What is nephrotic syndrome?

Nephroticsyndrome
Theglomerulus (plural: glomeruli) of the kidney is a minuscule cluster of tiny blood vessels
involved in filtering blood to form urine (pee). In nephrotic syndrome, the glomerulus allows
protein to leak into the urine.

Proteinuria

Proteinuria is when there are high levels of protein in the urine. Normally, there is
little to no protein found in urine. In nephrotic syndrome, the glomeruli (the filters
within the kidney) leak protein from the blood that flows through them into the
urine they produce.

Hypoalbuminemia

Hypoalbuminemia is when low levels of protein (albumin) are found in the blood.
When a large amount of protein leaks out into the urine, the body cannot make
enough new protein to keep up. This results in hypoalbuminemia.

Edema

Edema happens in nephrotic syndrome because of low protein levels in the blood.
Protein normally acts like a sponge to keep fluid in the blood vessels. With less
protein in the blood, fluid leaks out of the blood vessels into other tissues. Edema is
typically seen around the eyes, face and legs. Fluid can also build up in the
abdomen, around the genital area or in the lungs.

Treatment of nephrotic syndrome

The goal of treatment is to stop the kidneys from leaking protein into the urine. The
initial treatment of nephrotic syndrome is with prednisone or prednisilone, which is
taken every day for at least six weeks and then slowly decreased over several
months. Steroids work in many different ways for nephrotic syndrome, but the
exact way they work is not known. It is important to continuously take prednisone
as instructed without stopping suddenly. Sometimes, this first course of treatment
is all that is needed. However, for some children, the disease can return, which
requires further treatment.

Prednisone usually works very well to treat nephrotic syndrome. Most children
respond to prednisone; however, a small number of children do not. In these cases,
a kidney biopsy may be required. Some children may need to take other
medications that suppress the immune system, such
as cyclophosphamide, tacrolimus, mycophenolate or rituximab.
If there is severe edema, your child may need to be admitted to the hospital to
receive protein (albumin) intravenously. Your child may also be given a diuretic
(“water pill”) to help remove some of the extra fluid that has accumulated in the
body. It is very important for a child with nephrotic syndrome to eat a low-salt diet
to prevent fluid retention. Consuming more protein in the diet will have no effect
and is not recommended. While on prednisone, it is also important to eat a low-salt
and low calorie diet.

While on steroid medication, your child is at higher risk of infection. Seek

immediate medical attention if your child shows signs of fever or other signs of
infection. Let your dentist know that your child is on steroids, and speak to your
health-care team before giving any vaccinations.

Complications
Hyperlipidemia (high levels of blood cholesterol and triglycerides) is a common
consequence of nephrotic syndrome. If albumin levels are low due to leakage, the
balance of various fats in the body is altered, which leads to high levels of
cholesterol. This is usually temporary and does not cause long-term harm, as
cholesterol levels normally re-balance with treatment.

If nephrotic syndrome is left untreated, complications such as infection, fluid

overload (significant swelling causing discomfort), kidney injury and blood clots can
occur.

Long-term outcomes/future expectations

Most children respond well to treatment and have a good outlook. Nephrotic
syndrome rarely causes kidney failure and children are likely to grow out of it. It is
not uncommon for children to have at least one relapse. If your child has a relapse,
they will receive repeated treatment with prednisone or other medications.
Sometimes, continuous low-dose steroids every second day and/or an additional
medicine may be needed to stop relapses that occur too often.

When to call your health-care provider

 If your child has chicken pox, or has direct contact with someone who has chicken
pox, while on prednisone.
 If urine becomes positive for protein again.

Take your child to the nearest emergency department if your child:

 is swollen
  is unwell with a fever
 has a persistent headache
 has vomiting or abdominal pain and/or decreased urine output

Resources
The Kidney Foundation of Canada (www.kidney.ca)

NephCure® Kidney International (www.nephcure.org)

References
Childhood Nephrotic Syndrome: A Guide for the Parents on the Management and
Treatment of Childhood Nephrotic Syndrome. The Kidney Foundation of Cananda.
Retrieved from https://www.kidney.ca/document.doc?id=330.

Downie ML, Gallibois C, Parekh RS, Noone DG (2017). Nephrotic syndrome in

infants and children: pathophysiology and management. Paediatr Int Child Health,
37(4), 248-258.

Banh TM, Hussain-Shamsy N, Patel V, Vasilevska-Ristovska J, Borges K, Sibbald C,

Lipszyc D, Brook, J, Geary D, Langlois V, Reddon M, Pearl R, Levin L, Piekut M, Licht
C, Radhakrishnan S, Aitken-Menezes K, Harvey E, Hebert D, Piscione T, Parekh RS
(2016). Ethnic differences in incidence and outcomes of childhood nephrotic
syndrome. Clin J Am Soc Nephrol, 11(10), 1760-1768.

Your role as a caregiver

Dipstick bottle
It is important for you to monitor your child’s urine for protein because it is the first
sign of a relapse. Your health-care provider will teach you how to check your child’s
urine with a dipstick. You will need to check the first sample of urine every morning
and report back to your health-care team if the dipstick tests positive for protein
(anything greater than ‘trace’ amounts [greater than 0.3 g/L]). Your health-care
provider may recommend keeping a bathroom scale at home to look for sudden
changes in weight.

