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NEPHROTIC SYNDROME

Definition Nephrotic syndrome is a primary glomerular disease characterized by proteinuria, hypoalbuminemia, edema, and hyperlipidemia. It is seen in any condition that seriously damages the glomerular capillary membrane, causing increased glomerular permeability with loss of protein in the urine. Generally a disorder of childhood, it does occur in adults, including the elderly. Causes in clued chronic glomerulonephritis and diabetes mellitus, among other conditions.

Main Causes/ Etiology

1. Glomerulonephritis Glomerulonephritis is also known as glomerular nephritis (GN) or glomerular disease. It is a disease of the kidney, characterized by inflammation of the glomeruli. Glomeruli are very small blood vessels in the kidneys that act as tiny little filters - there are about one million glomeruli in each kidney. The disease damages the kidneys' ability to remove waste and excess fluids from the body. 2. Metabolic Diseases such as Diabetes Mellitus, Amyloidosis and Multiple myeloma.

3. Systemic sensitivity diseases such as systemic lupus erythematosus and periarteritis nodosa. 4. Circulatory diseases such as heart failure, renal vein thrombosis, and sickle cell anemia. Signs and Symptoms 1. Edema which is usually soft and pitting and commonly occur around the eyes, in dependent areas and in the abdomen. 2. Proteinuria ( > 3.5 g / day ) caused by abnormal permeability of the glomerular basement membrane results in loss of protein in the urine. 3. Hyperlipidemia occurs because of increased hepatic lipoprotein synthesis in response to decreased levels of serum albumin. 4. Hypoalbuminemia is a low level of albumin (a protein) in the blood due to proteinuria. Low albumin in the blood causes fluid to move from the blood into the tissue, causing swelling. The kidney perceives the decrease of fluid in the blood and aggressively retains as much fluid and salt as it can. This contributes to the body's fluid-overload state. 5. Weight gain from fluid retention 6. Shortness of breath may develop because fluid accumulates in the space surrounding the lungs (pleural effusion) Complications 1. Acute kidney failure. If your kidneys lose their ability to filter blood due to damage to the glomeruli, waste products may build up quickly in your blood. If this happens, you may need emergency dialysis an artificial means of removing extra fluids and waste from your blood typically with an artificial kidney machine (dialyzer). 2. Infections

People with nephrotic syndrome have an increased risk of infections especially cellulitis and, in 2 to 6%, spontaneous bacterial peritonitis. The contributing factors are unknown but possibly because of the loss of opsonins and immunoglobulins. A large number of immune globulin from urine loss, plasma protein reduced, affecting antibody formation. Adrenal cortex hormones and the application of cytotoxic drugs, in which patients resistant to systemic decline in vulnerable to infection, such as skin infections, primary peritonitis, respiratory tract infections, urinary tract infection, or even induced sepsis.

3. Coronary Heart Disease Nephrotic syndrome patients often have hyperlipidemia and hypercoagulability, so prone to coronary heart disease. It was reported that patients with nephrotic syndrome, the incidence of myocardial infarction 8 times higher than normal. Nephrotic syndrome with coronary heart disease has become the cause of death of the third factor (after infection and renal failure). 5. Hypercoagulability and Thromboembolism Especially renal vein thrombosis and pulmonary embolism, which occur in up to 5% of children and 40% of adults. A hypercoaguable state, in which the blood abnormally overclots, is also seen in some patients with NS. This means that they are at risk for developing a blood clot in the legs or in the renal veins that transport blood from the kidney. Some patients take blood thinners to prevent this complication. The contributing factors are (1) loss of antithrombin III, (2) Increased hepatic synthesis of clotting factors, (3) Platelet abnormalities, and (4) Hyperviscosity caused by hypovolemia. Thrombosis prone patients with nephrotic syndrome, particularly in the incidence of membranous nephropathy up to 25% ~ 40%. The

