Treatment of Nephrotic Syndrome MLTTP (case study) Bakur Ahmed Wedaa Ali 57752 Monday 31/12/2012

Nephrotic versus Nephritic Syndrome Nephritic syndrome is a collection of signs (syndrome) associated with disorders affecting the kidneys, more specifically glomerular disorders. It's characterized by having small pores in the podocytes of the glomerulus, large enough to permit proteins (proteinuria) and red blood cells (hematuria) to pass into the urine. By contrast, Nephrotic syndrome is characterized by only proteins (proteinuria) moving into the urine. Both nephritic syndrome and nephrotic syndrome result in hypoalbuminemia due to protein albumin moving from the blood to the urine.

Figure 25.7a

Figure 25.7b, c

Nephrotic Syndrome Marked loss of protein in the urine Urinary excretion of protein > protein production Protein level in blood falls Causes edema due to low plasma osmotic pressure Clinical manifestations Marked leg edema Ascites

Nephrotic Syndrome cont. Prognosis In children: minimal glomerular change, complete recovery In adults: a manifestation of severe progressive renal disease May result from Glomerulonephritis Diabetes (causing glomerular changes) Systemic lupus erythematosus, SLE Other kidney diseases

Physical Examination Edema is the predominant feature of nephrotic syndrome and initially develops around the eyes and legs. With time, the edema becomes generalized and may be associated with an increase in weight, the development of ascites, or pleural effusions. Hematuria and hypertension manifest in a minority of patients. Additional features on exam will vary according to cause and as a result of whether or not renal function impairment exists. Thus, in the case of longstanding diabetes, there may be diabetic retinopathy, which correlates closely with diabetic nephropathy. If the kidney function is reduced, there may be hypertension and/or anemia.

Diet Reduce sodium intake to 1000–2000 mg daily. Foods high in sodium include salt used in cooking and at the table, seasoning blends (garlic salt, Adobo, season salt, etc.) canned soups, canned vegetables containing salt, luncheon meats including turkey, ham, bologna, and salami, prepared foods, fast foods, soy sauce, ketchup, and salad dressings. On food labels, compare milligrams of sodium to calories per serving. Sodium should be less than or equal to calories per serving.

cont, Eat a moderate amount of high protein animal food: 3-5 oz per meal (preferably lean cuts of meat, fish, and poultry) Avoid saturated fats such as butter, cheese, fried foods, fatty cuts of red meat, egg yolks, and poultry skin. Increase unsaturated fat intake, including olive oil, canola oil, peanut butter, avocadoes, fish and nuts. Eat low-fat desserts. Increase intake of fruits and vegetables. No potassium or phosphorus restriction necessary. Monitor fluid intake, which includes all fluids and foods that are liquid at room temperature. Fluid management in nephrotic syndrome is tenuous, especially during an acute flare.

Monitoring kidney function: Supportive Monitoring and maintaining euvolemia (the correct amount of fluid in the body): Monitoring urine output, BP regularly. Fluid restrict to 1 L. Diuretics (IV furosemide). Monitoring kidney function: do EUCs daily and calculating GFR. Treat hyperlipidemia to prevent further atherosclerosis. Prevent and treat any complications [see below] Albumin infusions are generally not used because their effect lasts only transiently. Prophylactic anticoagulation may be appropriate in some circumstances.

Specific Immunosuppression for the glomerulonephritides (corticosteroids, ciclosporin). Standard ISKDC regime for first episode: prednisolone -60 mg/m2/day in 3 divided doses for 4 weeks followed by 40 mg/m2/day in a single dose on every alternate day for 4 weeks. Relapses by prednisolone 2 mg/kg/day till urine becomes negative for protein. Then, 1.5 mg/kg/day for 4 weeks. Frequent relapses treated by: cyclophosphamide or nitrogen mustard or ciclosporin or levamisole. Achieving better blood glucose level control if the patient is diabetic. Blood pressure control. ACE inhibitors are the drug of choice. Independent of their blood pressure lowering effect, they have been shown to decrease protein loss.

Complications Venous thrombosis: due to leak of anti-thrombin 3, which helps prevent thrombosis. This often occurs in the renal veins. Treatment is with oral anticoagulants (not heparin as heparin acts via anti-thrombin 3 which is lost in the proteinuria so it will be ineffective.) Hypercoagulopathy due to extravasation of fluid from the blood vessels (oedema) is also a risk for venous thrombosis. Infection: due to leakage of immunoglobulins, encapsulated bacteria such as Haemophilus influenzae and Streptococcus pneumoniae can cause infection. Acute renal failure is due to hypovolemia. Despite the excess of fluid in the tissues, there is less fluid in the vasculature. Decreased blood flow to the kidneys causes them to shutdown. Thus it is a tricky task to get rid of excess fluid in the body while maintaining circulatory euvolemia. Pulmonary edema: again due to fluid leak, sometimes it leaks into lungs causing hypoxia and dyspnoea.

Complications Growth retardation: does not occur in MCNS.It occurs in cases of relapses or resistance to therapy. Causes of growth retardation are protein deficiency from the loss of protein in urine,anorexia (reduced protein intake), and steroid therapy (catabolism). Vitamin D deficiency can occur. Vitamin D binding protein is lost. Hypothyroidism can occur. Thyroxine is reduced due to decreased thyroid binding globulin. Microcytic hypochromic anaemia is typical. It is iron-therapy resistant. Hypocalcemia can occur as a result of nephrotic syndrome. It may be significant enough to cause tetany. Hypocalcemia may be relative; calcium levels should be adjusted based on the albumin level and ionized calcium should be checked. Cushing's Syndrome

Prognosis The prognosis depends on the cause of nephrotic syndrome. It is usually good in children, because minimal change disease responds very well to steroids and does not cause chronic renal failure. However other causes such as focal segmental glomerulosclerosis frequently lead to end stage renal disease. Factors associated with a poorer prognosis in these cases include level of proteinuria, blood pressure control and kidney function (GFR).

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