NEPHROTIC SYNDROME

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Last Updated : 1/6/2011
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Kumud P Mehta
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What is nephrotic syndrome?
Nephrotic Syndrome is a common disease in children involving kidneys.

How does it occur?
It occurs due to loss of proteins in the urine in large quantities which reduces the amount of protein in blood. Normally, as little as 150 mg of protein is excreted in urine by kidneys in a day whilst in Nephrotic Syndrome 2-20 gm may be lost.

How shall the patient present?
Patients present with swelling around eyes in the morning in the early stage, which subsequently spreads to legs, back, abdomen and whole body. Child may also pass less urine during this time.

How do you diagnose the condition?
Diagnosis of NS is by a simple urine examination. Urine tests are positive for protein (3+ to 4+).

What is the treatment?
In majority of children the cause of nephrotic syndrome is not known hence there is no curative treatment. But oral prednisolone treatment is effective in controlling the protein loss in the urine which is the main problem in NS.

How do you monitor the patient?
Since the total course of prednisolone is for 2-3 months, weekly examination of urine to detect its effect on protein loss is important. Once the protein loss is controlled, once every 2-4 weeks the urine test should be done to diagnose recurrence of the disease or relapse which is diagnosed by reappearance of 3+ to 4+ proteins in urine. B.P.record, weight of the child, urine output also should be checked periodically.

What precautions does the patient take?
The main precautions to be taken are avoidance of infection because infections are known to cause relapses and in severe cases death. Boiling the drinking water, avoiding contact with patients with infections like chickenpox, jaundice, typhoid etc. and giving vaccinations against the above mentioned diseases are important steps to prevent infections in NS. There is increased risk of complications in a child with NS due to infections which may be mild in healthy children.

What are the chances of recurrence?
Chances of recurrence or relapse of NS is as high as 50-75% in a Nephrotic child.

What to do in case of a relapse?
In case of relapse the first step is contact your doctor to treat the infection which might be responsible for the relapse.

How do you treat a relapse?
Then again oral prednisolone therapy may be needed. The duration of course of prednisolone therapy for relapse is shorter (6-8 weeks).

When do you start steroids in a relapse?
If a child has proteinuria of 2+ or more for 2-3 weeks even if there is no swelling or edema it is safer to start steroids rather than wait, because all the complications of NS are related to edema.

When is a kidney biopsy required?
If the patient does not respond to oral prednisolone therapy within the prescribed time a kidney biopsy is a must.

What is the role of levamisole & when do you start & how to give?
Levamisole is a drug, which is useful if NS relapses frequently i.e. more than 3 relapses within 1 year or patient relapses while on steroids i.e. Steroid dependent NS. Levamisole can control proteinuria, which is reduced by steroids sparing effect so that fewer doses of steroids is needed and in some cases steroids can be omitted.

Levamisole is started when prednisolone has produced remission (ie. urine albumin shows nil or trace for 3 days consecutively). The dose recommended is 2.5 mg/kg on alternate day for 6-18 months with tapering dose of steroids. It is to be given after dinner at bedtime.

What is the role of Enalapril in NS?
Enalapril is used to reduce proteinuria in selected cases of NS with infections like hepatitis, peritonitis etc. in whom steroids are contraindicated.

If the child is Hbs Ag+ve do you give steroids? What is the treatment?
With HbsAg +ve, steroids are not indicated and may be harmful. Tab.Enalapril can be tried in such cases.

What is the role of immunisation?
Immunisations are advised when the child is not in relapse and the dose of prednisolone is very small or when prednisolone is stopped. Vaccines for chickenpox, hepatitis-B, H, influenzae and pneumococci are important to prevent these infections which increase risk to life in a child with NS.

What is the role of urinary EPP & 24 hrs urine protein? When are they required?
Urinary electrophoresis was once recommended to distinguish nephrotic patients who are likely to respond to steroids from those who are less likely to respond i.e. steroid resistant cases using selectivity index i.e. ratio of urinary albumin to urinary IgG or transferrin. But since there is an overlap and no clear cut differences reported between responsive and non responsive cases, this test is not recommended in practice. As regards 24 hours urinary protein excretion, to measure exact amount of protein loss is difficult in young children because of difficulty in actual collection of all samples of urine. Recently urinary protein:creatinine ratio in a spot sample of urine is found to be equally useful.

When do you give diuretics in NS? When is Albumin given?
Diuretic (Furosemide) is only given to those patients who have severe oedema with oliguria or high BP. Similarly IV Albumin followed by IV Lasix given to severely oedematous nephrotics by experienced paediatricians in specialised centers.

How do you screen for complications of Spontaneous Bacterial Peritonitis and how to you treat it?
Peritonitis is a common major infection in Nephrotic syndrome of childhood, which can even cause mortality if not diagnosed early enough. Usually it presents as distension of abdomen because of accumulation of infected fluid in the peritoneum, which is the covering of the intestines. The other manifestations like fever, pain and vomiting may be absent. Hence, the diagnosis is difficult if not suspected in a child with distension of abdomen and active NS. It becomes mandatory to tap this fluid by inserting the needle in the abdomen and send it for examination for presence of pus cells and organisms. It can be treated successfully by giving intravenous antibiotics for 7-10 days. Obviously, it can be treated only in a hospital. Recurrence of peritonitis is again a big problem and some of the organisms responsible for peritonitis can be prevented by Vaccination. Pneumococcal and HIB vaccine are recommended when the child is off steroids and does not have active NS.



Contributor Information and Disclosures Kumud P Mehta
Consultant Pediatrician & Pediatric Nephrologist, Jaslok Hospital & Research Centre, Bai Jerbai Wadia Hospital for children, Mumbai, India


First Created : 1/3/2001
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