reasons for the formation of thrombus edema, the patient's less venous stasis, high blood lipids, blood concentration to viscosity increase, high fibrinogen levels, and V, , , x factor increases and the use of adrenal cortex hormones and the blood-prone hypercoagulable state and so on. Diagnostic Tests 1. Urinalysis A finding of significant proteinuria (3 g protein in a 24-h urine collection) is diagnostic (normal excretion is < 150 mg/day). Besides proteinuria, urinalysis may demonstrate RBCs and casts (hyaline, granular, fatty, waxy, RBC, or epithelial cell). Lipiduria, the presence of free lipid or lipid within tubular cells (oval fat bodies), within casts (fatty casts), or as free globules, suggests a glomerular disorder causing nephrotic syndrome. Urinary cholesterol can be detected with plain microscopy and demonstrates a Maltese cross pattern under crossed polarized light; Sudan staining must be used to show triglycerides. Urine for a urinalysis can be collected at any time. The first morning sample is considered the most valuable because it is more concentrated and more likely to yield abnormalities if present. It is important to clean the genitalia before collecting urine. Bacteria and cells from the surrounding skin can contaminate the sample and interfere with the interpretation of test results. With women, menstrual blood and vaginal secretions can also be a source of contamination. Women should spread the labia of the vagina and clean from front to back; men should wipe the tip of the penis. As you start to urinate, let some urine fall into the toilet, then collect one to two ounces of urine in the container provided, then void the rest into

the toilet. This type of collection is called a midstream collection or a clean catch. 2. Blood Tests A blood sample for a blood test can be taken from either an artery or a vein. In the most common type of blood test, blood is taken from a vein. If the blood sample needed is very small, then a prick to the tip of a finger will allow enough blood to be taken. If the blood is taken from an artery or vein, then a needle is used. It is usually quite painless with only a small amount of discomfort. If you have nephrotic syndrome, a blood test may show low levels of the protein albumin (hypoalbuminemia) specifically and decreased levels of blood protein overall. Loss of blood protein may cause an increase in blood cholesterol and blood triglycerides. Serum creatinine and blood urea also may be measured to assess your overall kidney function. Full blood count and coagulation screen Renal function including plasma creatinine and estimated glomerular filtration rate Liver function tests to exclude concomitant liver pathology Bone profilecorrected (for albumin) plasma calcium There are many ways to perform a kidney biopsy. The most

3. Renal Biopsy common uses ultrasound guidance. This means the doctor uses an ultrasound image to locate the proper area in your kidney. The biopsy is done in the hospital, usually in the radiology suite. You will lie face down for at least 20 - 30 minutes. A towel may be placed under your upper abdomen so you remain in the correct position. Ultrasound will be used to find the proper biopsy site. The health care provider will then inject local numbing medicine (anesthetic) under the skin near the area.

The health care provider makes a tiny cut in the skin and

inserts a biopsy needle into the area and to the surface of the kidney. You will be asked to take and hold a deep breath as the needle is introduced into the kidney. If the health care provider is not using direct ultrasound guidance, you may be asked to take deep breaths to verify the needle is in place. The biopsy needle is then withdrawn, and pressure is applied to the biopsy site to stop the bleeding. The needle may need to be inserted again (possibly several times) before enough tissue is collected. After the procedure, a bandage is applied to the biopsy site. You will need to stay in bed for 6 - 8 hours after the procedure and will remain in the hospital for at least 12 hours. The health care team will give you pain medicines and fluids by mouth or a vein. Your urine will be checked for excessive bleeding. (A little bleeding usually occurs.) Blood counts and vital signs are monitored. A renal biopsy is the removal of a small piece of kidney tissue for laboratory examination. It is indicated in adults to diagnose the disorder causing idiopathic nephrotic syndrome. Idiopathic nephrotic syndrome in children is most likely minimal change disease and is usually presumed without biopsy unless the patient fails to improve during a trial of corticosteroids. Specific biopsy findings are discussed under the individual disorders. During a kidney biopsy, a special needle is inserted through your skin and into your kidney. Kidney tissue is collected and sent to a laboratory for testing. Ideal Diet The main aim of nutritional management of Nephrotic syndrome is to replace the protein loss by having an adequate intake of proteins. However high

intake of protein must be avoided to prevent any tubular damage to the kidneys caused by filtering of the excess proteins. Sodium intake in diet should be low. Fat intake should also be low. Fluid intake should be restricted as per the physician's advice.

