Nephrotic Syndrome

Kidneys receive approximately 1 liter of blood every minute. Of this, about 100 ml gets filtered in the kidney. This contains, small molecules and few large molecules. This is due to the intricate cellular structure that prevents proteins from getting filtered into the urinary space. A derangement of this filter results in passage of heavy amounts of proteins in urine. This condition is called heavy proteinuria or Nephrotic syndrome or Nephrosis.

Definition:

In the urine, the protein excretion is > 3.5 gm/day or in children, it is >50mg/kg of body weight. In most cases, the serum albumin is <3gm/dl (normal>4), and passage of lipids in the urine. The lipids in the blood increase and swelling of face and feet also occur.

Causes of Nephrotic Syndrome:

In children, most cases are due to Minimal Change Disease, FSGS, MPGN etc. In adults, most cases are due to primary illness while upto 30% may be due to secondary illness.

Clinical Features:

Apart from edema, there is a tendency towards clotting of the blood in blood vessels. If this involves a critical area, like heart, brain or kidneys complications may occur. Infections also are common. In addition, kidney function may be reduced due to either the disease or its treatment. Sometime the blood pressure may be elevated.

In some cases, continuation of excess proteins in the urine may lead to glomerular damage and Chronic kidney disease leading to renal failure.

Diagnosis:

After confirmation of excess protein in the urine, a kidney biopsy is required in most cases. In children between 2 to 8 years, a biopsy may be deferred if there is a good response to steroids as Minimal Change disease is presumed.

Treatment:

The treatment consists of steroids in primary cases and in case of poor response other immunosuppressants and ACE-Inhibitors. Disease often has remissions and relapses which need to be monitored and treated appropriately.

MYTHS AND FACTS ABOUT KIDNEY DISEASE

Myth : All kidney diseases are serious and incurable

Fact : Most kidney diseases are treatable. Some are self-limiting and occur only once in a lifetime. Some progress towards renal failure but this progression can be slowed down if the disease is detected early. Even in late cases, treatment may help in reversing or slowing down the loss of kidney function.

Myth : only one kidney is affected by kidney diseases.

Fact : All medical diseases (high BP, diabetes, glomerulonephritis, nephrotic syndrome, poisonings, infections of urinary tract etc) affect both kidneys.

Some structural diseases like tumours, stones, abscesses, renal artery or vein clots, ureteral narrowings, may affect only one kidney.

Myth : I am passing enough urine. Hence my kidneys are not obstructed.

Facts : Most obstructions of the bladder and partial obstructions of the ureter  (stones or accidental ligation during surgery) cause more urine to be formed and passed. Only bilateral total obstruction or obstruction below bladder neck cause reduced urine output.

Myth : I am passing enough urine. Hence my kidneys are healthy and I do not require treatment or dialysis.

Fact : Some kidney disease cause decreased urine output or oliguria (< 400 ml urine/day). Most, however, are nonoliguric or Polyuric (urine output > 3000 ml/day). Even with normal or large urine production, waste material like acids, potassium, urea, creatinine and many more may not be excreted. A person then may require treatment at times dialysis as well.

Myth : Drinking more water will keep my kidneys healthy. This is the treatment for kidney diseases.

Fact : Usual water intake is well managed by thirst in healthy people. Drinking 2-3 litres of fluid may avoid some stone formations and urinary tract infections. Continuing to drink fluids when kidneys are failing or have failed may cause fluid in the lungs (pulmonary edema) or poor control of hypertension. The consequences may be deadly. Follow your doctors’ advice.

Myth : Dialysis once started is required life long.

Fact : In temporary or reversible renal failure, dialysis is required till kidney recovers. In CKD or ESRD with no reversibility, dialysis is required for emergency treatment of high potassium or fluid in the lungs. Maintainance dialysis for uremic symptoms, neuropathy, encephalopathy, pericarditis etc usually means either lifelong dialysis or renal transplant.

Life After Kidney Transplant

Life After Kidney Transplant

Kidney transplant is preferable to lifelong dialysis as it usually provides a better quality of life. After initial 14 days, the risk of death is less in transplant recipients compared to patients on dialysis.

The human body tries to throw out the transplanted kidney as it is perceived as a foreign body by the tissues.

To overcome this tendency of the body, drugs are required to be taken lifelong by transplant recipients. These drugs are called immunosuppressives.

These drugs also reduce body’s reaction in case an infectious agent gains access to the body. Hence infections can occur more often, with smaller doses of bacteria and viruses. These infections may be severe and life threatening. At times bacteria and viruses which do not cause infections in other healthy individuals may also cause infections in transplant recipients.

These infections have to be suspected more often, investigated more aggressively and treated vigorously with appropriate antibiotics by a physician. In India, most  deaths take place with a functioning graft due to infections. Hence the importance of preventing these.

How to prevent infections in transplant recipients:

Infections spread by food, water, contact and by inhalation of droplets containing pathogens( bacteria, virus, fungi etc). They can also be introduced during surgery, by IV lines, tubes placed in the body and during various medical procedures.

Infections from water are prevented by drinking filtered / RO and clean water only. Tubewells, river, ponds, most municipal water in cities and towns may be having infectious agents. Hence it is not safe to drink untreated water. In case of dire necessity, boiled water as in tea can be consumed.

Food should be fresh, made from clean ingredients in clean utensils and consumed early. Food kept in refrigerator (especially as power cuts are common place) can be contaminated and when consumed may cause infectious diarrhoea.

Close contact of persons with obvious respiratory infections eg common cold, influenza, pneumonia, chickenpox, measles, sore throat etc should be avoided. Good quality masks (as used for preventing the spread of swine flu) are used for prevention of respiratory infections. Vaccines against pneumonia are similarly useful to protect against pneumonia.

Hygiene should be immaculate. In hospitals etc ensure absolute asepsis to prevent any infections during sampling, IV infusions, injections or surgical procedures.

If adequate care is taken a person can lead an active life. He can live a normal long, productive and useful life.

Next coming up

Various drugs after a transplant.