A Family Doctor's Tale - NEPHROTIC SYNDROME

DOC I HAVE NEPHROTIC SYNDROME

Nephrotic Syndrome is a rare disease characterized by the edema(accumulation of fluid in the body) and hypo-albumemia (low blood protein due to protein loss through the kidneys).

The cause is the increased capillary permeability of the glomeruli to plasma protein  due to the inflammation and allows loss of blood and protein in the urine and retention of salt, water and nitrogen.

Loss of negative charges on the filtering membrane allows negatively charged molecules including albumin to enter the urinary tract.

Reduced protein in the blood leads to edema.

There are 3 types of Nephrotic Syndrome:
1.Minimal Change Nephropathy (MCN) occurs in children with very little microscopic changes in the glomerular tissues of the kidney.

2.Focal glomerulonephritis has appearance of MCN above but develop sclerosis of the glomeruli later.

3.Diffuse proliferative glomerulonephritis and membranous nephropathy are more common in adults and be secondary to other diseases such as:


a.SLE and other connective tissues
b.diabetes
c.allergies to drugs such as penicillinamine, tolbutamide, probenecid
d.certain infections
e.cancer (carcinomas and lymhomas)

Symptoms:
1.Urine may show the presence of protein at least 3.5g/day

2.low protein in blood causes decreased plasma mass pressure resulting in retention of water and salts

3.swelling of the face and ankle occurs as a result

4.edema ranges from puffiness of eyes, ascites (water in abdominal cavity), pleural effusions(water in the lungs) to generalized edema

5.With increasing edema and nitrogen retention, headaches, nausea and vomiting may occur.

6.high blood pressure may occur in one third of patients

7.The blood in nephrotic syndrome tend to clot easily and the patient may develop blood clots in his leg veins.

8.Lipids may be raised in the blood especially triglicerides and cholesterol

9.Symptoms and signs of underlying diseases such as diabetes, SLE etc may be present

Diagnosis:
1.A history of recent kidney infection followed by swelling of face ang legs

2.Urinary protein loss of 15g per day

3.Full examination of fresh urine for red blood cells and casts(indicating glomerulonephritis) and lipid

4.Full blood count and tests for kidney function

5.Ultrasound of kidneys

6.Biopsy of kidney and histology of specimen

Complications:
1.Renal failure

2.hemolytic uremic stndrome

Treatment should be in hospital:
1.Minimal change nephropathy in children are treated with high doses of corticosteroids up to 1mg prednisolone per kg per day.
In most cases within 10 days there should be reduction of fluid in the body with increased urinary passage of urine.
Relapses may occur and should be treated with more corticosteroids or cyclophosphamide

2.In adults treatment with corticosteroids should help to treat the edema.

3.In addition thiazide diuretics or frusemide to reduce edema.
Spironolactone and amiloride may also help

4.Hypertension may require anti hypertensive. The use of corticosteroids may aggravate hypertension.

5.Diet with low salt and protein

6.Intake of fluids are restricted
These 2 measures will reduce the strain on the kidneys and prevent accumulation of fluids

Prognosis:
1.In most cases of MCN the illness will clear up with proper treatment.

Some may have relapses.

After 3 years of remission 99% of MCN will not have relapses
MCN cases seldom progress to renal failure.

2.In focal glomerulonephritis cases nephrotic syndrome may develop into chronic renal failure.

3.In Membranous nephropathy
25% complete remission
25% improves in renal function
25% have persistent nephrotic syndrome
25% will have chronic renal failure