Nephrotic Syndrome in Children
Nephrotic Syndrome in Children
Nephrotic Syndrome in Children
in Children
Kidney
This booklet has been prepared to answer most of your questions. It is meant to
to reassure you and allay misgivings. Your doctor will be happy to discuss with
you any other matter regarding your childs condition.
Bladder
Urethra
Figure 1
Each kidney contains about a million functioning units, called nephrons. The
nephron is composed of a glomerulus and a long coiled tube (tubule). Blood is
filtered by the glomerulus and the fluid (filtrate) flows down the tubule. The
tubules take back useful constituents and excrete the harmful ones (Fig. 2). At
the end of the individual tubule a few drops of urine form. The tubules join to
form larger channels, which in turn unite to form still bigger channels, ultimately
ending in the ureter, which carries the urine from the kidney into the bladder.
1
Filtrate
Tubule
(reabsorbs
what is
necessary and
excretes which
is harmful)
Blood
enters
Protein leaked
Blood
leaves
Normal
Blood
enters
Nephrotic
syndrome
Glomerulus
(blood is filtered
here)
Urine
The features of nephrotic syndrome, viz. swelling over face, legs and abdomen
(and abnormalities in the blood), result from the passage of large amounts of
protein in urine. To reemphasize, only negligible amount of protein is present in
urine in normal person.
Normally only very small amounts of protein and few red blood cells pass out of
the blood in the filtrate. It is abnormal for large amounts of protein to leak
through the kidney filters and appear in the urine. When this happens, we call it
as spilling protein in the urine (proteinuria) (Fig. 3).
The main protein in the blood is albumin. If large amounts of protein (albumin)
are lost through the urine, its level in the blood decreases. When the level of
protein in the blood is low, swelling (edema) will develop. This is because the
protein in the blood has a sponge-like effect, holding fluid within the blood
channels. With less protein in the blood, the sponge effect does not work as
well, and fluid leaks out into the body tissues. This is seen as swelling around the
eyes, face, feet, ankles and later on the abdomen.
The exact cause of the large amount of protein leak from the minute blood
channels (capillaries) of the glomeruli is not known. In about 95% cases of
nephrotic syndrome in children, there is no permanent damage to the capillaries.
With treatment, the leakage of protein stops.
In most cases, the exact cause of nephrotic syndrome is not known. The disease
occurs in children all over the world. It is not caused by bacterial or viral infection.
There is no relationship with diet or socioeconomic status of the family. It is not
infectious and does not transfer to other family members. Only in exceptional
instances, more than one child in a family may suffer from nephrotic syndrome.
In a very small proportion of children other diseases are present that cause
capillary damage in the glomeruli, which results in leakage of protein. The doctor
will carry out appropriate laboratory tests to diagnose these conditions.
on the face, around the eyes. It is most prominent in the morning when the child
gets up. Towards the evening the swelling disappears. In fact the accumulated
fluid shifts to the legs where it is not so easily noticeable. The swelling is sometime
mistaken to be due to allergy or eye-problem. In nephrotic syndrome there is
no itching or redness of the eyes.
The child is otherwise well and active and does not look ill. The swelling
gradually increases to involve the feet, legs, hands and abdomen. If untreated,
it may become enormous with distension of the abdomen (ascites) due to
collection of fluid. At this stage the quantity of urine decreases and urgent
treatment is required to decrease the swelling.
What tests will my child have?
Urine examination
Urine examination is the initial test in nephrotic syndrome. The results will usually
show 3+ or 4+ proteinuria. The details of this test are given on page 17. You
should learn how to do urine test for protein and monitor for proteinuria. Urine
is also examined by the microscope to look for red cells, white cells and bacteria.
A urine culture is done if urinary infection is suspected.
It may occasionally be necessary to measure the total amount of protein (and
other substances) in urine passed in 24 hours. Instructions about collection of
24-hour urine are given on page 19.
Blood tests
Blood is examined for hemoglobin, white cell count, protein, albumin, cholesterol
and electrolytes. Heavy loss of protein in urine leads to lower levels of proteins
in the blood. The level of blood cholesterol increases. However, once proteinuria
disappears with treatment, blood protein and cholesterol levels return to normal.
Blood urea and creatinine levels help evaluate kidney function. Special blood
tests such as complement level, antinuclear antibody (ANA), antistreptolysin O
(ASO) titer and hepatitis B antigen may sometimes be required.
(ii)
(iii)
Other tests
A x-ray chest and Mantoux (tuberculin) test are done at the first visit to rule out
underlying infections, which if detected would need treatment.
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Prednisolone
Prednisolone (a steroid drug) tablets are available under different names
(Wysolone, Deltacortril) and strengths (5, 10, 20, 30 mg). The amount of
prednisolone administered depends upon the weight of the child. Your doctor
will explain the dose, frequency of administration and duration of the treatment.
Prednisolone tablets are bitter! If the child has trouble taking the tablets,
sweetening agents (honey, sugar, jam) may be used to mask their taste. Liquid
preparations of prednisolone are also available.
i) Daily treatment
For the first 4 to 6 weeks, the required amount of prednisolone is given every
day in 1-3 divided doses (referred to as daily treatment). The tablets are taken
with a glass of milk or some food.