How to test urine for protein with dipsticks:

1. Collect first morning urine into a clean container.

2. Take one strip out of bottle, and close bottle tightly.
3. Dip the strip into the urine quickly. Be sure the square on the tip is wet with
urine.
4. Place the strip on a flat surface and wait 60 seconds.
5. Compare the colour of the stick to the colours on the bottle.
6. Record the value: the packaging should show you which range(s) indicate
“normal.”
If the dipstick results show as above the “normal” range (0.3 g/L or higher), contact
your health-care provider.

Note: Always check the expiry date of the dipstick bottle. Be sure to test the urine
while it is fresh (within one hour). Ensure that the dipsticks are stored in a cool, dry
place and that the bottle remains tightly sealed. Do not store dipsticks in the
kitchen or bathroom.

Diagnosis
Diagnosis is made by testing the urine for proteinuria, and the blood for
hypoalbuminemia, in the presence of edema. The disappearance of protein in the
urine after the use of prednisone helps to confirm the diagnosis of nephrotic
syndrome. Sometimes, the doctor may also recommend a kidney biopsy to help
with the diagnosis and treatment of nephrotic sydrome.

Course of nephrotic syndrome

For childhood nephrotic syndrome, most patients respond well to treatment with a
steroid medication called prednisone or prednisilone. About 80% of children will
improve with steroids. Even with successful initial treatment, some children with
nephrotic syndrome can have relapses. Treatment plans for children with relapses
are customized based on whether the child has frequent or infrequent relapses.
Prognosis is excellent—the majority of children, especially those with minimal
change disease, will outgrow nephrotic syndrome and become adults with normal
functioning kidneys.

Causes of nephrotic syndrome

Most nephrotic syndrome in children is idiopathic, meaning that the cause of
nephrotic syndrome is unknown. Nephrotic syndrome is more common in boys
than girls, and often appears for the first time in children under five years of age.

The most common types of nephrotic syndrome in children are minimal change
disease and focal segmental glomerulosclerosis (FSGS).

Rarely, some patients have nephrotic syndrome in the first few months of life. In
these cases, the cause is most likely genetic, meaning that the glomeruli did not
develop normally before birth.

Signs and symptoms

When a child first presents with nephrotic syndrome, they may be irritable and
have swelling around the eyes, abdomen, lower legs and sometimes the genitals.

You may also note the following changes in your child:

 Weight gain because of fluid retention

 Less frequent urination (peeing) or foamy urine
 Abdominal pain or discomfort
 Diarrhea (loose stools) and/or vomiting
 Feeling generally unwell and tired

Asking chronic kidney disease (CKD) patients to follow 6 tips may help them
manage their protein intake better than standard dietary advice, a small Italian
study suggests.

Investigators led by Eleonora Riccio, MD, and Antonio Pisani, MD, of

University Federico in Naples, Italy, randomly assigned 57
discharged CKD patients (stage 3b-5) to the 6-tips diet or a standard diet low
in protein (0.8 g/kg/day). None of the patients received individual counseling
from a dietitian.

The tips were based on the same principles of the standard diet (i.e., choose
foods low in protein, sodium, and phosphate, and consume moderate food
portions), but in easy-to-follow steps for patients eating typical Southern Italian
foods:

1. Do not add salt during cooking or at the table.

2. Avoid salami, sausages, cheese, dairy products, and canned foods.
3. Replace noodles and bread with low protein alternatives.
4. Eat 4–5 servings of fruits and vegetables daily.
5. Meat, fish, or eggs are allowed once a day in a reasonable quantity.
6. Once or twice a week, noodles with legumes may be eaten in place of
meat, fish, or eggs.

After 6 months, the metabolic profile of patients on the 6-tips diet was better
than those on the standard diet, according to results published online
in Clinical and Experimental Nephrology. Although both diets progressively
reduced protein intake and urinary excretion of nitrogen and phosphate, the
effect was more pronounced in the 6-tips group. Plasma levels of phosphate,
bicarbonate, parathyroid hormone, and urinary sodium chloride stayed stable
for all patients.
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At baseline, both groups were comparable with respect to sex, body weight,
blood pressure, protein intake, estimated glomerular filtration rate, and
treatments. Patients also took prescribed medication throughout the study,
including phosphate binders.

Additionally, researchers observed that more patients adhered to the 6-tips

diet: 70% vs. 44%. Adherence was defined by a consistent protein intake of
0.7-0.9 g/kg/day.

Patient adoption of low protein diets has been historically poor, prompting a
need for alternatives. “These data, however, clearly suggest that the [6-tips
diet], beyond its metabolic efficacy, is certainly better accepted than the usual
diet, probably for its simplicity…,” the researchers noted. They emphasized
that dietitians are valuable and should be sought when available, especially
when protein intake needs to be reduced to 0.6 or 0.3 g/kg/day.

The investigators acknowledged that the tips are best suited to patients eating
a Western-style diet. Future studies need to probe patients’ actual nutrient
intake, such as the quality of protein and the amount of produce eaten.