Q. My 8-year-old daughter has portal hypertension and
enlarged liver and spleen. Although she is eager to keep up with her
classmates, I donít allow her to participate in soccer or other
vigorous sports because Iím afraid she might injure her spleen. But
what about gymnastics, playing on the monkey bars or other
The spleen is like a bag filled with blood. This is because
the spleenís purpose is to filter the blood supply of bacteria and
remove old red blood cells. Normally, the spleen is safely
tucked up under the ribs; but when the spleen is enlarged
due to portal hypertension it can be felt below the ribs.
Whenever the spleen is felt below the ribs
it is not protected from injury. Abdominal trauma such as a kick or
direct blow with a blunt object, such as an elbow, stick, or bicycle
handlebars may cause the spleen to rupture and leak blood into
itself or into the abdominal cavity. This may occur rapidly and a
child could die suddenly.
Vigorous exercise, including gymnastics, sit-ups, push-ups, and
playground equipment are generally safe. A child with an enlarged
spleen should not participate in contact sports, such as kick
boxing, boxing, karate, hockey, and football. (Note: Wrestling and
Lacrosse may also not be considered safe with portal hypertension.)
On the other hand, sports like soccer, baseball, volleyball, and
basketball are generally safe (although elbowing can be dangerous.)
In some cases, an abdominal binder can be fashioned to help protect
the spleen from injury.
Dr. Berquist is Chief, Gastroenterology and Liver
Transplantation at Lucile Salter Packard Childrenís Hospital at
Stanford University and Chairman of the CLASS Scientific Advisory
From Archived ARPKD/CHF Newsletter
Many of these questions and answers are ADPKD related,
generally because not enough specific research has been done on
ARPKD and CHF. These comments should not be taken as medical
advice. Please refer to your specialists for specific options.
Finding A Doctor
Your first step should be finding a local medical professional who
has knowledge about the disease. To find a qualified professional,
What are typical treatment options?
Many, but not all infants/children need treatment to control high
blood pressure; this is done with medication(s). It is not unusual
for dosages to be decreased or weaned with age. (Kidneys generally
do not grow in size, but stay the same with ARPKD.) If needed,
urinary tract infections (UTIís) are controlled with antibiotics.
Other symptoms are treated as they occur.
Will this affect growth?
Some children experience slow growth and stature ("failure to
thrive"). In some cases, depending on the circumstances,
supplements are required and growth hormones used when children
consistently score below the 5th percentile of standard growth and
Will my child need a transplant?
It is likely at some point kidney transplantation will be needed,
but this varies greatly to very early in life to later in life. It
all depends on the rate of decline of kidney function.
Can surgery help this problem?
Not usually. It will not change the course of the disease. Banding
may help with the varcises and shunts may help with the blood flow.
How can diet help?
Eating a healthy diet is recommended. A low sodium diet may be
recommended (yet children may crave salt), depending on individual
circumstances. Generally children are NOT placed on a low protein
diet, as protein is needed for growth.
Find a nephrologist to help manage renal function, high blood
pressure and other clinical symptoms.
There will likely come a time to find a GI specialist who can
assist with clinical liver manifestations.
Take your medication(s) as directed.
Normalize lifestyle as much as possible.
Eat a healthy diet.
Donít take medicines or vitamins without first speaking with your
Ibuprofen (Advil) is thought to be detrimental to PKD kidneys.
Watch your diet restrictions during different phrases of the
disease- especially under dialysis.
Avoid contact sports
What tests can be done to monitor my kidney function?
There are 4 main tests used to determine kidney function:
test that measures levels of creatine in your urine. Creatine is
dependent on weight, age, physical activity and even weather. If
you are not drinking sufficient fluids, your creatinine will go up
due to dehydration; inactivity breaks down muscle, creating more
Blood Urea Nitrogen (BUN)
A test that measures levels of urea nitrogen in your blood. Urea
is the main waste product of protein metabolism. Blood levels of it
are referred to as blood urea nitrogen (BUN) and are used to
evaluate kidney disease. A high BUN is the hallmark of
kidney disease. It is also high in heart failure, increased protein
breakdown, diabetic acidosis, dehydration, intestinal bleeding, and
muscle degeneration. Certain drugs cause an elevated BUN, as will a
recent meal heavy in protein. Low BUN is associated with excess
fluid intake, malnutrition, liver disease, and pregnancy.
24 Hr. Creatinine Clearance
A 24 hour collection of urine samples that is measured for
creatine levels. Creatine is a waste product of normal metabolism.
with blood urea nitrogenóare the most common kidney function tests. High creatinine
is associated with any of a number of kidney diseases, but provides
only a rough approximation of the extent of disease and does not
identify its cause.
Radionuclide studies (GFR)
A test that uses an isotope solution injected into your vein,
with a gamma camera taking pictures to show the blood supply to each
What is GFR?
The filtering action of the kidneys processes about 120 milliliters
of blood per minute. Doctors measure this rate as an indication of
how well the kidneys are working and call it the glomerular
filtration rate or GFR for short.
Glomerular filtration rate (GFR) is considered by medical
professionals to be the best measure of kidney function.
What is a normal GFR?
The chart below lists values for GFR:
Glomerular Filtration Rate
60 ML/MIN: Failing Kidneys
30 ML/MIN: Chronic Renal Failure
6 ML/MIN: End-Stage Renal Failure
medicine, standard therapy is a treatment that experts agree is
accepted, appropriate and widely used. Healthcare providers are
obligated to provide patients with standard therapy. The terms
standard of care or best practice are also used.