After a few days of daily treatment the child becomes completely well. The
edema disappears and urine no longer contains protein (nil test). Once daily
treatment period is completed, prednisolone schedule is changed over to the
alternate-day treatment.
Diuretics are medications that help the body get rid of extra fluid. They will be
needed while your child has more than mild edema. Lasix (frusemide) is often
prescribed. Usually one dose in the morning is adequate. If swelling is severe,
other diuretics like Aldactone (spironolactone), thiazides (Dytide) or metolazone
may also be used. Once the swelling is controlled, the dose of the drug is reduced
and then stopped.
Diuretics should only be given under supervision of your doctor. They should
not be used if the child is having loose stools or vomiting.
Albumin infusions
If the child is very edematous, has large amount of fluid in the abdomen and the
blood albumin (protein) level is very low, he may require albumin infusion. Albumin
is given through a vein (intravenously). It helps replace the protein in the blood
temporarily and increases urine output, resulting in decrease of swelling. Albumin
infusions are very expensive and used only when unavoidable. They should be
administered under close supervision
Diet
Protein
The child should be given a diet with enough proteins. High protein dietary
articles include milk and milk products, dal, chana, soyabean, eggs, meat and
fish. Extra protein is necessary only while the child is passing large amounts of
protein in the urine. Subsequently he should have his usual diet.
Salt
The intake of salt should be reduced while the child has swelling. The amount
used in ordinary cooking is permitted, but he should not be given extra salt and
salty snacks. As soon as the swelling disappears, the child can have his usual
diet. It must be understood that salt has no role in the causation of nephrotic
syndrome. No benefit can be expected by prolonged restriction of salt, unless
advised by the doctor for some other reason (e.g., if the blood pressure is high).
General care and precautions
Once the urine does not show protein (nil or trace) the child is said to have
achieved a remission. Normal activity and usual routine should be resumed.
Schooling should be restarted. No dietary restriction is needed. He should be
considered a normal child.
Suspect infections in a child receiving prednisolone
The child with nephrotic syndrome is more likely to suffer from infections,
especially when receiving prednisolone. Usually the infections are mild, such as
cold, sore throat and diarrhea. The doctor should be consulted and treatment
carried out. Occasionally, the infection is serious and may develop rapidly.
If the child has pain in abdomen, vomiting, diarrhea and fever he should be
promptly shown to the doctor. If he has headache, vomiting, drowsiness and
fever, he must be immediately taken to the hospital. The likelihood of a serious
infection is more if the child is having edema and there is fluid in the abdomen.
A delay in the treatment of such infections may be dangerous.
Children receiving treatment with corticosteroids (or other drugs) can become
very ill if exposed to chickenpox or measles. Let the doctor know at once if
your child is in close contact with another child who has chickenpox or
measles.
Immunizations may be deferred
Administration of live vaccines (e.g., against polio, measles-mumps-rubella,
chicken pox) may be delayed till the child is off corticosteroids or receiving a
very small dose. Steroid therapy may also reduce the efficacy of immunizations.
The doctor will advise on the appropriate timing of vaccinations.
9
In most cases the child with nephrotic syndrome becomes completely well with
prednisolone treatment. He may remain well for several months or longer. During
this period the child should be regarded as being normal and managed accordingly.
Physical activity and games should be encouraged. He should not be regarded
as sick or delicate or made to feel different from other children. Usual
disciplinary measures, as for other children in the family, should be employed.
In a majority of cases, however, nephrotic syndrome recurs. The recurrence is
indicated by appearance of swelling around the eyes, which if untreated gradually
increases to involve the face, feet, legs and abdomen. Urine examination again
shows 3+ to 4+ of protein. This situation is called a relapse. A relapse can be
detected, before the appearance of swelling on the face, if urine test for protein
is done once or twice a week on a long-term basis, since edema occurs only
after several days of presence of protein in the urine. Usually, relapses occur
after a cold or sore throat or some other infection, but sometimes there is no
obvious cause for a relapse.
Treatment of relapse
On some occasions proteinuria develops when the child has a cold or sore
throat, but completely disappears within a week or so, along with recovery from
the infection. It is, therefore, important to perform daily urine tests during an
episode of infection, while the infection is being appropriately treated. Urine
may show 1+ or 2+ reaction for a few days and then gradually become nil. No
treatment is necessary for such a short spell of mild proteinuria.
On the other hand, proteinuria may continue even after the infection has subsided.
After 1 or 2 weeks of heavy proteinuria, swelling appears over the face and
gradually increases. Such an episode (relapse) will need treatment with
prednisolone, which must be started before the swelling becomes very prominent.
The drug is initially given daily in 1-2 divided doses, the number of tablets
depending upon the weight of the child. If the swelling has increased, Lasix may
be given for a few days. The daily treatment is usually needed for about 2
weeks, during which urine will gradually become free of protein, showing a nil
reaction. At this stage prednisolone is changed to the alternate-day schedule,
which is continued for 4-6 weeks and then stopped.
