EWJ Dec 2023 - Journal - Page 18
Nephrotic Syndrome in Children
Nephrotic syndrome is a condition that causes the kidneys to leak large amounts of protein into
the urine. This can lead to a range of problems, including swelling of body tissues and a greater
chance of catching infections.
Although nephrotic syndrome can affect people of
any age, it's usually first diagnosed in children aged
between 1 and 6 years old. It tends to be more common in those with an Asian background, although it's
unclear why.
Nephrotic syndrome can sometimes occur as a result
of a kidney problem or another condition, such as:
l glomerulosclerosis – when the inside of the kidney
becomes scarred
l glomerulonephritis – inflammation inside the
kidney
The symptoms of nephrotic syndrome can usually be
controlled with steroid medication. Most children with
nephrotic syndrome respond well to steroids and are
not at risk of kidney failure. But a small number of
children have inherited (congenital) nephrotic syndrome and usually do less well. They may eventually
have kidney failure and need a kidney transplant.
l an infection – such as HIV or hepatitis
l lupus
l diabetes
l sickle cell anaemia
l in
very rare cases, certain types of cancer – such as
leukaemia, multiple myeloma or lymphoma
What problems can it cause?
Most children with nephrotic syndrome have times
when their symptoms are under control (remission),
followed by times when symptoms return (relapses).
In most cases, relapses become less frequent as they
get older and often stop by their late teens.
These problems tend to be more common in adults
with nephrotic syndrome.
Diagnosing nephrotic syndrome
Nephrotic syndrome can usually be diagnosed after
dipping a dipstick into a urine sample. If there are
large amounts of protein in a person's urine, there will
be a colour change on the stick.
Some of the main symptoms associated with nephrotic
syndrome include:
l swelling – the low level of protein in the blood
reduces the flow of water from body tissues back into
the blood vessels, leading to swelling (oedema).
Swelling is usually first noticed around the eyes, then
around the lower legs and the rest of the body.
A blood test showing a low level of a protein called albumin will confirm the diagnosis. In some cases, when
the initial treatment does not work, your child may
need a kidney biopsy. This is when a very small sample of kidney tissue is removed using a needle so it can
be studied under a microscope.
infections – antibodies are a specialised group of
proteins in the blood that help to fight infection.
When these are lost, children are much more likely to
get infections.
l
Managing nephrotic syndrome
The main treatment for nephrotic syndrome is
steroids, but additional treatments may also be used if
a child develops significant side effects.
urine changes – occasionally, the high levels of
protein being passed into the urine can cause it to become frothy. Some children with nephrotic syndrome
may also pass less urine than usual during relapses.
l
Most children have relapses until their late teens and
need to take steroids when these occur. Your child
may be referred to a childhood kidney specialist (paediatric nephrologist) for tests and specialist treatment.
l blood
clots – important proteins that help prevent
the blood clotting can be passed out in the urine of
children with nephrotic syndrome. This can increase
their risk of potentially serious blood clots. During a
relapse, the blood also becomes more concentrated,
which can lead to clotting.
Steroids
Children diagnosed with nephrotic syndrome for the
first time are normally prescribed at least a 4-week
course of the steroid medicine prednisolone, followed
by a smaller dose every other day for 4 more weeks.
This stops protein leaking from your child's kidneys
into their urine.
Causes of nephrotic syndrome
Most children with nephrotic syndrome have "minimal change disease". This means that their kidneys appear normal or nearly normal if a tissue sample is
studied under a microscope.
When prednisolone is prescribed for short periods,
there are usually no serious or long-lasting side effects,
although some children may experience:
l increased appetite
l weight gain
l red cheeks
l mood changes
But changes to the tissue sample can be seen if
studied under an extremely powerful electron
microscope.
The cause of minimal change disease is unknown.
EXPERT WITNESS JOURNAL
16
DECEMBER 2023