Foods that can be taken: Cow's milk, skimmed milk Yogurt Wheat, cereals, sprouts, pulses and legumes such as tur dal, moong dal, rajmah, chana, lentils (masoor), etc. Eggs, fish, dry fish, chicken, lean meat, etc. Vegetables and fruits Soups, sauces, chocolate drinks, juices, etc (but with low sodium content) Wafers, popcorns, chutneys which are prepared in less salt. Moderate to low intake of vegetable oils, butter and mayonnaise. Noodles, spaghetti, pancakes, etc (low in salt) Foods to be avoided in nephrotic syndrome:

Excess of protein should be avoided because a very high protein diet may cause tubular damage to the kidneys as the kidneys will have to filter more of the proteins. But moderate protein intake (about 1 gm/kg body weight) is mandatory to compensate for the protein loss in the urine.

High amount of fats should be avoided as the cholesterol and triglyceride levels tend to be high in patients with Nephrotic syndrome. The diet must be high in calories so as to conserve proteins, yet low in fats. Excess of oily food and saturated fats (ghee, margarine, etc) should be avoided.

Sodium in the diet should be minimum so as to prevent fluid accumulation and oedema. The foods that are high in sodium content and thereby should be avoided are:

Salted wafers, popcorns, salted biscuits, snacks, chips, etc . Papads all varieties. Salted pickles, chutneys, curry powder commercial preparations. Commercial salad dressings and sauces. Soup cubes. Bakery products, bread, biscuits.

Salted cashew nuts, pistachio, walnuts, and peanuts. cheese, preservative containing foods, noodle mixes, pastas. Salted or canned meat. Foods containing baking soda and ajinomoto

Commercial

Medical Management

Blood pressure medications. Drugs called angiotensin-converting enzyme inhibitors reduce blood pressure and also reduce the amount of protein released in urine. Medications in this category include benazepril (Lotensin), captopril (Capoten) and enalapril (Vasotec). Another group of drugs that works in a similar way is called angiotensin II receptor blockers and includes losartan (Cozaar) and valsartan (Diovan).

Water pills. Water pills (diuretics) help control swelling by increasing your kidneys' fluid output. Diuretic medications include chlorothiazide, hydrochlorothiazide, furosemide (Lasix) or spironolactone (Aldactone).

Cholesterol-reducing medications. Medications called statins can help lower cholesterol levels. Statins include atorvastatin (Lipitor), fluvastatin (Lescol), lovastatin (Altoprev, Mevacor), pravastatin (Pravachol), rosuvastatin (Crestor) and simvastatin (Zocor).

Blood thinners. Medications called anticoagulants help decrease your blood's ability to clot and reduce your risk of developing blood clots. Anticoagulants include heparin or warfarin (Coumadin).

Immune-system-suppressing medications. Medications to control the immune system, such as corticosteroids, may decrease the inflammation that accompanies kidney disorders, such as membranous nephropathy.

Antibiotics. Antibiotics can help control infections caused by bacteria. Bed rest for a few days to promote diuresis and reduce edema. Diet with high biologic protein (0.8 g/kg/day) to replenish urinary losses. Low sodium, low saturated fat, liberal potassium diet to help control blood cholesterol levels and control swelling.

Surgical Management: NONE Nursing Management 1. Careful monitoring of daily weight and intake and output is important to assess extent of edema and fluid balance. A weight gain of more than 0.5 kg/day suggest fluid retention. 2. Monitor heart rate and blood pressure. Tachycardia and hypertension can occur because of failure of the kidneys to excrete urine. 3. Frequently check for protein in the urine which may appear frothy to check for proteinuria and for kidney functioning. 4. After kidney biopsy, watch for bleeding and shock since this may indicate complications with the diagnostic test done. 5. Provide good skin care because the patient with nephrtic syndrome usually has edema. 6. To avoid thrombophlebitis, encourage activity and exercise, and provide antiembolism stockings as ordered. 7. Instruct patient receiving corticosteroids or cyclosporine regarding medication, signs and symptoms and adverse reactions that must be reported to physician for immediate intervention. 8. Instruct patient regarding dietary regimen so that their condition may remain stable as long as possible. 9. Offer the patient and his family reassurance and support, especially during the acute phase, when edema is severe and the patients body image changes.

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