10
It is most important to treat a relapse early. Once the child develops gross
swelling with large amount of fluid in the abdomen, the management becomes
more difficult, and serious complications may occur.
Frequency of relapses
A child may not have a relapse for several months or longer. Some children get
one or more relapses in one year. Each relapse is treated with prednisolone as
mentioned above. If more than 3 relapses occur within a year, other forms of
treatment are considered. A frequent method is to keep the child on a small
dose of alternate-day prednisolone treatment for 1 year or more. If such a
regimen does not prevent relapses, other medications may have to be given.
The need for such treatment will be considered by the doctor and discussed
with you. Some of these medicines include:
(i) Methylprednisolone or dexamethasone given by intravenous injections, to
induce a remission.
(ii) Levamisole tablets given on every alternate-day for a prolonged period.
(iii) Cyclophosphamide. This is a drug often used in various forms of cancer.
Its use in nephrotic syndrome has nothing to do with cancer. This drug has
been found to be very beneficial in a large proportion of children with
nephrotic syndrome all over the world. Usually tablets are given daily along
with prednisolone for 12 weeks. Rarely cyclophosphamide may be given by
injection once a month for 6-8 months. The side effects of this drug and
other precautions will be explained to you by the doctor.
(iv) Cyclosporine. This drug is chiefly used in patients who undergo kidney
transplantation. However, it has been found to be very useful in many other
conditions. It is also effective in preventing relapses in nephrotic syndrome.
The doctor will discuss and explain various issues in case cyclosporine is to
be administered.
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periods causes slowing of growth. The childs height is monitored regularly and
if height gain is slow, other drugs are used.
Other side effects. Some children may show changes in mood and behaviour.
They may be excessively happy, quiet or abnormally active while on prednisolone.
Prolonged administration of prednisolone may result in development of tiny
opacities (cataracts) in the lens of the eye. These are rarely significant.
Occasionally, the blood sugar level may increase and urine examination show
glucose.
The parents need to keep a careful account of the childs treatment, urine tests
and other events. The information may be recorded in a diary, as shown below:
Date
Urine test
Prednisolone
Other drugs
Remarks
1.9.02
3+
30mg
Lasix 1 tablet
2.9.02
3+
30mg
cough better
3.9.02
2+
30mg
well
4.9.02
2+
30mg
well
5.9.02
30mg
well
Records that are complete and accurate give the doctor valuable information on
the childs health and progress. The treatment is mostly based on that and blood
tests are usually not required.
Know important facts about nephrotic syndrome
The parents should clearly understand the treatment schedule and become familiar
with the drugs used in the management. The child should not be given any
unknown drugs. All prescriptions from doctors should be retained.
A number of facts about nephrotic syndrome should be accepted.
It is likely to last over a period of several years.
The course of the disease is variable and whereas some children get only a
few relapses, others have frequent relapses.
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14
What is the long term outcome for children with nephrotic syndrome?
With correct management, the child will lead a normal life. If regular urine
tests are done, the relapse will be detected early and treated promptly.
Schooling will rarely be interrupted.
Most children with minimal change disease outgrow the disease sometime
during adolescence (or earlier), and do not get kidney damage. Unfortunately it
is not possible to predict at what age the child will be completed cured. The
severity of nephrotic syndrome is quite variable, and some children suffer
from very frequent relapses, while others get few or an occasional relapse.
The aim of management is to treat the relapse and keep the child in remission
using the minimum amounts of medications, and prevent (or treat) complications.
The focus of the family should be more on the growth and development and
normal activities (schooling, participation in games, etc.). The parents should
disregard unsolicited advice and desist from discussing their childs problem
with relatives and friends. Any clarification or information should be sought
from the pediatrician or pediatric nephrologist.
Your child is in close contact with another child having chickenpox or measles,
or he develops that illness.
ii) If he gets diarrhea, vomiting, pain in abdomen, high fever, or appears ill and
drowsy.
15
A.
Q.
A.
Yes, the child can participate in all sports within his capability.
Q.
Q.
A.
Children with the most usual type of nephrotic syndrome, which responds
to prednisolone have no risk of the kidney failure. In other uncommon
types, increasing kidney damage may occur.
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APPENDIX
1. Examination of urine for protein
Examination of urine for protein forms an important part of the long-term care.
The test is simple to perform. Urine, preferably the first specimen passed in the
morning, is collected in a clean container. The specimen should have a yellow
tinge; urine passed after having received diuretics (e.g., Lasix) looks like water
and is not suitable for testing.
Top part is
boiled
Urine
Protein
present
Protein
absent
Spirit
lamp
Spirit
lamp
If protein is present the heated part of the urine turns white. The appearance
can be compared with cold urine at the bottom which should be clear
(transparent). The intensity of whiteness is proportional to the amount of protein
in the urine and can be graded as follows:
Nil
1+
2+
3+
4+
=
=
=
=
=
The grading is not difficult and one becomes an expert after a little practice.
However, this method is a bit cumbersome and most parents prefer the dipstick
test.
Uristix test for protein
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18
4. Kidney Biopsy
i.
ii.
iii.
Child passes urine at 8 a.m. the next morning. Collect this specimen.
iv.
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