the condition. Also, some patients may have a spontaneous remission while others experience a gradual progression of the condition.
Other factors will include reactions or complications from the treatment modalities. Some of these complications include atherosclerosis (hardening of the arteries), and adverse reaction to steroids. These reactions include osteoporosis, cataracts, increased risk of infections and even diabetes.(c) 2005 Pat O'Connor - Lymphedema People
Childhood Nephrotic Syndrome
This fact sheet has been written to tell you some facts about a kidney disease called the nephrotic syndrome. This illness also is called nephrosis or minimal change disease. The brochure will give you and your family information about your child's illness. It will tell you what will happen with this illness. You also should talk to your doctor. The more you know, the more you can help your child.
What do the kidneys do?
The kidneys are two fist-sized organs found in the lower back. When they are working well, they clean the blood, and get rid of waste products, excess salt and water. When diseased, the kidneys may get rid of things that the body needs to keep, such as blood cells and protein.
What is the nephrotic syndrome?
This is an illness where the kidney loses protein in the urine. This causes protein in the blood to drop, and water moves into body tissues, causing swelling (edema). You will see the swelling around the child's eyes, in the belly, or in the legs. Your child will not go to the bathroom as often as usual and will gain weight with the swelling.
Do other kidney diseases cause edema and protein in the urine?
Yes. Edema and protein in the urine are common in other types of kidney disease, especially a disease called glomerulonephritis.
What causes the nephrotic syndrome?
In the majority or cases, the cause is not known. The National Kidney Foundation has active research programs into causes and treatments of the nephrotic syndrome.
Who gets it?
Usually, young children between the ages of 1 1/2 and 5. It happens twice as often in boys as girls. However, children of all ages and adults also can get it.
How can you tell if your child has it?
You may see that your child has swelling around the eyes in the morning. You may think that your child has an allergy. Later, the swelling may last all day, and you may see swelling in your child's ankles, feet and belly.
Also, your child may be:
• more tired & more irritable
• eating less
• pale looking
The child may have trouble putting on shoes or buttoning clothes because of swelling.
How is the nephrotic syndrome treated?
The treatment will try to stop the loss of protein in the urine, and increase the amount of urine. Usually, the doctor will start your child on a drug called prednisone. Most children get better with this drug.
What does prednisone do?
Prednisone is used to stop the loss of protein from the blood into the urine. After one to four weeks of treatment, your child should begin going to the bathroom more often. As your child makes more urine, the swelling will go away.
When there is no protein in the urine, the doctor will begin to reduce the amount of prednisone over several weeks. The doctor will tell you exactly how much prednisone to give your child each day. Never stop prednisone, unless the doctor tells you to do so. If you stop this drug or give your child too much or too little, he or she may get very ill.
Sometimes, your child will stay healthy after treatment. Your child may relapse (get sick again) at any time, even after a long time with good health. Getting sick may happen after a viral infection, such as a cold or the flu.
What problems call occur with prednisone?
Prednisone can be a very good drug, but it has a number of side effects. Some of these side effects are:
• being hungry
• gaining weight
• acne (pimples)
• mood changes (very happy, then very sad)
• being overactive* more chance of infection
• slowing of growth rate
Side effects are more common with larger doses and if it is used for a long time; once prednisone is stopped, most of these side effects go away.
What if prednisone does not work?
If prednisone does not work for your child or if your child has serious side effects, the doctor may order another kind of medicine, called an immunosuppressive drug. These drugs decrease the activity of the body's immune system. They are effective in most, but not all, children. Your doctor will discuss in detail with you the good and bad things about the drug. The side effects of these drugs include: increased susceptibility to infections, hair loss and increased blood cell production.
Parents also should be aware that children taking immunosuppressive drugs may become ill if they develop chicken pox. Therefore, you should notify your doctor any time that your child is exposed to chicken pox while on these medications.
Your child also may be given diuretics (water pills). These drugs help the kidney get rid of salt arid water. The most common water pill used in children is called furosemide. If your child starts to have a problem with vomiting or diarrhea, you should call your doctor as the child can lose too much fluid and become even sicker. Once protein disappears from the urine, diuretics should stopped.
What other problems happen with the nephrotic syndrome?
Most children will have problems only with swelling. However, a child with nephrotic syndrome can develop a serious infection in the belly. If your child has a fever or starts complaining of severe pain in the belly, you should call your doctor at once.
Sometimes, children with nephrotic syndrome get blood clots in their legs. If this happens, your child will complain of:
• severe pain in arm or leg
• swelling of arm or leg
• changes in color or temperature of arm or leg
If any of these things happens, you should call your doctor right away.
What can parents do?
Much of your child's care will be given by you. Pay attention to your child's health, but do not overprotect the child. If your child is ill or taking prednisone, the doctor will recommend a low salt diet. This type of diet will make your child more comfortable by keeping the swelling down. Try to give your child foods that he or she likes, but that are low in salt. Ask the dietitian for suggestions.
Usually, the child will be allowed to drink as much as he or she wants. A child's natural thirst is the best guide as to how much to drink. You should also weigh your child and keep a record of weight to spot a change in the disease.
The first sign that your child is getting sick again is the return of protein in the urine. Because of this, many doctors ask you to check your child's urine regularly. To do this, a special plastic strip with a small piece of paper on the end is dipped into the urine. The paper will change color when protein is in the urine. This test can be done easily at home and it can detect a relapse before any swelling is seen. Check with your doctor to learn how to do the test and how often to do it.
When there is swelling, check that your child's clothing is not too tight because the clothing can rub the child's skin over the swollen areas. This can make the skin raw, and it may get infected.
Your child will probably have this disease several years. It is very important to treat your child as normally as possible. Your child needs to continue his or her usual activities, such as going to school and seeing friends. Your child should be treated just like other children in the family in terms of discipline. Occasionally, your child may not go to school for a time. Your doctor will let you know if this is necessary. Keeping your child out of school or not letting him or her see friends will not change the illness.
Does the disease ever go away?
Sometimes. Even though the nephrotic syndrome does not have a specific cure, the majority of children "outgrow" this disease in their late teens or early adulthood. Some children will have only one attack of the nephrotic syndrome. If your child does not have another attack for three years after the first one, the chances are quite good that he or she will not get sick again.
Still, most children will have two or more attacks, The attacks are more frequent in the first one to two years after the nephrotic syndrome begins. After ten years, less than one child in five is still having attacks. Even if a child has a number of attacks, most will not develop permanent kidney damage. The major problem is to control their accumulation of fluid using prednisone and diuretics. Children with this disease have an excellent long-term outlook.
What else should I know?
1. Most children with the nephrotic syndrome respond to treatment.
2. Most children with the nephrotic syndrome have an excellent long-term outcome.
3. You should feel free to ask your child's doctor any questions.
What if I have more questions?
If you have more questions, you should speak to your doctor. You also can get additional information by contacting your local National Kidney Foundation Affiliate.
What is The National Kidney Foundation and how does it help?
Twenty million Americans have some form of kidney or urologic disease. Millions more are at risk. The National Kidney Foundation, Inc., a major voluntary health organization, is working to find the answers through prevention, treatment and cure. Through its 50 Affiliates nationwide, the Foundation conducts programs in research, professional education, patient and community services, public education and organ donation. The work of The National Kidney Foundation is funded entirely by public donations.
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